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

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.