An international/multicentre report on patients with cystic fibrosis (CF) over the age of 40 years

J Cyst Fibros. 2008 Nov;7(6):537-42. doi: 10.1016/j.jcf.2008.06.003. Epub 2008 Aug 19.


Background: The lifespan of patients with cystic fibrosis (CF) is increasing significantly. The objective of this international pilot study was to study the characteristics of these long-term survivors.

Methods: Four centres with large CF clinics from London (UK), Minneapolis (USA), Toronto (Canada) and Verona (Italy) identified 366 patients who had survived 40 years and longer.

Results: At all centres males survived longer than females. There were more pancreatic sufficient patients in Verona (60%) and Toronto (40%) than in London (16%) and Minneapolis (21%). The percentage of DeltaF508 homozygous patients varied between 47% in London and 45% in Minneapolis to only 26% in Toronto and 9% in Verona. Average FEV(1) and BMI values of the surviving population appeared to stabilise after 40 years of age. FEV(1) was on average 12% higher in patients who were pancreatic sufficient (p > 0.0001). There was no difference in survival between the centres. The overall median survival after the age of 40 was 13 years. The estimated annual death rate was approximately 3.4% from the age of 40-60 years.

Conclusions: Significant numbers of patients are now surviving to 40 years or more, and it is hoped that an in-depth study of these patients may identify the factors contributing to longer survival.

Publication types

  • Multicenter Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Age Distribution
  • Aged
  • Canada
  • Cohort Studies
  • Cystic Fibrosis / complications
  • Cystic Fibrosis / epidemiology*
  • Cystic Fibrosis / physiopathology
  • Female
  • Humans
  • Italy
  • Life Expectancy*
  • London
  • Longevity
  • Male
  • Middle Aged
  • Minnesota
  • Retrospective Studies
  • Sex Distribution
  • Survival Rate