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

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


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.

MeSH terms

  • Adult
  • Age of Onset
  • Aged
  • Body Mass Index
  • Comorbidity
  • Cystic Fibrosis* / epidemiology
  • Cystic Fibrosis* / genetics
  • Cystic Fibrosis* / microbiology
  • Female
  • Forced Expiratory Volume
  • Humans
  • London / epidemiology
  • Male
  • Middle Aged
  • Sputum / microbiology
  • Survivors / statistics & numerical data*
  • United Kingdom / epidemiology
  • United States / epidemiology
  • Vital Capacity
  • Young Adult