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Respir Med. 2009 Apr;103(4):629-35. doi: 10.1016/j.rmed.2008.10.011. Epub 2008 Nov 20.

Growing old with cystic fibrosis - the characteristics of long-term survivors of cystic fibrosis.

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1
Department of Cystic Fibrosis, National Heart and Lung Institute, Imperial College and Royal Brompton Hospital, London, UK. nickjamessimmonds@yahoo.com

Abstract

BACKGROUND:

The proportion of patients with cystic fibrosis (CF) who are middle-aged is increasing - and is likely to continue to do so. We surveyed a population of long-term CF survivors to assess their burden of illness and profile their disease characteristics.

METHODS:

A case series (n=112) of patients from one specialist centre who had reached their 40th birthday without transplantation. Hospital records and annual review data were examined.

RESULTS:

The median age of the group was 43.1 years (range 40-71.1); 57% were men. 68% were diagnosed before 16 years of age. 30% were DeltaF508/DeltaF508, 76% having at least one DeltaF508 allele. When compared with the total adult CF population, the older patients were significantly less likely to have a DeltaF508 mutation or colonisation with Stenotrophomonas maltophilia and MRSA; but more likely to have pancreatic sufficiency, colonisation with Pseudomonas aeruginosa or allergic bronchopulmonary aspergillosis. On average they required less than one hospital admission a year; lung function and body mass index were relatively well preserved. Many were married and working.

CONCLUSIONS:

We describe one of the largest surveys to date of CF patients aged more than 40 years. The full spectrum of disease is represented in this population and, importantly, 30% are DeltaF508 homozygous. Provision needs to be made for the healthcare needs of this increasing population of older patients.

PMID:
19022643
DOI:
10.1016/j.rmed.2008.10.011
[Indexed for MEDLINE]
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