Cystic Fibrosis

Cystic Fibrosis
Title of Guideline (must include

the word Guideline (not
protocol, policy, procedure etc)
Contact Name and Job Title
(author)
Directorate & Speciality
1a
2a
2b
3
Guideline for the management of patients with

Cystic Fibrosis
Dr Jayesh Bhatt
Consultant in Paediatric Respiratory Medicine
Directorate: Family Health Children
Speciality: Respiratory
Sept 2015
Sept 2020
2015
Children and young people suspected of having
cystic fibrosis and those with confirmed diagnosis
Date of submission
Date when guideline reviewed
Guideline Number
Explicit definition of patient group
to which it applies (e.g. inclusion
and exclusion criteria, diagnosis)
Abstract
Details of diagnosis and management
Key Words
Paediatrics. Children. Cystic fibrosis
Statement of the evidence base of the guideline has the guideline been peer reviewed
by colleagues?
meta analysis of randomised controlled
X
trials
at least one well-designed controlled study
without randomisation
at least one other type of well-designed
quasi-experimental study
well designed non-experimental
descriptive studies (ie comparative /
correlation and case studies)
expert committee reports or opinions and /
or clinical experiences of respected
authorities
recommended best practise based on the
clinical experience of the guideline
developer
Staff at Nottingham Childrens Hospital via the

Guidelines E-mail process.
Target audience
Staff at the Nottingham Childrens Hospital
This guideline has been registered with the trust. However, clinical guidelines are
guidelines only. The interpretation and application of clinical guidelines will
remain the responsibility of the individual clinician. If in doubt contact a senior
colleague or expert. Caution is advised when using guidelines after the review
date.
Consultation Process
Dr Jayesh Bhatt
September 2015
Document Control
Document Amendment Record
Version
V1
V2
Issue Date
Nov 2012
Sept 2015
Author
Dr Jayesh Bhatt
Prof Alan Smyth
Dr Carol Bertenshaw
General Notes:
Summary of changes for new version:
No major changes format improved.
Major review under way.
Dr Jayesh Bhatt
September 2015
NOTTINGHAM UNIVERSITY HOSPITALS NHS TRUST
CHILDREN AND YOUNG PERSONS CYSTIC FIBROSIS

UNIT
CYSTIC FIBROSIS MANAGEMENT

GUIDELINES
Dr Jayesh Bhatt
September 2015
Index
Section 1 - Diagnosis
1.1
1.2
1.3
1.3.1
1.3.2
1.3.3
1.3.4
1.4
1.4.1
1.5
1.6
1.7
1.8
1.9
1.10
Background
The diagnosis of CF
Diagnostic testing
New Born Screening
Necessary cautions with new born screening
Babies with CFTR mutations detected in both genes
Babies with only one mutation detected on new born screening
The Sweat Test
Stool testing
Testing for CF and initialling management in patients with meconium ileus
Failure to thrive
Chronic respiratory symptoms
Siblings
Antenatal diagnosis - genetic
Antenatal diagnosis - bowel abnormalities detected on ultrasound scan
Section 2 - Outpatient Clinic

2.1
2.2
2.3
2.4
2.5
Outpatient clinic arrangements

Items performed at every clinic visit
Multidisciplinary meeting
Post clinic administration
Holiday advice
Section 2a Transition
2a.1
2a.2
2a.3
2a.4
2a.5
2a.6
Definition
Background
Hospital Youth Service
Young Persons CF Clinic
Individual transition plan
Transfer
Section 3 - Annual Assessment

3.1
3.2
3.3
3.4
3.5
Annual assessment
Medical review
Documentation
Computer database
Multidisciplinary review
Section 4 - Ward Management of CF Patients

4.1
4.2
4.3
Segregation Policy
Admission procedure
Ongoing care
Dr Jayesh Bhatt
September 2015
Section 5 - Antibiotic Guidelines

5.1
5.1.1
5.1.2
5.2
5.2.1
5.2.2
5.2.3
5.2.4
5.2.5
5.2.6
5.3
5.3.1
5.3.2
5.4
5.4.1
5.4.2
5.4.3
5.4.4
5.4.5
5.5
5.6
5.7
5.8
Collection of specimens for respiratory culture

Cough swabs and sputum
Flexible bronchoscopy and bronchoalveolar lavage
Oral antibiotics
Staphylococcus aureus
Haemophilus influenzae
Mycoplasma or Chlamydia pneumoniae
Preventing chronic infection with Pseudomonas aeruginosa
Long term Azithromycin
Treatment of exacerbations in children with chronic P.aeruginosa infection
Nebulised antibiotics
Colistin
Nebulised Tobramycin
Intravenous antibiotic treatment
Haemophilus influenzae
Staphylococcus aureus
Multiply resistant Staphylococcus aureus (MRSA)
Pseudomonas aeruginosa
Burkholderia cepacia complex
Infection with atypical mycobacteria
Fungal Secpticaemia
Allergic reactions
Heparin and sodium chloride flushes
Section 6 - Home "IV's" & Home Oxygen

6.1
6.2
6.3
6.4
6.5
6.6
6.7
6.8
6.9
Home intravenous (IV) antibiotic therapy

Indications for home IV treatment
Teaching sessions
Starting a course of home IV's
Prescribing instructions
Checklist for children going home on IV antibiotics
Antibiotic levels
Monitoring clinical response
Home oxygen
Section 7 - Management of Other Respiratory Problems

7.1
7.2
7.3
7.4
7.5
Allergic bronchopulmonary aspergillosis

Haemoptysis
Pneumothorax
Referral for transplantation
Non-invasive ventilation (NIV)
Section 8 - Management of Other CF Related Disease

8.1
Abdominal Pain - Distal intestinal obstruction syndrome (DIOS)
8.2
Cystic fibrosis related liver disease
8.3
Management of bleeding oesophageal varices
Dr Jayesh Bhatt
September 2015
8.4
8.4.1
8.4.2
8.5
8.6
8.6.1
8.6.2
8.6.3
8.6.4
8.6.5
8.6.6
CF Bone disease
Assessment of bone health
Treatment recommendations
Joint pain and disease
Cystic fibrosis related diabetes (CFRD)
Diagnosis of CFRD
Dietary management of Diabetes in Cystic Fibrosis
Dietary management if patient is generally well and in good nutritional status
Dietary management if patient is unwell and in poor clinical status
Insulin
Oral hypoglycaemic agents
Section 9 - Dietetic & Nutritional Management

9.1
9.2
9.3
9.3.1
9.3.2
9.4
9.5
9.6
9.7
Diet and nutrition

Assessment of growth & nutritional status
Nutritional support
Oral nutritional supplements
Nasogastric and gastrostomy feeds
Pancreatic enzyme supplements
Vitamin supplements
Salt supplementation
Standards of dietetic care
Section 10 - Guidelines for Obtaining Intravenous Access

10.1
10.2
10.3
10.4
Pic line/ Longline insertion

Indications for port-a-cath insertion
Flushing a portacath
Administration of Medication via a portacath using an ANTT
Section 11 - Physiotherapy
11.1
11.2
11.3
11.4
11.5
11.6
11.7
Introduction and principles

Airways Clearance Techniques
Physiotherapy for postural and musculosketal problems
Physiotherapy for urinary incontinence
Physiotherapy Services - Inpatients
Physiotherapy Services - CF Clinic
Physiotherapy Services - Community
Dr Jayesh Bhatt
September 2015
Section 12 - Dornase alfa & Hypertonic saline

Dornase alfa
12.1 Introduction
12.2 Mode of action
12.3 Clinical efficacy
12.4 Dosage and administration
12.5 Indications
12.6 Initiating therapy
12.7 Adverse effects
Hypertonic Saline
12.8 Introduction
12.9 Mode of action
12.12 Indications
Section 13 - Pain & Palliative Care in Cystic Fibrosis
13.1
13.2
13.3
13.3.1
13.3.2
13.3.3
13.3.4
13.3.5
Symptom control in the well child

Pain relief
Palliative care
Anti-emetics
Physiotherapy/ assisted ventilation
Laxatives
Other treatments
Communication
Section 14 - Psychosocial support

14.1
14.2
14.3
14.4
14.5
14.6
14.7
14.8
14.9
14.10
14.11
14.12
14.13
14.14
The importance of psychosocial factors in CF

Specialist CF psychosocial provision
Shared / Network care
Role of the clinical psychologist
Role of the social worker and Sherwood Project
New diagnosis
Outpatient clinics
Annual review
Admissions to hospital
Starting nursery education / full time school
Transition to adult services
Transplant assessment
Cessation of active treatment / terminal care phase
Bereavement
Dr Jayesh Bhatt
September 2015
Section 15 Education
15.1
15.2
15.3
15.4
15.5
15.6
15.7
QMC Hospital School

Introduction
Statutory Guidance
School in Hospital
Going to School on D33
Liaison with mainstream school
D33 Hospital play specialist
Dr Jayesh Bhatt
September 2015
Appendix Index
Appendix 1 Management of Baby with Echogenic bowel at risk of CF
Appendix 2 Outpatient Clinic Record
Appendix 3 Clinic letter proforma
Appendix 4 GP Antibiotic Guideline
Appendix 5 Letter for GP & Family re sputum result and antibiotic required
Appendix 6 Scoring Systems
Northern CXR score
Shwachman score
Exercise tolerance Score
Appendix 7 Annual Assessment Sheets
Appendix 8 Gastrostomy Care
Appendix 9 Guidelines for unblocking a Portacath
Appendix 10 Paediatric guideline for once daily tobramycin in cystic fibrosis
Appendix 11 Prescribing nebulisers on the ward
Appendix 12 (a) Bronchoscopy Record Sheet
(b) Bronchoscopy Booking Form
Appendix 13 (a)
(b)
(c)
(d)
Transition boys transfer folder

Transition girls transfer folder
Transition - young person information leaflet
Transition - parent information leaflet
Appendix 14 CF Formulary
Appendix 15 Giving 1st dose of intravenous antibiotics at home
Appendix 16 UK Paediatric Lung and Heart-Lung Transplantation Referral
Proforma
Appendix 17 Patient Summary Sheet
Dr Jayesh Bhatt
September 2015
Section 1 Diagnosis
(Revised by Jayesh Bhatt and Sonal Kansra)
Dr Jayesh Bhatt
September 2015
DIAGNOSIS
1.1 Background
Cystic Fibrosis (CF) is a genetic disorder affecting about 1 in 2500 live births in the
UK. It is likely that majority of newborns with cystic fibrosis will present through
Newborn screening programme, antenatal diagnosis or as meconium ileus.
The Cystic fibrosis consultant (Dr Bhatt, Prof Smyth or Dr Bertenshaw contacted via
Secretary extension 64041 or switchboard) should be informed as soon as possible if
diagnosis of CF is suspected in a neonate.
1.2 The Diagnosis of Cystic Fibrosis

The three main situations when CF diagnosis needs to be considered are
1. Clinical manifestations
2. Positive newborn screening test result
3. Family history
In patients with the classical clinical picture, the diagnostic tests are mainly needed to
CONFIRM the diagnosis. In other cases the diagnostic tests are necessary to
SUPPORT OR EXCLUDE the diagnosis of CF or to POINT to an alternative
diagnosis
The European Diagnostic Working Group has proposed the following terminology for
cystic fibrosis 1.
Classical or Typical CF: if they have
One or more Phenotypic characteristics (chronic sinopulmonary disease specific
or characteristic GI or nutritional abnormalities, salt loss syndromes, and male
genital abnormalities resulting in obstructive azospermia)
AND
A sweat chloride concentration of > 60 mmol/L
The vast majority of CF patients fall in this category and usually one disease causing
mutation can be identified on each CFTR gene. The patients with classical or typical
CF can be pancreatic sufficient or insufficient. The disease can have a severe course
with rapid progression or a milder course with very little deterioration over time.
Non-classic or Atypical CF:
A CF phenotype in at least one organ system
AND
A normal (< 30 mmol/L) or borderline (30-60 mmol/L) sweat chloride level
In these patients confirmation of diagnosis requires detection of one disease causing
mutation on each CFTR gene or direct quantification by nasal potential difference
measurement. The patients with Non classic or atypical CF may have multiorgan or
single organ involvement and most are pancreatic sufficient.
Equivocal Diagnosis
The increased use of neonatal screening to identify babies with cystic fibrosis has
identified a small but significant number of cases where the diagnosis is equivocal. A
European consensus for the investigation and management of infants with an
equivocal diagnosis following newborn screening has been established.2
Dr Jayesh Bhatt
September 2015
These are babies who have either: - One or no CF mutation and an equivocal sweat test (sweat chloride in the range 3060mmol/L)
- OR 2 CF mutations of unclear significance.
It is recommended that these babies have a repeat sweat test and if the diagnosis
remains equivocal a baseline clinical assessment to look for evidence of cystic
fibrosis. If there is no clinical evidence of CF they should be offered FU with a repeat
sweat test in 6-12 months. Referral for further diagnostic investigations for e.g ion
transport studies or nasal potential difference may be required and should be
discussed with the CF consultant.
CF phenotype is a composite CFTR mutations, modifier genes, lifestyle, treatment,
environment and age.
1.3 Diagnostic testing
1.3.1 Newborn screening

In 2007 neonatal screening for CF was extended to include the whole of the UK.
Children whose parents consent are screened for CF shortly after birth by means of a
dried blood spot collected on the newborn screening dried blood spots(previously
known as Guthrie Card) on the 6th day of life. False negatives, often due to rare
genotypes, can occur.
All infants with CF have some pancreatic damage at birth and this leads to a leakage
of tryptic-like substances in to the blood detected by the immunoreactive trypsin
(IRT)3 blood test. If this is raised (i.e. > 99.5th centile) genetic testing is done on the
same blood spot. This initially looks for 4 CF mutations including F508.
If only one mutation is detected, further 29 mutation analysis is carried out.
1.3.2 Necessary cautions with newborn screening

I.
As not all possible CF mutations are a part of Newborn screening, false
negatives due to a rare phenotype can occur.
II.
IRT can be falsely elevated in a hypoxic insult to the fetal pancreas, congenital
viral infections, trisomy 18 and 13, renal insufficiency, congenital heart disease
spina bifida and Nephrogenic diabetes insipidus.
III.
6% of babies with CF (including those with meconium ileus )could be missed by

newborn screening.
1.3.3 Babies with CFTR mutations detected in both genes

Babies with CFTR mutations detected in both genes (either a homozygote or
compound heterozygote) have a presumptive positive diagnosis of CF and are
reported as CF SUSPECTED.
Dr Jayesh Bhatt
September 2015
They are either

I.
Referred by the screening lab to the CF paediatric service for evaluation

(clinical assessment, sweat test, confirmatory mutation analysis.) and should
be offered an appointment with the CF consultant the following day.
II.
Referred by the neonatal team to the CF consultant paediatrician for an

assessment.
III.
When the diagnosis is based on genotype, a written report stating the genotype
must be available, before a diagnosis of CF is confirmed to the parents. This
can be in the form of a fax from the molecular genetics department to the
neonatal unit.
The consultant will discuss the diagnosis with the family, usually together with the CF
specialist nurse before and the family will be seen outside the CF clinic.
(Telephone number below) should be contacted for the result of the day 6 IRT if this
has already been sent. The GP should be sent a copy of the CF antibiotic guidelines.
Consent for Port CF should also be obtained. If the diagnosis is confirmed, the
physiotherapist and dietician will be involved and appropriate follow up arranged. A
sweat test should always be performed at around 6 weeks of age
(This can often be done earlier if diagnosis has proven difficult).
Contact telephone number: 0114 271 7000 (ask for neonatal screening)
Useful leaflet titled CF is suspected is available to download from the national
neonatal
screening
website
http://newbornbloodspot.screening.nhs.uk/cms.php?folder=2460
Although designed for asymptomatic children it still gives useful information about
sweat tests and information on further support
1.3.4 Babies with only one mutation detected on Newborn screening

Most babies with only one mutation detected will be unaffected carriers but there is a
risk that they carry a second abnormal allele not detected by the mutation panel
used. They are therefore tested again (second IRT) on a repeat dried blood spot
specimen taken between day 21 and day 28 of life and assayed for IRT. The
screening lab will ordinarily contact the neonatal unit to organise this. They would
also usually intimate the CF consultants.
If IRT is > cut-off 2 in the second sample the baby has a presumptive positive
diagnosis of CF, is reported as CF SUSPECTED and is referred to the CF
paediatric service for evaluation (clinical assessment, sweat test, further mutation
analysis)
A baby whose second sample IRT result is < cut-off 2 is a Probable carrier with a
low likelihood of CF4. See CF screening flow chart (Appendix 2) 4.
1.4 The Sweat Test
Despite the availability of genotyping (and because of its limitations) the majority of
children in whom CF needs to be excluded will undergo sweat testing. This group will
include the following:
Dr Jayesh Bhatt
September 2015
A Child with suggestive history / symptoms/ examination.

Sibling of a known case (even if asymptomatic).
Babies found to have a raised IRT but only 1 CF mutation
This is still the gold standard method of diagnosis for cystic fibrosis.5
If the genotype is shown to be homozygous F508, one confirmatory sweat test
should still be carried out. In other situations, two positive sweat tests are required for
a confirmed diagnosis. To arrange a sweat test call ext. 64932. (Clinical Chemistry)
National guidelines recommend the following sweat chloride levels:
Sweat Chloride concentrations
<40mmol/L
30-60 mmol/L
>60mmol
Suggested outcome
Normal
Suggestive but not diagnostic of CF
Supports a diagnosis of CF
CF affected patients occur with similar frequency in the 30-40 mmol/L range, as in
the 40-60 mmol/range 6 so a chloride cut off of 30 mmol/L has been suggested.6
Sodium should not be interpreted without a chloride result. A sodium value below
60mmol/L is unlikely to be associated with cystic fibrosis. Sodium values above
90mmol/L support a diagnosis of cystic fibrosis.7 A greater than 5-15 mmol/L
discrepancy between sodium and chloride requires a repeat test.
1.4.1 Stool testing

At the time of diagnosis stool should be sent for pancreatic elastase (which is a more
sensitive indicator of pancreatic function than chymotrypsin and has replaced it as a
test to indicate pancreatic insufficiency).8 Normal values are >200 mcg/g of faeces.
Samples will be sent to Leeds, by arrangement with clinical chemistry.
1.5 Testing for CF and initiating management in patients with meconium ileus
Meconium ileus is the presenting feature in about 15% of children with cystic fibrosis.
Meconium ileus in the absence of CF does occur but is uncommon.9
The baby may present with the classical features of neonatal small bowel obstruction
i.e. failure to pass meconium, bilious vomiting and abdominal distension. Important
differential diagnoses include Hirschsprungs disease and midgut volvulus. The plain
abdominal X-ray may show a distended small bowel and a granular appearance, due
to bubbles of air within the meconium. Once perforation and meconium peritonitis
have been excluded, a contrast enema can help confirm the diagnosis and will, in
many cases, relieve the obstruction. Otherwise the baby will require surgery, which
may involve an ileostomy.
Once a diagnosis of meconium ileus is confirmed
I.
CFTR gene mutation analysis should be performed. Up to 25 % of infants with
meconium ileus do not have an IRT value above the cut off level.10
II.
Sweat test are required to confirm the diagnosis
Dr Jayesh Bhatt
September 2015
III.
Chest physiotherapy should be started and the physiotherapist should see the
parents as soon as possible to demonstrate percussion and postural drainage
to the parents.
IV.
An anti-staphylococcal antibiotic should be started immediately (intravenously if

the baby is not feeding).
It is better to stop these interventions if the baby does not have CF than to allow lung
damage to occur early in life.
Early treatment with ursodeoxycholic acid should be considered in babies who have
had meconium ileus and prolonged hyperbilirubinemia especially if they have
required parentral nutrition.11
1.6 Failure to thrive

Pancreatic insufficiency is common in CF and occurs in over 90% of patients.12 A
sweat test forms part of the investigations for many children with failure to thrive. The
reason for performing the test should always be clearly explained to the parent. Tests
for malabsorption, such as microscopy for fat globules, and pancreatic elastase
should also be performed. Rectal prolapse may be secondary to failure to thrive and
may be the presenting feature of cystic fibrosis.13 Once the diagnosis has been
made, with good dietary management surgery can usually be avoided.
1.7 Chronic respiratory symptoms

Severe recurrent wheeze in young children and a persistent productive cough in
older children are indications to perform a sweat test. This is particularly important if
the child is failing to thrive or has chronic atelectasis on the chest X-ray. Finger
clubbing and nasal polyps in an older child, isolation of S aureus or P aeruginosa
from the respiratory tract are other important pointers.
CF may occasionally be raised as a differential diagnosis in children who have
atypical respiratory course on NNU. Individual cases may be discussed with the
respiratory specialists if required.
1.8 Siblings
Other children in the family should have a sweat test when the diagnosis of CF has
been made in a sibling. Some parents may wish their children to have genetic testing
for carrier status. Generally speaking this is not indicated until the child in question is
old enough to make an informed decision. Siblings of the parents may wish to seek
genetic counselling and testing.
1.9 Ante natal diagnosis genetic
Parents of a child with CF are routinely offered genetic counselling after the birth of
that child. The timing of this should be negotiated with them. When planning further
children they may wish for counselling before and/or at the early stages of the next
pregnancy. Referral to the clinical genetics department should be made if required.
Dr Jayesh Bhatt
September 2015
Some families choose to have ante-natal CVS testing in subsequent pregnancies,

giving them the choice of termination of an affected fetus or being able to prepare
themselves for the birth of a child with CF, should that be the case.
Dr Jayesh Bhatt
September 2015
1.10 Ante natal diagnosis bowel abnormalities detected on ultra sound scan
Second trimester detailed ultrasound scans are able to identify a range of structural
anomalies. Occasionally, echogenic foci and/or dilated fetal bowel are found.
There is a 4% risk of the fetus being affected with CF in the case of echogenic bowel
and a 17- 24% risk if both echogenic foci and dilated loops are found.14-17 The risk
of CF in an individual case is assessed jointly between the genetic/obstetric/neonatal
and CF consultants, and takes into account factors such as family history, or parental
carriage of the CF gene. The parents are counselled. Due to advances in
ultrasonography, an increasing number of babies are being diagnosed antenatally.
Please see Appendix 1 for the management plan for antenatal bowel abnormalities
detected on Ultrasound scan.
REFERENCES
1. De Boeck K, Wilschanski M, Castellani C, Taylor C, Cuppens H, Dodge J, et
al. Cystic fibrosis: Terminology and diagnostic algorithms. Thorax 2006;
61:627-635.
2. Mayell S, Munck A, Craig JV et al. European consensus for the investigation
and management of infants with an equivocal diagnosis following newborn
screening for cystic fibrosis. Journal of Cystic Fibrosis 2008:doi10.1016/
3. Heeley AF, Bangert SK. The neonatal detection of cystic fibrosis by
measurement of immunoreactive trypsin in blood. Ann Clin Biochem
1992;29:361-76.
4. http://newbornbloodspot.screening.nhs.uk/nat_std_cf_protocol
5. Littlewood JM. The sweat test. Arch Dis Child 1986;61:1041-3.
6. Lebeccque P, Leal T, De Boeck K. Mutations of the cystic fibrosis gene and
intermediate sweat chloride levels in children. Am J Respir Crit Care Med
2002;165:757-63.
7. Report from a multidisciplinary working group. Guidelines for the performance
of the sweattest for the investigation and diagnosis of CF in the UK. 1-112003.
8. Wallis C, Leung T, Cubitt D, Reynolds A. Stool elastase as a diagnostic test
for pancreatic function in children. Lancet 1998;350:1001.
9. Fakhoury K, Durie PR, Levison H, Canny GJ. Meconium ileus in the absence
of cystic fibrosis. Arch Dis Child 1992;67:1204-6.
10. Massie R, Olsen M, Glazner J, Robertson C, Francis I. Newborn screening for
cystic fibrosis in Victoria: 10 years experience (1989-1998). Med J Aust
2000;172:584-587.
11. Siano M, De Gregorio F, Boggia B, Sepe A, Ferri P, Buonpensiero P, Di
Pasqua A, Raia V Ursodeoxycholic acid treatment in patients with cystic
fibrosis at risk for liver disease. Dig Liver Dis. 2010 42(6):428-31.
12. Morgan W, Butler S, Johnson C. Epidemiologic study of cystic fibrosis. Pediatr
Pulmonol 1999;28:231-241.
Dr Jayesh Bhatt
September 2015
13. Stern RC, Izant RJ, Jr., Boat TF, Wood RE, Matthews LW, Doershuk CF.
Treatment and Prognosis of rectal prolapse in cystic fibrosis. Gastroenterology
1982; 82:707-10.
14. Ghose I, Mason, G C, Martinez, D, Harrison K L et al. Hyperechogenic fetal
bowel: Prospective analysis of 60 consecutive cases. BJOG 2000;107:426-9.
15. Muller F, Dommergues M, Simon-Bouy B, Ferec C, Oury J-F, Aubry M-C et al.
Cystic fibrosis screening: a fetus with hyperechogenic bowel may be the index
case. J Med Gene 1998;35:657-60.
16. Muller F, Simon-Bouy B, Girodon E, Monnier N, Malinge M C, Serre J L and
the French Collaborative Group. Predicting the risk of cystic fibrosis with
abnormal ultrasound signs of fetal bowel:results of a French Molecular
Collaborative Study based on 641 prospective cases. Am J Genet
2002;110:109-15.
17. Yaron Y, Hasson S, Geva E, Kuferminic K, Yavetz H, Evans M I. Evaluation of
fetal echogenic bowel in the second trimester. Fetal Diagn Ther 1999; 14:17680.
Dr Jayesh Bhatt
September 2015
Dr Jayesh Bhatt
September 2015
Section 2 - Outpatients Clinic

(Revised by Jayesh Bhatt)
Dr Jayesh Bhatt
September 2015
2.1 Outpatient Clinic Arrangements
The CF clinic is held every Tuesday afternoon. Children with established

Pseudomonas aeruginosa infection are seen in separate clinics from those without
Pseudomonas. Pseudomonas and Non Pseudomonas clinics alternate every week.
Each child is allocated an individual room and should not wait in the waiting area.
Parents may bring the child any week on request and this is preferable to being
seen on the ward. If the child is to be seen in the wrong clinic, please arrange for
non-Pseudomonas patients to be seen first on a Pseudomonas week and
Pseudomonas patients to be seen last on a non-Pseudomonas week. Children with
Burkholderia cepacia complex infection should be seen at the end of a
pseudomonas clinic where possible and arrive at 16:00 hours. The same applies to
patients with MRSA who are also colonised with P.aeruginosa. Patients with MRSA
who do not have P.aeruginosa may be seen at the end of a non-pseudomonas
clinic.
A Respiratory exacerbation is defined as an increase in respiratory symptoms
(increased cough, sputum production or chest congestion, change in sputum colour
to green, diminished exercise tolerance or dyspnoea with exertion, increased
school/work absenteeism), reduced appetite, fall of 10% or more in lung function or
weight loss defines a respiratory exacerbation1. Symptoms are more predictive than
findings and on examination adventitial sounds may or may not be present. It is not
necessary to perform a chest X-ray. Consider oral or intravenous antibiotics.
All new isolates of Multi or Pan- resistant Pseudomonas and Burkholderia cepacia
complex need to be discussed with the Consultant. These will need sending to the
reference lab at Colindale or Edinburgh for typing by the Department of
Microbiology.
Children are usually seen every 2 months. Parents and child should see the
physiotherapist and dietician at least twice a year in clinic.
Compressors for nebulised drugs are lent from the clinic and should be serviced 12
monthly.
2.2 At every clinic visit:

Use the clinic proforma to record the relevant details (see Appendix 2).
The nursing staff will record height, weight and oxygen saturation individually in the
weigh room and perform lung function (>5years) using specified equipment and
obtain sputum (preferably) or cough swab for culture in the allocated room.
Bacterial filters are used when performing lung function. All children are asked to
use the alcohol gel prior to lung function and the machine is cleaned between
patients using Clinell Universal Sanitising wipes. Bacterial filters are disposed of in
the clinical waste bags in the room. For children with BURHOLDERIA CEPACIA
COMPLEX OR MRSA, all procedures are performed in the allocated room with
Dr Jayesh Bhatt
September 2015
portable equipment. These children can be weighed and heighted in the weigh
room, ONLY if no other children are due in clinic.
Surfaces are cleaned with Clinell Universal Sanitising wipes. The windows are
opened for airing (for a minimum of 30 minutes) CF Infection Control Group
reviewing Sept 09.
The play specialist will provide age appropriate activities for the rooms for the child
and their siblings.
A nebuliser and hypertonic saline will be available in clinic if a child requires an
induced sputum sample (Refer to section 5 /12 /13).
Other:
Advice e.g. flu immunisation (children over 6 months from September - November),
salt replacement in hot weather (see section 9.7)
Next clinic visit (give the parents a specific date do not overbook the clinics)
2.3 Multidisciplinary meeting:

Occurs immediately after the CF clinic. All staff attending the clinic should take part
in this meeting. Ensure any arranged admissions are notified to the ward. All
outcome forms are collected at the end of clinic to ensure all activity is recorded.
2.4 Post Clinic Administration:

Clinic letters to GPs (with copy to parents - see below) should be dictated and given
to the consultants secretary by the Monday following the Tuesday clinic. All cough
swab / sputum results are reviewed and appropriate action taken by the Consultant
on service for that week on the Monday following the Tuesday clinic. Trainees are
encouraged to participate in this meeting. The parents and GP can be notified of
any antibiotic treatment necessary using a standard letter. This is available from the
CF secretary (appendix 5). It is faxed the same day. Record that the letters have
been sent and what treatment has been given in the case notes. Telephone the GP
and the parents if the result is urgent. In any case a standard clinic letter should still
be written. A copy of the GP letter should be sent to the child's parents (and child if
appropriate).
2.5 Holiday advice
Reserve antibiotics these should be considered individually for each patient.

Oxygen requirements Patients with an FEV1 <50% predicted should receive
supplementary oxygen in flight at 2 L/min.2 This must be arranged in advance
with the airline, some of whom will make a charge.
Portable nebulisers these are available from childrens outpatients.
Discuss if enteral feeds can be discontinued whilst on holiday.
Suggest that portable wedges are available from the physiotherapists.
Dr Jayesh Bhatt
September 2015
Consider salt replacement for holidays in hot climates.

Advise the use of sun block or advise to stay in the shade if patient is currently
taking ciprofloxacin or will be using it as their reserve antibiotic.
Provide a letter for the patient to take through customs (if he or she is going
abroad) giving full details of the current medications.
The medical staff may be required to write a letter for the insurance company
with regard to the patients fitness to travel. Do this as close as possible to the
departure date.
REFERENCES
1. Rosenfeld M, Emerson J, Williams-Warren J, Pepe M, Smith A, Bruce
Montgomery A, et al. Defining a pulmonary exacerbation in cystic fibrosis. J
Pediatr 2001;139(3):359-365.
2. Buchdahl RM, Francis J, Bennett S, Sheehan D, Bush A. Hypoxia during flight an audit of the fitness to fly test in children with cystic fibrosis (CF). Arch Dis
Child 2000; 82(suppl I):A43.
Dr Jayesh Bhatt
September 2015
Section 2a Transfer to Adult Care

(Revised by Carol Bertenshaw)
Dr Jayesh Bhatt
September 2015
Transition
2a.1 Definition
A purposeful, planned process that addresses the medical, psychosocial and
educational needs of adolescents and young adults with chronic physical and
medical conditions as they move from child and family centred to adult orientated
health care systems. Transition is merely one part of the wider set of educational,
personal, family and social transitions young people make during adolescence.
2a.2 Background
The need for young person centred planned transition processes for all young people
with chronic disease has been highlighted by a number of recent governmental
reports. Ensuring a safe and effective transition from paediatric to adult health
services is a key quality issue for the NHS. This has become a particular issue in
cystic fibrosis care in recent years due to the huge improvement in survival. The vast
majority of children with cystic fibrosis will now survive to adult life. Poorly planned
transition can be associated with increased non adherence to treatment and lack of
follow up. This can lead to adverse consequences, in terms of morbidity and
mortality, as well as in social and educational outcomes. There is a national service
specification for CF transition in development. The Nottingham CF transition service
meets all the proposed criteria.
2a.3 Hospital Youth Service

There is a well developed and active Youth Service run by enthusiastic youth workers
for all hospital inpatients or those with chronic illness. In hospital there is a drop in
youth room. A regular youth club is based just off the hospital campus and can be
attended by inpatients who are well enough or outpatients. Regular activities, trips
and holiday camps are arranged. A youth forum is also coordinated by the youth
service. For patients with CF there are obviously infection control issues and these
should be considered when accessing the youth service.
2a.4 Young Persons CF Clinic

For Nottingham Patients: From 14 years of age patients are invited to a 6 monthly
clinic held at the site of the Adult CF Clinic at Nottingham City Hospital. At this clinic
they meet members of the adult CF Team along with the paediatric team. The focus
is on developing skills required for independence, such as encouraging self
management and confidence in conducting a consultation. Another primary aim of
the clinic is to support parents through the transition process. Clinical responsibility
for the patient remains with the paediatric team although over time the adult team
become more involved in the clinical assessment and negotiated management plan
for each patient. Immediately prior to the clinic both multidisciplinary teams meet to
share information regarding the families due to attend. At the end of the clinic team
members meet to discuss the patients seen and review those soon to be invited.
Dr Jayesh Bhatt
September 2015
For Shared Care Patients: Patients who currently have most of their care at a DGH
with visits from the Nottingham CF paediatric MDT are considered on a individual
basis due to the smaller number of patients. Patients and their families may be
invited to the young persons clinics held in Nottingham. The adult CF team visit
some shared care centres on an occasional basis to hold clinics alongside the DGH
team.
2a.5 Individual Transition Plan (see appendix 13)

The CF transition service has developed an individual transition plan which is used
for each patient attending the clinic. This document is based on a generic document
produced by an NUH transition working party. This is a useful tool to ensure that
there is an accurate record of the young persons medical condition and treatment
and also helps guide discussion on the knowledge and skills required for
independence.
When a patient is attending the transition clinic they have City hospital notes (North
notes) all subsequent correspondence is then copied to both the South and North
notes. By the time of transfer over to the adult service a comprehensive set of
medical notes exists ensuring that all important current clinical information is held by
the adult team.
2a.6 Transfer
When the patient, paediatric and adult team agree that the time is right to transfer to
adult services an appointment is made to attend the adult clinic. Patients who have
completed the transition process and have recently been transferred to adult services
will be discussed at the young peoples clinic. Issues that may occur early after
transfer such as non attendance can then be identified and addressed jointly.
Dr Jayesh Bhatt
September 2015
TRANSITION FROM DIAGNOSIS TO ADULT SERVICES
Informed of the CF Trust
Diagnosis
Given written information

about CF and the CF service
Regular invitation to
parents evenings
2yrly invitation to
parents evening with
the adult CF team
Ensure aware of
youth service
Over the first 2 or 3 clinic

appointments introduction to
all members of MDT
.
Sent copies of all clinic letters

and yearly annual assessment
report
4 years
and
11 years
CF Nurse and physio school

visit for individual children as
appropriate
From 13 years invited to

attend part of each medical
consultation on own
13 years
Individual transition plan

reviewed and updated at
each young persons clinic.
14 years
Opportunity to visit adult

Ward
Transfer to
adult services
Copy of this and further annual

assessment reports sent to
patient and parent
Invitation to attend 6 monthly

young persons CF clinic
When patient, paediatric and

adult team agree that the time
is right to transfer to adult
services.
Regular communication
with adult CF Team at the
YP clinic regarding
progress of transferred
adult
Dr Jayesh Bhatt
September 2015
Useful resources/references:
National Service Framework for Children, Young People and Maternity
Services. Department of Health, 2004
Transition: getting it right for young people. Department of Health, 2006
Bridging the gaps: Healthcare for adolescents. Report of the Intercollegiate
Working Party on Adolescent Health, 2003.
Youre Welcome Quality Criteria: Making health services young people
friendly. Department of Health, 2005
RCPCH: Adolescent Health Project
Viner RM. Transition of care from paediatric to adult services: one part of
improved health services for adolescents. Arch Dis Child 2008;93:160-163
www.yphsig.org.uk website of the young peoples health special interest group
28
Section 3 - Annual Assessment

(Revised by Amanda Ward & Jayesh Bhatt)
29
3.1 Annual assessment

This is undertaken around the childs birthday to assess progress and identify
areas where treatment can be improved. An individual treatment plan should
be formulated after the annual review. It should be postponed if the child
is unwell. It is usually organised at an out-patient visit, but can be done at the
end of a hospital admission. A separate annual assessment out patient clinic
is held on most Wednesdays of the year. This is planned a year in advance
and parents are sent out appointment letters nearer the time with a list of
topics that they may want to discuss at the annual assessment.
At the clinic attendance prior to the annual assessment an electronic x-ray
request should completed so that the chest x-ray can be carried out on their
arrival. The "annual assessment blood tests" are often performed at this
attendance so the results are available when the annual assessment is
performed. For children aged 10 years or over an oral glucose tolerance test
should be performed annually. This should be arranged as a day case
admission to D33, prior to the annual assessment clinic visit. (See section
8.6.1)
3.2 Medical review

Use the annual assessment proforma sheet to perform the review.
See appendix 7
3.3 Documentation (see Appendix 7)

When completing the report, please fill in the annual assessment summary
sheets and also ensure the general summary sheet is up to date. The
report is dictated as shown on the annual assessment sample letter.
(Appendix 7). The CF Trust Standards of care document recommends that It
is good practice if the CF Consultant writes the final report (not a training
grade doctor even if training in cystic fibrosis) and sends to the GP or
where appropriate to the referring hospital Consultant with copies to other
colleagues involved in the Annual Review or the patients care. Copies of the
annual assessment are sent to the parent (and the child if a teenager),
Parents and patients will vary in the amount of detail they will find useful.
Always offer to elaborate on any questions in the next clinic.
3.4 Computer database

All annual assessment details are entered on the UK CF registry (Port CF)
by the data entry clerk.
3.5 Multidisciplinary review
At the time of the annual assessment, a review of care should be carried out
by the dietician, physiotherapist, CF social worker and clinical psychologist if
appropriate.
30
Section 4 - Ward Management of CF Patients

(Revised by Dr. Jayesh Bhatt)
31
4.1 Segregation policy

There is a segregation policy on the ward. This reduces the risk of cross
infection between CF patients and also those with bronchiectasis. There is no
risk to patients without CF or bronchiectasis.
Pseudomonas aeruginosa
Patients colonised with P.aeruginosa should be admitted to a cubicle on D33
and should not mix with non- Pseudomonas CF patients (e.g playroom,
classroom etc).
Resistant strains of Pseudomonas aeruginosa
Where patients carry resistant strains of Pseudomonas, their inpatient
accommodation should be in on D33 in the negative pressure cubicles
MRSA
All patients who carry MRSA should be confined to a cubicle on D33 for the
duration of their admission. A patient may be considered to be free of MRSA
after 6 months of negative sputum results (minimum 3 negative sputum
results) and a negative nasal swab.
Burkholderia cepacia complex
Where patients carry Burkholderia cepacia complex, their inpatient
accommodation should be arranged on D34 /D35 following discussion with
the on call CF consultant.
4.2 Admission procedure

The most common indication for admission is for 2 weeks of intravenous
antibiotics to treat a pulmonary exacerbation. Other indications include DIOS
or pre-operation.
The following general guidelines apply:
Nursing responsibilities:
Weigh and measure all children on admission
Measure lung function (> 5 years) and SaO2 (all patients)
Collect a sputum sample / cough swab on admission and discharge
Collate the weight and lung function on the CF review sheet.
Needle the port-a-cath if present, at the same time blood is taken for renal
profile, FBC, magnesium and CRP (medical staff may request additional
blood tests).
Complete patient contract for adolescent patients and for younger ones if
appropriate.
32
Medical responsibilities:
Perform full history and examination
Record the weight and lung function on CF review sheet
Plot weight and height on the growth chart
FBC, magnesium and CRP, total Ige, aspergillus specific IgE and
aspergillus precipittins. If no port-a-cath in situ then a long line is
preferable to a cannula. However, consider each childs preferences and
past procedure history. (section 10).
Prescribe IV antibiotics either after discussion with the doctor who planned
the admission, or by checking the OPD review sheet if admitted following
a clinic review.
Check the drug doses from the CF guidelines initially. If the antibiotic
dosage is not present in the CF guidelines, use the BNF for children.
Indicate on the drug chart whether drug levels and blood test need to be
taken and when (e.g. When on Tobramycin, venous levels pre drug
required with UE on Dose 2 and 8)
Prescribe appropriate flushes of heparin and Saline, Pg 34
Prescribe oral nystatin for children with a port-a-cath for two weeks whilst
on antibodies and on TTO for one week after discharge. (see section 5.4)
Prescribe home IVs if indicated. See section 6
Liaise with the physio, CF nurse and dietician of the admission (see
section 11).
Chase recent cough swabs/ sputum result and put on flow sheet at front of
notes.
Make a note on review sheet regarding date and antibiotics used for
admission, to help with annual review.
4.3 Ongoing care

Nursing responsibilities:
Record weights on Monday, Wednesday.
Record lung function on Monday and Wednesday.
Send a sputum sample / cough swab mid treatment if poor clinical

progress or deterioration.
Liaise with the dietician where appropriate
Arrange home IVs if appropriate.
Oxygen should be administered to keep SaO2 > 90%.
Medical responsibilities:
Gel hands before and after entering each childs room.
Patients should be seen and examined daily by the ward team.
Consultant ward rounds are on either Tues 9am ,Wed 9am,Thurs 9am
Flow sheets should be used to collate sputum, blood results, antibiotic and
new therapies summaries.
If new therapies are used, please write down after liaising with the
consultant what lung function is used as the baseline to assess effect of
the treatment.
33
There is a multidisciplinary meeting held on Tuesday at 12pm in Ward D33

resource room. The ward F1/ SHO should summarise and present CF
patients using the proforma already in use.
If a parent requests a non-emergency review (please see flow chart **)
liaise with the respiratory SpR or CF nurse regarding the most appropriate
time for a review. Ideally, patients will be reviewed following the consultant
ward round or the most suitable next available clinic at the discretion of
the team.
If there are difficulties with venous access during the night or if the child is
particularly needle phobic and not clinically deteriorating, it may be more
appropriate to discuss this with the child, parents and nursing team and to
re-obtain venous access the following work day.
34
Section 5 Obtaining respiratory specimens for

microbiology & prescribing antibiotics
(Revised by Alan Smyth & Antony Mazzei)
Please see the UK CF Trust antibiotic guidelines for a more detailed

discussion of the management issues discussed in this section.1
http://www.cftrust.org.uk/aboutcf/publications/consensusdoc/Antibiotic_treatment_for_Cystic_Fibrosis.pdf
35
5.1 Collection of specimens for respiratory culture
5.1.1 Cough swabs & sputum

Each time children attend CF clinic, a cough swab or sputum sample is sent
for culture and so a culture result will usually be available from within the last
8 weeks. Empirical prescriptions should be guided by the last positive culture
result. When a positive result is received, antibiotic treatment should always
be given, even when the child is asymptomatic. (The only exception to this
rule are organisms designated as coliforms often found in young children.)
Culture results from the Tuesday CF clinic are usually available on the
following Monday. The results are collated by the CF Clinical Nurse
Specialist. Individual results should be checked by the consultant on
service.
When a child has a positive culture and antibiotics are prescribed it is
important that arrangements are made for the cough swab/sputum sample to
be repeated (at the end of the antibiotic course). Prescriptions are usually
arranged on a standard letter, which is sent to the child's GP and parents
(see appendix 5). A microbiology form should be sent to the family with this
letter. Cough swabs/sputum samples can be performed in children's OPD at
a time to suit the family. If this is inconvenient the family should contact one
of the CF nurses. Alternative arrangements can then be made.
Each time a child is seen for ward review it is essential to send a cough swab
or sputum and to measure lung function in children over 5 years.
5.1.2 Flexible bronchoscopy and bronchoalveolar lavage

Some children with CF, who have previously been free of cough, may
develop a wet cough, which persists for many weeks, in the face of negative
cultures and a poor response to oral antibiotics. Where this happens, it is
important to consider differentials such as allergic bronchopulmonary
aspergillosis (ABPA) (section 7.1) and cough related to bronchospasm.
However no child with CF, who has previously been symptom free, should be
allowed to develop a chronic wet, cough without full investigation which may
include induced sputum or flexible bronchoscopy. When the induced sputum
or bronchoalveolar lavage specimens are sent for culture, it is important to
request culture for a full range of CF pathogens, including atypical
mycobacteria.
Indications for sputum induction or bronchoscopy:
A wet cough has persisted for 6 weeks
Two courses of oral antibiotics (each for 2 weeks) have been given
without improvement
Respiratory cultures are negative
36
Perform sputum induction. Sputum induction can be performed after a

shorter period of cough, if there is clinical concern. If culture still negative
proceed to flexible bronchoscopy and lavage.
Also consider flexible bronchoscopy when a child is having a general

anaesthetic for another reason e.g. insertion of portacath
Consider combining flexible bronchoscopy with physiotherapist-directed
bronchial lavage. A non bronchoscopic bronchial lavage can be
requested from anaesthetic colleagues if bronchoscopy not possible.
Procedure for induced sputum:

This procedure is not suitable for pre-school children. Induced sputum
should always be collected with the help of a physiotherapist or specialist
nurse.
Administer Salbutamol 4 x 100mcg via MDI & spacer
Using a syringe, draw up 4ml of 7% saline (Nebusal Forest)
Nebulise, using the patients own nebuliser (or a Pari LC plus if
unavailable)
Withdraw 2 ml from a 4 ml vial of vial of hypertonic saline and dilute to
4ml with sterile water (3% saline solution)
Ask the child to expectorate (with assistance from the physiotherapist or
nurse)
If insufficient sputum obtained:
Repeat Salbultamol 4 x 100mcg
Nebulise a 4ml vial of hypertonic saline without dilution
Ask the child to expectorate again
5.2 Oral Antibiotics

5.2.1 Staphylococcus aureus (sensitive strains)
Children under 3 years: This organism is common in young children and is
often difficult to eradicate. Prophylactic antibiotics are therefore given
continuously from birth until 3 years of age.2 At 3 months of age, S. aureus is
present in the lower respiratory tract of up to one third of untreated infants
with CF.3 If S. aureus is isolated while on prophylactic antibiotics then the
child should be given the treatment dose of flucloxacillin for 2 weeks (see
below). If the repeat cough swab is clear then the child should restart the
prophylactic dose.
Prophylactic dose:
Flucloxacilli
Age
n
Up to 3 years
ORAL
Dose
Frequency (times daily)
125mg
Liquids are available as 125mg/5ml and 250mg/5ml. Some parents prefer to

have the higher strength to reduce the volume administered please specify
on the prescription if the 250mg/5ml strength is required. The Floxapen
brand (Actavis UK Ltd, not available in the hospital) tastes different to the
other brands and may be tolerated by young children who spit out other
37
brands. To obtain this brand it will need to be specifically prescribed as

Floxapen by the GP.
Older children: a 2 week course of treatment should be given whenever the
organism is isolated.
First Line:
Flucloxacillin
Age
Dose
Frequency
(times daily)
Duration
1 month 25mg/kg
4
2 weeks
18 years
Maximum single dose 1g.
Total daily dose can be given in 3 divided doses if necessary to improve
adherence
ORAL
If S.aureus continues to be isolated when the patient has been prescribed a

course of flucloxacillin first consider adherence to treatment. Then add a
second antibiotic for 2 4 weeks (e.g. fusidic acid or rifampicin). If S.aureus
continues to be isolated then discuss with consultant.
Fusidic
acid
ORAL
Age
Frequency
(times
daily)
Duration
2 - 4 weeks
250mg
5 -12 years
500mg
Over 12
years & Adult
750mg
(doubled
for severe
infections)
2-4
weeks
2-4
weeks
2-4
weeks
1 month 1
year
1 - 5 years
Dose
Suspension
(fusidic
acid)
15mg/kg
Tablets
(sodium
fusidate)
500mg
3
(doubled
for severe
infections
)
Suspension contains fusidic acid (250mg in 5ml) and tablets contain sodium
fusidate (250mg per tablet).
750mg fusidic acid is equivalent to 500mg sodium fusidate.
Clarify which salt is required at the time of prescribing.
38
Rifampicin
Age
Dose
ORAL
1 month
1 year
1 18
years
5 10mg/kg
10mg/kg (Maximum
450mg <50 kg & 600mg
>50kg)
Frequency
(times
daily)
2
Duration
2 weeks
2 weeks
5.2.2 Haemophilus influenzae

This is a frequent cause of exacerbations in school age children.4 Most forms
found in children with CF are non-encapsulated and so children are not
protected by HiB immunisation. Recommended first line treatment is coamoxiclav (Augmentin) (which will also treat concurrent infection with S.
aureus). Second line is cefaclor which is also suitable for infections with
Moraxella catarrhalis. Antibiotics are often given to children with CF at the
time of a cold. A cough swab or sputum sample should be taken and
antibiotics prescribed to cover H. influenzae. The antibiotic prescribed can
be reviewed in light of the culture result.
Coamoxiclav
(Augmentin)
ORAL
Age
Preparation
Dose
1 month
1 year
0.5ml/
kg
12-18
years
125/31 in
5ml
suspension
250/62 in
5ml
suspension
250/62 in
5ml
suspension
Or
500/125
tablet
500/125
tablet
Cefaclor
Age
Dose
Frequency
(times daily)
Duration
ORAL
0-1 year
1-7 years
over 7
years
125 mg
250 mg
500 mg
3
3
3
2 weeks
2 weeks
2 weeks
1- 6 years
6-12
years
Frequency
(times
daily)
3
Duration
5ml
2 weeks
10ml
2 weeks
1 tab
2 weeks
1 tab
2 weeks
2 weeks
39
5.2.3 Mycoplasma or Chlamydia pneumoniae

In children who are penicillin allergic, or where the chest X-ray and serology
suggest these organisms use clarithromycin.
Clarithromycin
Weight /
Dose
Age
< 8kg
7.5mg/kg
ORAL
8 11kg
62.5mg
12 19kg
125mg
20 29kg
187.5mg
30 40kg
250mg
12 18
250mg
years
Doses can be doubled in severe infections.
Maximum Adult dose 500mg bd
Frequency
(times daily)
2
2
2
2
2
2
Duration
2 weeks
2 weeks
2 weeks
2 weeks
2 weeks
2 weeks
5.2.4 Preventing chronic infection with Pseudomonas aeruginosa

Most patients with CF will eventually develop chronic infection with P.
aeruginosa. This is associated with more frequent exacerbations, productive
cough and a drop in lung function.5 Acquisition of P. aeruginosa can be
prevented at least temporarily by treatment with ciprofloxacin orally and
nebulised colistin.6 A 3 week course can achieve eradication in up to 80% of
newly infected patients.7
Nebulised colistin can provoke bronchospasm in some patients this usually
occurs immediately after the drug has been given.8 This should be
mentioned to the patient/parent. Salbutamol may be given prior to nebulised
colistin9 this may also aid drug delivery.
The schedule is as follows for asymptomatic children. For children with
increased cough or reduced lung function consider intravenous treatment.
Nebulised colistin should still be given as intravenous antibiotics alone are
successful in eradicating P.aeruginosa in only 20% of cases.10 Nebulised
antibiotics are not given on the ward. Therefore, nebulised colistin should be
given at home, once the child has been discharged.
TORPEDO-CF: Starting in 2010 our CF clinic will participate in a large
randomised controlled trial of intravenous antibiotics vs. oral
ciprofloxacin (both with nebulised colistin) for eradication of P.
aeruginosa in pseudomonas-nave and pseudomonas-free patients. All
patients previously negative, who have a new isolate of P. aeruginosa,
should be considered for this trial.
40
When P. aeruginosa is isolated this should be clearly documented in the

hospital notes with details of the eradication therapy prescribed. When the
prescription is written the "step" being prescribed should be stated. Discuss
new infections of P.aeruginosa with the consultant. The family may wish to
meet the consultant to discuss the isolation of P.aeruginosa. If the child has
problems taking the prescribed medication this should be discussed with the
consultant.
Step 1 Eradication Therapy:
Step 1
Oral
Ciprofloxacin
NEBULISED
Colistin
1month 18
years
1 month
18 years
Dose
20mg/kg
(max 750mg)
1 mega unit
Frequency
(times daily)
2
Duration
3 weeks
3 weeks
Also prescribe 1x5ml 0.9%sodium

chloride/dose (use 4ml of this to reconstitute
Colistin vial)
Repeat cough swab/sputum: negative - stop treatment; positive - step 2.
Step 2
ORAL
Ciprofloxacin
NEBULISED
Colistin
1 month 18
years
1 month 2
years
2 18 years
Dose
20mg/kg
(max 750mg)
1 mega unit
Frequency
(times daily)
2
Duration
3 weeks
3 weeks
2 mega units
3
3 weeks
Also
prescribe
1x5ml
0.9%
sodium
Colistin vial)
If repeat cough swab/sputum is negative, stop treatment. If it is positive then

proceed to step 3.The highest eradication rate for P.aeruginosa is achieved
with step 3.11 Patients on step 3 should be seen at the end of the clinic.
Step 3
Dose
ORAL
Ciprofloxacin
1 month
18 years
20mg/kg
(max 750mg)
NEBULISED
Colistin
1 month 2
years
2 18 years
1 mega unit
Frequency
(times daily)
2
Duration
3 months
3 months
2 mega units
3
3 months
Also
prescribe
1x5ml
0.9%
sodium
Colistin vial)
For some patients, nebulised tobramycin (300mg twice daily) for 28 days is
an alternative eradication regimen, which has been shown to achieve good
rates of eradication12.
41
Subsequent positive cultures for P. aeruginosa.

If after step 1 repeat cough swabs are negative but the patient has
subsequent positive cultures step 2 should be prescribed.
If the patient has a positive culture and has received step 2 at any time
previously then prescribe step 3.
If a patient has previously received step 3 then after a 6 month "clear"
period re-isolates P. aeruginosa they can repeat step 3.
The doctor who initiates eradication treatment must also arrange the
repeat cough swab or sputum specimen. The result will be reviewed at
the weekly microbiology meeting between the consultant and the CF
Clinical Nurse Specialist. The community nurses are usually happy to
arrange specimen collection.
Patients may be classified into one of four groups, based on Pseudomonas
status (UK CF Trust Registry Guidelines):
1. Never grown P. aeruginosa
2. Free of P. aeruginosa for at least 1 year
3. Intermittent grower of P. aeruginosa with <3 positive samples in the last
year.
4. Chronic P. aeruginosa infection with >3 positive samples in the last year.
If a patient isolates P. aeruginosa within 6 months of completing step 3 they
will be in the intermittent or the chronic category of infection. This
decision should be discussed sensitively with the parents (and child where
appropriate). The child should commence maintenance nebulised antibiotics
(see 5.3) and be seen in the "pseudomonas clinic". Before a patient moves
from category 3 into category 2 ensure that induced sputum or bronchial
lavage specimens have been collected on at least on occasion over the
previous 1 year and that they are negative for P. aeruginosa. Discuss with
consultant.
5.2.5 Long term Azithromycin

Clinical trials have shown long term azithromycin, may be of benefit to some
patients, with established P. aeruginosa.13;14 There is more recent evidence
that azithromycin is helpful in the absence of P. aeruginosa 15. In patients not
responding adequately to conventional treatment a 6 month trial should be
considered. In children who produce sputum, ensure that the last annual
assessment sputum specimen for non tuberculous mycobacterium was
negative, before commencing azithromycin.
Azithromycin
ORAL
Age
6 18 years
and less
than 40kg
6 18 years
and more
than 40kg
Dose
250mg
Frequency
3 times a week
e.g.mon,wed,fri
500mg
3 times a week
e.g.mon,wed,fri
Duration
Initial 6
month
period
Initial 6
month
period
42
5.2.6 Treatment of exacerbations in children with chronic P. aeruginosa

infection
Colds and mild exacerbations can be treated with oral ciprofloxacin. The dose
is the same as for eradication therapy (see section 5.2.4) but courses are
usually for 2 weeks. More severe exacerbations need intravenous treatment
(see below).
5.3 Nebulised antibiotics
5.3.1 Colistin
Maintenance Therapy:
Colistin
Age
Dose
0.5 1 mega
units. Discuss
with consultant
2 - 10 years
1 mega unit
> 10 years
2 mega units
Also prescribe 1x5ml 0.9%sodium chloride/dose
NEBULISED
1 month 2
years
Frequency
(times daily)
2
Duration
2
2
Lifelong
Lifelong
Lifelong
Colistin comes as a powder for injection, reconstituted in 4 ml of 0.9%

sodium chloride. Staff in childrens outpatients will supply the appropriate
nebuliser. A different brand of colistin, Promixin (Respironics), is supplied
with an Adaptive Aerosol Delivery system (AAD) this dispenses the drug in a
shorter time (around 4 mins). If the patient finds the administration time of
the standard nebuliser problematic then Promixin may improve compliance.
This drug is more expensive and before a prescription is issued it should be
discussed with the child's GP. The nominal dose of Promixin is the same but
the dose released from the nebuliser is less, as the nebuliser is more
efficient. The dose and fill volume for the iNeb device are summarised in the
table.
Nominal dose
of colistin
Number of
1MU vials
Vol. diluent
Fill volume
Concentration
1 mega unit
2ml
1ml
2 mega unit
1ml
1ml
0.5 mega
units/ml
1 mega units/ml
5.3.2 Nebulised Tobramycin

Consider prescribing the preservative free preparation of tobramycin
(TOBI/Bramitob) as an alternative maintenance therapy to colistin if colistin is
poorly tolerated, if there is additional chronic infection with S. aureus or if
43
clinical progress is unsatisfactory.

consultant.
(TOBI/Bramitob)
This should be discussed with the
Age
Dose
Over 6 yrs
300 mg
Frequency
(times daily)
2
TOBI/Bramitob is administered in cycles of 28 days followed by a 28 day

break.
5.4 Intravenous antibiotic treatment
The antibiotic used is determined by the most recent organism isolated. A

cough swab/sputum should always be sent before starting intravenous
antibiotics and the antibiotics used may need to be changed, depending
on the result.
Blood should be sent for electrolytes (including magnesium) and
creatinine when the long line is inserted or the port-a-cath accessed.
Lung function should be recorded in the notes and the discharge
summary at the beginning and the end of each course of intravenous
antibiotics.
Courses of intravenous treatment should be for at least 2 weeks.
Where P. aeruginosa has been isolated (even on one occasion) antipseudomonal antibiotics should be given.
A dose should be prescribed which can be conveniently administered e.g.
consider vial size. The dose prescribed should be within 5% of the dose
calculated on the patient's weight.
Patients with an indwelling intravenous device such as a port-a-cath
should receive oral nystatin (5ml qds) to slosh and swallow throughout
every course of intravenous antibiotics and for 1 week after the course is
completed. This will suppress oral and gut carriage of candida and may
prevent a port-a-cath becoming infected with candida.
5.4.1 Haemophilus influenzae

Where recent isolates have shown only H. influenzae give intravenous
cefuroxime as a single agent.
Cefuroxime
Dose
IV
5060mg/kg
Frequency
(times daily)
3 or 4
Administration
Bolus over 3
5 mins or 30
min infusion
Duration
(course)
2 weeks
Maximum dose 1.5g

For home treatment change to Ceftriaxone 50mg/kg (maximum 4g) once
daily
44
5.4.2 Staphylococcus aureus:

If a patient has previously isolated P. aeruginosa, give IV anti-pseudomonal
antibiotics (see 5.4.4 below) and oral flucloxacillin (see treatment dose in
section 5.2.1). Intravenous flucloxacillin may also be used for S. aureus. If a
patient has isolated MRSA in the past then prescribe teicoplanin.
First Line:
Flucloxacillin
Dose
Frequency
(times daily)
4
Administration
Frequency
(times daily)
2 (for first 3
doses)
Administratio
n
Bolus over 1
min or 30 min
infusion
Duration
(course)
2 weeks
Bolus over 3
5 mins or 30
min infusion
Dose can be doubled in severe infection (maximum single dose is 1g)
Total daily dose may be given in 3 divided doses
IV
25mg/kg
Second Line:
Teicoplainin
IV
Loading
dose
Dose
Continued
dose
10mg/kg
10mg/kg
(max
400mg)
Duration
(course)
2 weeks
(max
400mg)
Vial sizes available are 200mg and 400mg.
45
5.4.3 Multiply resistant Staphylococcus aureus (MRSA)

Eradication is difficult (sometimes impossible) and intravenous treatment
may be required. Discuss the treatment plan with the consultant. Take the
following samples before starting treatment: cough swab/sputum sample,
anterior nares swab and perineal swab. Add mupiricin cream if nasal
carriage is present, apply 3 times a day for 5 days. In addition the patient
should wash with chlorhexidine 4% scrub (or octenisan if chlorhexidine
contraindicated) for 5 days, during this time the hair should be washed twice.
After 2 days all swabs should be repeated. Discuss with consultant or the
microbiologist if skin carriage is present. Eradication should be attempted for
a new isolate of MRSA. If the patient is well enough to have oral antibiotics
then the options are:
First line: Oral fusidic acid and rifampicin. (The doses of fusidic acid &
rifampicin are given in section 5.2.1). Give for 5 days and repeat cultures. If
still positive give another 5 days. If still positive give 10 14 days of
intravenous teicoplanin. This regimen is reported to achieve a 94%
eradication rate16.
Second line: Nebulised vancomycin (55% eradication rate)17.
Vancomycin
Age
NEBULISED
1 month 18
years
Dose
Frequency
(times daily)
4
Duration
4mg/kg
5 days
(max
250mg)
Adult
250mg
4
5 days
500mg and 1g vials. Dilute with sodium chloride 0.9% or sterile water.
Precede dose with beta 2 agonist.
If IV treatment is to be given the choice of drug will obviously depend on the
sensitivity pattern. Most MRSA isolated at NUH are sensitive to teicoplanin
(see above for dose). For serious MRSA infections IV antibiotics should be
combined with oral rifampicin.
Linezolid is a new antibiotic, which may be given by the oral route in patients
with severe exacerbations due to MRSA. (Oral Linezolid has a high
bioavailability and so it is rarely necessary to give it IV). Linezolid should not
be prescribed if there is hepatic or renal impairment. Haematopoietic
disorders have been reported in patients receiving Linezolid.
It is
recommended that FBC is monitored weekly and that the treatment is given
for no more than 10 - 14 days. (See current BNFC for further information).
Discuss with consultant before commencing linezolid.
46
Linezolid
Age
IV / PO
Up to 12years
Over 12 years
Maximum dose 600 mg bd
Dose
10mg/kg
600mg
Frequency
(times daily)
3
2
Duration
10 - 14 days
10 - 14 days
5.4.4 Pseudomonas aeruginosa:

Antibiotics are always used in combination. Once daily tobramycin has been
shown to be just as effective and less nephrotoxic, than conventional 3 times
daily treatment.18 If the patient has had once daily tobramycin in the last 6
months, use the dose which achieved satisfactory levels last time. See
tobramycin prescribing guidelines (appendix 10).
First Line:
Ceftazidime
Dose
IV
50 mg/kg
Maximum dose 3 g tds

Tobramycin
Dose
Frequency
(times daily)
3
Doses / day
Administration
Bolus over 3-5
mins or 30 min
infusion
Duration
(course)
2 weeks
Administration
Duration
(course)
10 mg/kg
1
30 min infusion
2 weeks
IV
Round down dose to the nearest 10mg in children <20kg and to the
nearest 20mg in children> 20kg. Maximum starting dose 660mg
Tobramycin levels*
Timing
Peak (not routinely

performed see prescribing
guideline appendix)
Trough
30 minutes after the end of a

30 minute infusion
pre-infusion
Normal
range
20 - 30 mg/l
<1 mg/l
*All blood samples should be venous. A trough level and renal profile should
be taken before the 2nd dose. If the levels are satisfactory and the renal
function is normal, a trough level and renal profile should be performed
before the 8th dose.
47
Second Line:
Colistin
Dose
Frequency
Administration
(times daily)
30 min
infusion
Maximum dose 2 mega units (2 million units) tds
Combine with ceftazidime (above) or meropenem (below) according to
sensitivities
Meropenem (see section 5.4.5 Burkholderia cepacia complex) is also a
useful second line anti-pseudomonal antibiotic.
IV
25 000 units/kg
Duration
(course)
2 weeks
Other options:
Piperacillin Tazobactam
Should only be initiated by a consultant due to risk of hypersensitivity
reactions and blood dyscrasias.
Piperacillin Tazobactam
(Tazocin)
Age
Dose
Frequency
Administration
Duration
1 month
12
years
12 18
years
Adult
90mg/kg
(max
4.5g)
2.25
4.5g
4.5g
6 - 8 hourly
30 min
infusion
2 weeks
6 - 8 hourly
30 min
2 weeks
infusion
6 - 8 hourly 30 min
2 weeks
infusion
2.25 g (piperacillin 2 g and tazobactam 250 mg) 4.5 g (piperacillin 4 g and
tazobactam 500 mg) vials. Hypersensitivity reactions, gastrointestinal
reactions, blood dyscrasias.
Fosfomycin
Another option in patients with resistant organisms / drugs allergies.
Fosfomycin should only be initiated by a consultant.
Fosfomycin
Dose
Frequency
(times daily)
1-12years (1040kg)
> 12 years
100mg/kg
Infusion
Duration
30mins
5g (Total daily dose

2-3
30mins
can be increased to
20g)
Fosfomycin dosage needs adjusting in patients with renal insufficiency
seek advice from pharmacy. To prepare the infusion add 100ml water for
injection to each 5g vial of fosfomycin and withdraw the appropriate volume.
48
5.4.5 Burkholderia cepacia complex:

This organism is transmissible between patients and highly resistant to
antibiotics.19 Response to antibiotics is often poor but some isolates have in
vitro sensitivity to Meropenem.20 If a second line antibiotic is required discuss
with consultant.
First Line:
Meropenem
Dose
Frequency
(times daily)
3
Duration
Duration
(infusion)
(course)
40mg/kg
<1g dose: Bolus
2 weeks
IV
over 5 min
> 1g dose: 1530 min infusion
Maximum dose 2 g tds. The use of a dose of 2 g will require a prolonged
infusion.
Infection with B. cepacia complex is usually accompanied by P.aeruginosa.
Meropenem should be combined with a second antibiotic such as
ceftazidime to which the patients strain of P.aeruginosa is sensitive.
Second Line:
There are anecdotal reports of the use of cotrimoxazole and chloramphenicol
against B. cepacia complex.
Chloramphenicol
Dose
Frequency
Child & Adult
12.5mg/kg
6 hourly
Duration
(infusion)
10% solution
given by slow
bolus or infusion
Duration
(course)
2 weeks
Avoid in infants. Maximum dose 1g 6 hourly. Blood disorders including aplastic

anaemia. Monitor blood counts before and during treatment. Avoid, if possible,
in renal or hepatic impairment. Also gastrointestinal disturbances, peripheral
and optic neuritis. In view of possible toxicity stop or change this antibiotic as
soon as clinically indicated.
Co-trimoxazole
Dose
Frequency
Duration
Duration
(infusion)
(course)
6 mths-6years
240mg
12hourly
60 min
2 weeks
6-12 years
480mg
12hourly
60 min
2 weeks
>12years
960mg
12hourly
60 min
2 weeks
480mg in 5ml; 960mg in 10ml. 240mg = 2.5ml in 62ml diluent. 480mg = 5ml in
125ml diluent. 960mg = 10ml in 250ml diluent
5.5 Infection with atypical mycobacteria

A sample should be sent at each annual assessment requesting staining and
culture for atypical mycobacteria. The organism can only be detected on a
sputum sample and, if there is a strong clinical suspicion of atypical
mycobacteria, induced sputum or bronchoscopy and lavage should be
49
considered in patients who do not expectorate sputum (section 5.1.2).

Atypical mycobacteria are environmental organisms and careful evaluation is
needed to distinguish asymptomic colonisation from infection1. A high
resolution CT scan should be assessed for specific abnormalities and 2
positive sputum samples (or 1 lavage) are needed before starting treatment.
Mycobacterium abscessus is more likely to be associated with clinical
disease. Initially 3 weeks of intravenous treatment is given with amikacin,
meropenem (section 5.4.5), clarithromycin, imipenem and tigecycline. For
other atypical mycobacteria see the UK CF Trust Guidelines.1
Amikacin
Dose
1 month 18
years
Frequency
(times daily)
3
Duration
(infusion)
Slow
bolus
Duration
(course)
3 weeks
Frequency
(times daily)
2
Duration
(infusion)
60 min
Duration
(course)
3 weeks
60 min
3 weeks
Administration
Duration
(course)
3 weeks
10mg/kg
(max
500mg)
Adult
7.5mg/kg
2
Slow
3 weeks
(max
bolus
750mg)
100mg and 500mg in 2ml vials. Aim for trough level of <10 mg/l. Peak should
not exceed 25 to 30 mg/l at 1 hr.
Levels should be taken pre and one hour post dose after 24 hours of
treatment. If within range, pre dose levels should be taken twice weekly for
duration of course.
Clarithromycin
Dose
1 month 18
years
7.5 mg/kg
(max
500mg)
500 mg
Adult
Imipenem
(with cilastatin)
1 month 18
years
Dose
Frequency
(times daily)
25mg/kg
(max 1g)
30min infusion
(doses over 500mg
infuse over 4060mins)
For infusion dilute to 5mg/ml with sodium chloride 0.9%
Tigecycline
Over 12 years
Dose
Frequency
(times
daily)
2
Administration
Duration
(course)
1mg/kg
Infuse over
3 weeks
(max 50mg)
30mins
50mg vials reconstituted to produce 10mg/ml. For infusion - dilute further with
100ml sodium chloride 0.9% or glucose 5%
Initial treatment is followed by maintenance treatment with nebulised
amikacin and oral clarithromycin (section 5.2.3), for at least one year, with
careful microbiological surveillance.
50
Amikacin
Dose
Frequency
(nebulised)
Child <12years
250mg
12hourly
Child >12 years
500mg
12hourly
and Adult
250mg/ml vial. Make up to 4ml with 0.9% sodium chloride. Give first dose in
hospital, can cause bronchospasm, monitor lung function before and after.
5.6 Fungal septicaemia

Fungal septicaemia should be suspected in the following circumstances:
Indwelling intravenous access device (e.g. port-a-cath)
And
Patient receiving prolonged or repeated courses of intravenous antibiotics
And
Persistent spikes of fever
And
Serial C reactive protein climbing over 3 consecutive measurements in
spite of intravenous antibiotics
In these circumstances intravenous liposomal amphotericin may be
indicated. Discuss with consultant and with microbiology. For further details
see British National Formulary for Children. Discuss which brand and dose to
use with microbiology as different brands have different dose
recommendations. A test dose must be administered at the start of treatment
(see BNFC for details). Consult with pharmacy about reconstitution and
administration details. Amphotericin is incompatible with sodium chloride. A
flush of glucose 5% in the line used must be administered before and after
the amphotericin infusion. Successful treatment will involve line removal
(discuss with consultant).
Liposomal
Amphotericin
(Ambisome
brand)
IV
Dose
Frequency
(times daily)
Duration
(infusion
)
Duration
(course)
Test dose 100

One dose
15 min
One dose
micrograms/kg
only
only
Maximum test
dose 1mg
Start - 1
mg/kg/day
1
30
3 weeks
Increase by 1
60min
mg/kg/day
Maximum 3
mg/kg/day
Always consult pharmacy. Subtract test dose from 1st dose given on day 1.
51
5.7 Allergic Reactions

Repeated courses of IV antibiotics are associated with the development of
allergic reactions. This can severely limit antibiotic choice. The commonest
reaction is a rash but life-threatening anaphylaxis can occur. Drug allergies
should be clearly documented in the patient's notes and on all prescription
charts. With some antibiotics it is possible to prescribe a desensitising
regimen at the start of the antibiotic course, this should be done in liaison
with the ward pharmacist. For patients having home IV's see protocol in
section 6.
5.8 Heparin and Sodium Chloride Flushes

All patients prescribed intravenous antibiotics must have sodium chloride
0.9% and heparin 10units/ml or 100units/ml prescribed (see table). The
volume and frequency depends on the type of intravenous access.
Sodium chloride 0.9% is

given before & after each
antibiotic administered (15
ml in total for 2 antibiotics)
Heparin
Portacath
15ml TDS
Long Line
15ml TDS
Cannula
15ml TDS
100units/ml
4ml TDS
10units/ml
2ml 4hourly
Not
required
Portacaths do not need flushing in between antibiotic doses to maintain

patency. When the portacath is not being used for IV antibiotics it is needled
and flushed with 10mls Sodium Chloride 0.9%, followed by 4mls 100units/ml
of heparin every 4-6 weeks to maintain patency.
However, if a child under the age of 5 years has a portacath inserted an
individual plan for the dose and strength of heparin needed to maintain the
patency of the portacath must be discussed and agreed by the Respiratory
Consultant Paediatricans and the CF Nursing Team. This must be noted in
the medical notes.
References
(1) UK Cystic Fibrosis Trust Antibiotic Group. Antibiotic Treatment for
Cystic Fibrosis (3rd edition). London: UK Cystic Fibrosis Trust; 2009
(2) Smyth A, Walters S. Prophylactic antibiotics for cystic fibrosis.
Cochrane Database Syst Rev 2003;Issue 3. Art. No.: CD001912. DOI:
10.1002/14651858.CD001912.
(3) Armstrong DS, Grimwood K, Carzino R, Carlin JB, Olinsky, A et al.
Lower respiratory infection and inflammation in infants with newly
diagnosed cystic fibrosis. BMJ 1995; 310:1571-1572.
52
(4) Rayner RJ, Hiller EJ, Ispahani P, Baker M. Haemophilus infection in

cystic fibrosis. Arch Dis Child 1997; 65:255-258.
(5) Kerem E, Corey M, Gold R, Levison H. Pulmonary function and clinical
course in patients with cystic fibrosis after pulmonary colonisation with
Pseudomonas aeruginosa. J Pediatr 1990; 116:714-719.
(6) Langton Hewer SC, Smyth AR. Antibiotic strategies for eradicating
Pseudomonas aeruginosa in people with cystic fibrosis. Cochrane
Database Syst Rev 2009;Issue 4. Art. No.: CD004197.
DOI:10.1002/14651858.CD004197.pub3.
(7) Valerius N, Koch CHN. Prevention of chronic Pseudomonas
aeruginosa colonisation in cystic fibrosis by early treatment. Lancet
1991; 338:725-726.
(8) Maddison J, Dodd M, Webb AK. Nebulised colistin causes chest
tightness in adults with cystic fibrosis. Respir Med 1994; 88:145-147.
(9) Dodd ME, Abbott J, Maddison J, Moorcroft AJ, Webb AK. The effect of
the tonicity of nebulised colistin on chest tightness and pulmonary
function in adults with cystic fibrosis. Thorax 1997; 52:656-658.
(10) Steinkamp G, Tummler B, Malottke R, von der Hardt H. Treatment of
pseudomonas aeruginosa colonisation in cystic fibrosis. Arch Dis Child
1989; 64(7):1022-1028.
(11) Frederiksen B, Koch C, Hoiby N. Antibiotic treatment of initial
colonization with Pseudomonas aeruginosa postpones chronic infection
and prevents deterioration of pulmonary function in cystic fibrosis.
Pediatr Pulmonol 1997; 23(5):330-335.
(12) Ratjen F, Munck A, Kho P, Angyalosi G, for the ELITE study group.
Treatment of early Pseudomonas aeruginosa infection in patients with
cystic fibrosis: the ELITE trial. Thorax 2009; 65:286-291.
(13) Equi A, Balfour-Lynn IM, Bush A, Rosenthal M. Long term azithromycin
in children with cystic fibrosis. Lancet 2002; 360:978-984.
(14) Saiman L, Marshall BC, Mayer-Hamblett N, Burns JL, Quittner AL,
Cibene DA et al. Azithromycin in patients with cystic fibrosis chronically
infected with Pseudomonas aeruginosa: a randomized controlled trial.
JAMA 2003; 290(13):1749-1756.
(15) Saiman L, Anstead M, Mayer-Hamblett N, Lands LC, Kloster M,
Hocevar-Trnka J et al. Effect of azithromycin on pulmonary function in
patients with cystic fibrosis uninfected with Pseudomonas aeruginosa:
a randomized controlled trial. JAMA 2010; 303(17):1707-1715.
(16) MacFarlane M, Leavy A, McCaughan J, Reid AJM. Successful
decolonisation of meticillin-resistant Staphylococcus aureus in
paediatric patients with cystic fibrosis (CF) using a three-step protocol.
J Hosp Infect 2007; 65:231-236.
53
(17) Solis A, Brown D, Hughes J, Van Saene HK, Heaf DP. Methicillinresistant Staphylococcus aureus in children with cystic fibrosis: An
eradication protocol. Pediatr Pulmonol 2003; 36(3):189-195.
(18) Smyth A, Tan KHV, Hyman-Taylor P, Mulheran M, Lewis S, Stableforth
D et al. Once versus three-times daily regimens of tobramycin
treatment for pulmonary exacerbations of cystic fibrosis - the TOPIC
study: a randomised controlled trial. Lancet 2005; 365(9459):573-578.
(19) Govan JRW, Brown PH, Maddison J, Doherty CJ, Nelson JW, Dodd M
et al. Evidence for transmission of Pseudomonas cepacia by social
contact in cystic fibrosis. Lancet 1993; 342:15-19.
(20) Pitt TL, Kaufmann ME, Patel PS, Benge LCA, Gaskin S, Livermore DM.
Type characterisation and antibiotic susceptibility of Burkholderia
(Pseudomonas) cepacia isolates from patients with cystic fibrosis in the
United Kingdom and the Republic of Ireland. J Med Microbiol 1996;
44:203-210.
54
Section 6 - Home IVs & Home Oxygen

(Revised by Janice Mighten & Debra Forster)
55
6.1 Home intravenous (IV) antibiotic therapy

An admission for a course of IV antibiotics can be disruptive for the family
and to the childs education. With advances in CF treatment, home IV
therapy is now a routine occurrence for many children. However, some
families still prefer their treatment in hospital for a variety of reasons.
The first course of IV treatment can have a significant emotional,
psychological and physical impact on the child and family. Further, some
children require more intensive treatment regimes that can only be given in
hospital. In addition, the outcomes of an IV treatment course at home should
not vary too significantly from a course of IV treatment in hospital. Therefore,
home IV therapy is not suitable for every child on every occasion.
It must also be remembered that home IV therapy is not necessarily an easy
option. During IV treatment, many aspects of care have to be intensified,
which can be extremely tiring and disruptive to family life. Before the issue is
raised with the family, this should be discussed with the multidisciplinary
team. A joint decision is then made with the family and members of the team,
with regards to the most appropriate place for treatment to be given. In some
cases it is necessary for the child to remain in hospital for the full course due
to their general condition. A flexible approach to treatment is vital for effective
working with families, people with CF live exceptional lives: they deserve
exceptional care.
6.2 Indications for home IV treatment

Home IV therapy should be considered for children when:
1.
Hospital admissions interrupt education.
2.
The family is unable to visit and prefer home IVs in order to keep the
family together.
3.
The child and parents are unhappy about a two-week admission.
4.
The child needs elective IVs on a regular basis.
5.
Concerns about cross infection.
6.3 Teaching sessions

If 3 or more families are interested in home IV therapy, a study session can
be organised. The teaching session includes the following people:
1.
Pharmacist: re drug therapy and the importance of drug levels.
2.
Infection control nurse.
3.
Physiotherapist.
4.
Pharmaceutical manager from the home care company, regarding
types of infusion devices (This service is only available to patients with
funding and approval from the relevant community bodies).
Alternatively, the teaching sessions are all undertaken in the ward
environment.
56
Each parent is taught how to administer antibiotics safely. The CF

community nurses carry out the teaching and assessment, in conjunction
with the CF Nurse specialist & ward nurses. The programme is tailored to
meet each individuals need. Home IV therapy will only commence once the
individual has been assessed as being confident and competent (this will
usually take about a week). After successful completion of the teaching and
assessment process a certificate of competence is issued to the proficient
individual.
6.4 Starting a course of home IVs

Home IV courses can be given: 1. At home
2. Started in hospital and completed at home
3. Started and completed at home. (Not appropriate for every child)
The first dose: This can now be given at home using the relevant protocol
(Appendix 15)
Checklist: For first dose administration:
Ensure procedure is available for reference
Ensure parents are aware of the signs of anaphylaxis
Ensure parents received training re the use of an epipen
Give parents information sheets re the use of an epipen
When ordering IVs ensure that 2 epipens are prescribed on the drug
chart (see appendix)
Ensure Pharmacy dispenses 2 epipens with labels specifying when to
use
Send a letter of notification to the GP about the patient having home IVs
The nurse, parent/carer will observe the child for 30-60 minutes following
administration of the first dose
Check that the parents store the epipen below the 250 C and protect from
the light (a dark cupboard) and check the expiry before each course of IV
treatment.
6.5 Prescribing instructions

All ward patients who intend to go home on IVs need to be registered as
home IV users. The CF nurse specialist or community nurses usually carry
this out. If they are not available, please inform them as soon as possible
about new patients.
Ordering and contact details are available from the CF nurse specialist on
D33. Two working days are usually required before a delivery can be made.
However, the co-ordinators at the home care company are available to help
with enquiries such as delivery date and time, types of infusion devices, and
volumes or diluents for the antibiotics. If a prescription is received before
10am, the order can usually be delivered on the next working day. It is the
responsibility of the doctor who has advised the course of home IV antibiotics
to decide on a suitable antibiotic combination (section 5.4) and complete the
57
prescription form. Remember to prescribe oral Nystatin for children with

port-a-caths (to be taken throughout the IV course and for 1 week after
see section 5.4).
When the child is an inpatient for part of the course, the drugs for home IV
therapy will be ordered by the nurses on the ward. If the child is starting &
completing the full course at home, the CF community nurses will order the
drugs.
Help can also be offered with physiotherapy at home when having IV
therapy but the CF specialist physiotherapist must be contacted before each
child is discharged home on IV therapy.
The progress of every child having home IV therapy will be discussed at the
meeting following the CF clinic on a Tuesday.
6.6 Checklist for children going home on IV antibiotics
Prescription for home IV medication should be faxed & sent to the home
care company.
All children with a port-a-cath having IV antibiotics must have oral
Nystatin while having IV antibiotics and for 1 week after.
Ensure parents have a clearly written prescription chart with only the IV
medication and oral nystatin written up. (Remember to ensure that the
dose of aminoglycosides is the correct one, if altered following recent
levels).
The doses of IV antibiotics should be rounded to the nearest appropriate
dose for ease and safe administration. (Discuss with CF team if in doubt)
Ensure home assessment sheet also given to parent.
Ensure given any other TTO's and supplies as necessary.
6.7 Antibiotic levels

Antibiotic levels need to be monitored for those children having
aminoglycosides (see appendix 7). For tobramycin this is done before the
2nd and 8th dose. For those starting antibiotics in hospital the first trough level
will be done before discharge home. Children having either the complete
course or the second week of treatment at home will need arrangements
making for the levels to be taken. The results will be phoned to D33. It is the
responsibility of the junior medical staff to inform one of the CF doctors
promptly (or the duty registrar at the weekend). It is important that the CF
community nurse is contacted, as soon as possible, if there is a need to
repeat
aminoglycoside
levels.
Parents
of
children
having
aminoglycosides at home should be informed of the need for any dose
adjustment or the need for repeat levels, as soon as possible. This is
the responsibility of the medical staff.
58
6.8 Monitoring clinical response

The CF community nurses will visit each child at home based on individual
circumstances. For those having the very first course of home IVs, contact
will be made as required.
Home assessments include the monitoring of lung function on day 7 and 14,
weight and oxygen saturation. A cough swab or sputum specimen should be
obtained at the beginning and at the end of the IV treatment. The home
assessment results will be entered into the medical notes (outpatient section)
at the end of treatment. On completion of the IV course the CF community
nurse removes the longline or gripper needle.
6.9 Home oxygen

A discussion with members of the CF team will take place before a child is
discharged home on oxygen therapy. Each childs oxygen requirements are
variable. Overnight oximetery is often performed to determine oxygen
requirements overnight. This should be done when the child is well (e.g. at
the end of a course of IVs).
Children needing home oxygen therapy will usually require an oxygen
concentrator. If a child only needs oxygen occasionally, such as after
physiotherapy, the light weight option will be sufficient. Portable cylinders
are also available on prescription so the child can go out of the house.
Arrangements can be made for oxygen therapy in school if necessary, either
by a cylinder or a concentrator.
The new home oxygen service specification places a greater emphasis on
assessment, and safety, with the responsibility of selecting the right
equipment solution resting with the health care professional, based in the
hospital.. The new contract is no longer a fixed cost per year, but rather there
is a separate cost for each item ordered. Therefore, careful ordering is vital.
Air Liquide is the supplying company for this region.
All relevant forms including the HOOF and consent form can be found at
www.primarycarecontracting.nhs.uk.. Each ward area will have an oxygen
resource file containing all the relevant information. Once the HOOF form is
completed, it must then be faxed to Air Liquide on; 08708632111. It is also
essential that this is followed up with a telephone call to Air Liquide to
confirm receipt of the fax and clarify the requirements. Failure to do so can
result in no delivery to the child concerned.
The CF team should provide details such as flow rate in litres, the number of
hours a day the child will require oxygen therapy and whether humidification
is needed. Air Liquide provide a back up cylinder, which is needed in case of
an emergency such as a power cut. Up to 2 litres of oxygen can be
administered via nasal cannulae. If less than one litre is required the child will
need a low flow meter. This must be specified when prescribing. Light weight
cylinder options should be discussed with Air Liquide. Further support,
advice and guidance about home oxygen use can be obtained from Air
59
Liquides prescriber support number 08082022099 or the clinical nurse

specialist for oxygen therapy; Briony James on 07810527513
Once the concentrator is installed the engineer will teach the parent/carer
how to use the concentrator/cylinder and how to change a cylinder. The need
to monitor oxygen saturation levels at home is not required as standard,
however, each individual case should be discussed with the consultant
before discharge home. Parents are provided with information packs
containing emergency contact numbers and information about the safety
aspects of home oxygen therapy.
The CF community nurses must be contacted before discharging a patient
home on oxygen therapy.
Please ensure that, when home oxygen is commenced, it is documented on
the summary sheet in front of the medical notes.
NB: When well, if the childs O2 saturation is less than 93% / FEV1 is less
than 50% predicted, they are having home oxygen or they desaturate with
shuttle or step test, the doctor should arrange overnight saturation monitoring
with CF community nurses. This can then be undertaken at home.
Bibliography
Mighten J. (2007) Home intravenous therapy training for carers of children
and young people, British Journal of Nursing vol 6 no5 p272
Iara Maria Sequeiros and Nabil A. Jarad (2009) Home intravenous antibiotic
treatment for acute pulmonary exacerbations in cystic fibrosis Is it good
for the patient? Ann Thorac Med. Jul-Sep; 4(3): 111114
60
Section 7 - Management of Other Respiratory Problems

61
7.1 Allergic bronchopulmonary aspergillosis

Aspergillus fumigatus is a common mould found in soil, water, and air and
decaying vegetable matter, which liberates spores. ABPA is a lung disease
resulting from hypersensitivity to aspergillus species, clinically characterized
by impaired mucociliary clearance, mucoid impaction and airway obstruction,
and pulmonary infiltrates1.In one UK series the prevalence was estimated to
be 6%, in a large CF clinic.2 Other clinical presentations include aspergillus
bronchitis, aspergilloma and invasive pulmonary aspergillosis.
The following diagnostic criteria are based on a combination of the UK CF
trust and US CF foundation guidelines for diagnosis of ABPA in CF.3,4
1.
Acute/ sub acute clinical deterioration ( cough, wheeze, exercise

intolerance, exercise-induced asthma, decline in lung function or
increased sputum production) not attributable to other aetiology
2.
Total serum IgE > 500 IU/ml or a four-fold increase in IgE titres
3.
Immediate cutaneous reactivity to A. fumigatus >3 mm or raised

aspergillus specific IgE to A. fumigatus greater or equal to than grade 4
Precipitating IgG antibodies to A. fumigatus
4.
5.
New or recent (infiltrates, mucus plugging or central bronchiectesis )

changes on chest radiograph/CT that have not cleared with antibiotics
and standard physiotherapy
Positive respiratory culture for aspergillus is not an essential pre-requisite for

diagnosis of ABPA 5.
The diagnosis of ABPA can be fulminant or insidious and annual screening
will help to identify allergic sensitization and progression to disease. This
should also be considered in an acute exacerbation when the response to
treatment is poor or atypical despite appropriate antibiotic.
62
Treatment: The aim is to attenuate the allergic inflammation with oral

corticosteroids and reduce the antigen burden with antifungal therapy.
Drug
Route
Dosage
Duration
0.5-1mg/kg
0.5-1mg/kg
Frequency
(times daily)
1
alt daily
Prednisolone
Followed
by
Taper off
Itraconazole
PO
PO
<12 years 3-5mg/kg

>12 years 200-400mg*
1
1
For 3 to 6
months
Voriconazole
PO
2-12 years 200 mg
Maintenance
12-18 years
weight < 40kg
200 mg
then 100 mg
(increase to 150 mg if
necessary)
2
2
2 doses
Maintenance
(Review in 6
months)
12-18 years
weight > 40kg
400 mg
then 200 mg
(increase to 300 mg if
necessary)
2
2
2 doses
Maintenance
(Review in 6
months)
1-2 weeks
1-2 weeks
2-3 months
*The initial dose of itraconazole should be 5 mg/kg/day, which may be given

once daily unless the dose exceeds 200 mg/day, in which case it should be
given twice daily. The daily dose should not exceed 400 mg/day unless low
serum itraconazole levels are obtained.
A resolution of symptoms, a return of respiratory function to previous levels,
resolution of X-ray changes and a fall in total IgE of >35% by 2 months is
indicative of remission.6
General advice about reducing exposure to environmental sources of the
fungal spores e.g. avoiding construction and renovation work, rotting
vegetation, dust, mucking out stable etc) should be given
There is insufficient evidence to recommend the use of inhaled
corticosteroids for initial therapy or for prevention of pulmonary fibrosis and
chronic pulmonary dysfunction from ABPA in patients with CF.5 For patients
who are on ICS, these should be discontinued when started on oral steroids
and especially when on itraconazole and this can increase the systemic
bioavailability of ICS.
For patients on Itraconazole therapy, Liver Function Tests should be
monitored before starting therapy, 1 month and 3 to 6 months later.
Absorption may be impaired in patients on ranitidine or omeprazole as it is
sensitive to gastric pH. Voriconazole is a new antifungal agent with better
63
absorption than Itraconazole. It may be used as a second line agent in

patients who fail to respond to itraconazole7 (consultant decision).
There are some case reports of other therapy (pulsemethyl prednisolone,
omalizumab) and this should only be a consultant decision.
7.2 Haemoptysis
Small haemoptysis: This is a common complication in patients with cystic
fibrosis and is presumed to be due to erosion of small bronchial blood
vessels due to localised areas of infection. The management is conservative.
If the episodes of haemoptysis have been persistent or if more than a
minimal amount of blood is present, check haemoglobin, clotting and
platelets. Perform a chest X-ray and treat underlying infection as
haemoptysis may be a manifestation of an infective exacerbation.
Massive haemoptysis: is defined as acute bleeding greater than 240mL in a
24-hour period or recurrent bleeding greater than 100 mL/d over several days.
There is a high risk of subsequent episodes of massive haemoptysis. Nearly
26% of patients will have more than one occurrence8.
Uncontrolled bleeding into the airway can result in significant impairment of
ventilation (drowning in blood). Initial management consists of administration
of oxygen and vigorous resuscitation with colloid and blood. Use group
specific uncross-matched blood if there is any delay in obtaining crossmatched units. Early consultation with the thoracic surgeons and /or
interventional radiologists is very important. Urgent rigid bronchoscopy under
general anaesthetic may be required (to suck out clots/define source of
bleeding/achieve tamponade) and lung resection may be required if the
bleeding is not controlled. Stop non-steroidal anti-inflammatory drugs
(NSAIDS), stop bi-level positive airway pressure (BiPAP) for as long as there
is bleeding and stop airway clearance therapies. Hypertonic saline should be
withheld while there is active haemoptysis.
Chest Physiotherapy as active cycle breathing or autogenic drainage might be
preferable to high-frequency chest compression or intrapulmonary percussive
ventilation9.
Bronchial artery embolisation along with treatment of a CF pulmonary
exacerbation may be used in selected patients. There are reports of
alternative successful therapies, including tranexamic acid, vasopressin9 and
Recombinant Activated Factor VII10.
Pneumothorax9
Pneumothorax occurs in patients with CF when a sub-pleural bullus ruptures
into the pleural cavity. Tension pneumothoraces are uncommon but require
emergency chest drainage when they occur. More commonly there is a
history of chest pain, shortness of breath in a teenage or young adult patient
with a drop in lung function and a chest X-ray reveals the diagnosis. Many
patients (~ 5090%) may suffer a recurrence, (on the ipsilateral side more
than 7 days after the resolution of the initial pneumothorax), and there is a
high rate (46%) of subsequent contralateral pneumothorax. Some patients
will die acutely as a result of pneumothorax, with an attributable mortality
64
estimated at 6.314.3%, and it also may be a prognostic factor, as the 2-year

mortality rate in patients following a pneumothorax is high (48.6%)
Management
Small pneumothoraces may be managed conservatively with observation
however chest drain placement is the recommended initial treatment. The
affected lung should rapidly reinflate. When the chest drain has stopped
swinging or bubbling for several days it should be clamped and the chest Xray repeated. If the lung remains inflated, the drain can be removed.
There is a high rate (estimated 37%) of treatment failure with chest tube
drainage alone and as there is a high recurrence rate, ~ 70% of patients may
ultimately require more definitive treatment (i.e., pleurodesis).Surgical
pleurodesis appears to be the most effective, but if the patient is unable to
tolerate surgery, chemical pleurodesis (e.g., talc) should be performed.
Although contralateral pneumothorax is common, prophylactic pleurodesis
on the opposite side is not considered standard therapy. For those patients
who may be referred for lung transplantation, previous pleurodesis is not an
absolute contraindication to lung transplantation.
Alternative airway clearance therapies such as active cycle breathing, or
autogenic drainage, rather than positive expiratory pressure should be
encouraged.
Occasionally the pneumothorax may still be present even though the drain is
bubbling vigorously in which case the patient may have a bronchopleural
fistula. This is a troublesome complication and should be discussed with the
thoracic surgeons. Where the pneumothorax persists and the drain is not
bubbling then the drain may have become blocked or may need to be
repositioned.
7.4 Referral for transplantation11, 12
Lung transplantation (bilateral sequential lung) is indicated for patients with
end stage CF-related lung disease, whose lung function parameters and
quality of life are declining despite maximal medical therapy. However lung
transplantation is a palliative procedure in CF. However, where the procedure
is successful, it may produce a huge improvement in the childs quality of life
for a limited period.
Patients will usually be considered for lung transplantation if:
the patient has a predicted life expectancy, without transplant, of two years
or less when a childs FEV1 has fallen to <30% predicted;
the patient has a poor quality of life, which is likely to be improved by
transplant;
there are no specific contraindications to transplant;
the patient / family is fully informed about the procedure, and is committed
to proceeding.
However, females, very young patients, subjects declining quickly and
subjects who have other relevant clinical factors such as a history of massive
haemoptysis or increasing frequency of respiratory exacerbations should be
considered for transplant referral before they reach this point.
65
Where a childs lung function is consistently below the suggested level, their
suitability for transplant should be discussed with the multidisciplinary team.
The CF / Barnardo's social worker will be involved here. This will usually lead
to a meeting between the consultant and the family (to include the child in the
case of teenagers). Younger children should have their referral discussed with
them separately.
If the child and family wish to proceed, then a referral should be made to Dr
Paul Aurora or Dr Helen Spencer, Consultants in Lung Transplantation and
Respiratory Medicine at Great Ormond Street (GOS). Please use the UK
Paediatric Lung and Heart-Lung Transplantation Referral Proforma (Appendix
16 )
This will usually lead to a 4 day admission to GOS for assessment, after
which the child will be either placed on a provisional list, an active list or
reassured. Children should be well when they go to GOS for assessment, as
they are not admitted formally as inpatients. In the case of older teenagers,
referral should be made to Dr David Spencer at the Freeman Hospital
Newcastle, using the same referral proforma.
Of those listed for transplantation, about 30% will receive a transplant. More
recent data suggest that more than 50% of those transplanted will survive 5
years and this keeps improving.11
Patients on the active list will require vigorous treatment for pulmonary
exacerbations, including non-invasive ventilation (NIV) in some cases.
However there may reach a point where, despite the hope of a transplant,
active treatment should be discontinued and a switch made to palliative care
(see chapter 14). This should be discussed in full with the child and their
family.
Post transplant management is carried out as shared care with the transplant
centre.
7.5 Non-invasive ventilation (NIV)

Non-invasive ventilation13,14
may be a useful adjunct to other airway clearance techniques,
particularly in people with CF who have difficulty expectorating sputum
can be used in addition to oxygen, may improve gas exchange during
sleep to a greater extent than oxygen therapy alone in moderate to
severe disease. These benefits of NIV have largely been demonstrated
in single treatment sessions with small numbers of participants.
Long term NIV has been used for patients awaiting lung transplantation
A number of factors are important to the success of non-invasive ventilation:
The mask must fit correctly
The child / young person must be allowed to learn how to use the
ventilator at a time when they are not acutely unwell. Discuss it with the
family and allow the child / young person to try the technique when there
is evidence of type II respiratory failure but before the patient is receiving
terminal care.
66
CF specialist Physiotherapists and Pulmonary function lab can advise on

mask fitting and NIV ventilator settings.
Oxygen may be entrained into the system.
1. Diagnosing allergic bronchopulmonary aspergillosis in children with cystic fibrosis.
Thia LP, Balfour Lynn IM. Paediatric Respiratory Reviews 2009 (10) 3742.
2. Simmonds EJ, Littlewood JM, Evans EGV. Cystic fibrosis and allergic
bronchopulmonary aspergillosis. Arch Dis Child 1990;65:507-11.
3. Stevens DA, Moss RB, Kurup VP et al. Allergic bronchopulmonary aspergillosis in
cystic fibrosis _ state of the art: Cystic Fibrosis Foundation Consensus Conference.
Clin Infect Dis 2003; 37(Suppl 3): S225S264.
4. The Antibiotic Consensus Group. Antibiotic Treatment for Cystic Fibrosis - Report of
the UK Cystic Fibrosis Trust Antibiotic Group (3nd Edition). 2009. London, UK Cystic
Fibrosis Trust.
5. Geller DE, Kaplowitz H, Light MJ, Colin AA. Allergic broncho-pulmonary aspergillosis
in cystic fibrosis: reported prevalence, regional distribution, and patient
characteristics. Scientific Advisory Group, Investigators, and Coordinators of the
Epidemiologic. Study of Cystic Fibrosis Chest 1999; 116: 639646
6. Marchant JL, Warner JO, Bush A. Rise in total IgE as an indicator of allergic
bronchopulmonary aspergillosis in cystic fibrosis. Thorax 1994;49:1002-5.
7. Hilliard T, Edwards S, Buchdahl R, Francis J, Rosenthal M, Balfour-Lynn I et al.
Voriconazole therapy in children with cystic fibrosis. J Cyst Fibros 2005;4:215-20.
8. Flume PA et al Massive Hemoptysis in Cystic Fibrosis Chest 2005;128(2):729-738
9. Flume PA. Pulmonary complications of Cystic Fibrosis Respiratory care 2009 ;(54) 5;
618625
10. Dentice R, et al. Recombinant Activated Factor VII for Massive Hemoptysis in
Patients with Cystic Fibrosis. Chest 2009;136;277-281.
11. Burch M,.Aurora P. Current status of paediatric heart, lung, and heart-lung
transplantation. Arch Dis Child 2004; 89:386-9.
12. Balfour-Lynn IM, Martin I, Whitehead BF, Rees PG, Elliott MJ, de Leval MR. Heartlung transplantation for patients under 10 with cystic fibrosis. Arch Dis Child
1997;76:38-40
13. Moran F, Bradley JM, Piper AJ. Non-invasive ventilation for cystic fibrosis.Cochrane
Database Syst Rev. 2009 Jan 21;(1):CD002769. Review.
14. Efrati O, et aLong-term non-invasive positive pressure ventilation among cystic
fibrosis patients awaiting lung transplantation. Isr Med Assoc J. 2004 Sep;6(9):52730.
67
Section 8 - Management of Other CF Related Disease

8.1 Abdominal pain
(Jayesh Bhatt)
8.2 Cystic Fibrosis Related Liver Disease
(Jayesh Bhatt)
8.3 Management of Bleeding Oesophageal Varices
(Jayesh Bhatt)
8.4 Low Bone Mineral Density
(Jayesh Bhatt))
8.5 CF Joint Pain and Disease
(Jayesh Bhatt)
8.6 Cystic Fibrosis Related Diabetes
(Jayesh Bhatt & Robyn Huggins)
68
8.1 Abdominal Pain

Recurrent abdominal symptoms are common despite adequate enzyme
doses. The commonest symptom is abdominal pain and patients may not
have infrequent stools despite constipation. The pain is often relieved by
passing stools and responds to lactulose.
Distal intestinal obstruction syndrome (DIOS) - (previously called
Meconium Ileus Equivalent) 1
Intestinal obstruction may occur in CF patients, outside the neonatal period,
due to the accumulation of viscid mucofeculent material in the terminal ileum
and caecum. Patients with a history of meconium ileus, previous laparotomy
and post lung transplant are at high risk of developing DIOS2.The
pathophysiology is poorly understood. It may be related to inadequate
dosage of pancreatic enzymes, poor compliance and episodes of
dehydration, though why some patients suffer recurrent episodes is not
always clear. It can also occur in those who are taking regular enzyme
supplements and diabetes mellitus may be a contributory factor in some.3
Clinical features are of abdominal pain, constipation, and poor appetite, with
a palpable right iliac fossa mass. In children who have simple constipation,
faecal loading is often felt in the left iliac fossa. Vomiting is a late feature and
it is important to intervene before vomiting occurs. A plain abdominal X-ray
shows absence of the normal gas pattern, faecal loading with a granular or
bubbly appearance in the right lower quadrant.
Management
Patients who present early with a short history of abdominal pain and
constipation can be managed with lactulose or movicol.
When symptoms have been prolonged, or when abdominal examination
reveals distension and a right iliac fossa mass, then admission is required
and oral gastrograffin should be given. N-acetylcysteine can be also used
as an alternative to gastrograffin. Oral granules or dilute injection solution
(200mg/ml); orange or blackcurrant juice may be used to mask the bitter
taste. Biochemistry should be checked. Adequate hydration and pain
relief should be ensured. Nasogastric tube will need to be placed to
ensure fluid and gastrograffin intake.
When an appropriate dose of gastrograffin has been given, without the
patient passing stool or when gastrograffin is not tolerated because of
vomiting, then a prompt surgical review is indicated.
Consider other diagnoses: Intussusception, appendicitis, fibrosing
colonopathy. biliary tract or gallbladder disease, acute pancreatitis,
urinary tract infection.
Following an episode of DIOS, review to the CF specialist dietician is
appropriate. The dietician will review and advise on: appropriate use of
Pancreatic Enzyme Replacement Therapy, adequate fluid intake, fibre
intake and regular meal pattern.
Proton Pump inhibitors should be considered to improve the efficacy of

enteric-coated enzyme preparations. In order to achieve a high
intraduodenal enzyme concentration, the dissolution of these
preparations in the duodenum can be optimized by increasing
intraduodenal pH by adding a PPI 4..
69
Drug
Lactulose
Movicol
Paediatric plain
Gastrograffin
N Acetylcysteine
Age
<7 years
>7 years
<6years
>6 years
Dosage
10 ml
20ml
1 sachet ( max 4 sachets)
2 sachets (max 4 sachets)
Doses/day
bd
bd
Daily
daily
< 10 kg
<25 kg
>25 kg
< 7years
>7 years
25 ml in 100 ml water/ juice

50 ml in 200 ml water/ juice
100ml in 400 ml water/ juice
2-3 g
4-6 g
single dose
single dose
single dose
single dose
single dose
8.2 Cystic fibrosis related liver disease

With the increased survival rate of patients with cystic fibrosis, complications
other than lung disease are becoming an increasing problem. CFTR (cystic
fibrosis transmembrane regulator) is expressed in the epithelial cells lining the
intra and extra-hepatic bile ducts and gall bladder but not in the hepatocytes
or other cells of liver and its main role is to participate in the ductal secretion.
Absent or dysfunctional CFTR leads to dehydrated viscous secretions. This
may lead to obstruction of the bile ducts with sludge leading to fibrosis and
ultimately biliary cirrhosis, a process, which is usually asymptomatic. As in
other liver diseases characterized by initial involvement of bile ducts and later
impairment of hepatocytes function, the hemodynamic consequences of
cirrhosis, mainly portal hypertension, are often prominent, whereas liver failure
tends to be a late event. In infancy, presentation may be with bile duct
obstruction (neonatal cholestasis) due to inspissated bile or with fatty change
that may cause abdominal distension. Gallstones and cholecystitis can occur
in later childhood. Recent best practice guidance for the diagnosis and
management of cystic fibrosis associated liver disease5 mentions a
cumulative incidence of liver disease ranging between 27 and 35 % , without
incident cases after the age of 18 years. 5- 10 5 of all CF patients will develop
multilobular cirrhosis during the first decade of life and liver cirrhosis accounts
for 2.5 % of overall CF mortality. No specific CFTR mutations have been
associated with the presence and severity of CFLD and environmental and
modifier genes might play a role.
Identification
Regular screening for CLFD is necessary to detect pre-symptomatic

signs of liver disease and to initiate treatment with ursodeoxycholic
acid. The presence of CFLD should be considered if at least two of
the following are present5
Hepatomegaly: palpable liver edge of > 2 cm below costal margin in
mid clavicular line or a palpable left lobe in epigastrium.
spleenomegaly on palpation or on ultrasound.
Elevated transaminases ( ALT and AST) and GGT above the upper
normal limits on 3 consecutive tests over 12 months ( exclude other
causes of liver disease)
70
Abnormal liver ultrasound (increased and /or heterogenous

echogenecity, irregular margins, nodularity) Or portal hypertension Or
biliary tract abnormalities. Recent guidance recommends annual
abdominal US5.
Management
Ursodeoxycholic acid
The water-soluble bile acid ursodeoxycholic acid (urso) is widely used in
the treatment in CF liver disease. This has been shown to improve liver
function tests, biliary drainage, early ultrasonographic changes in the liver
and even liver histology5. Urso should be started if liver damage has been
demonstrated by means of liver ultrasound (parenchymal abnormality or
frank cirrhosis) and/or persistent raised liver function tests are documented.
If prothrombin time is prolonged on 2 occasions the patient should also be
started on vitamin K see below. The dose of urso is given below. Once it
has been started, it should be continued indefinitely. Liver can be
morphologically normal or show steatosis (fatty infiltration), focal biliary
fibrosis and multilobular cirrhosis on liver US scan. Gallstones are also a
common finding, seen in between 24 to 50 % and do not require any further
imaging or treatment6.Nutrition should be optimised as development of liver
disease can exacerbate the malnutrition.
ORAL
Dose
10-15 mg/kg
Frequency
(times daily)
2
1- 12 years
5 - 10 mg
12 -18 years
10 - 20 mg
Vitamin K
(Prescribe as
Menadiol Sodium
Phosphate)
Preparations
150 mg & 250 mg
Suspension (250
mg /5 ml)
10 mg tablet
Or suspension 10
mg / 5 ml)
Referral to supra regional liver Unit

A referral should be made to the Supraregional Liver Unit at Birmingham
Childrens Hospital (Dr Indra Van Mourik) if there is evidence of portal
hypertension (reverse blood flow in the portal vein, oesophageal varices on
liver ultrasound, or splenomegaly), or progressive liver disease.
8.3 The management of bleeding oesophageal varices
Oesophageal varices may remain undetected until they bleed or patients with
known varices may bleed unexpectedly.
Resuscitation of the patient with bleeding varices follows the usual rules of
ABC.
1. Patients will need high flow face mask oxygen.
2. Intravenous access should be achieved immediately (2 sites). Call for help
from the anaesthetic team if necessary.
3. Take blood for group and cross match, clotting and platelets.
71
4. Administer colloid - 4.5% Human albumin solution 20 ml/kg until blood is

available. If necessary give group specific uncross-matched blood initially.
Correct shock but take care not to over-transfuse as this may precipitate
rebleeding.
5. Transfer the patient to Paediatric HDU/PICU.
6. Commence octreotide infusion.
7. Correct thrombocytopenia (often secondary to splenomegaly from portal
hypertension) and prolonged clotting (fresh frozen plasma 10 ml/kg and
vitamin K 10 mg IV)
IV
Dosage
Infusion
Octreotide
Contact pharmacy for advice on dilution and administration
Octreotide is a somatostatin analogue7 which stops bleeding from

oesophageal varices by reducing pressure in the portal vein. When there is
no active bleeding reduce dose over 24 hours.Patients with bleeding
oesophageal varices should be discussed with the pediatric gastroenterology
team (Dr Charlton & Dr Kirkham), to assess whether urgent endoscopy and
injection of varices is indicated. All children should be discussed with the
Liver Unit in Birmingham and, if necessary, transferred for assessment.
8.4 CF Bone Disease8,9
People with CF may develop low bone mineral density (BMD) from either
osteoporosis or vitamin D deficiency osteomalacia. Osteoporosis is a systemic
skeletal disease characterised by low bone mass and microarchitectural
deterioration of bone tissue with a consequent increase in bone fragility and
susceptibility to fracture. Osteomalacia is a disorder where there is an
increase in the proportion of non-mineralised bone.Low bone mineral density
(BMD) was first reported in CF patients in 1979.10 It is now emerged as a
common complication in the long term survivors of CF. Although the
pathophysiology of low BMD in people with CF has not been wholly defined,
both increased bone resorption and decreased bone formation have been
described. Delayed pubertal maturation (as growth and mineral accrual is
most rapid and expected gains may be compromised in individuals with CF),
malabsorption of vitamin D and K, poor nutritional status, diabetes mellitus,
CF related liver disease, physical inactivity, hypogonadism and glucocorticoid
therapy are all potential aetiological factors. Possibly most importantly, chronic
pulmonary infection increases bone resorption and suppresses bone
formation through the activity of inflammatory cytokines.11 Low BMD in CF
patients can lead to pathological fractures and kyphosis in adolescence, and
exclusion from lung transplant.12 In addition to causing pain and debilitation,
rib and vertebral fractures produce chest wall deformities that reduce lung
function, inhibit effective cough, hinder airway clearance.
The prevalence of bone disease appears to increase with the severity of lung
disease and malnutrition, though more recent studies suggest that the origin
72
of CF bone disease in early childhood ( < 6 years of age) may be

independent of nutritional status or disease severity.It is unclear whether there
is a link between bone pathology and the CFTR gene mutation. Low BMD
also occurs among 10 % of CF patients who are pancreatic sufficient,
indicating the role for chronic infection and other factors in causing poor bone
health.
8.4.1 Assessment of Bone Health

1. Nutritional status : Good nutrition is vital and should be optimised
through frequent liaison with a specialist CF dietitian.
2. Calcium intake should be at current recommended level for age
Age
Calcium (mg/day)
0 - 6 months
210
7 - 12 months
270
1 - 3 years
500
4 - 8 years
800
9 - 18 years
1300
Adults
1300 - 1500
3. Vitamin D status should be assessed from diagnosis by measuring the
serum level of 25-hydroxyvitamin D taken at annual assessment. Vitamin D
supplementation should be individualised with the aim of achieving serum 25
hydroxyvitamin D (25OHD) levels between 30 and 60 ng/ml (75150 nmol/L).
4. Hormonal Status- annual clinical assessment of pubertal staging using
Tanner staging should ideally be conducted starting at 9 years in females
and 10 years in males. Pubertal delay is defined in girls as no breast
development by the age of 13 years or no menarche by 16 years. Pubertal
delay in boys is defined as no testicular enlargement by the age of 14 years.
Patients with delayed puberty should be referred to a paediatric
endocrinologist and considered at increased risk of bone disease.
5. Glucocorticoid exposure the cumulative dose of glucocorticoids should
be assessed annually. More frequent assessment of bone health is
recommended in patients taking significant doses of prednisolone (> 5mg
/day >90 days /year)
6. Bone density measurement : DEXA scan (lumbar spine and proximal
femur) may underestimate BMD in children with CF who have suboptimal
growth and short, narrow bones. A DXA scan should be performed from
about 10 years of age and repeated every two years, determined by clinical
need, to ensure that bone accrual is occurring at a satisfactory rate.
The results for any individual should be compared to Z-scores (mean value
for age and sex obtained from DEXA studies of the general population). Tscores (mean bone mineral density of the young adult normal reference
population must not be used in children.
The CF trust suggests that the term CF related low BMD be applied to
children or adults with CF with a BMD Z-score below 2, (<2SDs below the
73
age and gender matched mean reference value) with the caveat that Z-scores
may be unreliable in individuals of small body size.
CXR at annual assessment should be examined for presence of vertebral
fractures. If a fracture is present DEXA scan should be performed.
8.4.2 Treatment recommendations

The central principles for preventing and treating low BMD-associated fracture
are heightened surveillance through screening for low BMD and fragility
fracture, and the optimisation of clinically modifiable factors that are likely to
affect bone health. Prevention is important and the following should be
encouraged.
1. Optimize nutrition
2. Exercise: encourage regular weight bearing exercise, as this will increase
bone mineral content. Sunlight exposure should be encouraged and just a
few minutes a day would suffice.
3. Calcium see above table: an increased calcium intake may improve BMD14
Vitamin D
Age
Dose
Duration
Routine supplementation - Ergocalciferol or Cholecalciferol
< 1 year
400 IU/day
ongoing
1 - 12 years
400 - 800 IU/day
ongoing
> 12 years
800- 2000 IU/day
ongoing
Higher doses may be required if serum 25-hydroxyvitamin D levels are below
30ng/mL
If the 25-hydroxyvitamin D level increases to 30ng/mL then continue with
routine therapy. If the level remains low please discuss with consultant.
4. There is not yet sufficient evidence to recommend universal vitamin K
supplementation for bone health in CF, but consideration should be given
to individuals with low BMD, liver disease and/or a prolonged prothrombin
time.
5. Minimise the use of glucocorticoid therapy.
6. Antiresorptive agents
These agents can treat established osteoporosis mostly by decreasing
bone resorption with some possible effects on bone formation. A recent
trial of Alendronate has demonstrated its efficacy and safety in a small
group of adult CF patients with low BMD.15 Bisphosphonates are not
licensed for use in children and although they appear to be relatively safe
even when used for long periods in other paediatric bone disease,
experience is limited. There are no published data reporting the outcome
of bisphosphonate use in children with CF. However, bisphosphonates
may be beneficial in children:
74
with a history of fragility fracture

and those listed for/post transplantation.
Some authorities suggest bisphosphonates for children who
have low BMD and continuing bone loss despite implementing
general measures for optimising bone health.
Some children starting on bisphosphonates may develop severe bone pain
and will require short course steroid therapy. Discuss with
Consultant.Patients treated with bisphosphonates should be monitored
with repeat DXA at 6-12 monthly intervals.
Alendronic acid (Alendronate)15
ORAL
Tablets available
Adult dose 10 mg od. Once weekly dose of 70
5mg,10mg and 70mg
mg may improve compliance.
Tablet should be taken in the morning
Administration
30 minutes before any food or drink and
the patient needs to stay upright for 30
minutes after taking the tablet to ensure rapid
passage down the oesophagus and stomach.
Hypocalcaemia, abnormality of oesophagus or
Contra-indications and
any factor which delays oesophageal emptying.
warnings
Caution with upper GI disease as may
cause irritation
Sex steroid replacement therapy should be considered in consultation with
Paediatric endocrinologist in individual cases.
8.5 Joint Pain and Disease:

Arthropathy may be associated with cystic fibrosis in three ways16
1. Cystic fibrosis arthropathy (CFA) , a complication characteristic of CF. It
affects large joints and is episodic. It can be quite disabling with sudden
onset. There can be associated high swinging fevers and skin rashes.
Most patients show a good response to NSAIDS. It follows a remitting and
relapsing course and X-Rays show no abnormality. There is no permanent
damage in most cases.
2. Hypertrophic Pulmonary osteoarthropathy: It is insidious in onset and may
begin as a continuous ache. The clinical picture may vary from a minimally
swollen joint to tender, warm swollen joints/ symptoms are worse in cold
weather. X-rays may show a periosteal reaction and some new bone
formation. These changes can slowly progress and can result in
permanent bone destruction. Symptomatic relief is with NSAIDS. HPOA
may resolve if the underlying chest disease is intensively treated. Both
these forms of arthritis can be worse with infective exacerbations.
3. Coincidental joint problems such as juvenile idiopathic arthritis.
75
8.6 Cystic fibrosis related diabetes (CFRD)17

CFRD occurs when progressive fibrosis and fatty infiltration of the exocrine
pancreas leads to progressive disruption and destruction of endocrine cells. It
is more common and develops at an earlier age in individuals who are
pancreatic insufficient. Now as patients with CF are living longer, this is
emerging as a major complication. CFRD is a distinct type of diabetes with
features of both Type 1 and Type 2 diabetes17. It differs from Type 1 in that
onset is usually insidious; many individuals are asymptomatic at diagnosis
and reliance upon clinical symptoms will result in failure to recognize CFRD in
the majority of patients. In others the first sign may be a decline in pulmonary
function, poor growth velocity, or delayed puberty. It differs from Type 2
diabetes in that weight loss despite nutritional intervention is often an early
feature. Delaying the diagnosis can result in an unnecessary deterioration in
both pulmonary function and clinical status. Prevalence rates for CFRD are
reported as 3% in 5-9 years, 11% between 10-19 years, with 50% aged more
than 30 years being affected. Prevalence of impaired glucose tolerance is
20% of patients at 10 years, 50% at 15 years, 75% at 20 years, 82% at 30
years18; There is a clear association between diabetes and increased
morbidity and mortality, with survival being significantly lower in patients with
diabetes. Female patients with CFRD but without chronic Pseudomonas tend
to have lower FEV1 when compared to sex matched subjects with NGT19 and
a remarkably poorer prognosis as compared to all male subjects with CF and
female subjects with CF but without diabetes20.
Prediabetic state may also be deleterious because a greater decline in
pulmonary function and nutritional state was found as early as 6 years before
the diagnosis of diabetes21.
Early diagnosis and aggressive treatment have played a major role in
improving survival in patients with CRFD and previously noted gender
differences in mortality have disappeared, and the gap in mortality between
CF patients with and without diabetes has narrowed considerably22.
8.6.1 Diagnosis of CFRD

An oral glucose tolerance test (OGTT) should be performed on patients aged
10 years or over annually (as a day case admission to the ward around their
annual assessment ).Children aged 5-9 should have HBA1c and random
blood glucose and have OGTT if the random glucose is elevated or there is
unexplained decline in pulmonary function or growth.
CFRD is diagnosed if there is
1) persistent random glucose levels >11.1 mmol/L,
2) persistent fasting glucose levels > 7.0mmolL, or
3) by OGTT.
Oral glucose tolerance test:
The OGTT in type 2 diabetes involves only baseline and 120-min samples
and the diagnostic cut-offs were designed to forecast microvascular
complications of diabetes rather than CF-specific outcomes such as decline in
76
weight and lung function27. Peak glucose in CFRD occurs earlier than the
routinely measured 120 min sample and the 120-min sample fails discriminate
between CF patients and healthy controls. It also did not correlate with weight
or lung function decline in the preceding year. Glycemic cut-offs designed to
detect a decline in weight standard deviation score in the preceding year have
been proposed ( see table). The cut-offs early stages of insulin deficiency
(CFID1 and 2) CFID3 and CFID4 correspond to CFRD without and with
fasting hyperglycemia, respectively and Insulin treatment is now
recommended as standard care for both of these categories27.
The patient should be fasted overnight. If possible they should remain seated
or in quiet play throughout the test.
Time 0:
Take blood for glucose.

Administer glucose 1.75 g/kg or maximum 75g
75g glucose = 394 ml Lucozade Original Energy (73 kcal/100 ml)
Younger children: volume of lucozade = 394/75 1.75 wt (Kg)
Time 30, 60, 90: Intervening samples for glucose every 30 min ( to detect
peak blood glucose BGmax) .
Time 120: Take blood for glucose
Interpretation of OGTT (adapted from Ref 27):

Category Origins
Peak Blood
Glucose BGmax
CFID1
CFID2
Detection of weight and

lung function decline in CF
Detection of weight and
lung function decline in CF
Detection of microvascular
disease in type 2 diabetes
8.2
<11.1
11.1
<11.1
CFID3
<7
(CFRD
FH-)
CFID4
Detection of microvascular 7
(CFRD
disease in type 2 diabetes
FH+)
Possible ACTION on results of screening OGTT:
Blood Glucose
BG120 min
11.1
Not required
Normal glucose tolerance: Repeat OGTT in 1 year.

CFID1 and 2: Repeat OGTT in 1 year or sooner if clinical parameters
worsen e.g. lung function, unexplained weight loss and consider
treatment ( see below)
CFID 3 and 4: Start insulin.(d/w Consultant)
It is important to determine any factors that may have affected the OGTT
result e.g. concomitant infection, use of steroid treatment etc. before a
diagnosis of CFRD is made. Once diagnosis has been made, inform the
diabetic team including the specialist nurse.
77
8.6.2 Dietary Management of Diabetes in Cystic Fibrosis

CF patients are often underweight, with reduction in both fat and protein
stores. The degree they are underweight correlates inversely with survival.
Progressive clinical deterioration and weight loss have been reported up to 4
years prior to the onset of overt diabetes. With appropriate insulin therapy
and dietary management, body mass index and pulmonary function can be
restored to levels found before the onset of diabetes. Patients with CFRD
must therefore continue to follow an energy-dense diet because a low sugar,
low fat, high fibre diet typically promoted in the management of Type 1 and
Type 2 diabetes will not provide sufficient calories for the CF patient. A more
liberal approach to management with minimal dietary restrictions is therefore
recommended, and insulin regimens are adjusted to suit individual patients.
8.6.3 If the patient is generally well and in good nutritional status17
Recommend regular meals/snacks to ensure an even and consistent
distribution of carbohydrate throughout the day. (Carbohydrate exchanges
are no longer used in the dietary education of these patients). Complex
carbohydrates (bread, potatoes, pasta, rice, and cereals) should be a part of
each meal in order to maintain stable blood sugars. Carbohydrates with high
fibre content are to be encouraged in well-nourished children, but not
necessarily with poorly nourished children, as energy intake may be
compromised.
Ordinary squashes and fizzy drinks may be used, if taken in moderate
amounts and spread evenly throughout the day - preferably after meals. If
blood sugar levels are difficult to control, it may be necessary to use low
calorie/diet drinks (squash/fizzy drinks).
Sugar need only be restricted if consumed in large quantities throughout the
day, or if quantities are inconsistent from day-to-day. Otherwise, allow sugar
as usual.
Sweet foods e.g. chocolate/sweets should be spread evenly throughout the
day and taken preferably after meals. This will help avoid large swings in
blood sugar levels. Difficulties in controlling blood sugar levels should
be resolved by a multidisciplinary approach, with liaison between the
diabetes and CF team members.
Meal planning should be individualised to account for lifestyle, activity level
and food preference.
Advice should be given about hypos and extra carbohydrate should be taken
prior to exercise.
A reduction in fat is not recommended;
mono/polyunsaturated fats may be advisable.
but
change
to
78
Nutritional supplements/enteral feeding should be continued as prescribed

by the dietician. Insulin therapy should be adjusted as appropriate. Glucose
supplements e.g. Polycal, etc. are not recommended.
Dietary treatment of a hypo
If an insulin treated child has symptoms of hypoglycaemia (e.g. trembling,
pallor, sweating, impaired thinking, mood change),the blood glucose should
be tested and the patient should immediately be given some sugar, glucose
tablets or a sweet food or drink.
The quantity of sugar needed will vary from one child to another, as follows:
3-6 glucose tablets e.g. Dextrosol

cup (50ml) Lucozade
1 cup (100ml) ordinary fizzy drink (not diet) or fruit juice
2-4 teaspoons of sugar
If the child does not feel better after 10 minutes the above should be
repeated. Following the initial treatment of the hypo, if it is close to a meal
the meal should be brought forward, or, a starchy snack should be given e.g.
a sandwich or two biscuits.
8.6.4 If the patient is unwell and in poor clinical status

No dietary restrictions.
Encourage small regular meals and snacks
throughout the day. Nutritional supplements (including glucose polymers if
necessary) or enteral feeding as prescribed by the dietician.
Adjust insulin therapy to suit diet and to keep the patient `symptom free (no
need for `strict control over blood sugar levels).
8.6.5 Insulin17
Treatment should be considered in (CFID 1 and 2):
When impaired glucose tolerance on OGTT is associated with weight loss
or deteriorating clinical condition.
When there are episodes of transient hyperglycaemia.
When a diabetic glucose tolerance on OGTT, but normal glucose
monitoring, is associated with weight loss or deteriorating clinical condition
.
Definite indications for initiating treatment are CFID 3 and 427:
Insulin therapy should be started early and is the mainstay of treatment.
Optimal control of diabetes will reduce the chances of long-term complications
and should be the aim in most patients. Treatment adjustments may be
required during respiratory exacerbations, steroid therapy and enteral tube
feeding.
Insulin therapy should be cautious. Newer long acting insulins (detemir or
glargine) have theoretically ideal profile (no peak, prolonged duration of
action, stable profile from day to day and once daily s/c injection) and may be
suitable as single agents for treatment in the prediabetic stage(CFID 1 and
79
2) as well as early stages of frank CFRD23. Low dose glargine leads to

improved weight and pulmonary function in CF patients with abnormal glucose
tolerance without causing significant hypoglycaemia.24, 25
There is a
significantly greater reduction in fasting plasma glucose with glargine
compared to intermediate acting insulin 26.
Insulin preparation S/C
Glargine (lantus)
Total daily dose

0.25 U/kg
CFRD with raised fasting glucose will require both basal insulin and extra
mealtime insulin in order to achieve tight control. Patients with predictable
meal times and reliable food intake can sometimes be managed on twice daily
mixed insulin. Many people with CF have variable food intake due to loss of
appetite, nausea and the need to fit in other treatments especially in the
mornings. Patients with variable food intake usually require a basal bolus
regimen, where rapid acting insulin is given with meals and intermediate or
long acting insulin at bedtime17. Patients taking long acting insulin should
occasionally check blood glucose in the night. Patients who have enteral
feeding should check 1-2 times a week, at the beginning, and end of feed and
once during feeds. With intermittent bolus feeds, check 1 to 2 hours after
feed. Blood sugar also needs to be checked 9 hours after taking steroids and
before and after exercise. These patients should be discussed with the CF
Consultant for the optimal insulin regimen.
Before starting insulin therapy; an insulin starter pack should be prescribed;
and the patient should be reviewed by the Paediatric diabetic specialist
nurse, who will provide training on insulin administration.
Patients with CFRD may have increased insulin needs during acute infection
secondary to worsening insulin resistance and these patients must be
treated vigorously. Diabetic ketoacidosis is very rare in CFRD and should be
treated according to the local protocol. If patients with CFRD are going for
surgery and are on glargine, they will not need any other treatment. Ideally
blood glucose should be monitored more frequently.
8.6.6 Oral hypoglycaemic agents

The use of oral agents, including insulin secretagogues (such as
sulphonylureas) or insulin sensitizers (such as metformin or
thiazolidinediones), is not recommended in CFRD.
The onset and diagnosis of diabetes in patients with CF adds to the burden of
monitoring and treatment and may have important psychological implications.
Appropriate psychosocial support should be ensured.
References:
1. Kopelman H. Gastrointestinal and nutritional aspects. In: Shale DJ, editor. Cystic
Fibrosis. London: BMJ Publishing Group, 1996: 102-119.
2. Morton JR,Ansari N,Glanville AR,Meagher AP,Lord RV. DIOS in patients with CF
after lung transplantation J Gastroentert Surg.2009 may 22..
80
3. Abdominal Pain in Cystic Fibrosis. Littlewood JM J of Royal soc Med 1995;88;25;9-17

4. New modalities in the treatment of exocrine pancreatic insufficiency in cystic fibrosis.
Heijerman HG. Neth J Med 1992;41(3-4):105-9.
5. Debray D, Kelly D, Houwen R, Strandvik B, Colombo C.Best practice guidance for the
diagnosis and management of cystic fibrosis-associated liver disease.J Cyst Fibros.
2011 Jun;10 Suppl 2:S29-36.
6. Liver manifestations of cystic fibrosis. Akata D, Akhan O. Eur J Radiology 2007;61:
1117
7. Stringer MD, McClean P. Treatment of oesophageal varices. Arch Dis Child 1997;
77:476-477.
8. Aris RM, Merkel PA, Bachrach LK et al CONSENCUS STATEMENT: Guide to bone
health and disease in Cystic Fibrosis. J of Clin Endocrin & Metabolism. 2005
90;1888-96.
9. Bone Mineralisation in Cystic Fibrosis Report of the UK Cystic Fibrosis Trust Bone
Mineralisation Working Group February 2007
10. Mischler EH, Chesney PJ, Chesney RW, Mazess RB. Demineralization in cystic
fibrosis detected by direct photon absorptiometry. Am J Dis Child 1979; 133:632-635.
11. Aris RM, et al. Adverse alterations in bone metabolism are associated with lung
infection in adults with cystic fibrosis. Am J Respir Crit Care Med 2000; 162(5):16741678.
12. Aris RM, Barbers RG, Barst R, et al. International guidelines for the selection of lung
transplant candidates. Am J Respir Crit Care Med 1998; 158:335-339
13. Sermet-Gaudelus I et al. Low Bone Mineral Density in Young Children with Cystic
Fibrosis Am J Respir Crit Care Med 2007; Vol 175. pp 951957
14. Cadogan J, Eastell R, Jones N, Barker ME. Milk intake and bone mineral acquisition
in girls: randomized controlled intervention trial. BMJ 1997;315:1255-1260
15. Aris RM, et al. Efficacy of Alendronate in cystic fibrosis adults with Low Bone Mineral
Density. Am J Respir Crit Care Med 2003
16. Phillips BM, David TJ. Pathogenesis and Management of arthropathy in cystic
fibrosis. J of R Soc of Med 1986;12(79):44-50
17. Management of cystic fibrosis related diabetes mellitus Report of the UK Cystic
Fibrosis Trust Diabetes Working Group June 2004
18. Bismuth E et al Glucose Tolerance and Insulin Secretion, Morbidity, and Death in
Patients with Cystic Fibrosis J Pediatr 2008;152:540-5)
19. Sims E J et al Decreased lung function in Female but not Male subjects with
established Cystic Fibrosis-related Diabetes. Diabetes Care; 2005; 28, 7;1581
20. Milla CE et al Diabetes Is Associated With Dramatically Decreased Survival in
Female but Not Male subjects with Cystic Fibrosis. Diabetes Care; Sep 2005; 28, 9;
2141-44.
21. Lanng S, Thorsteinsson B, Nerup J, Koch C. Influence of the development of
diabetes mellitus on clinical status in patients with cystic fibrosis. Eur J Pediatr
1992;151:684-7
22. Moran a et al Cystic Fibrosis Related Diabetes: Current Trends in Prevalence,
Incidence and Mortality Diabetes Care published online June 19, 2009
23. L Dobson, A T Hattersley, S Tiley, S Elworthy, P J Oades, and C D SheldonClinical
improvement in cystic fibrosis with early insulin treatment Arch. Dis. Child., Nov 2002;
87: 430 431
24. P Grover,W Thomas,A Moran.Glargine versus NPH insulin in cystic fibrosis related
diabetes.Journal of cystic fibrosis 7(2008)134-136.
25. Mozzillo E, et al: One year glargine treatment can improve the course of lung
disease in children and adolescents with cystic fibrosis and early glucose
derangements. Pediatric Diabetes 200910:162- 167,
26. Bizzarri C, Lucidi V, Ciampalini P, Bella S, Russo B, Cappa M: Clincial effects of
early treatment with insulin glargine in patients with cystic fibrosis and impaired
glucose tolerance. 2006 J Endocrinol Invest 29:1-4
27. Hameed S, Jaff A, Verge CF.Cystic fibrosis related diabetes (CFRD)-the end stage
of progressive insulin deficiency.Pediatr Pulmonol.2011;46(8):747-60.
81
Section 9: Dietetic & Nutritional Management of Paediatric

Cystic Fibrosis
(Revised by Robyn Huggins 2012)
Nutritional management for children with CF - a summary
Nutrition requirements are often higher than normal in CF and a high calorie, high
protein balanced, nutritious diet is encouraged (9.1).
Growth and nutritional status needs to be closely monitored (9.2)
Nutrition support with oral supplements (9.3a) and/or enteral feeding (9.3b) may be
needed to meet nutrition demands for normal weight gain and growth in CF.
The majority of CF patients are pancreatic insufficient and require pancreatic enzyme
replacement therapy (PERT) to treat malabsorption (9.4).
Supplements of the fat-soluble vitamins A, E and D are commonly required to prevent
deficiency (9.5).
Sodium supplements may be required by infants particularly those with ileostomys and
by children with increased sweat losses due to exercise or in hot weather (9.6).
82
9.1 Diet and nutrition (1, 2, 3, 4, 5)

Nutritional state plays a major part in maintaining respiratory health. Good
growth and nutritional status is associated with better lung function and
survival for children with CF. Conversely malnutrition increases susceptibility
to infection and compromises growth, lung function and survival. Numerous
factors increase risk of malnutrition in CF including maldigestion and
malabsorption due to pancreatic insufficiency; increased energy demands of
progressive lung disease and catabolic and anorexic effects of infection.
Active nutritional management is vital to ensure all children with CF achieve
optimal weight gain, growth and vitamin, mineral and essential fatty acid
(EFA) status.
Nutritional requirements depend on age, clinical and nutritional status and
generally increase with age and disease severity. Energy (calorie) and protein
requirements of children with CF are often increased and it is commonly
recommended to aim for 100 -150% of the EAR for energy and 200% of the
RNI for protein intake. Dietary fat is essential to achieve a high energy intake
and should provide 35% - 40% of total energy (1) with emphasis on increasing
mono and poly-unsaturated fats to prevent deficiency of essential fatty acids.
Fibre is to be encouraged in the well-nourished patient to help prevent gut
complications such as constipation and distal ileal obstruction syndrome but it
may compromise energy intake in poorly nourished patients. Fluids and salt
are also to be encouraged for good hydration.
Breast milk is ideal for CF infants for the first 4-6 months of life and breast
feeding has been shown to protect against infections in the first three years of
life (1). Alternatively normal infant formula can be used. Nutrient enriched
infant formulas are not needed routinely but can be helpful for the infant with
slow growth. Weaning is often needed a little early (17 weeks) to satisfy the
increased appetite and nutritional needs of infants with CF but progression of
food types and textures is as normal. For toddlers and children with CF a
good eating pattern of regular meals and snacks and a varied balanced diet is
recommended. Nutrient dense foods and fortifying foods is helpful if appetite
is poor and/ or nutritional requirements are increased.
Excessive focus on food, feeding and weight gain can lead to abnormal
feeding patterns and eating behaviour in children with CF thus preventative
and supportive counselling is needed and care plans must be individualized to
child and family needs. With increased life expectancy it is also important to
minimize risk of developing CF bone disease, achieve good control of cystic
fibrosis related diabetes (CFRD) and support development of selfmanagement skills for progression to adult life.
9.2 Assessment of growth & nutritional status

Growth is considered by many to be the best indicator of a childs nutritional
status. Assessment of growth requires serial measurements of height and
weight (and head circumference for infants) and use of growth percentile
charts appropriate for age and gender. An individual childs progress can be
assessed and monitored from plotted longitudinal data - variation from a
consistent percentile can indicate problems. Body mass index percentiles
(BMI centile) assess weight-for-height status and are recommended to screen
83
for malnutrition (3, 5). American data suggests that a BMI of 50th percentile
is associated with best lung function in CF (5) and that a BMI percentile
between the 10th and 25th represents nutritional risk and a BMI percentile
below the 10th represents nutritional failure (3). However percentiles by
definition describe the distribution of growth in a population and therefore not
all patients in the at risk category will be nutritionally compromised. Caution
should be applied when interpreting growth data in pubertal delay or during
the pubertal growth spurt. Weight, height and BMI Standard Deviation Scores
(sds) can be used to audit the nutritional status of the CF clinic as a whole.
Weight and height should be recorded at every clinic visit and plotted onto
individual percentile charts in the medical notes. Head circumference should
be recorded for infants. For all children >2 years BMI percentile should be
determined at annual review to allow for greater accuracy in assessing
individual nutritional status. All annual review measures should be recorded in
the dietetic files, medical notes and on the Port CF database.
9.3 Nutritional Support

The majority of children with CF grow well on a normal to high-energy diet. A
sub-group require oral nutritional supplements and/or enteral feeding to
support growth. Nutritional risk is increased at times of high growth (infancy
and puberty), with progressive or advanced lung disease, established
bacterial colonisations particularly some Pseudomonas and Burholderia
Cepachia species and by co-morbidities notable CFRD.
Guidelines for nutritional intervention
Child's Nutritional State
Intervention Required
Good nutritional status,

Maintaining normal growth &
weight gain
BMI centile 50th
Regular review
Monitor weight and growth
Preventative advice to optimize
diet & PERT
Declining nutritional status

Weight loss over 4 months or
plateau in weight over 6 months.
BMI centile 25th
Thorough review and intervention

Optimize diet & PERT
Consider oral nutritional
supplements
Malnourished
Weight falling 2 centiles or inability
to gain/maintain weight on diet
and oral supplements over a 6month period.
BMI centile < 9th
Aggressive nutritional support

Optimize diet, oral nutritional
supplements & PERT
Consider enteral feeding
84
9.3.1 Oral nutritional supplements

Nutritional supplements are not needed routinely and studies of long-term
use of commercially available supplements did not improve nutritional status
compared with dietary advice (3, 6). Supplements may be recommended
when a child is failing to grow or gain weight satisfactorily (on normal diet
and appropriate PERT) and/or during illness when the child may be catabolic
and have a poor appetite.
A useful guideline is to increase the existing energy intake by a further

20-30% or the following is a `rough indication of how much extra to give
daily:
1 to 2 years - 200kcal
3 to 5 years - 400kcal
6 to 11 years - 400 to 600kcal

> 12 years - 800kcal
The dietician will select the most appropriate nutritional supplement to

suit individual needs. The use of supplements must be regularly reassessed by an experienced dietician. The supplements listed below can
be prescribed on ACBS.
Supplements can be given 1 - 3 times daily either after meals or at snack
times. The quantity and timing of supplements is critical such that
sufficient is given to promote weight gain without causing reduction in
normal appetite.
Enzymes must be taken with supplements containing fat or protein. Dose
should be individualized however common doses are listed below for
reference.
Oral nutritional supplements and PERT Doses
Nutritional Value
Common PERT
dose
Creon
Creon
10000
25000
Energy
Fat
(Kcals)
(grams)
1. Nutritionally complete or protein/energy supplements
Fortini or Pediasure Plus (200ml)
300
14-15
2
Fortisip or Fresubin Energy
300
~12
2
(200ml)
Fortijuce or Provide Xtra (200ml)
300
0
0-1
Scandishake or Calshake
600
30
5
(300ml)
2. Energy supplements
Calogen (30ml)
140
15
2-3
Polycal or Maxijul powder
20
0
0
(per 5 gram scoop)
1
1
0-1
2-3
1
0
9.3.2 Nasogastric and gastrostomy feeds

A number of studies have shown that nocturnal enteral feeding of
malnourished patients with CF improves nutritional status and improves,
stabilises or slows the rate of pulmonary function decline without major side
effects (7, 8, 9). Enteral feeding should be considered in CF children who are
unable to achieve or maintain adequate nutritional intake, weight gain and
85
growth with a high-energy diet and oral nutritional supplements. This includes
infants who are failing to thrive, children with more severe chest disease and
children who are being considered for transplantation. See table in 9.3 above
for selection criteria (3, 10). However the CF multidisciplinary team should
discuss each individual case before discussing the concept of home enteral
feeding with the child and family. Gastrostomy buttons are the method of
choice for long term enteral feeding in children with CF. Nasogastric tubes are
suitable for short term feeding such as in acute illness.
Guidelines for selection of feed, regimen and PERT doses
General recommendations:
Enteral tube feeding is usually given overnight allowing patients to eat
normally through the day.
Use a whole protein infant, paediatric or adult feed appropriate for age
(see below).
Commence with 50% of estimated energy requirements from the overnight
enteral feed. The level of nutrition support (volume and/or type of feed) can
then be adjusted to attain and maintain (catch-up) weight gain, growth and
good tolerance of feeds.
The enzyme requirement for continuous enteral feeds is often lower than
the patients usual doses with food (dietary fat). 1000-1500U of lipase per
gram of fat is usually adequate or 1 Creon 10000 per 7-10 grams of fat.
Doses should be rounded out for simplicity. The dose can then be adjusted
if necessary depending upon tolerance and weight gain.
Infants and Neonates
Use the infants normal milk feed (breast milk or infant formula). CF infants
require a minimum of 150ml/kg/day of fluid but typically need 180250ml/kg/day of breast milk or standard formula to meet energy, nutrient
and sodium requirements.
Consider using high-energy infant formula (such as SMA High Energy or
Infatrini) at 120-180ml/kg/day if the infant has lost weight, has poor growth
or poor tolerance of volume. In CF, protein hydrolysate formulas containing
MCT such as Pepti Junior require PERT and have not been found to be of
benefit over standard formula (11) thus should only be used if clinically
indicated post-surgery.
Enzymes should be given with each bolus feed or 3-4 hourly during
continuous feeds. For suggested doses see table Guideline for
commencing PERT in CF Infants. The enzyme micro-spheres should be
given orally in a very small quantity of fruit puree from a baby spoon.
For infants on the Neonatal Unit see Nottingham Neonatal Service Clinical
Guideline F5 Pancreatic Enzyme Replacement Therapy and Feeding of
Cystic Fibrosis Infants on the NNU for specific recommendations.
Children
For children >1 year use whole protein products providing 1.5-2kcal/ml
(appropriate for age) such as Nutrini Energy or Nutrison Energy
(1.5kcal/ml) or Nutrison Concentrated (2kcal/ml). Pack presentation is
preferable for ease of use and infection control at home.
Commence with 50% of estimated energy requirements from the overnight
enteral feed. The feeding regimen should be matched to the child/families
routine to minimise disruption i.e. commence feed at the childs usual
86
bedtime and calculate rate of delivery to finish at usual waking time. Allow
for the child to have at least one night off the feed per week to reduce the
burden of care on the child and family.
Divide the total enzyme required for a continuous overnight feed into 2-3
doses given at the start (before sleep), during the night (if awake) and in
the morning.
Dose should be individualised however common doses are listed below for
reference.
Very occasionally it is necessary to use a peptide based feed such as
Peptisorb (Nutricia Clinical Care) due to poor feed tolerance (worsening
abdominal symptoms, vomiting or failure to gain weight). A lower enzyme
dosage may be needed due to the lower fat content.
Common Overnight Enteral Feeds and PERT
Doses
Feed
Nutrini Energy
(1 x 500ml pack)
Nutrison Energy
(1 x 1000ml pack)
Nutrison
Concentrated
(1x 500ml pack)
Energy
(kcals)
750
1500
1000
Common Regimen
50ml/hr x 10hrs
(or 60ml/hr x 8hrs)
100ml/hr x 10hrs
(or 125ml/hr x 8hrs)
50ml/hr x 10hrs
(or 62ml/hr x 8hrs)
Common dosage
of Creon 10000
Start of
feed
3
End of
feed
1-2
Home enteral feeding

CF patients are taught how to tube feed whilst in hospital. The dietician, CF
and stoma specialist nurses, paediatric community nurse, ward nursing staff
and Homeward nurse are all involved in patient-training, and the patient
should not be discharged home until all staff members are satisfied with the
patients understanding and technique. Written information including a contact
telephone number should be provided by the dietician. The Homeward Nurse
can also provide follow-up teaching on use of the feeding pump at home. The
paediatric community nurse will register the child with Vygon Home Service
for supply of gastrostomy buttons and extension sets or alternative buttons
and extension sets can be supplied via Homeward. The dietician will register
the child with Homeward Clinical Support Services for supply of a feeding
pump and monthly deliveries of feed, giving sets and syringes. It may be
necessary to provide the first weeks supply of feed and equipment from the
ward at discharge. Patient progress should be regularly reviewed by the
dietician at clinic, home and by telephone. See also Appendix 8 - Gastrostomy
care.
9.4 Pancreatic enzyme supplements

Approximately 90% of children with CF are pancreatic insufficient and require
pancreatic enzyme replacement therapy (PERT). Following diagnosis
pancreatic insufficiency should be confirmed by measurement of faecal
pancreatic elastase. Cease enzyme supplements if faecal elastase is normal.
Enteric-coated micro-sphere preparations are recommended. The pH87
sensitive coating protects the enzymes from inactivation by stomach acid,

dissolving only when the pH exceeds 5.5 within the small bowel.
Studies (12) show enzyme supplements improve stool frequency and
consistency and can achieve good absorption of dietary fat of over 80%
coefficient of fat absorption (CFA). However satisfactory absorption and
control of bowel symptoms cannot always be achieved. Gastrointestinal
dysfunction in CF is complex with gastric, intestinal and hepatobiliary factors
as well as pancreatic insufficiency contributing to maldigestion and/or
malabsorption (13, 14). This includes inactivation of enzymes by prolonged
exposure to gastric acid and delayed release of enzymes from enteric coated
microspheres as far distal as the ileum due to acidification of the duodenum
(1). Problems with digestion including stomach pains, abnormal or frequent
stools and difficulty with enzyme dosing were reported by over 50% of parents
of children with CF (15). Non-adherence can also be significant contributor.
Children with CF and carers including schools need considerable support with
managing enzymes to optimize weight gain, growth and control of bowel
symptoms.
Enzyme preparations used at Nottingham Childrens Hospital
1. Standard Preparations recommended for: newly diagnosed
patients, infants and children or patient/GP preference.
2. High lipase preparations recommended for: Older children/adults,
patients with higher lipase requirements or patient preference. Creon
40000 is not typically recommended for children.
Preparation
1. Standard
Creon Micro*
Creon 10 000*
Enzyme Concentration (per scoop or capsule)

Lipase
Amylase
Protease
5000
3600
10 000
8000
Lipase
Amylase
200
Per
scoop
600
Per
capsule
Protease
2. High Lipase
Creon 25000*
25000
18000
1000
Creon 40000*
40000
25000
1600
Per
capsule
Per
capsule
* Abbott Healthcare
Dosage recommendations for PERT:
Evidence supports the efficacy of PERT to improve maldigestion due to
pancreatic insufficiency however guidelines for PERT dosage are consensus
based as there is insufficient evidence to make specific recommendations (5).
Dosage recommendations should take into account:
1. Committee on Safety of Medicines Recommendations (16) May 1995,
to minimize risk of fibrosing colonopathy (colonic strictures) in CF.
88
i. Enzyme dosage should not usually exceed 10,000 units of lipase

per kg per day.
ii. The high strength preparations Pancrease HL and Nutrizym 22 are
contra-indicated in children 15 years of age.
NB: Creon preparations have not been implicated in fibrosing
colonopathy.
2. American guidelines for enzyme dosage in relation to dietary fat
(17)
i. Infants: 450 - 900 units of lipase per gram of dietary fat
ii. Children & Adults: 500 - 4000 units of lipase per gram of dietary fat
with a mean requirement of 1800 units of lipase/g fat/day.
Enzyme doses should be determined on an individual basis and take into

account nutritional intake, abdominal symptoms and growth/weight gain.
The specialist dietician will see each CF child at least twice a year to
assess/ re-assess dosage requirements and adherence. Adjustment of
doses requires close supervision in order to achieve good weight gain,
growth and control of bowels.
Some patients require higher doses than the CSM recommendation of
<10,000 units of lipase/kg/day particularly those with a high energy
intake, large appetite or on enteral feeding. In these cases the patient
should be monitored. Dietary fat should not be restricted as the ultimate
goal is to promote normal nutrition, good weight gain and growth.
Good bowel control cannot always be achieved by increasing enzyme
dosage. For those exceeding the current CSM recommendation it is also
useful to consider:
i. Is dosage appropriate for nutritional intake? Check against the USA
recommendation for enzyme dosage in relation to dietary fat (see above)
and reduce/redistribute doses if necessary.
ii. Are adjunct therapies such as omeprazole or ranitidine required?
These reduce gastric acidity and hence duodenal pH to enhance enzyme
efficiency. NB. Higher than the routine recommended doses of
Omeprazole may be required.
iii. Other GI disorders occasionally complicate management for example
Coeliac disease, Crohns disease, food allergies and intolerances or short
bowel syndrome. Ensure any co-existing gastro-intestinal disorders are
effectively treated.
Practical administration of PERT

1. Infants
Creon Micro is the preferred pancreatic enzyme supplement for

infants (1). The enzyme granules from Creon 10,000 capsules can
also be used.
The enzyme micro-spheres should be given orally pre-feeds. The
prescribed dose should be mixed with a little ( teaspoon) fruit puree
and be given from a baby spoon. Check that micro spheres do not
remain in the mouth post-feed.
Commence with suggested doses below. These are practical doses to
administer and provide in the order of 700-1200 IU of lipase per gram
of fat.
89
For infants on the Neonatal Unit see Nottingham Neonatal Service

Clinical Guideline F5 Pancreatic Enzyme Replacement Therapy
and Feeding of Cystic Fibrosis Infants on the NNU for specific
recommendations.
Guideline for commencing PERT in CF Infants

Milk Type
Breast Milk
Standard Infant
Formula
High Energy Infant
Formula SMA High
Energy or Infatrini
Semi-elemental
Formula
e.g. Pepti Junior
Feed Size
100ml
scoop Creon Micro
short breast feed
scoop Creon Micro
(or Creon 10000 cap.)
1 scoop Creon Micro
scoop Creon Micro
120 - 180ml
scoop Creon Micro
long breast feed
1 scoop Creon Micro
1 -2 scoops Creon
Micro
(or 1 Creon 10000 cap.)
- scoop Creon Micro
Increase the dose gradually by - scoop of Creon Micro (or Creon

10000 capsule) at each milk feed until bowel symptoms, weight gain and
growth is good. NB Excessive doses can result in anal excoriation or
constipation.
Aim to keep lipase use below the CSM guideline of 10000IU of lipase per
kg per day however more may be required during growth spurts or for
catch-up weight gain as high feed intakes need commensurate enzyme
dosing. CF infants commonly take above average energy intakes of 120160kcals/kg that is 180-240ml per kg per day of breast milk or term infant
formula (or 120-160ml per kg per day of high energy infant formula) thus
fat intake can be up to 8-12g per kg per day. Compare lipase use to both
dietary fat and CSM guidelines to assess if dose is appropriate.
In CF infants who have undergone bowel surgery for meconium ileus (MI)
symptoms will be related to factors such as length of bowel resected not
just to PERT. Satisfactory bowel control cannot always be achieved by
increasing enzyme dosage. Other investigations should be considered if
there is persistent poor weight gain, growth and/or poor control of bowel
symptoms despite appropriate PERT.
2. Children
The aim of PERT is good weight gain, growth and control of bowel symptoms
(that is formed stools with reasonable frequency, odour, wind and abdominal
discomfort).
Enzyme supplements should be taken with all foods containing fat,
protein or starch i.e. meals, snacks (such as crisps, chocolate, biscuits
and sandwiches) and drinks (such as milk and milkshakes). Enzymes are
not needed with foods or fluids based on simple sugars such as squash,
fizzy drinks, fruit juice, fruit or boiled/jelly sweets.
Doses should be individualized and patients taught to adjust doses
depending upon quantities and fat content of food consumed (18). Fat
based dosing or matching enzyme doses to the fat content of foods can
90
be a useful tool for teaching dose adjustment of enzymes to some

patients and parents/carers. The mean lipase requirement in CF of
1800U per gram of dietary fat equates to 1 Creon 10000 for 5-6 grams of
fat or 1 Creon 25000 for 12-15 grams of fat. However individual
requirements can vary between 500 and 4000IU of lipase per gram of
dietary fat and thus from as little as 2 - 3 capsules with main meals up to
8 - 10 capsules.
Ideally enzyme doses should be split at main meals with - before and
- during the meal to maximize efficacy and to allow dose to be titrated
to the amount eaten and fat content of the meal.
Enzyme capsules should be swallowed whole at as early an age as
possible and most children can manage this from the age of 4-5 years.
The micro spheres should not be crushed or chewed as this will reduce
enzyme effectiveness. For young children or those who are unable or
unwilling to swallow whole capsules:
1. The enzyme micro spheres may be mixed with a small quantity of food
preferably with acid pH such as fruit pure or
2. Some children choose to swallow the micro spheres from opened
capsules directly with a drink.
Enzyme preparations should be changed to suit needs for example
progress to Creon 10000 when a young child is able to swallow capsules;
move on to Creon 25000 when enzyme dose is inconvenient (6+ per
meal). Aim for overall lipase dosages to remain comparable:
1. 1 scoop Creon Micro Creon 10000 capsule
2. 1 Creon 10000 capsule - Creon 25000 capsule.
3. Late Diagnosed Infants and Children
Commence with practical amounts to administer (ie whole scoops of Creon
Micro or Creon 10000 capsules) at a low lipase dose of 1000-1500 IU per
gram of fat. Increase doses slowly to avoid excessive weight gain or drastic
changes in bowel habit. Grade up by -1 scoop of Creon Micro or -1
Creon 10000 capsule until bowel symptoms, weight gain and growth are
good.
9.5 Vitamin supplements
The majority of CF patients require supplements of the fat-soluble vitamins
A, D and E in order to maintain normal vitamin status (2). This is partly
related to fat malabsorption, and possibly to an increased metabolic
requirement. Vitamin K supplementation may also be important to achieve
and maintain normal homeostasis, especially because of its role in bone
metabolism. A recommended dose for vitamin K in CF is not yet established
- see (2) for recommendations on monitoring of vitamin K status and section
8.4 CF Bone Disease.
Fat-soluble vitamins can accumulate in body tissues. In particular elevated
levels of vitamin A are found in the liver of CF patients at autopsy (2).
Further, an expert review (16) found potential adverse effects of high vitamin
A intakes on bone health and has recommended population sub-groups at
risk of osteoporosis should not consume more than 1500 micrograms
(4500U) of RE per day. Thus it is prudent to avoid excessive supplemental
vitamin A in CF patients.
91
Summary of vitamin supplementation doses in CF (2, 3):

Vitamin A
Vitamin D
Vitamin E
Age
units
units
milligrams
(micrograms)
(micrograms)
<1
1500*-4,000
400
10 - 50
Year
(450*-1200)
(10)
1 -10
4,000 - 10,000
400 - 800
50 - 100
Years
(1,200- 3,000)
(10 - 20)
>10
years
4,000 - 10,000
(1,200 - 3,000)
400 - 800
(10 - 20)
Vitamin K
milligrams
10
suggested
dose
100- 200
10
suggested
dose
*< 1 year: see (3) for USA guideline on vitamin A supplementation

General recommendations for fat-soluble vitamin supplementation (2):
Pancreatic sufficient patients should have plasma vitamin levels
checked and vitamin supplements commenced when low levels are
detected.
Pancreatic insufficient patients should commence supplemental
vitamins A, D & E on diagnosis.
Vitamin supplements should be taken at a mealtime when pancreatic
enzyme supplements are used as this may improve absorption.
The supplemental doses of vitamins A, E & D should be determined
on an individual basis and be guided by plasma levels.
Monitoring of vitamin status:
Annual assessment of vitamin status (blood levels, dietary assessment, and
adherence) is important so that dosage can be titrated to suit individual
needs.
Plasma vitamin A, E & D levels should be measured for Annual Review
during a period of clinical stability.
Adjust vitamin supplement dose according to plasma levels. Plasma
levels should be re-checked within 3 - 6 months after any change in
supplement regimen. NB. Adherence should be checked before
increasing doses.
Progress to tablet or capsule preparations when children are able to
swallow enzyme capsules taking care to ensure vitamin doses remain
comparable.
Guideline for daily fat-soluble vitamin supplementation of CF Children
Multivitamin
(Vitamin A & Vitamin D)
Age
Vitamin E
0.6ml Abidec
50mg Vitamin E
Infants* & Toddlers
10
drops
Healthy
Start
(0.5ml)
liquid preparations
Childrens Vitamin Drops
0.6 - 1.2ml Abidec
0.5-1ml Vitamin E
Children 4 - 10 Years
or 1-3 MV capsules
or 1-2 Vitamin E gel
liquid or tablet
caps
preparations
1-2 A&D capsules
1-4 Vitamin E gel caps
Children 10+ Years
or 1-3 MV capsules
or 1-2 Swiss Ephynal
tablet preparations
* For infants on the Neonatal Unit see Nottingham Neonatal Service Clinical
Guideline F5 for specific recommendations.
92
Composition of vitamin preparations used at Nottingham Childrens Hospital

i. Multivitamins
Dalivit
Multi-vitamin
capsule
(BPC)
Forceval
A&D
Capsule
per 0.6ml
Healthy Start
Childrens
Vitamin
drops
per 10 drops
Per 0.6ml
Per capsule
Per capsule
Per capsule
1,333
400
0
0
40
1,400
600
0
0
40
5,000
400
0
0
50
2,500
300
0
0
0
2500
200
10
0
60
4,500
450
0
0
0
Abidec
Vitamin
A (U)
D (U)
E (mg)
K (mg)
C (mg)
Notes
For prescribing purposes Multi-vitamin Capsule (BPC) is listed in the BNF
and BNFC section 9.6.7 Multivitamin preparations as Vitamins Capsules
Forceval contains water-soluble vitamins (B group and C) iron and zinc
which can be useful when a child has an overall poor nutritional intake
and requires a multivitamin/mineral preparation to improve nutritional
adequacy. However iron supplementation is cautioned against as it may
enhance growth of Pseudomonas.
ii. Vitamin E*
Vitamin
E (mg)
Vitamin E
suspension
per ml
Vitamin E gel capsule

75IU ACBS
per capsule
Vitamin E gel capsule

400IU ACBS
per capsule
100
50
268
* Vitamin E is also known as alpha tocopheryl.
9.6 Salt supplementation (2, 20)

The CF defect gives risk of increased loss of sodium and chloride in sweat
with exercise or in hot conditions. Infants with CF especially those with an
ileostomy are at risk of salt depletion due to low levels of sodium in breast
milk and infant formula; a large body surface area; a greater requirement for
sodium due to the rapid growth rate and with an ileostomy there is increased
stool losses of electrolytes. Sodium deficiency can result in anorexia and
growth impairment in infancy and in children hyponatraemic dehydration may
contribute to thicker and more difficult to expectorate sputum and predispose
to DIOS in susceptible patients.
There is a lack of evidence and consensus regarding the need for sodium
replacement and dosage in CF however clinically additional sodium may be
required:
In infants on breast milk or normal infant formulas particularly those with
an ileostomy (see details above).
During illness when febrile episodes can increase losses and poor
appetite can reduce intake. NB oral supplements and enteral tube feeds
are low in sodium.
When undertaking strenuous exercise or work
When holidaying or living in hot or humid conditions
93
In infants a spot urine analysis (Na <10mmol/l) and measurement of serum

electrolytes can confirm sodium deficiency. Commence sodium supplements
of 2-3mmol/kg/day, monitor clinical response and titrate dose to normalise
urine (Na >20mmol/l) and plasma sodium levels. Preterm infants and infants
with ileostomies may require high doses - monitor symptoms and
individualize doses.
Suggested doses for sodium supplementation (20)
Age
< 1 year
Sodium dose
2-3mmol/kg
1 - 10
years
> 10
years
10-20
mmol/day
30-40
mmol/day
Suggested preparation(s)
40-50ml/kg of Dioralyte
0.4-0.6ml/kg of 30% Sodium Chloride Solution
1-2 sachets (200-400ml) of Dioralyte or
1-2 x Slow Sodium tablets
3-4 x Slow Sodium tablets
Dioralyte: 1 x 6g sachet contains 12mmol Na and is made up to 200ml

30% Sodium Chloride Solution contains 5mmol Na in 1ml
Slow Sodium: 1 tablet contains 10mmol Na.
For children sodium intake can also be increased by adding salt to foods and
in cooking and eating high salt foods such as crisps, cup-soups, ketchup and
marmite.
9.7 Standards of dietetic care

The specialist dietician should see each CF child for nutritional
assessment at least twice a year. Infants, children using nutritional
support, and those with specific nutritional concerns such as CFRD will
be seen more frequently, as determined by an individualized care plan.
Children will have a dietary annual assessment around their birthday
month. A written report will be provided to the specialist Consultants and
verbal feedback will be given to the parents/ child in clinic.
Standards of care, as outlined in the Annual Report, and in line with
recommendations from the CF Trust (2, 21) will be maintained and
service to the Nottingham centre and shared care hospitals will be
audited.
References:
1. Kalnins, D and Wilschanski, M. Maintenance of nutritional status in
patients with cystic fibrosis: new and emerging therapies. Drug Design,
Development & Therapy, 2012; 6: 151-161.
2. UK CF Trust Nutrition Working Group. Nutritional management of CF. 2002
3. Borowitz D et al. Consensus Report on Nutrition for Pediatric Patients with
CF. American Consensus Committee. J of Pediatric Gastroenterology &
Nutrition 2002; 35: 246-259.
4. Sinaasappel M et al. et al. Nutrition in patients with cystic fibrosis: a European
Consensus Report. J of Cystic Fibrosis 2002;1: 51-75
94
5. Stallings V et al. Evidence based practice recommendations for nutrition

related management of children & adults with cystic fibrosis and pancreatic
insufficiency: results of a systematic review. J Am Diet Assoc 2008; 108:832-9
6. Poustie V. et al. Oral protein energy supplements for children with CF:
CALICO multicentre randomised controlled trial. BMJ 332; 632-636, 2006
7. Steinkamp G, von der Hardt H, Improvement of nutritional status and lung
function after long term nocturnal gastrostomy feedings in cystic fibrosis,
1994, Vol. 124, pg. 244-249
8. Walker S.A, Gozal D, Pulmonary function correlates in the prediction of longterm weight gain in cystic fibrosis patients with gastrostomy tube feedings,
Journal of Pediatric Gastroenterology and Nutrition, 1998, Vol. 27, pg. 53-56
9. Williams S.G.J, Ashworth F, McAlweenie A, Poole S et al, Percutaneous
endoscopic gastrostomy feeding in patients with cystic fibrosis, Gut, 1999,
Vol. 44, Iss.1, pg. 87-91
10. Ramsey BW, Farrell P, Pencharz P and the Consensus Committee. Nutrition
assessment & management of CF: a consensus report. Am J Clin Nutr 1992;
55:108-116
11. Ellis L et al. Do infants with cystic fibrosis need a protein hydrolysate
formula? A prospective, randomized, comparative study. J of Pediatr
1998; 132:270-6
12. Sytematic review: efficacy and safety of pancreatic enzyme supplements
for exocrine pancreatic insufficiency. Taylor JR, Gardner TB, Waljee AK,
Dimagno & Schoenfeld PS Alliment Pharmacol Ther 2010; 31: 57-72.
13. Borowitz, D et al. Gastrointestinal Outcomes and Confounders in Cystic
Fibrosis. , Journal of Paediatric Gastroenterology & Nutrition 2005; 41; 273285.
14. Taylor CJ, Hillel PG, Ghosal S, Frier M, Senior S, Tindale WB & Read N; et al.
Gastric emptying and intestinal transit of pancreatic enzyme supplements in
cystic fibrosis. Arch Dis Child 1999: 80:149-152
15. Lake, E. Food for thought Patients & carers views on dietetic care in
cystic fibrosis. Cystic Fibrosis Trust UK, January 2010.
16. Report of the pancreatic enzymes working party. 1995. London, Committee on
safety of medicines.
17. Borowitz DS, Grand RJ, Durie PR. Use of pancreatic enzyme supplements for
patients with CF in the context of fibrosing colonopathy. Consensus
Committee Review. Journal of Paediatrics 1995; 127(5): 681-684.
18. Anthony H et al. Pancreatic replacement therapy in cystic fibrosis: Australian
guidelines. J. Paediatr. Child Health; 1999; 35: 125-129.
19. SACN (2005) Review of dietary advice on Vitamin A. TSO, London.
(http.//www.sacn.gov.uk/pdfs/sacn-vita-report.pdf).
20. MacDonald A, Nutritional management of cystic fibrosis. Archives of Disease
in Childhood, 1996; 74:81-7.
21. Cystic Fibrosis Trust UK. Standards for the Clinical Care of Children and
Adults with Cystic Fibrosis in the UK. Second edition December 2011.
95
Section 10 - Guidelines for Obtaining Intravenous Access

(Revised by Amanda Ward, Janice Mighten & Debra Forster)
96
10.1 Pic line/ Longline insertion

The preferred route of administration for intravenous antibiotics is a longline.
It is recommended that a Registrar or an appropriately experienced SHO
inserts longlines in young children or anyone with a significant needle phobia
because of the implications for their long-term management. It may be
appropriate for SHOs to gain experience in the technique on an older child
with appropriate supervision from a registrar. Make sure Emla cream or
Ametop have been applied to several sites where you feel confident you can
insert a line. (The site may require negotiation with some children). Sedation
may be necessary for a few children (see table below) but a gentle manner
and the presence of the parent during the procedure usually make this
unnecessary. The skills of the play specialist are invaluable for providing
distraction therapy and should be used whenever possible. A few children
benefit from the use of Entonox, which may be used if administered by an
appropriately trained nurse or doctor. Always try to plan an admission for
when a Registar is available to insert the line.
Type of
drug
Drug
Route
Dose
Sedative
Midazolam
Oral
Midazolam
Intranasal
Ametop
Topical
500mcg/kg
(max dose 20mg)
200 300 mcg/kg
dose each
nostril
7.5-10 mg/kg
(max dose
200mg)
Blob
Emla cream
Topical
Blob
Secobarbital*
Oral
Quinalbarbitone
Topical
analgesia
Time
before
procedure
30 -60
min
10 min
20 min
30 min
60 min
*This is a controlled drug

Procedure for Pic line or Vygon longline insertion
1. Make sure you have an experienced nurse to help you.
2. Allow the parent to be present if they wish.
3. Prepare a trolley with; Pic line or Vygon longline pack, suitable skin
preparation, 5ml syringes containing heparinarised saline and 0.9%
saline, bung, steristrips and tegaderm or equivalent dressing.
4. Choice of catheter: A vygon neonatal line is recommended for children
under 5 years of age. In children aged 5-12 years use a 2F Vygon Pic
line and for 12 years use a 3F Vygon PIC line.
5. Remove Emla or Ametop.
6. Gown and glove up.
7. Prime the line with 0.9% saline.
8. Apply the tourniqui. Cleanse the site with skin prep.
9. Insert the butterfly and remove stylet.
97
10. If you feel confident and the patient is cooperative take blood samples
from the butterfly with the stylet pulled back prior to removing it
completely.
11. Thread the line through the butterfly using forceps.
12. Flush the line with saline and clamp.
13. Remove the butterfly by snapping the wings and peeling the butterfly in
two.
14. Secure the line with steristrips and tegaderm.
15. Place a bung on the end of the line.
16. Flush first with 5ml 0.9% sodium chloride then with 5ml heparinised
saline.
17. An X-ray is NOT required to confirm line position.
18. Flush 4 hourly with 2ml heparinised saline.
The patient can receive their first dose of antibiotics.
If inserting a Vygon neonatal longline the following points are different:
7. Flush the line with the hub and port assembled then remove the screw on
hub.
9. No stylet to remove.
12 &13. Before flushing line need to remove butterfly by threading back over
the line, then reattach the screw on hub. Line can now be flushed.
Cannulation
If a Pic line or Vygon longline cannot be sited it may be necessary to place a
peripheral cannula. Ideally this should be placed in the non-dominant hand
or antecubital fossa. It should be carefully secured. If a cannula is to be
used the child and parents should be advised that it is likely that the cannula
will need to be resited during the antibiotic course.
10.2 Indications for port-a-cath insertion

Insertion of an indwelling intravenous access device such as a port-a-cath
is indicated when all of the following criteria have been fulfilled:
1. Intravenous access using a long line has been attempted unsuccessfully
due to the excessive distress experienced by the child or because
accessible veins have thrombosed.
2. The child and their parents have been counselled about the risks of
indwelling devices (infection and endocarditis) and are aware that
needling the port-a-cath must be done 4-6 weekly to allow it to be
flushed.
3. The decision for insertion of a port-a-cath is made following discussions
with the multi-disciplinary team.
4. Informed consent has been obtained.
With Hickman and Broviac lines, infection will invariably occur with
prolonged use and these devices are therefore not suitable for long-term
antibiotic administration in patients with CF.
98
Nottingham Childrens and Young Peoples Hospital

Nursing procedure:
P7 Care and Management of Children with A Totally Implantable Venous
Access Device (Portacath).
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Introduction
A Portacath is a central venous access device system that can be used for the
delivery of both I.V. medications and fluids. Once accessed, it can usually be
used for the collection of blood specimens without venepuncture. It is
particularly useful in patients that have poor vascular access, who require
frequent intravenous medication,
for various reasons such as regular long term antibiotics or chemotherapy. A
Portacath must be flushed regularly in order to minimize complications. Once
needled the Portacath can be accessed like any other central venous access
device. However, needling requires additional training and assessment.
Standard statement
A Registered Nurse (Child), who has been appropriately trained, completed
the IV and Portacath Working in New Ways Expansion to Practice Packages,
will be able to safely access a Portacath.
A parent or carer who has been appropriately trained and assessed
competent may access a Portacath that has been inserted in the child in their
care.
Structure
Where possible this procedure should be a two person technique, however,
in the community setting a parent carer of a co-operative child and a
community nurse may do the procedure alone. Needling a portacath is done
using a traditional aseptic technique, however once needled then an aseptic
non-touch technique (ANTT) can be used for all access to the line.
This procedure is carried out in accordance with General Principles for All
Procedures (2006) NUH NHS Trusts and Childrens Services Nursing
procedures F (2007).
10.3 Flushing a portacath (which requires needling using an aseptic

technique)
Portacaths should be routinely flushed every 4-6 weeks.
Equipment
Prescription card/Patient Specific Direction
Tray or trolley
Dressing Pack
Sterile gloves
Chloraprep 2% 3ml (Chlorhexidine gluconate applicator) or Sterexidine
200 if baby under 3 months.
99
Gripper needle of the appropriate size

10mls Sodium Chloride (NaCl) 0.9%
4mls Heparinised Saline (100 units in 1 ml)
Blue needles x 2
10 ml syringes x 2
Alcohol hand rub
Procedure:
ACTION
RATIONALE
1.
Apply local anaesthetic cream if required Minimise pain on needle insertion.
2.
Wash hands and then use alcohol hand

rub. Clean trolley or tray, gel hands and
apply sterile gloves.
Prepare all equipment This includes:
drawing up 10mls sodium chloride 0.9%
and 4mls heparinised saline (100 u/ml)
using an aseptic technique.
Prime gripper needle with NaCl and
close clamp.
Expose and clean around port area with
chloraprep or Sterexidine 200 applicator
in a circular motion, working from inside
out for 30 seconds, and leave to dry
Immobilise port with fingers and insert
needle into port at a 90-degree angle
until the base of the port is felt.
Withdraw a small amount of blood to
ensure correct position and instil 10mls
of sodium chloride, observing for
localised swelling.
3.
4.
5.
6.
7.
8.
9.
To reduce risk of infection (NUH

Hand Hygiene Policy 2008)
To ensure preparation prior to
procedure
To clear dead space and reduce

risk of embolism.
To minimise introduction of infection
and
ensure
effectiveness
of
cleaning agent.
To ensure correct position
To confirm correct position and

patency of portacath 10ml syringe
or larger should be used to prevent
damage to the catheter through
pressure
(Smiths
Medical
International Ltd. 2005)
If in doubt remove the needle
To prevent infiltration of medication
into subcutaneous tissue.
If it is positioned correctly, flush line with To prevent port and line occlusion
4mls of heparinised saline under
positive pressure. Clamp line.
10. Remove needle, pushing down on the

portacath with two fingers.
11. If there is any bleeding, apply clean
piece of gauze or plaster.
12. Dispose of all equipment according to
trust policy. Wash hands.
13. Sign for heparinised saline and sodium
chloride on prescription card and report
any problems encountered to hospital or
community
staff.
Document
as
appropriate.
To minimise risk of damage

To stop the bleeding
Waste management policy (NUHT)
To keep record of medication given
and any problems encountered.
100
IF BLOOD SAMPLING IS REQUIRED:

Extra Equipment needed:
10mls sodium chloride 0.9%
8mls heparinised saline (100u/ml)
6 x 10 ml syringes (will need more if more than 10mls blood required)
Procedure:
ACTION
Follow steps 1-6 in procedure for
needling portacath.
7. Withdraw 3 mls of waste blood. If
unable
to
withdraw
blood
immediately then instil 5mls of
sodium chloride (0.9%) followed by
withdrawing 5mls of waste blood and
discard. Clamp line. Change syringe
and withdraw sample of blood
required. Clamp the line and remove
syringe. Request that assistant
decants
blood
samples
into
appropriate
bottles
and
label
correctly. Send to lab.
8. Flush line with 5mls sodium chloride
0.9% and clamp line using positive
pressure. (Pharmacia 1998).
9. Flush line with 4mls of heparinised
saline under positive pressure and
clamp line.
10. Remove needle, pushing down on
the Portacath with two fingers
If continuing on regular prescribed
IV medication do not remove
needle.
11. If there is any bleeding, apply clean
piece of gauze or plaster.
RATIONALE
Study showed no influence on blood

results if taking 3 mls blood rather
than 5mls. Veal at el (2007)
To ensure specimens are correctly

processed.
To further help disperse blood in the
line.
To prevent port and line occlusion and
create a lock (West 1998).
To minimise risk of damage
To allow IV medication to continue to
be given as prescribed.
To stop the bleeding
12. Dispose of all equipment according

to the trust waste management
policy. Wash hands.
Sign for heparinised saline and To keep record of medication given
sodium chloride on prescription card and any problems encountered.
and report any problems encountered
to hospital or community staff.
Document as appropriate.
101
10.4 Administration of Medication via a Portacath using an ANTT
Equipment
Prescription card
Sodium Chloride 0.9%
4mls Heparin (100 units in 1 ml)
10 ml syringes
Blue needles
Green needles
Tray
Bionector
Alcowipe
Non sterile gloves
Appropriate drugs
Procedure:
ACTION
1.
2.
3.
4.
5.
6.
Wash hands with soap and water

and then use alcohol hand rub.
Clean tray, gel hands and apply
non sterile gloves.
Prepare and check prescribed
medications in accordance with
Trusts Medicines Management
Policy.
If
administering
medications via an infusion pump,
ensure that the line is primed.
Wash hands or use alcohol hand
rub ??change gloves
Clean Bionector cap with alcowipe
and leave to dry.
Attach the syringe and flush with
5mls of sodium chloride 0.9%.
Clamp using positive pressure.
Administer prescribed medications
using positive pressure. Ensure
bolus drugs are given first. Flush
with 5mls of sodium chloride 0.9%
between each drug.
RATIONALE
To minimise risk of infection. NUH
Hand Hygiene policy 2008.
To ensure patients safety.
To minimise cross infection.

To minimise the risk of line sepsis.
To ensure patency of the line and
the positioning of the needle.
To ensure that the medications do
not mix in the line.
102
7.
The tissue surrounding the site

should be monitored throughout
the procedure for:
Redness
Swelling
Discomfort surrounding the port
It is inadvisable to use the port in
these circumstances inform
medical staff and document.
8. On
completion
of
the
administration of medications,
flush with 5mls sodium chloride
0.9% and clamp the line.
9. Attach the syringe containing 4mls
of heparinised saline (100u/ml),
flush and clamp the line using
positive pressure.
10. Dispose
of
all
equipment
according to trust policy. Wash
hands.
11. Sign the drug prescription card to
denote that they have been
administered.
12. Report any problems encountered
and record in nursing notes.
To ensure that appropriate steps

are taken to prevent port infection
and any other problems associated
with the Portacath.
To ensure all medication is infused.
To prevent port and line occlusion.
To maintain an accurate record of

medications given.
To maintain an accurate record of
any change
References
Aitkin ML, Tonelli MR. (2000) Complications of indwelling catheters in cystic
fibrosis: a 10year review Chest ;118:1598-1602
Infusion Nurses Society (2004) Policies And Procedures For Infusion, SASH
Guidelines 2nd Edition, USA.
Nottingham University Hospital NHS Trust (2006a) Nursing Practice
Guidelines. General principles for all guidelines
Nottingham University Hospital NHS Trust (2006b) Childrens Services
Procedure F. Care of Children Undergoing Nursing or Medical Procedures
Pratt R.J. Pellowe, C.M. Wilson, J.A. Loveday, H.P. Harper, P.J. Jones,
S.R.L.J.
McDougall, C. Wilcox, M.H. (2007) Epic2: National Evidence-Based
Guidelines for Preventing Healthcare-Associated Infections in NHS Hospitals
in England. Journal of Hospital Infection 65S, S1S64
Primhak RH (1998) Pressures used to flush central venous catheters,
Archives of Disease in Childhood Fetal & Neonatal edition 78 F234.
Rowley, S. (2001) Aseptic Non-Touch Technique. NTPlus 97 (7) VI-VIII
Smiths Medical International Ltd. (2005) Port-a-cath and Port-a-cath II Vascular access
systems. Instructions for use.
Weiner, E.S. & Albanese, C.T. (1998) Venous Access in Pediatric Patients. Journal of IV
Nursing. 21 (5S) Supplement ppS122-S133
Authors:
Janice Mighten Respiratory Nurse Specialist, Debra Forster
Respiratory Nurse Specialist, Amanda Ward CF Nurse Specialist
103
Section 11 Physiotherapy
(Revised by Helen Holden & Marie Bolton)
104
11.1 Introduction and principles

Chest physiotherapy is an essential part of the management of CF. Airway
Clearance Techniques (ACT) enhances the clearance of excessive bronchial
secretions. By removing obstructive secretions they aim, in the short-term, to
reduce airway obstruction, airway resistance and improve ventilation. In the
long-term chest physiotherapy aims to delay the progression of respiratory
disease and maintain optimal respiratory function, as removal of
mucopurulent secretions may help to reduce the elastase mediated damage
to the airways. Physiotherapy includes aerobic exercise, which increases
sputum clearance and maintains/improves exercise tolerance. In addition,
assessing and treating complications of CF such as urinary incontinence,
musculoskeletal pain and postural abnormalities arising from chronic lung
disease may also be required.
All CF patients and their families/carers should have access to advice from a
senior physiotherapist who has specialised knowledge of CF. It is important
that all patients/carers are seen by an experienced physiotherapist as soon
as is appropriate at diagnosis in order to formulate an effective management
plan, which is suitable for the age, background and disease severity of the
individual. The treatment regimens should be reviewed at regular intervals
through clinic visits, community visits and ward reviews, and adapted
according to their changing needs.
11.2 Airway Clearance Techniques

Different techniques are used for different ages with a gradual change from
the passive treatment of babies to independence in adulthood.
Techniques in pre-school children
All babies with CF commence on a daily programme of ACT. Parents are
taught how to assess for signs of respiratory exacerbations and to increase
treatment frequency as appropriate. Treatment techniques for the first two
years consist of modified positioning/postural drainage and chest percussion.
Parents are also taught to incorporate daily movement positioning/assisted
exercise from diagnosis. Other techniques such as baby PEP and assisted
AAD may be used. From around the age of two blowing games to encourage
and teach deep breathing exercises and directed coughing are introduced
into the treatment session. From the age of three years, the forced expiratory
technique (FET) can be started by practising huffing through peak flow tubes.
At around the age of three to five years the incentive spirometer can be used
(see below). These techniques are then incorporated at a later age into the
Active Cycle of Breathing Techniques (ACBT).
Incentive Spirometer
This is a device that encourages and teaches thoracic expansion exercises/
deep breaths, whilst monitoring flow rate and volume and providing visual
feedback to the patient and carers. It can be incorporated into the ACBT
technique and modified postural drainage. This device can be started with
children from the age of about three to five.
105
Active Cycle of Breathing

This consists of three breathing techniques: Thoracic expansion exercises - to loosen secretions
Breathing control
- to encourage relaxation
Forced expiration technique - to mobilise and clear secretions
This is not a rigid treatment technique but is modified to suit each patient.
The cycle is repeated as often as necessary and is often combined with
modified postural drainage positions, or in conjunction with other treatment
adjuncts.
Positive Expiratory Pressure (PEP Mask / Pari PEP Device)
This technique involves breathing out through the PEP mask, or through a
mouthpiece with the pari-PEP device, against a resistance. PEP therapy
increases intra-bronchial pressure in central and peripheral airways splinting
the airways open and preventing compression induced by airway collapse.
This promotes inflow of air behind mucus obstructions either via a bronchial
route or collateral airway channels. Smaller bronchial airways are prevented
from collapse thus permitting the continuing upward movement of secretions.
This treatment can be performed in the sitting or modified postural drainage
positions. The technique involves deep breathing through the mask followed
by huffing, coughing and breathing control. The correct expiratory pressure
can be measured with a manometer. The PEP mask is usually introduced
from the age of six although using baby PEP with tidal volume breathing is
an emerging technique in the UK.
Oscillating PEP Devices (The Flutter and The Acapella)
The Flutter is a pipe like device containing a large ball bearing. During
expiration through the device, the steel ball initially impedes airflow. As
expiratory pressure increases and exceeds the effect of gravity the ball is
displaced, the pressure is released the ball drops back down. The Acapella
is a plastic hand held device that works similarly but using a plastic arm and
a magnet. These devices combine the effects of PEP, as explained above,
with a cyclic oscillatory and vibratory effect during expiration that is
transmitted through the tracheobronchial tree. This helps to break up and
reduce the viscosity of secretions, so they can be cleared more easily.
A treatment session consists of a series of deep breaths through the device,
usually in sitting, followed by a combination of huffing, coughing and
breathing control. The acapella can be used in any position whereas the
flutter can only be used in sitting. These devices are usually used with the
older children / adolescents as the technique is more difficult to perfect than
using a PEP mask.
Autogenic Drainage
This technique consists of a three phase breathing regimen, which uses high
expiratory flow rates while avoiding airway closure. The utilisation of high
airflow produces shearing forces within the airways that may tear mucus
from bronchial walls. The treatment can be performed in any position, and
involves breathing at different lung volumes, which maximises the expiratory
flow at that particular generation of the bronchial tree. The technique is
106
combined with the forced expiratory technique, coughing and breathing

control. This technique again is used with older children / adolescents as
good self-awareness of breathing control, lung volumes and secretions are
essential.
Intermittent Positive Pressure Breathing (IPPB)
IPPB is a technique used to provide positive pressure throughout inspiration.
The Bird Mark 7 ventilator is the pressure limited ventilator normally used to
provide IPPB triggered by the patients spontaneous effort, and is a useful
adjunct to physiotherapy. Through providing intermittent mechanical
ventilation it has been shown to augment lung expansion, reduce work of
breathing, and assist in the clearance of secretions when more simple airway
clearance techniques alone are not maximally effective. This is only used
during inpatient admissions and the need for IPPB will be based on careful
assessment and consideration by an experienced Physiotherapist, and by
agreement of the Consultant. There is a full hospital guideline on IPPB to
refer to for more information.
Exercise
The importance of regular exercise in maintaining a healthy life style is
recognised in both health and disease. Short-term and now long-term studies
of exercise training programmes or activity in CF have been shown to have
considerable therapeutic benefit. Everyone can exercise: patients with mild
to moderate disease (FEV1 > 55% predicted) can exercise to the same level
as their peers. Those with more severe disease (FEV1 < 55% predicted) will
require careful assessment. Exercise programmes therefore must be tailored
to the individual, based on disease severity, level of fitness and patient
preference. Daily movement positioning/assisted exercise is encouraged
from diagnosis, progressing to more independent aerobic activities from
toddler age to promote compliance and enjoyment of exercise early, and as
an additional means of airway clearance.
11.3 Physiotherapy for Postural and Musculoskeletal Problems

Children with CF are at risk of developing postural problems as a
consequence of lung disease, together with the risk of suffering CF related
arthropathy. From about the age of seven postural screening is performed at
annual assessment. Children and parents are educated about the
importance of good posture to optimise lung expansion and prevent
secondary musculoskeletal problems. Specific postural correction exercises
and thoracic stretches are taught if required. Out-patient musculoskeletal
physiotherapy can be arranged for more complex problems.
11.4 Physiotherapy for Urinary Incontinence

Urinary incontinence is recognised as a common problem in girls with CF,
and although may cause significant problems may not be readily reported.
Screening for this is included in the physiotherapy annual assessment in girls
over the age of 6. If problems are identified advice is provided about self
management during ACT and daily activities, and pelvic floor exercises can
107
be taught. Patients can also be referred to the paediatric specialist urology

nurse.
11.5 Physiotherapy Services- Inpatients

A normal physiotherapy service is provided to the CF patients between the
hours of 8.30am and 4.30pm Monday to Friday. All CF patients are assessed
and an appropriate treatment plan is devised. This is reviewed and adapted
throughout the admission as appropriate, and may include airway clearance
techniques and exercise.
Contact details:
Senior Paediatric CF Physiotherapist: Phone extension 65326/61887
Bleep 7413
Physiotherapy Clinical Specialist:
07771623927
Referral:
An open referral system to physiotherapy operates for the CF patients during
normal working hours, but it is essential that physiotherapy staff are made
aware of an admission.
Weekends:
A weekend physiotherapy service is provided to the CF patients between
8.30am and 4.30pm as part of the paediatric physiotherapy weekend rota. If
patients are admitted over the weekend please bleep the paediatric
physiotherapist on for the weekend via switchboard in working hours to
inform them of the admission.
On call / Emergency physiotherapy:
An emergency service is provided between the hours of 4.30pm and 8.30am
each day. The physiotherapist can be contacted through switchboard. The
out of hours physiotherapy policy applies, which states the criteria for referral
is That the patient would significantly deteriorate or die if not treated. A
Registrar or above must make the emergency call out. Arranged on-call or
late shift physiotherapy, or Nurses to call will be discussed and agreed with
the ward physiotherapist if appropriate.
11.6 Physiotherapy Services - CF Clinic

All patients are assessed by an experienced physiotherapist every CF clinic
where possible. The aims of the service are to assess, review and progress
physiotherapy airway clearance techniques and exercise as appropriate,
provide equipment as required, and to give advice and educate children and
carers as necessary. The physiotherapist will closely liaise with the CF multidisciplinary team and shared care teams as appropriate.
A Physiotherapy Annual Assessment is performed around the time of the
childs birthday in either the Annual Assessment Clinic for non-pseudomonas
patients, or the normal CF clinic for pseudomonas patients. This assessment
will include:
108
A thorough subjective and objective respiratory assessment.

A review and modification of current techniques including demonstration.
A review of current exercise/activity. An exercise test is performed from
the age of six.

Reviewing, supplying and updating equipment as appropriate.
Screening for musculoskeletal and postural problems.
Screening for urinary incontinence.
Advice and education.
11.7 Physiotherapy Services - Community

Visits by the community physiotherapist, the clinical specialist in CF, or a
senior CF physiotherapist are offered and arranged for a variety of reasons.
These include:
Patients receiving IV antibiotic therapy at home.
Newly diagnosed patients.
Routine support for patients with severe lung disease; predominantly
those under review for lung transplantation

Care of the terminally ill.
Social problems e.g. parental illness, poor compliance.
Organising and monitoring the provision of physiotherapy within schools.
Contact details:
Physiotherapy Clinical Specialist: 07771623927
1. CF Trust Clinical Guidelines for the physiotherapy management of CF. 2002.
2. Flume, PA, Robinson K, OSullivan B, Finder JD, Vender FL, Willey-Courand D, White TB,
Marshall BC & The Clinical Practice Guidelines Committee. Cystic Fibrosis Pulmonary
Guidelines: Airway Clearance Therapies. Resp Care 2009; 54; 522-537
3. Main E, Prasad A, Van der Schans C. Conventional chest physiotherapy compared to
other airway clearance techniques for cystic fibrosis. The Cochrane Database of Systematic
Reviews 2005, Issue1. Art. No: CD002011. DOI: 10.1002/14651858. CD002011.pub2
4. Elkins S, Jones A, Van der Schans CP. Positive expiratory pressure physiotherapy for
airway clearance in people with cystic fibrosis. Cochrane Database of Systematic Reviews
2006, Issue 2. Art. No: CD003147. DOI: 10.1002/14651858. CD003147.pub3
5. Morrison L, Agnew L. Oscillating devices for airway clearance in people with cystic
fibrosis. Cochrane Database for Systematic Reviews 2009, Issue 1. Art. No: CD006842.
DOI: 10.1002/14651858. CD006842.pub2
6. Prasad SA, Main E, Dodd ME. Finding consensus on the physiotherapy management of
asymptomatic infants with cystic fibrosis. Paed Pulmonol 2008; 43; 236-244 #
7. Massery M. Musculoskeletal and neuromuscular interventions: a physical approach to
cystic fibrosis. J Royal Soc Med 2005; 98 (Suppl 45): 55-66
8. McVean RJ, Orr A, Webb AK, Bradbury A, Kay L, Phillips E, Dodd ME. Treatment of
urinary incontinence in cystic fibrosis. J Cyst Fibros 2003; 2: 171-176
109
Section 12 - Dornase alfa and Hypertonic saline

(Revised by Marie Bolton, Alan Smyth)
110
Dornase Alfa
12.1 Introduction
The use of DNase in cystic fibrosis is not new. In the 1960s, bovine DNase
was administered in nebulised form. Its use was discontinued after a report
of an allergic reaction with bronchospasm. In recent years recombinant
DNase or Dornase alfa (Pulmozyme, Roche) has become an established
mode of treatment in CF patients.
12.2 Mode of action

Dornase alfa breaks down extracellular DNA. DNA released by neutrophils is
responsible for much of the viscidity of CF sputum. Hence Dornase alfa
allows more effective sputum clearance.

Randomised controlled trials in patients with moderate lung disease have
shown that lung function improves over a 3 month period in patients
receiving Dornase alfa. (FEV1 increases by a mean of 6%).1 Some benefit is
still seen after 2 years of treatment.2 Patients report an improvement in
symptoms and there is a reduction in the need for intravenous antibiotics of
about 30%, which may help offset some of the cost of the drug.1

Dornase alfa 2.5 mg once daily can be administered via a compressor
nebuliser. It can also be administered by an iNeb nebuliser (Respironics) or
an eFlow nebuliser (Pari), which are more modern and efficient nebulisers,
but this is normally only in patients who are already on other nebulised
treatments and will be arranged by the CF team as a separate chamber is
needed. The optimal timing of Dornase alfa in relation to performing
physiotherapy techniques remains uncertain. Studies have shown varied
results but none have shown a significant difference between the time it is
given and lung function. Therefore, advice given to patients is largely based
on pragmatic reasons and individual preference.12 If it is given before
physiotherapy there must be a long enough interval for it to work, for
example one hour, though it has been reported that a much longer time
interval is better.8, 9, 10 Many families therefore find it convenient to give
Dornase before bed or after school. Patients may be switched to alternate
day dosage if there is a positive response after a trial period. If there is
clinical deterioration the dose can be switched back again.3
Dornase is denatured by nebulised antibiotics and hypertonic saline.
Therefore it is important to consider the timings of each nebuliser if a patient
is on multiple treatments. For children on multiple treatments the order
111
should be hypertonic saline before physiotherapy, followed by nebulised

antibiotics.
Then Dornase can be given at a separate time as above, but a minimum of
one hour after nebulised antibiotics.
12.5 Indications
The product is licensed for CF patients with an FVC of >40% who are over 5
years of age. At NUH the following protocol is used when initiating therapy.
The following criteria must all be met:
1. Patient is productive of sputum
2. FEV1 <70% &
3. FVC >40%
4. No bronchodilator response (i.e. bronchodilators do not improve FEV1 to
>70%)
5. Patient has required or is likely to require 4 or more courses of
intravenous antibiotics over a 1year period OR frequent exacerbations
(>8) needing oral or IV treatment.
There is now evidence that dornase alfa may be effective in younger
children. A randomised, placebo controlled trial, which enrolled children of
mean age 8 years (mean FEV1 95% predicted) found significant
improvements in pulmonary function over a 2 year treatment period.4 This
study showed a reduction in the number of pulmonary exacerbations similar
to that seen in older patients. However this benefit must be weighed against
the need for indefinite daily treatment in young children. However, where
there is a need to explore other treatment options in patients with good lung
function, the above requirement for an FEV1 <70% can be waived.

Four weeks trial therapy should be prescribed by the hospital but only when
the GP has been contacted and has agreed to fund the prescription, if the
trial is successful. (Arrange this through the Paediatric Directorate Business
Manager). Contact should be made by phone initially and later confirmed in
writing. FEV1, FVC, weight, and symptomatology are recorded at the start of
treatment.
The patient must be given an appointment for 4 weeks time and the above
assessment then repeated. Any new pulmonary maintenance treatment
initiated should be documented in the annual assessment proforma
(appendix 7). Copies are available in childrens out patients and on the ward.
If an improvement of 10% or more in FEV1 has not been achieved then
the treatment should be stopped. If a child does not improve on Dornase
alfa, that does not preclude a further trial on a future occasion. If possible do
not initiate Dornase alfa treatment when the child is on intravenous
antibiotics. If this is unavoidable, then look for a greater improvement in
FEV1 (e.g. 20%).
112

Few are reported but some patients experience: pharyngitis, hoarseness or
skin rash. Anaphylaxis has not been reported.
Hypertonic saline
12.8 Introduction
Hypertonic saline has been used for many years to induce sputum in children
with a variety of respiratory disorders. It has previously been shown to
improve mucociliary transport in small numbers of patients, although this
effect had been assumed to be short lived. A recent controlled trial of longterm inhaled hypertonic saline has shown however, that there are long term
benefits from its use including reduced pulmonary exacerbations and a
moderate improvement in lung function.5
12.9 Mode of action

Hypertonic saline increases the volume of the airway surface liquid layer by
an osmotic effect which results in improved mucus clearance. This has the
effect of reducing pulmonary exacerbations and producing moderate
improvements in lung function.4

A randomised controlled trial of nebulised hypertonic saline given twice daily
for 48 weeks found it reduced pulmonary exacerbations by 56%.5 The effect
on lung function was less dramatic with a small improvement in FEV1 and
FVC but no difference in the rate of change in lung function. In a randomised
crossover trial with DNase, hypertonic saline was found to have a less
significant effect on lung function6. Its effect on reducing pulmonary
exacerbations was comparable to DNase although this study may have been
inadequately powered and had too short a period of follow-up to adequately
assess this secondary outcome measure.

The brand of hypertonic saline used in NUH is Nebusal 7% (Forest). The
dosage is 4 mls, which is one ampoule, twice daily. It is given via compressor
nebuliser or through an eFlow nebuliser (Pari) or the iNeb (Respironics). If it
is given through an iNeb a larger lilac chamber needs to be ordered from
Respironics, which the patient should then fill twice for each treatment.
Bronchodilators should be given before hypertonic saline as there is a risk of
bronchospasm, and it is always given before physiotherapy. A recent
randomised controlled trial looked at timing of hypertonic saline in relation to
physiotherapy treatment. The time of treatment did not affect lung function
113
measures, but patient satisfaction and perceived efficacy was higher when
hypertonic saline was given before or during physiotherapy techniques.11
12.12 Indications
Children with frequent infective exacerbations. However consider other
alternatives (including dornase alfa and azithromycin).

Most patients will start treatment in hospital, where the response for any
adverse effects can be more closely monitored. It can be started in outpatients but be cautious about using it for the first time in children known to
suffer from bronchospasm. Bronchodilators should be given pre hypertonic
saline and may need to be repeated post physiotherapy. In children known to
be wheezy or those with severe airway obstruction, consideration should be
given to using lower concentrations of saline (3% saline) for the first dose
with the aim to increase to 7% as tolerated. Assessment of response should
be made following a 6 month trial.

Bronchospasm and cough are the most commonly reported side effects,
other reported symptoms are pharyngitis, haemoptysis, sinusitis, vomiting. A
reduction in FEV1 and oxygen saturations is sometimes seen initially.
However, tolerance usually improves with repeated doses.
References
1. Fuchs HJ, Borowitz DS, Christiansen DH, Morris EM, Nash ML, Ramsey
BW et al. Effect of aerosolised recombinant human DNase on
exacerbations of respiratory symptoms and on pulmonary function in
patients with cystic fibrosis. N Engl J Med 1994;331:637-42.
2. Jones AP and Wallis CE. Recombinant human deoxyribonuclease for
cystic fibrosis (Cochrane Review). In: The Cochrane Library (4). 2003.
Chichester, UK, John Willey & Sons,Ltd.
3. Quan JM, Tiddens HAWM, Sy JP, McKenzie SG, Montgomery MD,
Robinson PJ et al. A two-year randomized, placebo-controlled trial of
dornase alfa in young patients with cystic fibrosis with mild lung function
abnormalities. The Journal of Pediatrics 2001;139:813-20.
4. Donaldson SH, Bennett WD, Zeman KL, Knowles MR, Tarran R, Boucher
RC. Mucus Clearance and Lung Function in Cystic Fibrosis with
Hypertonic Saline. N Engl J Med 2006;354:241-50.
5. Elkins MR, Robinson M, Rose BR, Harbour C, Moriarty CP, Marks GB et
al. A Controlled Trial of Long-Term Inhaled Hypertonic Saline in Patients
with Cystic Fibrosis. N Engl J Med 2006;354:229-40.
114
6. Suri R, Metcalfe C, Lees B, Grieve R, Flather M, Normand C et al.

Comparison of hypertonic saline and alternate-day or daily recombinant
human deoxyribonuclease in children with cystic fibrosis: a randomised
trial. Lancet 2001;358:1316-21.
7. Elkins MR, Tingpej P, Moriarty CP, Yozghatlian V, Rose BR, Harbour C et
al. Tolerability of hypertonic saline when delivered rapidly via the eFlow
rapid nebulizer in subjects with cystic fibrosis. Pediatr Pulmonol
2006;S29:292.
8. Fitzgerald DA, Hilton J, Jepson, B, Smith L. A crossover, randomised,
controlled trial of dornase alfa before versus after physiotherapy in cystic
fibrosis. Pediatrics 2005; 116; e549-554.
9. Van der Giessen LJ, Gosselink R, Hop, WCJ, Tiddens HAWM.
Recombinant human DNase nebulisation in children with cystic fibrosis:
before bedtime or after waking up? European Respiratory Journal 2007;
30; 763-767.
10. Wilson CJ, Robbins, LJ, Murphy JM, Chang AB. Is a longer time interval
between recombinant huhuman deoxyribonuclease (dornase alfa) and
chest physiotherapy better? A multi-center, randomised crossover trial.
Paediatric Pulmonology 2007; 42; 1110-1116.
11. Dentice RL, Elkins MR, Bye PT. Adults with cystic fibrosis prefer hypertonic
saline before or during airway clearace techniques: a randomised
crossover trial. Journal of Physiotherapy 2012; 58:33-40.
12. Dentice R, Elkins, M. Timing of dornase alfa inhalation for cystic fibrosis
(Review), The Cochrane Library 2011.
115
Section 13 - Pain & Palliative Care in Cystic Fibrosis

(Revised by Harish Sutrave)
116
3.1 Symptom control in the well child /young person

Pain and discomfort are frequent symptoms in a child with CF and there can
be multiple aetiologies. This is where pain assessment is vital to establish
the location, description and intensity of the pain.
Chest pain may be due to the following;
Pleuritic pain frequently occurs during pulmonary exacerbations
Muscle fatigue due to prolonged coughing
Pulled or torn intercostal muscles
Costochondritis
Spontaneous pneumothorax
Spontaneous rib fracture
Chest wall disease
General pains
Abdominal pain DIOS(Distal Intestinal Obstruction Syndrome)/
chronic/recurrent pancreatitis (see section 8.1)
Oesophageal pain
Maxillo-facial pain due to para-nasal sinusitis
Headaches due to hypercarbia, muscle tension or migraine
Post-operative pain
Hernia repairs
Bowel obstructions
Thoracotomies
Lobectomies
Cholescystectomies
Appendicectomies
Gastrostomy insertion
Port-a-cath insertion
13.2 Pain Relief

The Childrens Pain Team should be involved at an early stage.
Procedural pain
Venepuncture and other painful or uncomfortable procedures are avoided if
at all possible. The Play specialist should be involved with play, distraction,
relaxation and guided imagery techniques. Consider local anaesthetic
creams or Entonox for short procedures. Use long lines/port-a-caths
whenever possible.
Oral analgesia is the route of choice if the child/young person is able to
tolerate this route. The following table is for guidance in selecting analgesia
for children in acute pain. Oramorph is available in 10 mg, 30 mg or 100 mg
dose vials and 100 mg/5ml solutions. Sevredol tablets in strengths of 10, 20
and 50 mgs.
A slow release preparation (e.g. MST) may be used. MST is also available in
sachets (liquid). The total daily dose is the same - divide into 2 daily doses. It
117
is usually easier to establish a childs daily requirement for morphine with 4

hourly dosing and then change to a slow release preparation.
Mild
Moderate
Severe
Pain severity
PARACETAMOL (avoid in liver impairment)
Loading dose 20 - 30 mg/kg THEN 20 mg/kg 6

hourly for up to 48 hours then review dose as
per BNFC
(Maximum daily dose 90 mg/kg up to 4 grams)
Oral
Rectal
Loading dose 30-40 mg/kg THEN 20 mg/kg 6

hourly
(Maximum daily dose 90 mg/kg up to 4 grams)
Intravenous
NO LOADING DOSE 15mg/kg 6 hourly

(Maximum 60mg/kg up to 4 grams)
NSAID (Be aware of contraindications and cautions re renal

impairment, thrombocytopaenia and/or oesophageal varices)
Ibuprofen
5 mg/kg 6 hourly
(Maximum daily dose 20mg/kg up to 2.4
OR
grams)
Diclofenac
1mg/kg 8 hourly
(oral / rectal)
(Maximum daily dose 3mg/kg up to 150mg)
WEAK OPIOID
Codeine
phosphate
(oral / rectal)
1mg/kg 4-6 hourly

(Maximum daily dose 240 mg)
OR
STRONG OPIOID - MORPHINE
Oral
IV Loading
Dose
IV Infusion
2 12 yrs 200 400 mcg/kg/dose x 6

12 18 yrs 10 15 mg x 6
See note re: breakthrough pain
50-100 micrograms/kg
10-30 micrograms/kg/hr
*Refer to NCA/PCA protocols*
Please refer to Pain Team or Pharmacy for further advice.

118
Patient Controlled Analgesia (PCA) programme

Drug: Morphine
Concentration: 1 milligram/kilogram morphine made up to 50 millilitres Normal
Saline = concentration of 20 micrograms/kilogram/millilitre. Maximum limit of
50 milligrams/50 millilitres.
Background infusion: 0.2 millilitres/hour (4 micrograms/kilogram/hour)
Bolus dose: 1 millilitre (20 micrograms/kilogram)
Lockout period: 5 minutes
Nurse Controlled Analgesia (NCA) programme
Drug: Morphine
Concentration: Age over 6 months -1 milligram/kilogram of morphine made up
to
50 millilitres
of
Normal Saline = concentration of 20
micrograms/kilogram/millilitre. Maximum limit of 50 milligrams/50 millilitres
Concentration: Age under 6 months 0.5 milligram/kilogram of morphine
made up to 50 millilitres of Normal Saline = concentration of 10
micrograms/kilogram/millilitre.
Background infusion: 1 millilitre per hour (10/20 micrograms/kilograms/hour)
Bolus dose: 1 millilitre (10/20 micrograms/kilogram/hour)
Lockout period: 30 minutes
Conversion from oral morphine to parenteral morphine
The total oral morphine taken in 24 hours should be converted to an
equivalent total daily dose of IV Morphine. 3mg oral morphine is equivalent
to 1mg parental I/V Morphine.
This is the starting dose only. Some children receiving terminal care
have required doses of over 300 mcg/kg/h IV during their final illness.
Breakthrough pain should be reduced to a minimum. Morphine is the drug of
choice for the relief of pain, dyspnoea and the constant urge to cough, but
should be used with caution as they may further depress respirations.
In most cases it should be given as an intravenous infusion, however the oral
route can be helpful for ambulatory patients. Fentanyl patches may also be
considered if pain is controlled and stable.
There should always be prescribed one sixth of the twenty-four hour dose for
breakthrough pain as necessary. When several doses have been given for
breakthrough pain throughout the day then the twenty-four hour dose should
be increased by the extra doses. The doses given are starting doses only
and may need to be increased several times per day. There is no upper limit
to Morphine. The dose is whatever relieves pain, limited only by the side
effects of respiratory depression and sedation.
Other analgesic techniques that may be of benefit may include TENS
machines, Local Anaesthetic blocks (Pleural blocks, epidural infusions) low
dose
tricyclic
antidepressants
(Amitriptyline)
or
anticonvulsants
(Gaberpentin) for neuropathic pain.
119
13.3 Palliative care

Some children with severe infection and end stage respiratory failure will
suffer from a combination of dyspnoea, pain and anxiety, which can be
extremely distressing. When the child has suffered a long illness, lung function
is severely compromised and there has been a poor response to a good trial
of intravenous antibiotics and physiotherapy, palliative care should be
discussed with the family.
Team communication is an intrinsic part of the decision to change to palliative
care. All team members should feel able to initiate discussion regarding this
decision. Each team member has an important contribution to make and the
views of the team can be helpful in facilitating discussion with the child and
the family. End of life plan (ACT 2004) recommendations must be discussed
with the family (and when appropriate the child as well) by the consultant.
Conclusions of the discussion must be documented clearly in the notes.
Once the issues have been discussed with the child and the family and it has
been agreed that palliative care is appropriate, then the emphasis changes.
The child should no longer have to suffer pain or discomfort in the hope of
achieving long- term improvement. The objectives of treatment are now to
relieve symptoms such as pain and dyspnoea and allay anxiety. The family
may find the involvement of the CF social worker or hospital chaplain
particularly valuable at this stage. The medical team may need to see the
child more frequently not less, but privacy should always be respected.
Some families may wish to care for a dying child at home. This requires
careful liaison between the community paediatric nurse, named nurse,
paediatrician and GP.
Treatment should anticipate symptoms.
13.3.1 Anti-emetics
Where a Morphine infusion is used an anti-emetic may be necessary.
Ondansetron (oral/IV) can be used. Dose for <12 years is 100mcg/kg 8-12
hourly (maximum 4mg). Maximum dose >12 years is 8mg.
OR
IV Cyclizine
Age
1 month6 years
6 years18 years
Dose
0.5 -1 mg/kg/dose
Maximum dose 25mg
0.5 -1mg/kg/dose
Maximum dose 50mg
Frequency
3
3
13.3.2 Physiotherapy / Assisted ventilation

If physiotherapy is to continue, it should be gentle and for the relief of
symptoms. Humidified oxygen may be required.
120
Endotracheal intubation and ventilation is inappropriate. Non-invasive

ventilation may help relieve breathlessness and should be discussed with the
parents and child at an early stage.
13.3.3 Laxatives
Constipation is a common side effect of opiates, and laxatives should always
be prescribed.
13.3.4 Other treatments

Intravenous antibiotics and other drug treatment should be reviewed and
unnecessary treatment stopped after discussion with the family.
13.3.5 Communication
It is essential that there is ongoing discussion between the CF team and
ward staff (nursing and medical). The resuscitation status and changes in
management should be clearly documented in both nursing and medical
notes.
If you are unsure of the management of a dying child you may contact
the consultant, CF nurse specialist or community nurse at any time.
121
Section 14 Psychosocial Support

(Revised by Phil Brewin & Jackie Hooley)
122
14.1 The Importance of Psychosocial Factors in CF

Children with Cystic Fibrosis (CF) and their families are obviously subject to
very significant psychological and social stresses due to the progressive and
usually fatal nature of the disease and its complications, along with the
complex and time-consuming daily treatment regimen. These stresses impact
many aspects of living including school, body image, relationships with peers,
intimate relationships, having children and developing careers. There is also
a huge impact on parents and other family members. The way young people
and their families cope with these stresses is known to influence their
psychological and physical well-being (Abbott, 2003).
In addition to the direct impact of the illness, children with CF and their
families are subject to the same range of problems and stresses common in
those without a chronic illness (e.g. housing, financial, bereavement, bullying).
Psychosocial factors are known to be strongly associated with physical
outcomes of people with CF (e.g. Balmer et al, 2008, Shechter et al, 2009).
The Clinical Psychologist and Social Worker specialising in children with CF
work closely as an integral part of Multi-Disciplinary Team (MDT) for children
and young people, and their families. Together, they will endeavour to provide
a service that meets the CF Trust Standards for the Clinical Care of Children
and Adults with CF in the UK (2011), taking into account the particular needs
both of patients whose full care is provided at the Specialist CF Centre in
Nottingham and those whose care is shared with Network Clinics.
14.2 Specialist CF Psychosocial Provision
Access to clinical psychology and social work services (which are part of the
CF team) is a requirement of the CF Trust Standards (p7).
14.3 Shared / Network Care
Shared care is provided for children at network clinics at Derby Childrens
Hospital; Chesterfield Royal Hospital; Kings Mill Hospital, Mansfield; Lincoln
County Hospital and Pilgrim Hospital, Boston.
Care delivered by Network CF Clinics should be to the same standard as that
delivered by the Specialist CF Centre in Nottingham (CF Trust Standards, p4).
There currently are no Social Workers or Clinical Psychologists specialising in
CF at any of the Network Clinics. Psychosocial care for children and families
at these clinics will therefore be provided as follows.
1. As integral members of the Specialist Centre MDT the specialist
psychologist and social worker will see all patients at least twice a year
as part of shared care clinics (CF Trust Standards, p4). If either is not
available they will liaise with each other and/or other team members to
ensure that any psychosocial issues are followed up.
2. The Psychologist and Social Worker will liaise closely with the Network
MDT and provide care directly, or through local services as
appropriate.
123
14.4 The Role of the Clinical Psychologist

The team Clinical Psychologist is employed by Nottingham University
Hospitals NHS Trust specifically to work with children with CF. The
psychologist will attend most CF clinics and annual assessments in order to
get to know all the families and understand the range of difficulties as well as
successes achieved by many young people. In addition, the psychologist will
meet most children when they are staying as in-patients on the ward. When
young people or their families need or request extra help from the
psychologist this can be arranged either as part of normal clinics or at
separate times.
The psychologist may be able to help with problem where emotional or
behavioural problems are involved, whether or not they are related to having
CF. The psychologist may also become further involved when a child, parent
or member of staff wants to discuss matters with someone who is not directly
involved in the young persons physical care.
Common problems in CF include, fear of medical procedures or needles,
worries about the future, difficulties with school, forgetting, not wanting or
refusing to do all the recommended treatment, not knowing how or if to
explain things to children, problems with brothers or sisters. Children with CF
can of course have the same problems as other children, (e.g. feeling low or
being bullied), but they are sometimes made worse, or more complicated by
having CF.
The psychologist works by listening and talking: to young people, parents and
team members, and can help identify others who can help where necessary.
Contact may be by phone as well as face-to-face.
When a child or young person is known to be worried about a medical
procedure, the psychologist should ideally be involved in preparing the child
and parents for the procedure days or weeks in advance, although it is
recognised this is not always possible.
The Psychologist can be contacted via any member of the team, or via the
Dept of Clinical Psychology: 0115 924 9924 x66165
14.5 Role of the Social Worker and Sherwood Project
Social Workers:
Jackie Hooley (Paediatric CF Clinic)

Ann Dealtry & Angela Mills (Adult CF Clinic)
Contact telephone number: 0115 969 1177

Barnardos Sherwood Project offers support to all families affected by cystic
fibrosis who attend Nottingham University Hospital and the hospitals who
provide shared care (Kings Mill Hospital, Lincoln County Hospital , Pilgrim
Hospital Boston, Chesterfield Royal Hospital, Derby Childrens Hospital)
124
The project attempts to meet the needs of children and families living across
the area, working closely with the health teams in the shared care hospitals.
The project recognises that cystic fibrosis remains a serious condition
despite the many improvements in treatment, and that it can at times
significantly impinge on family life, creating stress and other difficulties.
The project aims to work with families, whenever they feel we can be of
assistance. At all times the familys rights, decisions, and choices will be
respected. Help/support will be offered as often, and for as long, as the
family feel the support is needed. The project worker can see people
wherever they prefer i.e. at home, in hospital (ward or clinic) or at the project
offices.
The social worker will respond to direct requests from families as well as the rest of
the MDT.
Services available:
A range of services and information are provided, and these include:
Advice on rights to welfare benefits ensuring that the family receives
all the benefits they are entitled to.
Obtaining financial assistance for holidays or other items that will
benefit the child/family, and ease stress or improve quality of life.
Liaison with other agencies who may be able to offer help i.e. Social
Services, Family Fund etc.
Liaison with schools/colleges to promote better understanding of
CF.
Advice/support for families and children when their treatment
becomes difficult for them to accept.
Individual work with children in relation to their understanding of
aspects of CF and/or around bereavement, death and dying.
14.6 New Diagnosis

Following diagnosis, the Medical Consultant will inform the CF Social Worker,
who will normally arrange to see the family within the following week.
The Social Worker will:
Inform the family of the Project services and offer emotional and practical
support. If support is accepted this will usually involve a follow-up home
visit to enable the family to talk through their feelings/reactions, and to
reinforce information given at initial meeting. Subsequently home visits are
offered to the family, if they wish this.
125
Give advice to the family about the disability benefits they are eligible to
claim, and assist them in making applications for these.
Ensure that the family have access to up-to-date written/audio information
about CF as appropriate.
Offer contact with other families or adults affected by CF and arrange a
meeting where appropriate.
He/she will advise the family of the New Diagnosis Support Group and give
information regarding meetings.
The Psychologist will meet the family at the first full MDT (or shared care)
clinic to and respond to psychosocial difficulties through consultation with the
rest of the MDT and/or through additional direct contact as appropriate.
14.7 Outpatient Clinics

1. The specialist psychologist and social worker will be available at all CF
MDT clinics (Tuesday afternoons at the time of writing).
2. As core members of the MDT, the psychologist and social worker will
see all patients at least twice a year. (CF Trust Standards, p11)
14.8 Annual Review

The psychologist (or social worker) will see all children at Annual Review (CF
Trust Standards, p 31) and will contribute to the MDT letter to the family and
GP.
14.9 Admissions to hospital

Patients will have access to a clinical psychologist within the CF team when
on the ward. The Social worker will offer practical and emotional support
(particularly during a first admission), and will liaise with medical and nursing
staff where appropriate. (CF Trust Standards, p37)
14.10 Starting nursery education / full-time school

Starting education can create considerable stress and anxiety for a family
with a well child, and this is magnified when the child has a chronic condition.
The social worker and clinical psychologist will offer support to the family as
appropriate and, in conjunction with the rest of the MDT, will give relevant
information to the school to increase their awareness and understanding of
CF.
126
14.11 Transition to Adult Services

Planning for and moving from childrens to adult CF services is a complicated
process for young people, their families and the services themselves, and
successful negotiation of this transition is vital to young peoples physical and
emotional wellbeing. Preparation for this process should begin in early
teenage years, and to some extent from birth.
The psychologist and social worker will work closely with young people, their
parents, the childrens and adult CF teams to prepare, plan and successfully
negotiate the process. They will continue to offer support in the immediate
stages after transition where this is helpful in making the process as positive
for the young person as possible.
14.12 Transplant assessment

When a child/young person reaches the stage when transplant assessment
is being considered as an option, the Social Worker or Psychologist may
undertake some work with the child/young person around their
understanding of the process and their wish (or not) to proceed with
assessment. They will liaise with the transplant centre as well as the rest of
the local team acting as an advocate for the young person and their family.
The decision to go ahead with transplant assessment is a major one,
acknowledging the possibility of death. This has an enormous emotional
impact on the family dynamics, and there is a need to ensure that the child
has space to make an informed choice (dependent on his/her age and
understanding) without feeling that they have to conform to their parents
decision. (This will also be overtly discussed as part of the transplant
assessment).
The social worker and psychologist will offer emotional support to the family
before, during and after the transplant assessment. The social worker will
also offer practical help with travelling costs and accommodation as
appropriate.
14.13 Cessation of active treatment / terminal care phase

The decision to stop active treatment and move to palliative or terminal care
Is likely to create a very emotionally charged situation for the family and
professionals. The length of this period can be very variable, and the family
and the child/young person need to be fully involved in the management of
this phase, if they are to be helped towards living with dying in as positive
and comfortable way as possible. The individual child/young persons and
familys choices and wishes should always be respected, accepting that
everyone copes in different ways, when dealing with dying and death and we
cannot prescribe an individual's coping strategies.
127
Support will be offered to the child/young person, and members of their

family and this will include practical as well as emotional support where
appropriate and necessary.
14.15 Bereavement
Following the death of a child or young person, ongoing support will be offered
to family members, (including siblings) for as long as they feel they require it (up to two years following the death). Over their lifetime, young peoples
families are likely to have developed close relationships with different
members of the team, and this will influence who offers support.
Family members are encouraged to express their grief, in whatever way is
appropriate and acceptable to them, and to recognise that each may grieve
in different ways at different times, and that there is no right or wrong way.
Often expressing grief together is facilitated, by the compiling of a Memory
Book of the childs life, which can be done by family members together.
Siblings may sometimes compile their own Memory Book, which is special to
them, and reinforces the value of their relationship with their dead sibling.
Bereavement support will usually take the form of Home Visits and telephone
contact, or families may on occasions prefer to come to the Sherwood
Project offices.
References
Abbott, J. (2003) Coping with cystic fibrosis. Journal of the Royal Society of
Medicine, 96(supplement 43), 42-50.
Balmer DF, Schall JI, Stallings VA. (2008). Social disadvantage predicts
growth outcomes in preadolescent children with cystic fibrosis. Journal of
Cystic Fibrosis, 7(6), 543-50.
Cystic Fibrosis Trust (2011). Standards for the Clinical Care of Children and
Adults with CF in the UK (2nd Ed).
Schechter MS, McColley SA, Silva S, Haselkorn T, Konstan MW,
Wagener JS; (2009). Association of socioeconomic status with the use of
chronic therapies and healthcare utilization in children with cystic fibrosis.
Journal of Pediatrics, 155(5), 634-639
128
Section 15 Education
(Revised by Julie Overend)
129
15.1 QMC Hospital School
QMC Hospital School Manager Eleanor Tweedie

Ward Teacher: Julie Overend
Contact telephone number 0115 9709753 or internal extension 62348
15.2 Introduction
Children with CF will have the full range of ability and will all benefit from full
involvement in school life and a broad, stimulating and challenging curriculum.
The aim of the hospital school is to:
1. Provide a welcoming, happy and safe environment in which to learn.
2. Help all young people to achieve their full learning potential.
3.Support the needs of all our pupils.
4.Treat all people equally - with dignity, respect, fairness and honesty.
15.3 Statutory Guidance

ry guidance set out in Access to
Education for children and young people with medical needs ref. DfES
0732/2001
All pupils should continue to have access to as much education as their
medical condition allows so they are able to maintain the momentum of
their education and keep up with their studies.
and
Education should be available on the day of admission in recurrent cases.
15.4 School in Hospital

Pupils will attend the hospital school during their admission and will benefit
from one to one tuition.
School in hospital provides routine and normality to the day.
By attending the hospital school pupils will keep up to date with their studies
and be able to integrate smoothly back into their mainstream school.
Pupils will be taught the full range of subjects and will follow an individual
programme, which will mirror as closely as possible the work they would be
doing in their mainstream schools.
The hospital schoolteachers are fully qualified and are employed by
Nottingham City Council.
130
15.5 Going to school on D33

All children of school age who are well enough (as assessed by the
medical
and nursing staff) should attend the hospital school.
open during term times between 9.30am and
3.00pm.
Teaching can take place in the schoolroom or in cubicles or at the
bedside.
guidelines.
The ward teacher will normally contact the mainstream schoolteacher for
all
stays of 7+ days to ensure continuity of education.
The hospital school is a registered exam host centre so exams including
GCSEs, SATs , AS and A2 exams can be completed in hospital.
After a 2-week admission a record of work completed will be sent to the
mainstream school and the parent/carers.
The teachers will supervise the use of internet access during the school
day and may be able to support internet use outside school hours
Siblings may attend the school by arrangement.
Specialist teaching, for example in modern foreign languages, can be
provided.
The schoolroom on E floor may be available for pupils to work in.
15.6 Liaison with Mainstream Schools

The ward teachers work very closely with mainstream colleagues to provide
the best possible education for CF pupils.
Study day - There is an annual study day to which all mainstream schools
with CF pupils are invited. Presentations are given by members of the CF
team to inform, educate and discuss a wide range of topics relating to CF
pupils in mainstream schools
The ward teachers will also support pupils and their families in liaison with the
mainstream school to promote continuity of education and address any
problems that arise.
This may involve:
Monitoring academic progress and attendance at mainstream school.
Attendance at education review meetings
Setting up Home tuition and linking with home tutors
Attendance at social services meetings
Arranging school visits and giving presentations.
Discussions with the SENCO or named teacher
Reviewing statements, individual education plans, and progress
files.
Sharing information with the CF team.
Requesting targets and attainment information.
131
team at clinic should be fed back to the ward teachers or Hospital school
manager.
132
15.7 D33 Hospital Play Specialist Lisa Wright

Contact telephone number 0115 9249924 ex 69033
Introduction
For CF patients on D33, the aim of the play team is
To provide a friendly and relaxed environment
To offer a variety of activities and toys for all ages
To prepare patients for procedures and treatments
o and provide distraction during these procedures
To liaise with the CF team on behalf of the patient
To use play for relaxation and recovery
Why play in hospital?
It is familiar to children
It is relaxing
Play can help children to work through experiences in hospital
It relieves boredom
It promotes positive feelings about being in hospital
Specialist Play Techniques

Preparation - We use dolls, photo albums and books to explain procedures
before they occur.
Distraction Unusual and interesting toys are used to divert the childs
attention away from the procedure, therefore encouraging them to cope
better.
Post Procedural Figures or dolls are used to play out medical procedures in
the same way as preparation play but after the event.
General ward activities on D33 are to
organise daily play and art activities in the playroom or at the bedside
provide play to achieve developmental goals

help children master and cope with anxieties and feelings
use play to prepare children for hospital procedures
support families and siblings
contribute to clinical judgements through their play-based observations
teach the value of play for the sick child
encourage peer group friendships to develop
organise parties and special events
133
APPENDICES
134
Appendix 1: Antenatal Diagnosis Management Plan

(Dr C Gardiner, Consultant Clinical Geneticist)
During pregnancy, the couple may either elect to be tested themselves for CF
mutations or may elect to have invasive prenatal testing. Currently the
Regional DNA laboratory test for the common CF mutations, which account
for over 90% of the mutations in the Mid-Trent region.
Baby has 1 Detectable CF Mutation on invasive Prenatal Testing
1. Family reviewed antenatally by the Clinical Genetics Service and given a
1/6 chance of the baby having CF. Family offered referral to Pediatric CF
Team
2. Letter written to the Consultant Neonatologists so the baby is put in the
Neonatal Alert Book
3. Baby started on flucloxacillin at birth. Baby should not be discharged until
the baby has opened its bowel due to risk of meconium ileus
4. Baby to be referred to Pediatric CF Team for follow up and a sweat test at
6 weeks of age
5. If the sweat test is raised or there are any other features suggestive of CF
the family should be referred to Clinical Genetics to discuss analysis of
further rare CF mutations
1.
2.
3.
4.
Couple do not opt for Invasive Prenatal Testing

Both members of the couple tested for the common CF mutations
One member of the couple is found to be a carrier for a CF mutation
Couple referred to the Clinical Genetics Service
The couple opt for the baby to have CF mutation analysis on a cord blood
sample
5. Letter written to the Consultant Neonatologists so the baby is put in the
Neonatal Alert Book
6. Cord blood sample taken at birth and sent urgently to the Molecular
Genetics Laboratory in EDTA by Pediatricians for CF mutation analysis.
Laboratory telephoned by Pediatricians about sample. Once sample
received by laboratory, analysis can be performed in 3 working days
7. Baby to stay on postnatal ward until results of analysis received. Make
sure baby opens bowel because at risk of meconium ileus.
8. Baby found to be a carrier for a common CF mutations
9. Baby started on flucloxacillin. Baby to be referred to Pediatric CF Team for
follow up and a sweat test at 6 weeks of age
10. If the sweat test is raised or there are any other features suggestive of CF
the family should be referred to Clinical Genetics to discuss analysis of
further rare CF mutations
11. Baby does not have a detectable CF mutation
12. Risk of baby having CF is 4 in 1,000. Baby can be discharged and no
further follow up is required
135
Algorithm for Management of Baby with Echogenic bowel at risk of CF

Baby identified with Echogenic
Focus in Bowel
Couple opt for Invasive Prenatal

Testing
No CF mutation Identified
Detailed US scan for structural

anomalies
Congenital infection screen
involving toxoplasmosis, CMV,
HSV and parvovirus B-19. Ask
about varicella-zoster
exposure
Chromosome analysis
Abnormal
Result.
Implications
discussed
Normal
Results.
Regular
Growth
scans due to
risk of IUGR
Couple do not opt for Invasive

Prenatal Testing
Baby has 2 CF
mutations
Baby has CF
Couple offered
referral to
Paediatric CF
Team
Couple
offered
referral to
Clinical
Genetics
Service
Baby has 1 CF
mutation
Baby has
1/6chance of
being affected
with CF
Couple
offered
referral to
Paediatric
CF Team
No CF Mutation
identified
Baby does not
have CF. No
follow up
required
Couple tested for CF

Mutations
One of both
members of
couple CF
carrier
Baby started on
flucloxacillin at
birth. Baby not
to be
discharged until
bowel open.
Baby to be
referred to
Pediatric CF
Team for follow
up and a sweat
test at 6 weeks
of age
Two CF
Mutations
identified. Baby
to be started on
Flucloxacillin
and referred to
Pediatric CF
Team
No CF
mutations
identified
Letter to
Neonatologists so
baby in Neonatal
alert book.
Cord blood sample
taken at birth and
sent urgently to the
Molecular Genetics
Laboratory in EDTA
by Pediatricians for
CF mutation
analysis. Laboratory
telephoned by
Pediatricians about
sample. Once
sample received by
laboratory, analysis
can be performed in
3 working days
Baby to stay on
postnatal ward until
results of analysis
received. Make sure
baby opens bowel
because at risk of
meconium ileus.
Consider
other
causes of
echogenic
bowel
One CF Mutation
identified. Baby to be
started on Flucloxacillin
and referred to
Pediatric CF Team for
follow up and a sweat
test at 6 weeks of age
136
Appendix 2: Outpatient Clinic
Affix Sticker here
Nottingham University Hospitals

Cystic fibrosis clinic
Date:
School attendance:
Age:
Weight:_____
kg
Centile_______
Height: :_____
cm
Centile_______
Lung Function:
Actual
Predicted
% Predicted
FEV1
FVC
Oxygen saturation ___________%
Symptoms:
Physiotherapy:
Type
Frequency
Regular exercise
Examination:
Nasal polyps
Throat
Ears
Clubbing
Chest
Hyper-expansion
Abdomen
Distension
Asymmetry
Tenderness
Scars
Scars
Portacath
Gastrostomy
Air entry
Ascites
Wheeze
Hepatomegaly
Crackles
Splenomegaly
137
Treatment:
Medication
Name
Strength
Dose/Frequency
Gastrointestinal/Liver
Pancreatic Enzyme
Creon / Pancrease/
Multivitamins
Abidec/ADEK/
Vitamin E
Vitamin D
Vitamin K
Proton Pump inhibitor
Units/kg/day
Nutritional
supplement(s)
Pulmonary
Inhaled Corticosteroid
Short Acting 2 Agonist
Long Acting 2 Agonist
DNAse
Antibiotic: Oral
Nebulised
Others
Endocrine/Others
Changes to treatment:
Annual review (due):
Date of Next clinic review:
Hospital admission required:
Y / N.
If Y
Date
same day semi-elective (within 5 of deciding

that admission is needed)
elective future
date( specify)
Comments:
Microbiology (next week meeting):
Pathogen
Treatment
138
Affix Sticker here
Appendix 3: Clinic letter proforma

Please ask for: Paediatric Cystic Fibrosis Secretary
Ref:
{Click here to enter date}
Date of Clinic:
Cystic Fibrosis Clinic
Department of Paediatrics
E Floor, East Block
QMC Campus
Derby Road
Nottingham
NG7 2UH
0115 924 9924 ext 64041
Fax: 0115 9709763
Minicom: 0115 962 7749
www.ncht.org
{Click here to enter address}
Dear Dr
Re:
Diagnosis:
Treatment:
Recent History:
Growth and Lung Function:
Findings on examination:
Results of sputum or cough swab
Changes in Management:
Date of next appointment
Yours sincerely
Dr
139
Appendix 4: GP Antibiotic Guideline

This Guideline should be sent to all GPs who care for a child with CF.
It should also be given to all parents, for their information, and for
documentation in the event they should need to confirm this
recommendation for their child's management.
Introduction
Children with CF are much more susceptible to chest infections and the infection
is often slow to clear. Penetration of antibiotics through lung secretions is
difficult. Therefore antibiotics are recommended:
Much earlier in an initial infection, than for children without CF, due to the
almost inevitable progression to a bacterial lower respiratory tract infection
(LRTI).
For a longer course than is usual.
In higher doses than usual.
Cough suppressants should not be given.
When should antibiotics be prescribed?
1.
2.
3.
4.
5.
Treat all LRTI's and moderately severe upper respiratory tract infections
(URTI). Viral infections allow bacteria to become invasive in CF.
Treat symptoms of LRTI as signs may not be present e.g. the lung fields
may sound clear.
Treat if a chronic cough increases in frequency and severity, especially if
activity and sleep are affected. Treat if sputum production increases and
becomes more purulent.
Treat Staphylococcus aureus and Haemophilus influenza when they are
isolated on cough swabs or sputum culture irrespective of symptoms.
Recommendations for treatment of Pseudomonas aeruginosa are given
later.
Which Antibiotics Should Be Prescribed?

1.
2.
3.
4.
Send cough swab or sputum sample

Cover recent pathogens isolated from previous cultures
In the absence of recent positive cultures, in a patient who does not have
established P.aeruginosa, cover S.aureus and H.influenzae.
(for prescribing regarding P.aeruginosa, see below)
Consider the patients drug allergies and previous response to treatment.
Treatment of Staphylococcus aureus

Children under 3 years: This organism is common in young children and is
often difficult to eradicate. Prophylactic antibiotics are therefore given
continuously from birth until 3 years of age. If S. aureus is isolated while on
prophylactic antibiotics then the child should be given the treatment dose of
flucloxacillin for 2 weeks (see below). If the repeat cough swab is clear then the
child should restart the prophylactic dose.
140
Prophylactic dose:
Flucloxacillin Age
ORAL
Dose
Neonate
125mg
Frequency
(times daily)
2
1 month to 3 3 years
125mg
Older children: a 2 week course of treatment should be given whenever the

organism is isolated.
First Line:
Flucloxacillin
Age
Dose
Frequency
Duration
(times daily)
ORAL
1 month 25mg/kg
4
2 weeks
18 years
Maximum single dose 1g.
Total daily dose can be given in 3 divided doses if necessary
If S.aureus continues to be isolated when the patient has been prescribed a

course of flucloxacillin first consider adherence to treatment. Then add a second
antibiotic for 2 4 weeks (e.g. fusidic acid or rifampicin). If S.aureus continues
to be isolated then the patient should be discussed with the CF Consultant.
Fusidic
acid
ORAL
Age
1 month
1 year
1 - 5
years
5 - 12
years
Over 12
years &
Adult
Dose
Frequency
(times daily)
Duration
2 - 4 weeks
250mg
500mg
2
weeks
2
weeks
2
weeks
Suspension
(fusidic
acid)
15mg/kg
Tablets
(sodium
fusidate)
4
4
750mg
500mg
3
4
(doubled
(doubled
for severe for severe
infections)
infections)
Suspension contains fusidic acid (250mg in 5ml) and tablets contain sodium
fusidate (250mg per tablet).
750mg fusidic acid is equivalent to 500mg sodium fusidate.
Clarify which salt is required at the time of prescribing.
Rifampicin
Age
Dose
ORAL
1 month
1 year
1 18
years
5 10mg/kg
10mg/kg (Maximum
450mg <50 kg & 600mg
>50kg)
Frequency
(times daily)
2
Duration
2 weeks
2 weeks
141
Treatment of Haemophilus influenzae

This is a frequent cause of exacerbations in school age children. Most forms
found in children with CF are non-encapsulated and so children are not
protected by HiB immunisation. Recommended first line treatment is coamoxiclav (Augmentin) (which will also treat concurrent infection with S.
aureus). Second line is cefaclor which is also suitable for infections with
Moraxella catarrhalis.
Co-amoxiclav Age
(Augmentin)
ORAL
1 month 1
year
1- 6 years
6-12 years
12-18 years
Preparation
Dose
125/31 in 5ml
suspension
125/31 in 5ml
suspension
250/62 in 5ml
suspension
or
500/125
tablet
500/125
tablet
0.5ml/
kg
10ml
Cefaclor
Age
Dose
ORAL
0-1 year
1-7 years
over 7 years
125 mg
250 mg
500 mg
Frequency
(times
daily)
3
Duration
2 weeks
10ml
2 weeks
1 tab
2 weeks
1 tab
2 weeks
2 weeks
Frequency
(times daily)
3
3
3
Duration
2 weeks
2 weeks
2 weeks
Treatment of Mycoplasma or Chlamydia pneumoniae

In children who are penicillin allergic, or where these organisms are suspected
use clarithromycin.
Clarithromycin
ORAL
Weight / Age
Dose
Frequency
(times daily)
< 8kg
7.5mg/kg
2
8 11kg
62.5mg
2
12 19kg
125mg
2
20 29kg
187.5mg
2
30 40kg
250mg
2
12 18 years
250mg
2
Doses can be doubled in severe infections.
Maximum Adult dose 500mg bd
Duration
2 weeks
2 weeks
2 weeks
2 weeks
2 weeks
2 weeks
142
Preventing chronic infection with Pseudomonas aeruginosa

Most patients with CF will eventually develop chronic infection with P.
aeruginosa. Acquisition of P. aeruginosa can be prevented at least temporarily
by treatment with ciprofloxacin orally and nebulised colistin (colomycin).
If a patient, who does not have established infection, isolates P.aeruginosa
this should be discussed with the CF Consultant. The hospital team will
arrange the appropriate treatment. For your information this is detailed
below.
Nebulised colistin can provoke bronchospasm in some patients this usually
occurs immediately after the drug has been given. Salbutamol may be given
prior to nebulised colistin.
The schedule is as follows for asymptomatic children. Children with increased
cough or reduced lung function will probably be admitted to hospital for
intravenous treatment.
Dose
Step 1
Oral
Ciprofloxacin
NEBULISED
1month
years
Colistin
18
Frequency
(times daily)
2
Duration
20mg/kg
3 weeks
(max 750mg)
1 mega unit
2
3 weeks
Also prescribe 1x5ml 0.9%sodium
chloride/dose (use 4ml of this to
reconstitute Colistin vial)
If repeat cough swab/sputum is negative, treatment is stopped. If it is positive

step 2 is prescribed.
Step 2
Age
Dose
ORAL
Ciprofloxacin
NEBULISED
Colistin
1month
18
years
1 month 2
years
2 18 years
20mg/kg
(max 750mg)
1 mega unit
Frequency
(times daily)
2
Duration
3 weeks
3 weeks
2 mega units
3
3 weeks
Also prescribe 1x5ml 0.9% sodium
If repeat cough swab/sputum is negative, treatment is stopped. If it is positive

then step 3 is prescribed.
143

Step 3
Age
Dose
ORAL
Ciprofloxacin
NEBULISED
Colistin
1month 18
years
1 month 2 years
20mg/kg
(max 750mg)
1 mega unit
2 18 years
2 mega units
Frequency
(times
daily)
2
Duration
3
months
3
months
3
months
3
3
Also prescribe 1x5ml 0.9% sodium

Chronic infection is defined as the culture of P.aeruginosa on 2 or more
occasions extending over a 6 month period. If a patient isolates P.aeruginosa
within 6 months of completing step 3 they will be considered as chronically
infected. The patient will then usually be prescribed colistin maintenance
therapy.
Maintenance Therapy:
Colistin
Age
Dose
NEBULISED
Up to 1year
1 - 10 years
> 10 years
0.5 mega units

1 mega unit
2 mega units
Frequency
(times daily)
2
2
2
Duration
Lifelong
Lifelong
Lifelong
Also prescribe 1x5ml 0.9%sodium chloride/dose

Colistin comes as a powder for injection, reconstituted in 4 ml of 0.9% saline.
The hospital team will initiate the prescription and supply the appropriate
nebuliser.
This is an alternative maintenance therapy to colistin if colistin is poorly
tolerated, if there is additional chronic infection with S.aureus or if clinical
progress is unsatisfactory. A decision to commence TOBI is usually made by
the CF Consultant.
Nebulised Tobramycin (Bramitob)
Age
Dose
Over 6 yrs
300 mg
Frequency
(times daily)
2
Nebulised tobramycin is administered in cycles of 28 days followed by a 28 day

break.
144
Treatment of exacerbations in children with chronic P.aeruginosa

infection
Colds and mild exacerbations can be treated with oral ciprofloxacin. More
severe exacerbations will need hospital admission for intravenous treatment.
Ciprofloxacin
Age
Dose
ORAL
1month-18 years
20mg/kg
(maximum
750mg)
Frequency
(times daily)
2
Duration
2 weeks
Burkholderia cepacia complex:

This organism is transmissible between patients and highly resistant to
antibiotics. If a new isolate of this organism is found in one of your patients
or a patient known to carry the organism has a pulmonary exacerbation
please discuss with the CF Team.
Prescription requests following CF Outpatient Clinic
When patients are seen in the CF Clinic they always have a cough swab or
sputum sample taken. The culture results are available on the Monday after
the Tuesday Clinic. When a child has a positive culture a prescription is
arranged on a standard letter, which is sent to you (the child's GP) and to the
parents.
The cough swab/sputum sample should be repeated at the end of the
antibiotic course. A microbiology form is sent to the family with the letter.
They are asked to organise this with children's OPD or the community CF
nurses
Contact Details:
CF Consultant Secretary: 0115 9249924 ext 64041
Fax: 0115 9709763
Children's Outpatients: 0115 924 9924 ext 65292
Janice Mighten:
Debra Forster:
Amanda Ward:
0115 924 9924 ext 62986

0115 924 9924 ext 62501
Mailbox: 924 6414
0115 924 9924 ext 62286
145
Appendix 5: Letter to GP and family re sputum result and antibiotic

required:
Ref: JMB/ARS/TBN
Queens Medical Centre Campus

Family Health Division
Childrens Services
E Floor, East Block
Derby Road
Nottingham
NG7 2UH
Date
Telephone: 0115 924 9924 ext 64041

Fax: 0115 9709763
www.nuh.nhs.uk
Dear Parent
was seen recently in the cystic fibrosis clinic.
A sputum/cough swab specimen has grown the following organism:
We would recommend treatment with the following medication:
at a dose of
weeks.
mg
times daily for
A repeat cough swab/sputum specimen should be taken at the end of the

antibiotic course. A microbiology form is enclosed. Please could you contact
the children's outpatients department to arrange a convenient time for you to
attend. If it will be difficult for you to come to the outpatient clinic could you
please contact the CF nurses who will make alternative arrangements.
Yours sincerely,
Dr
Telephone Numbers:
Children's Outpatients: 0115 924 9924 ext 65292
Janice Mighten:
0115 924 9924 ext 62986
Debra Forster:
0115 924 9924 ext 62501
Mailbox:
924 6414
Amanda Ward:
0115 924 9924 ext 62286
146

Ref: JMB/ARS/TBN
Date
Queens Medical Centre Campus

Family Health Division
Childrens Services
E Floor, East Block
Derby Road
Nottingham
NG7 2UH
Telephone: 0115 9249924 ext: 64041
Fax: 0115 9709763
www.nuh.nhs.uk
Dear Doctor
Your patient as named above was seen recently in the cystic fibrosis clinic. A
sputum/cough swab specimen has grown the following organism:We would recommend treatment with the following medication :at a dose of
mg
times daily for
weeks.
I would be very grateful if you could provide a prescription for this antibiotic. I
have asked the parents to come and pick it up. A repeat cough swab/sputum
specimen should be taken at the end of the antibiotic course. I have asked
the family to arrange this with the Children's Outpatients Department or CF
Community Nursing team.
Yours sincerely,
Dr
147
Appendix 6: Scoring Systems
Northern Chest X ray Score

PA film only required.
Divide each lung into upper and lower zones by a horizontal line from the
middle of the hilum.
SCORE each quadrant on 0 - 4 scale.
Score
Radiological changes
Normal: no CF lung disease evident.
Mild: minimal increase in linear markings and/or

nodular-cystic densities up to 0.5 cm diameter.
2
and/or
lesions.
Moderate:
Severe: prominent increase in linear markings,

profuse nodular-cystic lesions, large areas of
collapse/consolidation.
Very severe: little or no area of normal lung,

dense infiltration.
more pronounced linear markings

more
widespread
nodular-cystic
ADD 0-4 points for overall severity, including acute changes and
complications e.g. enlarged hilar glands, degree of
hyperinflation,
pneumothorax.
TOTAL Max score - 20
148
Shwachman Score: System of clinical evaluation of patients

POINTS
25
20
15
10
05
25
20
15
10
05
25
20
15
10
05
25
20
15
05
General activity
Full normal activity; plays ball, goes to school regularly
Lacks endurance and tires at end of the day; good school
attendance
May rest voluntarily during the day; tires easily after exertion; fair
school attendance.
Home teacher, dyspnoeic after short walk; rests a great deal
Orthopnoeic; confined to bed or chair
Physical examination
Normal; no cough, pulse and respirations normal; clear lungs,
good posture
Resting pulse and respirations normal; rare coughing or clearing
of throat; no clubbing; clear lungs; minimal emphysema
Occasional cough, perhaps in morning on rising; respirations
slightly elevated; mild emphysema; coarse breath sounds; rarely
localised rales; early clubbing
Frequent cough, usually productive; chest retraction; moderate
emphysema; may have chest deformity; rales usually present;
clubbing 2 to 3+
Severe coughing spells; tachypnoea and extensive pulmonary
changes; may show signs of right -sided cardiac failure; clubbing
3 to 4+
Nutrition
Maintains weight and height at above 25th centile; well formed
stools, almost normal; good muscle mass and tone
Weight and height at approximately 15th to 20th centile; stools
slightly normal; fair muscle mass and tone
Weight and height above 3rd centile; stools usually abnormal,
large and poorly formed; very little, if any, abdominal distension;
poor
muscle tone with reduced muscle mass
Weight and height below 3rd centile; poorly formed, bulky, fatty
offensive stools; flabby muscles and reduced mass; abdominal
distension mild to moderate
Malnutrition marked; large protuberant abdomen; rectal
prolapse, large, foul, frequent, fatty movements
X-ray findings
Clear lung fields
Minimal accentuation of bronchovascular markings; early
emphysema
Mild emphysema; widespread areas of atelectasis with
superimposed areas of infection; minimal bronchial ectasia
Extensive changes with pulmonary obstructive phenomena and
infection, lobar atelectasis and bronchiectasis
Grading:
6-100 = Excellent, 71-85 = Good, 56-70 = Mild, 41-55 =
Moderate <40 = Severe
149
Affix Sticker here

Appendix 7
Nottingham Childrens Hospital Cystic Fibrosis Unit

ANNUAL ASSESSMENT
Genotype:
Date:
Age:
CF related diagnoses
Non CF related diagnoses

GROWTH:
Height (cms)_______
Growth Velocity in the last year (cms/year)______
Weight (Kgs) ______
BMI (kg/meter2) _____
Centile_________
Centile_________
Centile_________
Centile_________
LUNG FUNCTION:
Oxygen saturation ___________%
Arrange overnight saturation monitoring, if when well, O2 saturation <93% / FEV1 <50%
predicted/ on home oxygen/ or desaturations with shuttle or step test
Actual (Litres)
Predicted (Litres) % Predicted
Date
Current FEV1
Current FVC
Best FEV1
Best FVC
Worst FEV1
Worst FEVC
Bronchodilator reversibility:
SYMPTOMS REVIEW:
Respiratory Exacerbation?
Y / N
Action:
Cough:
none/ occasional/
daily/ nocturnal/
SOB:
none/ occasional/
exertional/
persistent
Wheeze:
none/ occasional/
exertional/
persistent
Sputum:
Consistency
Quantity
Colour
Frequency: daily/
with physio only/
with infections
Hemoptysis:
none/ streaking/
moderate/
Nasal symptoms:
obstruction/
discharge/
with physiotherapy
massive
history of polyps
150
Gastro-intestinal:
Appetite:
good/ moderate/
poor/ variable
Distension:
none/ occasional/
persistent /
Stools:
___/day
Abdominal pain:
none/ occasional/
recurrent
Type: Normal/ malabsorption/

persistent /
constipation
recurrent
Site: _______
Reflux symptoms: Y/N

Other: (e.g. joints / diabetes / liver)
IV ADMISSIONS:
No. Date
Date
Duration
Indication
Antibiotic 1
Antibiotic 2 Antibiotic 3
HOME IV ANTIBIOTIC TREATMENT

No. Date
Date
Duration
Indication
Antibiotic 1
Antibiotic 2 Antibiotic 3
Total IV antibiotic treatment days in the year: _______

No. of Oral antibiotic courses: _________
Hospital admissions other than for IVs: _______________
MICROBIOLOGY:
Date
Pathogen
Treatment
Does the patient grow Pseudomonas: CHRONIC: Yes / No
INTERMITTENT: Yes / No
If Intermittent what step treatment in treatment: STEP: I / II / III

Does patient grow Staph.aureus:
CHRONIC: Yes / No
Immunistations up to date: Influenza / Pneumoccoccal /
INTERMITTENT: Yes / No
Others
151
TREATMENT:
Medication
Name
Strength
Dose
Frequency
Gastrointestinal/Liver
Pancreatic Enzyme:
Creon / Pancrex /
Micro / 10000 /
Other / Nutrizyme
25000 /40000
Lipase:
Vitamins:
Abidec / Dalivit /
Multivitamins / Other
Vitamin E
Vitamin A
Vitamin D
Vitamin K
Ursodeoxycholic Acid
Acid Suppression
Omeprazole / Ranitidine /
Other:
Nutritional supplement(s):
Oral / NG tube / NJ tube
Gastrostomy / TPN
Pulmonary
Long Term Oral
Quinolone /
Antibiotic(s)
Flucloxacillin /
Cotrimoxazole /
Tetracycline /
Other:
Chronic Macrolide
Azithromycin
Antibiotic
Nebulized Antibiotic
Colomycin /
Promixin /
Tobi /
Bramitob /
Other:
High Dose Ibuprofen
/continued overleaf
152
Medication
Name
Strength
Dose
Frequency
DNAse
Hypertonic Saline
Inhaled Corticosteroid
Beclomethasone /
Budesonide
Flutcasone
Other:
Inhaled Corticosteroid in
combination with a
bronchodilator
Seretide
Symbicort
Short Acting 2 Agonist

Long Acting 2 Agonist
Short Acting Anticholinergic
Long Acting Anticholinergic
Long term Oral Steroid
Leukotriene Modifiers
Antifungals
Nasal Steroid
Home Oxygen Y / N
Non Invasive Ventilation Y / N
Oestrogen / Testosterone
Endocrine / others:
153
MAINTENANCE PULMONARY TREATMENT INTRODUCED / MODIFIED:

Yes / No
If yes, please complete:
Indication
Date Started : Date Reviewed:
Weight
FEV1 in liters ( % Predicted)
FVC in liters ( % Predicted)
Symptoms:
Ongoing wet cough
Sputum production
Minimal
Tablespoon
to pot
Shortness of breath
On exercise
On walking
At rest
Imaging
Number of Exacerbations ( PO Abx)
Number of Exacerbations ( IV Abx)
Side effects
DRUG INTOLERANCE & ALLERGY:

Drug name
Description of problem
Approx. date
154
EXAMINATION:
Nasal polyps
Clubbing:
Throat
Ears
none/ early/ obvious
Chest
Abdomen
Hyper-expansion
Distension
Asymmetry
Tenderness
Scars
Scars
Portacath
Gastrostomy
Air entry
Ascites
Wheeze
Hepatomegaly
Crackles
Splenomegaly
Pubertal Staging (use 4 in 1 chart): not applicable/
stage___________
Other positive findings:
OTHER ISSUES:
Clinic attendances in the past year:
School name:
Missed School:
< 2 weeks/
2 weeks-2 months/
> 2 months
Family composition:
Parents Occupation:
Parents educational level on leaving full time education:
Mother: GCSE / A Level / College / University
Father: GCSE / A Level / College / University
No. of siblings with CF:

Adherence to treatment:
Good
/Average
Smoking:
Mum/
Dad/
Transition: Discussed
/Not discussed
Fertility:
Discussed
/non adherent
both/
young person
/not applicable
/Not discussed
/not applicable
Participation on a Clinical Trial within the last 12 months:

CF Banding:
OTHER SPECIALIST CENTRE CARE:

Transplantation:
Not applicable
/Lung-heart
/Liver
Surgery:
155
MULTIDISCIPLINARY ASSESSMENTS COMPLETED:

Yes
Dietitian
Physiotherapist
Clinical Psychologist
Social Worker
No
WHAT PHYSIO TECHNIQUE IS BEING USED (CAN USE MORE THAN ONE):
Active Cycle of Breathing Techniques
Postural drainage with Active Cycle of Breathing Techniques (+/-clapping)
Postural drainage with clapping
Autogenic Drainage
Assisted Autogenic Drainage
Exercise
Positive Expiratory Pressure (PEP)
High Pressure PEP (HiPEP)
Oscillating PEP (eg. Flutter, acapella)
High frequency chest wall compression (eg. VEST)
Other (specify): .
No formal airway clearance carried out
None of the above
EXERCISE TESTING:
Has an exercise test been carried out since last annual data set?
Yes
No
Unknown
Yes, check one of the following:

Incremental cycle / treadmill ergometry
Submazimal endurance cycle / treadmiss ergometry
Shuttle Test (e.g. Modified Shuttle Walk Test)
Walk Test (e.g. 6 Minute Walk Test)
Step Test (e.g. 3-minute)
Other, specify
Other Pulmonary Related
Urinary incontinence Yes
No
Unknown
Yes
No
Unknown
Postural abnormality Yes
No
Unknown
Faecal incontinence
156
INVESTIGATIONS:
Chest X-ray score (Northern):
_______/20
Schwachman Score:
_______/100
General activity
____/25
Physical examination
____/25
Nutrition
____/25
X-ray findings
____/25
Liver Ultrasound Scan:
Y/N
(If on 2 occasions: raised liver function tests (ALT or GT) /Prolonged PT/ Hepatomegaly)
Date:
DEXA Scan:
Date:
Result: Normal / Abnormal
Action:
Y/N
Result: Normal / Abnormal
Action:
157
BLOOD TESTS:
Test
Result
Hemoglobin
Platelets
WCC
Eosinophils
Prothrombin time
Alkaline phosphatase
ALT
GGT
Bilirubin
Albumin
Creatinine
Na
K
Mg (if IV in last year)
Calcium
Phosphate
Glucose
Glucose tolerance test ( >10 yrs)
Fasting:
Action if abnormal
2 hour:
HbA1C
Vitamin A
Vitamin E
25 hydroxyvitamin D
Aspergillus precipitins
Total IgE
Aspergillus specific IgE
IgA
IgG
IgM
Urine: sugar, protein, blood
158
COMPLICATIONS:
Yes
No
Absence of vas deferens

Allergic Bronchial Pulmonary Aspergillosis (ABPA)
Arthritis
Arthropathy
Asthma
Atypical mycobacteria (requiring treatment)
Bone fracture
Cancer confirmed by histology
CFRD
Chronic Pseudomonas aeruginosa
Chronic Staph Aureus
Cirrhosis with no portal hypertension
Cirrhosis with portal hypertension
Depression
Dist. Int. Obst. Synd. (DIOS)
Fibrosing colonopathy/colonic Strict. (report incidence only)
Gall bladder disease requiring surgery
GERD (Gastro-Esoph. Ref.Dis)
GI bleed requiring hospitalisation (non-variceal)
GI bleed requiring hospitalisation (variceal)
Haemoptysis (massive)
Hearing loss
Hypertension
Kidney stones
Liver disease (non-cirrhotic)
Liver enzymes elevated
Nasal polyps requiring surgery
NTM
Osteopaenia
Osteoporosis
Pancreatitis
Peptic ulcer disease
Pneumothorax requiring chest tube
Port inserted or replaced
Rectal prolpase
Renal failure requiring dialysis
Sinus disease
Cough Fracture
Pulmonary Abscess
Acute chest tightness & wheezing related to medication
since last annual data set
Septicaemia
Haemoptysis
Other (specify)
159
MDT SUMMARY AND ACTION PLAN:
Medical:
Dietetic:
Physiotherapy:
Psychosocial:
Education:
Signature:
Designation:
160
Annual assessment letter proforma:

Ref: JMB/ARS/TBN
{Click here to enter date}
Date of Clinic:
Cystic Fibrosis Clinic
Department of Paediatrics
E Floor, East Block
QMC Campus
Derby Road
Nottingham
NG7 2UH
0115 924 9924 ext 64041
Fax: 0115 9709763
www.nuh.nhs.uk
www.ncht.org
{Click here to enter address}
Dear Parent/Carer
ANNUAL ASSESSMENT
Re:
Growth:
Bacteriology in the last year:
Blood Tests:
Lung Function:
Shwachman score:
X-ray changes:
Treatment:
Findings on Examination:
Other issues discussed:
Summary:
Yours sincerely
Dr
Cc GP
161
Appendix 8: Gastrostomy Care

(Revised by Janice Mighten)
The Mickey Skin level gastrostomy device, with a silicone retain balloon is the
gastrostomy button used for the children with cystic fibrosis in Nottingham at
present.
The child will be seen in the outpatient clinic by the surgeon and specialist
nurse following referral by the CF team. The button sizes range from 14 to 24
Fr diameter and there are various shaft lengths. The length required is
determined by the use of a measuring device during surgery.
NB. Remember to look at the manufacturers guide that is included in each
mickey button pack before using the button for the first time.
Just like any gastrostomy device the Mickey button has associated problems:Leaking This often occurs due to an insufficient amount of water in the
balloon. Each balloon should contain a minimum of 5 mls of water. This
should be checked by the medical or nursing staff (or parent), who are familiar
with the device. Leaking can also occur if the gastrstomy button is not flushed
through with water after use, in particular with overnight feeds. Therefore it is
paramount that the importance of this is stressed to the child and family.
A dressing may be used in the short term for excessive leaking. Generally
dressings are not recommended for long-term use due to the risk of infection.
For further advice please contact Ali Wright, Stoma nurse specialist.
Site Infection: Obvious signs of infection, such as discharging exudate
should be swabbed. The site will require daily cleaning and a dressing should
be applied whilst the site is discharging. Oral antibiotics or topical creams can
be used if appropriate. Creams and lotions that are Vaseline based should
not be used, as these can cause the rubber to perish.
Overgranulation: Gastrostomy sites often have a small amount of
overgranulation tissue. In most cases this is best left alone, in time it will clear
up. If the Mickey button is the correct size and fits close to the skin,
overgranulation is less of a problem. However, if the overgranulation tissue is
excessive, Maxitrol eye ointment applied daily is the initial treatment of choice.
Lyofoam dressing has also been shown to be effective; this can be used in
conjunction with Maxitrol eye ointment.
The use of silver nitrate is not advocated because:
it is painful and traumatic for the child
It is harmful to the surrounding skin if not correctly used.
Silver Nitrate should only be used in the very extreme case where
overgranulated tissue has not responded to other measures. This should be
done by someone experienced with its use.
162
Balloon deflation / gastrostomy dislodgement

For the first six weeks following insertion the child must be seen by the
surgeon on the ward if the button becomes dislodged or falls out.
Each child must have a spare button, which is provided by the home delivery
service Vygon UK. If the button appears loose and no water can be withdrawn
from the valve, this indicates that the balloon has burst. Tape should be
applied over the top of the button, securing it to the skin and allowing the
patency of the shaft to be maintained, until the button can be replaced. If the
button becomes displaced at any time it must be replaced urgently,
otherwise the gastrostomy track will close and the child will have to undergo
surgery to replace it.
(Each button contains a manual giving clear instructions on replacing the
button)
WHO TO CONTACT FOR ADVICE:
1. The CF community nurses: Janice Mighten (ext 62986) or Debra Forster
(ext 62501). The community nurse on call can also be contacted between
8.30am-4.30pm, Monday-Friday for advice.
2. The surgeon on call must be contacted immediately if there are any
problems replacing the button.
163
Appendix 9: Guidelines for unblocking a Portacath

Common problems such as catheter occlusion can occur in children with
central venous access devices such as portacaths (Harris & Maguire 1999).
Consequently resistance when flushing or using a portacath occurs. If this
continues, it generally means that the portacath is blocked.
Occlusion of a portacath occurs for several reasons;
(A) Incorrect or infrequent flushing
(B) Build up of crystallised drug in the portal chamber
(C) Formation of blood clot at the catheter tip, which is often characterised by
sudden catheter occlusion when previous flushes have shown no
resistance
(D) Catheter rupture.
Practical measures can be taken to try and alleviate the problem for example;
Ensure that the needle is sited correctly; check clamps if necessary

remove the needle and replace with a new one.
Ask the patient to change position or move upper part of body and
straighten arms, to relieve any kinking of the catheter.
Ask the patient to cough or take deep breaths, as the catheter tip may be
lodged against the wall of a blood vessel.
Following the above if the problem continues then it is likely that the
catheter is occluded with a growth of thrombus around the tip
A linogram can be requested by the Doctors, if the line is blocked and not
flushing back, as there could also be a fractured line. Once occlusion of the
catheter is confirmed by a linogram, then the use of urokinase should be
considered. The use of thrombolytic drugs such as urokinase has
demonstrated a cost effective method of restoring central venous catheters
without interventions such as surgery (Wachs 1990).
To proceed with urokinase follow steps below

Unblocking a Portacath using Urokinase
(Amended July 2008)
Step 1
Irrigate and aspirate the port with 2mls of heparinised saline (100units/ml).
Use a 10ml syringe. This may be repeated using 5mls of heparinised saline
(100units/ml). The to and fro action may clear the occlusion.
Step 2
Reconstitute one vial of Urokinase to prepare a solution of 5000 units per ml.
Use water for injection.
164
Step 3
Using a strict sterile technique and a 1ml or 2ml syringe slowly inject 1ml of
the Urokinase solution.
Wait at least one hour, preferably 2 hours, and then attempt to aspirate the
clot.
This step can be repeated if blockage remains.
Step 4
If still blocked, reconstitute one vial of Urokinase to prepare a solution of
25,000 units per ml. Use water for injection.
Using a strict sterile technique and a 1ml or 2ml syringe slowly inject 1ml of
the Urokinase solution.
Leave for 24 hours, attempt to aspirate the clot and flush as per normal
procedure.
If remains blocked, step 3 may be repeated. However, referral to the surgeons
is necessary at this point.
A Urokinase infusion is only used in those patients with normal coagulation
and platelet function. It is not suitable for lines which are completely blocked
to a manual flush. This is a Consultant decision.
Once unblocked it may be necessary to flush the portacath with 1ml of
Urokinase, 25,000 units per ml every 4-6weeks with the routine flush for 3
months.
References;
1.
2.
3.
4.
5.
6.
Babu R & Spicer RD (2002) Implanted Vascular access devices (Ports) in children:
Complications & their prevention, Pediatric Surgical International 18 50-53.
Duncan-Skingle (2005) Procedures & Practice of intravenous cannulation: A Guide
for nurses, Royal Brompton Hospital London.
Harris JL, & Maguire D (1999) Developing a protocol to prevent and treat pediatric
Central Venous Catheter occlusions, Journal of intravenous nursing vol 22 No 4
July/August p 194
St James & Seacroft (2003) A method of managing problems with Totally implantable
access devices (TIVAD appendix 11)
CF in children & adults; The Leeds method of management.
Wach T (1990) Urokinase administration in pediatric patients with occluded central
venous catheters, Journal of Intravenous Nursing Vol 13 No 2.
165
Appendix 10: Paediatric guidelines for once daily tobramycin in cystic

fibrosis
There is now robust evidence that aminoglycoside antibiotics, such as
tobramycin, should be given once daily, as this is less nephrotoxic1;2. The
following guideline is for use with children with cystic fibrosis.
Dosage
10 mg/kg given as a 30 minute infusion made up to 30mls with 0.9% saline.
Maximum dose 660mg. Round down dose to nearest 10 mg for younger
children or 20mg for children > 20kg.
Current timing for Tobramycin levels and renal function
Renal function should be performed before the 1st dose of tobramycin and
again before the 8th dose. Trough tobramycin levels are measured before the
2nd and 8th doses of tobramycin. All bloods should be venous.
Target levels
Trough level < 1mg/L
If the initial renal function is normal, do not wait for the trough level to come
back before giving the next dose of tobramycin.
Patients receiving
intravenous tobramycin at home may need to come to the ward for the blood
tests. This should be discussed with the Community CF nurses and a plan
made prior to the patients discharge.
Suitability of patients
Some patients who are already on an extended regimen for tobramycin (e.g.
twice daily), are not suitable for once daily tobramycin. Patients who have
impaired renal function or hearing may not be suitable for tobramycin on any
regimen and these cases should be discussed individually with the consultant
in charge.
Interpreting tobramycin levels
A raised trough level is an indication for stopping tobramycin. Then check
renal function and send a further (random) tobramycin level. Raised trough
levels should occur very infrequently. If the renal function is normal and the
repeat level very low, the raised trough may be an error. If the repeat level is
still raised, continue to monitor until it is <1mg/L.
References
(1) Smyth A, Tan KH, Hyman-Taylor P, Mulheran M, Lewis S, Stableforth D et al. Once
versus three-times daily regimens of tobramycin treatment for pulmonary exacerbations
of cystic fibrosis--the TOPIC study: a randomised controlled trial. Lancet 2005;
365(9459):573-578.
(2) Smyth A, Bhatt J. Once daily versus multiple daily dosing with intravenous
aminoglycosides for cystic fibrosis. The Cochrane Database of Syst Rev 2000; Issue 4.
Art. No.: CD002009. DOI: 10.1002/14651858.CD002009.pub2. (updated 2009).
166
Appendix 11: Prescribing nebulisers on the ward

Points to consider when prescribing bronchodilators:
Bronchodilators should be administered via MDI and spacer whenever

possible.
Bronchodilators are prescribed to dilate the airways, therefore if required
are generally used prior to airway clearance techniques/physiotherapy.
When prescribing establish with the patient and/or physiotherapist the
approximate time and frequency of physio whilst in hospital. When
prescribed on a nebuliser chart ensure that the additional information box
is filled in with the instruction pre physio.
Points to consider when prescribing dornase alfa (Dnase):
Dornase alfa is a drug that assists with reducing the viscosity of

secretions. Therefore it should be given at least 2 hours prior to any
airway clearance/physiotherapy.
Dornase alfa will be prescribed at an individual time to suit the patient,

therefore please establish with the patient their usual time of having
dornase alfa. Ensuring that this corresponds to hospital physiotherapy
times.
N.B dornase alfa and nebulised antibiotics should be given with a minimal
interval of 30 minutes.
Points to consider when prescribing nebulised antibiotics:
Nebulised antibiotics are prescribed as part of eradication therapy of

Pseudomonas aeruginosa or if established to reduce the bacterial load.
Nebulised antibiotics are now rarely given when a child is an inpatient.
Nebulised antibiotics should be given after physio when the chest is as

clear as possible. Ensure that the additional information box is filled in
with the instruction post physio and vented out of the window.
167
Appendix 12 (a)
Paediatric Bronchoscopy record sheet
Age:
Weight:_______ Kg
Patient Sticker
Date of procedure:
Date:
Bronchoscopist:
Indication: (Please circle the appropriate indication)
Stridor
Persistent wheeze
Persistent cough
Haemoptysis
Persistent lobar collapse
Selective bronchography
Cystic fibrosis
Difficult intubation
Tracheostomy assessment
?H type fistula
?Opportunistic pneumonia
?Interstitial lung disease
?Foreign body
Other (specify)
(in conjunction with rigid bronchoscopy)
Bronchoscope size:
2.2mm
Airway:
Laryngeal mask
Tracheostomy
Size: 1
2
3
3.5mm
Endotracheal tube
5.0mm
Size:
Chest Xray / CT appearance:

Bronchoscopy findings:
Procedures performed:
Procedure
Result
Suction
Bronchoalveolar Volume of saline used
________ mls
lavage
No .of aliquots
Returns: 1st aliquot (bronchial )
________ mls
nd
2 aliquot
________ mls
3rd aliquot (pool 2 and 3 ________mls
alveolar)
Samples sent
M,C&S
for
Virology
Mycobacterium TB and NonTB
mycobacterium
Lipid Laden Macrophage index (not all
children will have LLMI performed)
Quantitative differential cytospin
Staining for Pneumocystis carinii
Complications:
Desaturation
Y/N
If Y, Duration_______&
level_________
Laryngo/Bronchospasm
Y/N
Coughing
Y/N
Bleeding
Y/N
0
PostBronchoscopy fever(>38 c)
Y/N
If Y, Temp._____Duration________
Others
168
Appendix 12 (b)
NAME
HOSPITAL
NUMBER
DATE
LIST
4.4mm
BRONCHOSCOPE
(Model: GIF XP260N
Use Sony stack

(1 trolley)
3.6mm
BRONCHOSCOPE
(Model: BF 3C40
Please ensure correct

camera is available
and suction port
connectors and caps
are available
Use Evis Lucera

stack (2 trolleys)
5.9mm
BRONCHOSCOPE
(Model: BF-6C260
Use with the Evis
Lucera stacks
(2 trolleys)
Please ensure
correct camera is
available and suction
port connectors and
caps are available
BOOKING FORM FOR BRONCHOSCOPES
THEATRE
2.8mm
BRONCHOSCOPE
XP260F
Use with the Evis Lucera
stacks (2 trolleys)
camera is available and
suction port connectors
and caps are available

camera is available
and suction port
connectors and caps
are available
169
Appendix 13 (a)
Nottingham Cystic Fibrosis Transition

Clinic
Individual Transition Folder

(Boys)
170
Section 1, Patient details:

Name:
Date of Birth:
First Transition attendance/age:
Chronic sputum organism:
Significant CF related complications (with date):

CFRD
M. Abscessus
Enteric feed
NIV
ABPA
Treatment concordance
Good
Port: Yes
Moderate
Poor
No
Current IV frequency:
/year.
171
Patient trajectory.
1: Lung function.
FEV1 (%predicted)
120
-24
-18
-12
-6
95
70
45
12
18
24
Time vs First transition (months)
2: Nutrition.
172
Section 2, Transition topics.

1: Independence/self-sufficiency.
Assessment of involvement with self-management at
entry.
Targets for development (early to late transition).

o Able to correctly list current treatment .
o Asks questions in clinic .
o Confident to be seen on own in clinic .
o Obtains own prescriptions .
o Knows how to book clinic .
o Does treatment without assistance .
o Travels to clinic independently .
Assessment of CF knowledge.
o Apply questionnaire (??Modified Quitner??)
o Address knowledge gaps with targeted
education .
Topics covered
173
o Happy to openly discuss concordance .

o Makes informed choices re treatment agreed
with the team .
o Has visited the adult clinic and ward .
2: Sexual Health and Fertility.
Topics to be covered (early to late transition).
Puberty and growth .
Fertility and Contraception.
o Aware of gender specific CF fertility .
o Assessment of sexual activity and
contraception .
o Aware of safe sex/STDs .
Pregnancy.
o Knows effects of pregnancy on CF
health
(girls) .
o Aware of ICSI (boys) .
o Knows about genetics of CF and implications for
partner screening
3: Psychological wellbeing and Lifestyle.
Topics to be covered (early to late transition)
Family structure/support.
174
Assessment of psychological wellbeing and support.

o Confident to discuss their mood .
o Aware of how to seek help .
Areas of concern/interventions:
Use of street drugs, alcohol and smoking .

Education re exercise/diet/independent living
skills .
Review of relevant benefits/allowances .
Review of financial wellbeing .
4: Education and Employment
Topics to cover (early to late transition).
Review of current school/work attendance/progress.
o School Work: Part time Full time
Apprentice/work based training .
Able to discuss plans for further education or work
place development .
Specific career planning with CF impact taken into
account .
5: Getting to know the adult team
Meet all members of the MDT with paeds opposite
number (+date).
o Physiotherapist(s) .
o Dietician .
o Specialist nurses .
o Social workers
175
Visit to the adult wards/outpatient facilities .

Knows how to contact the Adult team .
Final transition plan.
Agreed first Adult clinic date.
(Ensure date is for an AAC clinic)
Transfer letter completed .

Other important information.
Please use the following links from the CF Trust website

for factsheets on transition from paediatric to adult clinics
and factsheet for parents
http://www.cftrust.org.uk/aboutcf/publications/factsheets/Transition__Young_People_-_Sept_2010_-_web.pdf
http://www.cftrust.org.uk/aboutcf/publications/factsheets/Factsheet__Transition_Parents-WEB-sept_2010.pdf
176
Appendix 13 (b)
Nottingham Cystic Fibrosis Transition

Clinic
Individual Transition Folder

(Girls)
177
Section 1, Patient details:

Name:
Date of Birth:
First Transition attendance/age:
Chronic sputum organism:
Significant CF related complications (with date):

CFRD
M. Abscessus
Enteric feed
NIV
ABPA
Treatment concordance
Good
Port: Yes
Moderate
Poor
No
Current IV frequency:
/year.
178
Patient trajectory.
1: Lung function.
FEV1 (%predicted)
120
-24
-18
-12
-6
95
70
45
12
18
24
Time vs First transition (months)
2: Nutrition.
179
Section 2, Transition topics.

1: Independence/self-sufficiency.
Assessment of involvement with self-management at
entry.
Targets for development (early to late transition).

o Able to correctly list current treatment .
o Asks questions in clinic .
o Confident to be seen on own in clinic .
o Obtains own prescriptions .
o Knows how to book clinic .
o Does treatment without assistance .
o Travels to clinic independently .
Assessment of CF knowledge.
o Apply questionnaire (??Modified Quitner??)
o Address knowledge gaps with targeted
education .
Topics covered
o Happy to openly discuss concordance .

o Makes informed choices re treatment agreed
with the team .
o Has visited the adult clinic and ward .
180
2: Sexual Health and Fertility.

Topics to be covered (early to late transition).
Puberty and growth .
Fertility and Contraception.
o Aware of gender specific CF fertility .
o Assessment of sexual activity and
contraception .
o Aware of safe sex/STDs .
Pregnancy.
o Knows effects of pregnancy on CF
health (girls) .
o Aware of ICSI (boys) .
o Knows about genetics of CF and implications for
partner screening
3: Psychological wellbeing and Lifestyle.

Topics to be covered (early to late transition)
Family structure/support.
Assessment of psychological wellbeing and support.

o Confident to discuss their mood .
o Aware of how to seek help .
181
Areas of concern/interventions:
Use of street drugs, alcohol and smoking .

Education re exercise/diet/independent living
skills .
Review of relevant benefits/allowances .
Review of financial wellbeing .
4: Education and Employment
Topics to cover (early to late transition).
Review of current school/work attendance/progress.
o School Work: Part time Full time
Apprentice/work based training .
Able to discuss plans for further education or work
place development .
Specific career planning with CF impact taken into
account
5: Getting to know the adult team
Meet all members of the MDT with paeds opposite
number (+date).
o Physiotherapist(s) .
o Dietician .
o Specialist nurses .
o Social workers .
Visit to the adult wards/outpatient facilities .
Knows how to contact the Adult team .
182
Final transition plan.

Agreed first Adult clinic date.
(Ensure date is for an AAC clinic)
Transfer letter completed .

Other important information.
Please use the following links from the CF Trust website

for factsheets on transition from paediatric to adult clinics
and factsheet for parents
http://www.cftrust.org.uk/aboutcf/publications/factsheets/Transition__Young_People_-_Sept_2010_-_web.pdf
http://www.cftrust.org.uk/aboutcf/publications/factsheets/Factsheet__Transition_Parents-WEB-sept_2010.pdf
183
Appendix 14: CF Formulary (reviewed by Antony Mazzei 2012)

DRUG
ROUTE
AGE/WEIGHT
DOSE
FREQUENCY
(Times daily)
MAX
DOSE
Abidec
PO
Birth - 3 years
4 - 10 years
PO
1
1
1
1.2ml
Alendronic
acid
Alendronate
0.6ml
0.6 - 1.2ml
Adult dose 10mg
daily or 70mg
weekly
10mg/kg
500mg
3 weeks
250mg
500mg
100mcg/kg
1mg/kg/day
1mg/kg/day
3mg/kg/day
2
2
1 dose
1
1
1
max test
dose 1mg
max dose
3mg/kg
1 dose
250mg
500mg
3 times a week
e.g.mon,wed,fri
125mg
250mg
500mg
50 -60mg/kg
50mg/kg
20mg/kg
3
3
3
3 or 4
3
2
1.5 g
3g tds
750mg
2 weeks
2 weeks
2 weeks
Steps 1 &
2 for 3
weeks
Step 3 for
3 months
3 weeks
(+ forms
available)
DUDURATION
continuou
s
continuou
s
10mg
(5mg, 10mg &

70mg tablets)
-see section
8.4
Amikacin
IV
Amikacin
Nebulised
Amphoteracin
(liposomal)
(important
see section
5.5)
Azithromycin
IV
PO
200mg/5ml
250mg tabs
500mg tabs
Cefaclor
PO
125mg/5ml
250mg/5ml
Cefuroxime
Ceftazidime
Ciprofloxacin
(treatment of
exacerbation)
IV
IV
PO
1 month- 18
years
<12 years
>12 years
Test dose
Start
Increase by
Maximum dose
Above 6 years
&
< 40kg
> 40kg
0 - 1 year
1 - 7 years
> 7 years
1month 18
yrs
3 weeks
Initial 6
month
period
2 weeks
250mg/5ml
250mg tabs
500mg tabs
Ciprofloxacin
(eradication
therapy see
section 5.2.4)
PO
1month 18
yrs
20mg/kg
750mg
Clarithromycin
IV
7.5mg/kg
500mg
Clarithromycin
PO
1 month- 18
years
< 8kg
8 - 11 kgs
12 - 19 kgs
20 - 29 kgs
30 - 40 kgs
12 - 18 years
7.5mg/kg
62.5mg
125mg
187.5mg
250mg
250mg
Doses can be
doubled in severe
infection
125/31 0.5ml/ kg
125/31 susp 10ml
250/62 susp 10ml
Or 500/125
tablets 1 tab
500/125 tablets 1 tab
2
2
2
2
2
2
125mg/5ml
250mg/5ml
250mg tabs
500mg tabs
Co-amoxiclav
125/31 liquid
250/62 liquid
250/125 tablet
500/125 tablet
PO
1 month - 1year
1 - 6 years
6 - 12 years
12 - 18 years
2 weeks
500mg bd
3
3
3
3
2 weeks
3
500/125
184
DRUG
ROUTE
Colistin
IV
Colistin
(maintenance)
Nebulised
Colistin
(eradication
therapy see
section 5.2.4)
Nebulised
AGE/WEIGHT
DOSE
25 000 units/kg
FREQUENCY
(Times daily)
3
MAX
DOSE
2
megaunits
DURATION
0 - 1 year
1 - 10 years
> 10 years
Step 1
0.5 mega units

1 mega unit
2 mega units
1 mega unit
2
2
2
2
continuous
Step 2
1 month - 2 yrs
2 yrs - 18 years
1 mega unit
2 mega units
3
3
3 weeks
Step 3
1 month - 2 yrs
2 yrs - 18 years
1 mega unit
2 mega units
3
3
3 months
2 weeks
3 weeks
Creon (see
section 9.4)
Cyclizine
IV
1 month- 6 yrs
0.5-1mg/kg
25mg
Diclofenac
PO/ Rectal
6 yrs -18 years

> 6 months
0.5-1mg/kg
0.3-1mg/kg
3
3
50mg
Max daily
dose
150mg
2.5mg
1 (in evening)
Continuous
125mg
Prophylactic
Birth - 3 yrs
25mg tabs
50mg tabs
(dispersible)
Dornase alfa
Nebulised
Flucloxacillin
(prophylactic
dose)
PO
Birth - 3 years
Flucloxacillin
PO
1 month 18 yrs 25mg/kg
1g qds
2 weeks
Flucloxacillin
IV
2g qds
2 weeks
Fosfomycin
IV
1 month 18 yrs 25mg/kg

(Dose can be
doubled to 50
mg/kg in severe
infection)
1-12years
100mg/kg
(10-40kg)
> 12 years
5g
2-3
PO
1 month- 1 year
1-5 years
5-12 years
>12 years
3
3
3
3
PO
<10 kg
15mg/kg
250mg
500mg
750mg (Equivalent
to 500mg sodium
fusidate tablets)
25ml in 100 ml
water/ juice
50ml in 100 ml
water/ juice
100ml in 400 ml
water/ juice
125mg/5ml
250mg/5ml
250mg caps
500mg caps
Fusidic acid
(see section
5.2.1)
250mg/5ml
250mg tabs
Gastrograffin
< 25 kg
> 25 kg
(Total daily
dose can
be
increased
to 20g)
2-4 weeks
2-4 weeks
2-4 weeks
2-4 weeks
Single dose
Single dose
Single dose
185
DRUG
ROUTE
AGE/WEIGHT
DOSE
Healthy Start
Childrens
Vitamin Drops
Heparin flushes
Imipenem
PO
< 4 weeks
> 4 weeks 4
yrs
See sections
1 month 18
years
5 drops
10 drops
PO
Lactulose
PO
Linezolid
IV/PO
Insulin
(see section
8.6.5)
Itraconazole
IV
IV
50mg/5ml
100mg caps
100mg/5ml
600mg tabs
Meropenem
Midazolam
Sedation for
procedure (see
section 10.1)
Morphine
(see section 14)
Morphine
(see section 14)
Movicol
Paediatric Plain
sachets
Octreotide
(see section 8.3)
Contact
pharmacy for
advice on
dilution
Omeprazole
10mg, 20mg &
40mg caps
or
10mg, 20mg
MUPS dispersible
tablets
FREQUENCY
(Times daily)
MAX
DOSE
5.8 or 10.3
25mg/kg
1g qds
< 12 years
> 12 years
3-5mg/kg
200-400mg
1
1
< 7 years
> 7years
< 12 years
> 12 years
10ml
20ml
10mg/kg
600mg
2
2
3
2
40mg/kg
IV
<1g bolus
>1g 15-30
mins infusion
PO
Intra-nasal
PO
2 - 12 years
12 - 18 years
IV infusion
PO
<6 years
>6 years
IV
PO
1month - 2
years
Weight 10-20kg
Weight > 20kg
DURATION
3-6 months
600mg bd
10 - 14 days
2g tds
2 weeks
500mcg/kg
200-300mcg/kg
half dose each
nostril
200-400mcg/kg
10-15mg
Loading dose
50-100 mcg/kg
then
10-30mcg/kg/hr
1 sachet
2 sachets
1 dose
1 dose
20mg
0.5-1mcg/kg/hr
(increasing to
5mcg/kg/hr if
necessary)
Continuous
infusion
700mcg/kg
increase to
3mg/kg if
necessary
20mg
10mg
increase to
20mg if
necessary
20mg
20mg
increase to
40mg if
necessary
40mg
6
6
1
1
4 sachets
4 sachets
186
DRUG
ROUTE
AGE/WEIGHT
DOSE
PiperacillinTazobactam
(Tazocin)
Prednisolone
(treatment of
ABPA, see
section 7.1)
Rifampicin
IV
1 month -12
years
12 18 years
90mg/kg
FREQUENCY
(Times daily)
3 or 4
2.25 4.5g
0.5-1mg/kg
0.5-1mg/kg
3 or 4
1
alternate days
2 weeks
2 - 3 weeks
2 - 3 months
1 month - 1
year
5 - 10mg/kg
2 weeks
10mg/kg
Nebulised
100-400mcg (14 puffs)

2.5-5mg
PO
7.5-10mg/kg
Single dose
PO
PO
100mg/5ml
150mg caps
300mg caps
Salbutamol
Secobarbital
Quinalbarbitone
1 - 18 years
Inhaled
MAX
DOSE
4.5g
DURATION
2 weeks
450mg if
<50 kg
600mg if
>50kg
2 weeks
single dose
200mg
Sodium Chloride
< 1 year
* 10-12 mmols
Give in divided
In hot
(Slow sodium
doses
weather
tablets contain
1 - 7 years
* 20 mmols
10 mmols Na,
(2 tablets)
1 dioralyte
sachet has 12
> 7 years
40-60 mmols
mmols Na)
(4-6 tablets)
* Pharmacy can make up a solution(30% - 5mmol/ml) for children unable to take tablets but this takes time to prepare
and has a 28 day shelf life. Dioralyte sachets are an alternative and also contain 4 mmols of potassium which may be
beneficial.
Teicoplanin
IV
Loading dose
Continue on
10mg/kg
10mg/kg
2
1
400mg
400mg
X 3 doses
2 weeks
Tigecycline
IV
Over 12 years
1mg/kg
50mg
3 weeks
Tobramycin
IV (30 min
*10mg/kg
1
Max
(drug levels
infusion)
starting
must be
dose
monitored see
660mg
section 5.4.4)
*Round dose down to the nearest 10mg in children < 20kg and to the nearest 20mg in children > 20kg
2 weeks
Tobramycin
(Bramitob)
Nebulised
Cycles of
28 days on
28 days off
Tranexamic
Acid
> 6 years
300mg
PO
15-25mg/kg
2 or 3
PO
10mg/kg
1.5g
500mg tabs
500mg/5mls solution
continuous
250mg/5ml
150mg tabs
250mg caps
187
DRUG
ROUTE
AGE/WEIGHT
DOSE
Vancomycin
(see section 5.4.3)
Vitamin D
(see section 8.4)
(As either
ergocalciferol or
cholecalciferol)
Nebulised
1 month 18
years
Routine
supplementation
< 1 year
1 - 12 years
> 12 years
4mg/kg
FREQUENCY
(Times daily)
4
400 IU/day
400 - 800 IU/day
800- 2000 IU/day
1
1
1
PO
3000 units/ml
20000 capsules
MAX
DOSE
250mg
DURATION
5 days
continuous
continuous
continuous
Higher doses may be

required for serum
levels
< 30ng/mL
Weekly dosing of
20000 units ONCE
WEEKLY is available
if daily dosing not
tolerated
Vitamin E
(Alpha
Tocopheryl)
PO
< 1 year
1 - 10 years
> 10 years
10-50mg
50-100mg
100-200mg
1
1
1
1- 12 years
5 - 10mg
continuous
12 -18 years
10 - 20mg
continuous
2 -12 years
200mg
Review in 6
months
12 -18 years
Weight < 40kg
200mg
Then 100mg
(Increase if
necessary to
150mg)
2
2
2 doses
Review in 6
months
12 -18 years
Weight > 40kg
400mg
Then 200mg
(Increase if
necessary to
300mg)
2
2
2 doses
Review in 6
months
100mg/ml
50mg gel caps
Vitamin K
- Menadiol or
phytomenadione
PO
continuous
continuous
continuous
Required
dose
dependent
on levels
10mg tabs
Voriconazole
(treatment of
ABPA, see section
7.1)
PO
188
Appendix 15: Giving 1st dose of intravenous antibiotics at home

M3 NOTTINGHAM CHILDREN'S SERVICES PROCEDURE
Medicines Giving 1st Doses of Intravenous Antibiotics to Children at Home
or in a Community Setting
Standard Statement
Emergency treatment of an acute or severe anaphylactic reaction will be
carried out by an RSCN/RN Child Branch nurse with the appropriate
community qualification, or a parent following the appropriate training, within
the community or home environment when administering first doses of
antibiotics.
Introduction
Many children with chronic illnesses such as Cystic Fibrosis (CF) often have
long periods of intravenous antibiotic therapy and over the years are exposed
to the same drugs.
Some CF patients and patients with other respiratory conditions who are
stable may now have their first dose of IV antibiotics at home if they have
previously received the same antibiotic. A nurse with the appropriate
qualification as identified by the guide to intravenous therapy (2011), will
administer the antibiotics and adhere to the principles of the five rights for
drug administration. The first dose of any newly prescribed antibiotic should
be administered in the hospital setting.
What is anaphylaxis?
This is an acute reaction to an allergen such as antibiotics, foods e.g. nuts
and latex. This is caused by a rapid release of histamine from mast cells and
basophils following interaction with an allergen such as penicillin (Miles & Bain
1992). This can be potentially life threatening and requires prompt action.
Community nurses need to be appropriately trained and equipped should
such a reaction occur.
Structure
When undertaking this procedure there must be two people present at all
times, one of who may be a parent/carer of the child. The Community Nurse
must ensure that the childs GP is aware that IV antibiotics are being
administered at home.
Equipment
Antibacterial hand washing solution
Two Auto-injector pens Epipen (Adrenaline with clear guidelines for use
in the home as an aid memoir)
Prescription chart
Antibiotics
189
Telephone for emergencies

Process
Action
Rationale
1. Parents need to be given the relevant

drug information regarding both the
antibiotics prescribed and the
Adrenaline (Epipen), which may be
required before accepting responsibility
of home care. The nurses should
undertake this when teaching and
assessing of the parent/carer occurs.
Give parents written and practical
advice about the use of the Epipen.
To ensure parents/carer are aware of the

responsibility that they are undertaking.
2. Explain the procedure to the

child/young adult and parents, also
ensure that the parents are aware and
taught how to recognize the signs of
anaphylaxis.
Best Practice:
Signs and symptoms of anaphylaxis,
during first 30 60 minutes after
antibiotics.
Itching of the skin
Rashes
Sudden difficulty in breathing
Feeling faint
Sweating
Pallor
Cold extremities
3. The child needs to be observed by the
nurse, parent or carer for signs of
anaphylaxis for 30-60 minutes after the
first dose of antibiotics
4. In the event of an anaphylactic reaction
mild or severe.
Stop the treatment.
Assess the airway, breathing and
circulation
Lay the child flat with feet elevated, if
having a severe reaction and
administer Adrenaline as prescribed.
Clear away and dispose of sharps
safely.
Sign for the drugs given on the
prescription chart, reporting any
problems to medical staff.
To reduce anxiety and ensure that the

parent recognizes the need to call for an
ambulance for medical assistance
promptly.
To allow for appropriate understanding

and the necessary action needed with
second and subsequent doses if problems
arise
To ensure signs of a reaction whether

minor or severe are recognised and acted
upon
To ensure quick action if a reaction should

occur whilst waiting for medical
assistance.
To prevent contamination and needle stick

injury.
To maintain accurate records of drugs
administered or problems encountered
190
Dosage Guidelines:
1.
0.15mg Epipen junior and 0.3mg if child over 30kg in the auto injector pen
(Epipen) into the side of the thigh.
2.
Repeat the dose 5-15 minutes later if there is no response
3.
Call 999 for an ambulance, a maximum of 2 doses only should be

administered whilst waiting for medical assistance, and transfer the child
to hospital.
Outcome:
The child will be observed during and after IV antibiotics therapy for a drug
reaction and anaphylaxis is appropriately managed.
References:
Anaphylaxis (2009) @http/ www.medinet.com/anaphylaxis. accessed on 18.05.09
Henry J (1988) Guide to Medicines and drugs. Dorling and Kindersley,
London
McNulty T J (1993) Initiation of Antimicrobial therapy in the Home. American
Journal Of Hospital Pharmacy, Vol 50 (April) pp 773-774.
Miles Am and Bain B (1992) Penicillin Anaphylaxis: A review of
Sensitatization, treatment and Presentation. Journal of association of Minor
Physiology. Vol 13(2) pp 506.
Nottingham University NHS Trust, (2011) Guide To Intravenous Therapy Third
Edition pp 7 & 11..
Nursing and Midwifery Council. Standards for medicines management.
2010 London: Nursing and Midwifery Council. Available from:
http://www.nmcuk.org/Documents/Standards/nmcStandardsForMedicinesMan
agementB
Resuscitation Council UK (2008) Emergency Treatment of anaphylactic
reactions; guidelines for health care providers; available at
www.resus.org.uk/pages/reaction.pdf
Janice Mighten (Childrens Respiratory Nurse ) November 2012
Document information:
Royal College of Nursing Administering IV Therapy to Children in the
Community Setting, 3rd Edition 2001, RCN Publications, London.
Type of document
Procedure
Index number
X_Link
191
Title: M3 Medicines - Giving 1st Doses Of Intravenous Antibiotics To

Children At Home Or In A Community Setting
Version
Services
Source
Publication date: Jan 2006

Author: Janice Mighten
QMC
Nottingham Childrens
Review date: 2015
Consultation process: All wards and Depts ,
Contact name: Miranda Witchell Department: Childrens Services

Filename and path:
192
Appendix 16: Transplantation Referral Proformal
UK Paediatric
Lung and Heart-Lung
Transplantation
Referral Proforma
STRICTLY CONFIDENTIAL
THIS FORM MAY BE USED TO REFER TO ANY OF THE UK

CENTRES THAT PERFORM LUNG & HEART-LUNG
TRANSPLANTATION. PLEASE RETURN THE FORM TO THE
CENTRE OF YOUR CHOICE:
GREAT ORMOND STREET

Dr Paul Aurora and Dr Helen Spencer
Cardiothoracic Transplant Office
Great Ormond Street Hospital
Great Ormond Street
London
WC1N 3JH
Tel:
Fax:
020 7813 8563

020 7813 8440
NEWCASTLE
Dr David Spencer
Cardiopulmonary Transplant Unit
Freeman Hospital
High Heaton
Newcastle Upon Tyne
NE7 7DN
Office:
Fax:
0191 223 1132

0191 223 1439
193
GUIDANCE NOTES FOR

COMPLETION OF REFERRAL PROFORMA
This proforma has been designed to streamline the referral process

for potential lung and heart-lung transplant recipients. As a result
potential transplant candidates can be identified more easily, be
formally assessed more quickly and duplication of investigations will
be avoided. The information required has been agreed by all UK lung
transplant centres and this form can be used to refer to any UK
centre.
Thank you for your co-operation.
KEY POINTS
Please complete all sections - any questions which are not applicable
should be marked as N/A.
When specific results are not available but have been requested
please mark as awaited.
Copies of Imaging (CT, coronary angiography, etc) should be
sent on CD with this form
Copies of complete reports of investigations can be appended to
this proforma, but the clinical summary should be completed by a
member of the multidisciplinary team in the appropriate proforma
section. Serial lung function tests are very helpful and should be
included when available.
Any questions about this proforma or its use can be addressed by
contacting the transplant co-ordinators at the hospital to which you
intend to send the referral.
194
PERSONAL DETAILS
PATIENT NAME:
.
NHS Number:
AGE:
DOB:
ELIGIBILITY FOR NHS CARE:..
NEED FOR INTERPRETER:
YES / NO
LANGUAGE:..
ADDRESS:
(Include Postcode)
TELEPHONE NUMBER
MOBILE: ..
REFERRING CONSULTANT:..
REFERRING CENTRE:.
(Include Postcode)
TELEPHONE NUMBER
PCT:
FAX: ..
..
GP NAME:
GP ADDRESS:
(Include Postcode)
GP TELEPHONE NUMBER: FAX: ..
IS PATIENT AWARE OF REFERRAL FOR TRANSPLANT ASSESSMENT?
YES
NO
(please circle)
195
RESPIRATORY HISTORY
Primary Diagnosis:
Secondary Diagnoses
Respiratory.
Non respiratory
1.
2.
3.
Respiratory Diagnoses made by: Clinical /CT chest/Histology/Genotype/ Sweat Test

Details.
Any household members smoke? : YES
Microbiology:
NO
(Please Circle)
Have these organisms ever been isolated?
Burkholderia cepacia
YES
specimendate
NO
Pan-resistent Pseudomonas
YES
specimendate
NO
MRSA
YES
specimendate
NO
Mycobacteria (TB or atypicals)

YES
specimendate
NO
Aspergillus
YES
specimendate
NO
If YES, please give further

details.
Oxygen at home
YES
Amount .L/min
NO
(Please Circle)
Average daily use hrs
Respiratory Past History

Haemoptysis
YES
NO
Details:
Pneumothorax:
YES
Details:
Thoracic Surgery:
YES
Details:
..
NO
NO
(Please Circle)
(Please Circle)
(Please Circle)
196
Has the patient ever required ventilation?

If yes
YES
NO
(Please Circle)
(duration days)
NIV / formal ventilation in ITU
Details:.
Current Exercise Capacity

Exercise tolerance (distance)
Formal 6 minute walk test performed ?
If yes
YES
Max distance metres
NO
(Please Circle)
Lowest saturation%
Performed on air / oxygen at litres per minute

Wheelchair
YES
NO
Progress pre and post diagnosis (Free Text)

Include details on rate of decline, life threatening exacerbations, frequency of iv
antibiotics, etc
Is family aware of prognosis?
YES / NO
Is patient aware of prognosis?
YES / NO
197
PAST MEDICAL HISTORY

Current or previous :
Details:
Heart Disease
YES
NO
Renal Disease
YES
NO
Liver Disease
YES
NO
Diabetes
YES
NO
Malignancy
YES
NO
GI problems
YES
NO
Portacath
YES
NO
Gastrostomy
YES
NO
Current Medication
1.
Dose
Frequency
2.
Dose
Frequency
3.
Dose
Frequency
4.
Dose
Frequency
6.
Dose
Frequency
7.
Dose
Frequency
8.
Dose
Frequency
9.
Dose
Frequency
10..
Dose
Frequency
ALLERGIES:
NO
YES
(Please Circle)
1.
2
Oral Corticosteroids?
YES
NO
(Please Circle)
Date commenced
Max dose
Current dose
Date stopped
Response.
198
Family and Social History

Compliance Good
YES
NO
(Please Circle)
Attendance Record Good
YES
NO
(Please Circle)
Family support available:

Social Services input:
YES
NO
Details..
School details:.
School attendance:..
Siblings?....................................................................................................................
Relevant Family Medical or Social History:..
Psychological assessment
Current or Previous History of:
Depression:
Panic attacks:
Anxiety:
YES
Needle phobia:
Other psychological concerns?:
YES
YES
NO
NO
NO
YES
YES
NO
NO
Details...
199
CLINICAL INVESTIGATIONS
Weight.kgs
ECG
Height.m
BMI..
Date performed:
Result. .
Echocardiogram
Date performed:
Result...
Chest x-ray
Last performed:
Result...
HRCT Thorax Date performed
Result...
..
Arterial/Caplillary/Venous (please circle) Blood Gas (ON AIR)

pH ..
pO2 ..
pCO2 .. BXS ..
HCO3 .. Sats .
Bone Densitometry
Spine Z score = ..
Femur Z score = ...
Abdominal ultrasound
..
Coronary angiography
..
Right heart catheter
..
GORD Testing
Glomerular Filtration Rate
..
Others (if available)
Respiratory Function Tests

Date
(attach trend values if possible)
FEV1
FVC
FEV1/FVC
Value
.
.
.
%
.
.
.
Value
.
.
.
%
.
.
.
TLC
FRC
RV
.
.
.
.
.
.
.
.
.
.
.
.
TLCO
KCO
.
.
.
.
.
.
.
.
200
Haematology
Biochemistry
Virology
Date:
Date:
Date:
Na
Hb
HIV
WCC
CMV
Urea
Platelets
Hepatitis B
Creatinine
PT
Hepatitis C
eGFR
APTT
Bilirubin
Fibrinogen
Immunology
ALT
ESR
IgE
ALP
GGT
Glucose (fasting)
Chol
(fasting)
Trig
(fasting)
Additional Microbiology
Date & Details
MRSA screen
Total Calcium
Asp. precipitins
CRP
Asp. culture
Blood group (if known) ..

Anti crossmatch antibodies (if known)
YES
NO
Details ..
ANY OTHER COMMENTS
Signed.NAME:..
POSITION:.DATE:
201
Appendix 17
Nottingham University NHS Trust
CYSTIC FIBROSIS UNIT - PAEDIATRICS
PATIENT SUMMARY SHEET
Diagnosis: Cystic Fibrosis
Date of Diagnosis:
/
Method of Diagnosis:
Affix Patient Label Here

Genotype:
/
Highest Sweat Test - Date:
Na:
Cl:
Sweat weight
Feacal elastase: Date:
Medical History
Management
Date
Ps aerug - 1st isol

Ps aerug - 2nd isol
Ps aerug - 3rd isol
Ps aerug - 4th isol
Ps aerug - 5th isol
Ps aerug - 6th isol
Ps aerug established
B cepacia - 1st isol
B cepacia confirmed
MRSA - 1st isolated
Pneumothorax
Drug allergies
Asthma
Diabetes
ABPA
Liver disease
(date started urso)
Liver US
Inj Varices
Bowel surgery
Child protection
register
Child in need
Date
Ps eradication step 1
Neb Antibiotics
DNase
Hypertonic saline
IV 1st treatment
Portacath insertion (1)
Portacath removal (1)
Portacath insertion (2)
Portacath removal (2)
Gastrostomy inserted
Bronchoscopy
Referred transplant lung/ liver
Transplanted
Frequency of regular
IVs
Home oxygen
Port CF consent
202
203

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Title of Guideline (must include

Guideline for the management of patients with

Staff at Nottingham Childrens Hospital via the

NOTTINGHAM UNIVERSITY HOSPITALS NHS TRUST

CHILDREN AND YOUNG PERSONS CYSTIC FIBROSIS

CYSTIC FIBROSIS MANAGEMENT

Section 2 - Outpatient Clinic

Outpatient clinic arrangements

Section 3 - Annual Assessment

Section 4 - Ward Management of CF Patients

Section 5 - Antibiotic Guidelines

Collection of specimens for respiratory culture

Section 6 - Home "IV's" & Home Oxygen

Home intravenous (IV) antibiotic therapy

Section 7 - Management of Other Respiratory Problems

Allergic bronchopulmonary aspergillosis

Section 8 - Management of Other CF Related Disease

Section 9 - Dietetic & Nutritional Management

Diet and nutrition

Section 10 - Guidelines for Obtaining Intravenous Access

Pic line/ Longline insertion

Introduction and principles

Section 12 - Dornase alfa & Hypertonic saline

Symptom control in the well child

Section 14 - Psychosocial support

The importance of psychosocial factors in CF

QMC Hospital School

Transition boys transfer folder

1.2 The Diagnosis of Cystic Fibrosis

1.3 Diagnostic testing

1.3.1 Newborn screening

1.3.2 Necessary cautions with newborn screening

6% of babies with CF (including those with meconium ileus )could be missed by

1.3.3 Babies with CFTR mutations detected in both genes

They are either

Referred by the screening lab to the CF paediatric service for evaluation

Referred by the neonatal team to the CF consultant paediatrician for an

1.3.4 Babies with only one mutation detected on Newborn screening

A Child with suggestive history / symptoms/ examination.

1.4.1 Stool testing

Sweat test are required to confirm the diagnosis

An anti-staphylococcal antibiotic should be started immediately (intravenously if

1.6 Failure to thrive

1.7 Chronic respiratory symptoms

Some families choose to have ante-natal CVS testing in subsequent pregnancies,

Section 2 - Outpatients Clinic

2.1 Outpatient Clinic Arrangements

The CF clinic is held every Tuesday afternoon. Children with established

2.2 At every clinic visit:

2.3 Multidisciplinary meeting:

2.4 Post Clinic Administration:

2.5 Holiday advice

Reserve antibiotics these should be considered individually for each patient.

Consider salt replacement for holidays in hot climates.

Section 2a Transfer to Adult Care

2a.3 Hospital Youth Service

2a.4 Young Persons CF Clinic

2a.5 Individual Transition Plan (see appendix 13)

TRANSITION FROM DIAGNOSIS TO ADULT SERVICES

Informed of the CF Trust

Given written information

Over the first 2 or 3 clinic

Sent copies of all clinic letters