A survey to explore factors relating to longevity in cystic fibrosis
Research type
Research Study
Full title
International Study Group of Ageing in Cystic Fibrosis (ISAC): A survey to explore factors relating to longevity in cystic fibrosis
IRAS ID
113960
Contact name
Nicholas Simmonds
Contact email
Sponsor organisation
Royal Brompton and Harefield Foundation NHS Trust
Research summary
Life expectancy for patients with cystic fibrosis (CF) is improving despite the lack of any cure for the underlying basic defect. Currently, survival is estimated at 40.1 years, and children born today with CF are predicted to have a median survival beyond 50 years of age.
As a result of this improvement in survival, the number of adults with CF is now greater than the number of children. Moreover, some individuals are significantly exceeding survival predictions and living into and beyond middle age - the most recent data from the Cystic Fibrosis Trust demonstrate that ~7% of all CF patients in the UK are aged over 40 years. This can only in part be explained by a milder disease course from nonclassic phenotypes as data show that long-term survivors are a highly heterogeneous group. Knowledge of patients’ clinical, physical, psychological and social characteristics will be vital to facilitate the provision of appropriate and effective future healthcare.
A key purpose of surveying older CF patients is to collect information on their healthcare needs and also to explore factors that may have contributed to their longevity. Determining this is vital as the benefits may then be extendable to the CF population as a whole. The survey will be particularly important for improving our understanding of non-genetic, or environmental, factors which are considered to play a key role in determining outcomes (eg. access to care, socioeconomic status, treatment concordance, activity levels, and exposure to tobacco smoke).
At present, there is not enough experience in any single centre to give an authoritative opinion on the complications of ageing in CF, nor the factors that determine such longevity. Conducting a large multi-centre survey would facilitate this and provide stimulus for important future studies.
REC name
London - Brighton & Sussex Research Ethics Committee
REC reference
14/LO/1607
Date of REC Opinion
12 Sep 2014
REC opinion
Unfavourable Opinion