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Clinical Guidelines: Care of Children with Cystic Fibrosis 2011

The 5th edition of these guidelines (see PDF version) has been written by members of the Royal Brompton Hospital Paediatric Cystic Fibrosis Team.

Contributors over the years include:

Saji Alexander, Khalid Alshafi, Ian Balfour-Lynn, Roger Buchdahl, Fran Beresford, Cara Bossley, Nicola Bridges, Andrew Bush, Nicola Collins, Christina Courtney, Jacqui Cowlard, Finella Craig, Jane Davies, Katie Dick, Emma Dixon, SiânEdwards, Sarah Elkin, Amanda Equi, Jackie Francis, Frances Goodhart, Alex Handford, Jonny Harcourt, Tom Hilliard, Nicola Hirsch, Wanda Kozlowska, Su Madge, Sukeshi Makhecha, Angela McCullogh, Donna McShane, Caro Minasian, Caroline Pao, Sarah Pike, Michèle Puckey, Lucy Reed, Mark Rosenthal and Pat Stringer, Rivanna Stuhler.

 

2nd to 5th 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 we anticipate that 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 Drugs & Therapeutics Committee of Royal Brompton & Harefield NHS Foundation Trust.

 

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

 

Next revision will be 2013 so this edition should not be used from 2014. Please destroy all 2007 editions.


 

> What's new in 5th edition?

 

> Introduction

 

> Department staff and contact numbers

 

> How the service runs

> Clinics

> Annual review

> Transition Clinic

> Homecare Service (nursing & physiotherapy)

> Clinical Psychology

> Social work support

> Family liaison team

 

> Admission to hospital

> Admitting the child

> Investigations

> Venous access & long line insertion

> Procedural distress

> Discharge

> Infection control

 

> Making the diagnosis

> Newborn screening

> Clinical presentation

> Sweat testing

> Genetic analysis

> Other tests

> Routine in-patient investigations for newly diagnosed patients

 

> Respiratory care

> Chest exacerbations

> Antibiotics

> Policies

> Home IV antibiotics

> Portacaths (Totally Implantable Venous Access Devices)

> Corticosteroids

> RhDNase (Pulmozyme)

> Hypertonic Saline

> Mannitol

> Long term azithromycin

> Aspergillus lung disease

> Haemoptysis

> Pneumothorax

> Intractable wheezing / severe small airways disease

> 'Challenging CF' protocol

> Bronchoscopy

> Chest physiotherapy

> Oxygen

> Non-invasive positive pressure ventilation (NIPPV)

 

> Gastrointestinal & nutritional care

> Nutritional care & oral calorie supplements

> Pancreatic enzyme replacement therapy (PERT)

> Supplementary enteral feeding & gastrostomy

> Management of feeding difficulties

> DIOS and constipation

> Liver disease

> Iron status

> Other non-pulmonary complications of CF

> Cystic Fibrosis-Related Diabetes

> Growth and puberty

> Bone Metabolism

> ENT complications

> 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

> Prophylactic antibiotics

> Oral antibiotics for treatment of a pulmonary exacerbation

> Intravenous antibiotics for treatment of a pulmonary exacerbation

> Antifungal therapy

> Other respiratory treatments

> Drugs for the gastrointestinal tract

> Pancreatic Enzymes

> Fat soluble vitamins

> ‘Antacids’

> Gastro-oesophageal reflux

> Distal Intestinal Obstruction Syndrome (DIOS)

> Constipation

> Liver disease

 

> Appendix I -  Transition document: paediatric to adult care

> Appendix II - Treatment of Non-tuberculous Mycobacteria

> Appendix III -  Antibiotic protocol for NBS Early Detection of Lung Disease Study

> Appendix IV - Social security benefits

> Appendix V -  Tables for body surface area

> Appendix VI -  SHO competency assessment for PICC line insertion

> Appendix VII - GP Shared cared document for rhDNase (pulmozyme)

> Appendix VIII -  GP Shared care document for Bramitob / TOBI

> Appendix IX -  GP Shared care document for Ccolomycin

> Appendix X -  Travel letters

> Appendix XI - Gene mutation nomenclature 

> Appendix XII -    CF Trust consensus statements & information

> Appendix XIII -   Useful telephone numbers

 

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

Harefield