Understanding cancer in individuals with cystic fibrosis
Research type
Research Study
Full title
Understanding cancer in individuals with cystic fibrosis
IRAS ID
333299
Contact name
Daniel Peckham
Contact email
Sponsor organisation
University of Leeds
Duration of Study in the UK
2 years, 11 months, 31 days
Research summary
The Problem:
Cystic fibrosis (CF) is an inherited disease that damages a person’s lungs, digestive tract, and other organs. There are over 11,000 People with CF (pwCF) in the UK and the average age is presently 22 years. Predicted life expectancy continues to rise significantly from 34.4 years in 2009 to 64.1 years in 2023. Survival is expected to increase exponentially following the introduction of highly effective CFTR modulator drugs.
PwCF have a higher risk of developing cancer and at a younger age than the general population. For instance, pwCF in England are five times more likely to develop bowel cancer. There is also growing evidence that CF is associated with an increased risk of developing other cancers such as oesophageal, small intestinal, pancreatic, skin, breast and cervical cancers. More worryingly, the prevalence of cancer is likely to further increase as people with CF enter the ages where cancers are more common.
Past studies have struggled to identify cancer risks and cancer outcomes in pwCF. This reflects a lack of accurate information about cancer in both CF clinical records and CF Registries. In addition, it can be difficult to identify pwCF in cancer registries as well as important risk factors, such as organ transplantation.
This study will link data from the ‘gold standard’ UK CF Registry, the National Cancer Registry and hospital admissions to identify, the true age-related incidence of all cancers in pwCF. By linking these datasets, we will also be able to look at the types of treatment people receive for their cancer, the stage of cancer at diagnosis, outcomes such as survival as well as a reliable prospective tool for long-term cancer surveillance.The results will determine the best approaches to cancer education, prevention, screening, testing, and treatment to minimise future risk.
REC name
East of England - Cambridge Central Research Ethics Committee
REC reference
25/EE/0168
Date of REC Opinion
12 Aug 2025
REC opinion
Favourable Opinion