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 (
Primary Health Care. 25, 6, 18-24. doi: 10.7748/phc.25.6.18.e714
Correspondence Peer reviewThis article has been subject to double-blind review and checked using antiplagiarism software
Conflict of interestNone declared
Received: 24 October 2012
Accepted: 22 September 2014
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