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Clinical Guidelines: Care of Children with Cystic Fibrosis 2014

The sixth edition of these guidelines (2014) is available in PDF format. You can view or download the latest guidelines (2014) here.

The sixth edition of these guidelines has been written by members of the Royal Brompton Hospital Paediatric Cystic Fibrosis Team. Contributors over the years include:


Saji Alexander, Khalid Alshafi, Anna-Karenia Anderson, Ian Balfour-Lynn, SiânBentley, Roger Buchdahl, Fran Beresford, Diana Bilton, Cara Bossley, Nicola Bridges, Sarah Brown, Andrew Bush, Siobhán Carr, Nicola Collins, Christina Courtney, Jacqui Cowlard, Finella Craig, Jane Davies, Katie Dick, Emma Dixon, Jane Docker, Sarah Elkin, Amanda Equi, Jackie Francis, Frances Goodhart, Gabriela Grigore, Chris Grime, Alex Handford, Jonny Harcourt, Laura Hayers, Karen Henney, Tom Hilliard, Nicola Hirsch, Sam Irving, Mary Jurd, Wanda Kozlowska, Kenny Macleod, Su Madge, Sukeshi Makhecha, Angela McCullogh, Andrea McKee, Donna McShane, Caro Minasian, Nicola Murray, Adam North, Suzie Nolan, Caroline Pao, Sarah Pike, Michèle Puckey, Lucy Reed, Mark Rosenthal, Nick Simmonds, Vikki Stone, Pat Stringer, Rivanna Stuhler, and Carole Wingett.


2nd to 6th editions were edited by Dr Ian Balfour-Lynn.

1st edition (1994) was edited by Dr Pat Oades.


These guidelines are based on published evidence as well as the extensive clinical experience of our Paediatric CF Team. This is how we do things, but it does not mean that other regimens are necessarily wrong just because they are different. However patients who come to the Royal Brompton Hospital, either for full or shared care, will be looked after using these guidelines.


These guidelines have been endorsed by the Medicines Management Board of Royal Brompton & Harefield NHS Foundation Trust in December 2013. 


If there are any comments, queries or errors noticed, please contact Ian Balfour-Lynn on i.balfourlynn@ic.ac.uk.


Please note, the next revision will be published in 2017 so this edition should not be used after that date. Please destroy all 2011 editions.


> What's new in 6th edition?


> Introduction


> Department staff and contact numbers


> How the service runs

> Clinics

> Annual review

> Transition from paediatric to adult care

> Home care & outreach service (nursing & physiotherapy)

> Clinical psychology

> Social work support

> Family liaison team & welfare rights adviser

> Payment by results 

  - the mandatory tariff


> Admission to hospital

> Admitting the child

> Investigations

> Venous access & long line insertion

> Procedural distress

> Self administration of medicines

> Discharge

> Infection control


> Making the diagnosis

> Newborn screening

> Clinical presentation

> Sweat testing

> Genetic analysis

> What happens when newborn screening results are not clear cut?

> Antenatal screening

> Pre-implantation diagnosis

> Other tests

> Routine investigations for newly diagnosed patients


> Respiratory care

> Chest exacerbations

> Antibiotics

> Policies & specific organisms

> Drug allergy & desensitisation

> Home IV antibiotics

> Portacaths (Totally Implantable Venous Access Devices)

> Corticosteroids

> DNase (Dornase alfa, Pulmozyme)

> Hypertonic saline

> Mannitol

> Long term azithromycin

> Ivacaftor

> Aspergillus lung disease

> Haemoptysis

> Pneumothorax

> Intractable wheezing / severe small airways disease

> 'Challenging CF' protocol

> Bronchoscopy

> Chest physiotherapy

> Airway clearance techniques

> Inhaled antibiotic bronchoconstrictor challenge

> Nebulisers

> Dry powder inhaled antibiotics

> Induced sputum

> Oxygen

> Non-Invasive Positive Pressure Ventilation (NIPPV)


> Gastrointestinal & nutritional care

> Nutritional care & assessment

> Pancreatic enzyme replacement therapy

Oral nutritional support

> Enteral nutritional support

> Management of feeding difficulties

> DIOS and constipation

> Liver disease

> Iron status


> Other non-pulmonary complications of CF

> Cystic Fibrosis-related diabetes

> Growth

> Puberty 

> Bone Metabolism

> ENT complications

> Nasal polyps

> Sinusitis

> Arthropathy

> Pseudo-Bartter’s syndrome

> Fertility

> Stress incontinence


> Transplant Assessment


> Miscellaneous

> Preparation for surgery

> Immunisation

> Chicken pox

> Travel abroad

> Terminal Care


> Drug Formulary

> Drugs for the respiratory tract

> Oral antibiotics - prophylactic antibiotics

> Oral antibiotics - treatment doses

> Inhaled antibiotics

> Intraveneous antibiotics

> Antifungal antibiotics

> Other respiratory treatments

> Drugs for the gastrointestinal tract

> Pancreatic Enzymes

> Fat-soluble vitamins

> ‘Antacids’

> Gastroesophageal reflux

> Distal Intestinal Obstruction Syndrome (DIOS)

> Constipation

> Liver disease

> Home delivery of medicines


> Appendix I - Transition Integrated Care Pathway

> Appendix II - Treatment of Non-tuberculous Mycobacteria

> Appendix III - Home visit report for 'challenging CF' protocol

> Appendix IV - Ivacaftor: proform for commencing / monitoring treatment

> Appendix V - Social security benefits

> Appendix VI - National Service specification

> Appendix VII - Payment by Results Guidance 2013-14

> Appendix VIII - Guide for parents starting a child on a nebulised therapy

> Appendix IX - Tables for body surface area

> Appendix X -  Travel letters

> Appendix XI - Gene mutation nomenclature 

> Appendix XII - CF Trust consensus documents, factsheets & leaflets

> Appendix XIII -   Useful telephone numbers


Royal Brompton

Sydney Street,
London SW3 6NP
Tel: +44 (0)20 7352 8121