Understanding and managing cystic fibrosis
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Understanding and managing cystic fibrosis

Kamilla Dack Clinical nurse specialist, Department of cystic fibrosis, Royal Brompton Hospital, London
Alan Peres Clinical nurse specialist, Department of cystic fibrosis, Royal Brompton Hospital, London
Linda Thrift Clinical nurse specialist, Department of cystic fibrosis, Royal Brompton Hospital, London
Susan Talbot Clinical nurse specialist, Department of cystic fibrosis, Royal Brompton Hospital, London
Susan Madge Consultant nurse, Department of cystic fibrosis, Royal Brompton Hospital, London

The growing number of people living longer with the disease means that care is increasingly shifting to community teams. Kamilla Dack and colleagues describe the main features of cystic fibrosis, and its treatment and management

Cystic fibrosis (CF) is one of the most common inherited diseases in the UK. It is a multi-system disease, mainly affecting the lungs and gastrointestinal tract, but also the liver, pancreas, joints, sinuses and male reproductive system. Diagnosis is commonly through screening newborns, although older children and adults are also diagnosed with CF. Treatment is daily, complex and time-consuming and, although most of it is conducted at home, there is an increasing need for hospital admission as individuals age and the disease progresses.

CF is life-limiting, with no cure. However, life expectancy throughout Europe is improving thanks to early and aggressive treatment, specialist centre care and novel therapies. Of the 32,248 patients registered in Europe with CF, 49.3% are more than 18 years old, although the median age of death across Europe is 28 years (European Cystic Fibrosis Society 2014) based on the latest available figures. Children born with CF today are expected to live into their fifties and sixties.

Primary Health Care. 25, 6,18-24. doi: 10.7748/phc.25.6.18.e714

Correspondence

s.madge@rbht.nhs.uk

Peer review

This article has been subject to double-blind review and checked using antiplagiarism software

Conflict of interest

None declared

Received: 24 October 2012

Accepted: 22 September 2014