Understanding new research on bowel cancer and cystic fibrosis

Advances in our understanding of CF mean that there are many more effective treatments out there, so people with the condition are living longer than ever. But the aging process can also bring its own health challenges to contend with

In the last few years, international registry data has started to emerge showing that people with CF are at an increased risk of developing gastrointestinal (GI) cancer, specifically bowel cancer. These early studies reported that more people with CF than they expected are developing GI cancers. In addition to this, people with CF are also developing GI cancer at a younger-than-average age.

Dr Daniel Peckham, director of the Adult CF Centre in Leeds, and colleagues, have been exploring this further in recent research, published earlier this month. The research studied whether people with CF in England are at higher risk of developing GI cancers compared to the general population.

In this study, the number of people with bowel cancer and cystic fibrosis was calculated using the Trust’s CF Registry, a cancer registry and NHS England data. And then using data from the 100,000 Genomes Project, the presence of CFTR mutations in people with bowel cancer was also measured. 

The researchers found that people with CF and bowel cancer had an average age of diagnosis of 52 years, which is significantly younger than in people without CF, which is an average of 73 years. The study also found that people with CF are five times more likely to develop bowel cancer than people without, and that the overall rate of CFTR mutations in the bowel cancer population was a lot higher than was expected. However, it is very important to note that the risk of an individual with CF developing bowel cancer remains low.

So what does this research mean? The study is in agreement with studies from other countries and shows that CF is linked to an increased risk of bowel cancer. It also shows, in the control population studied, that the rate of CFTR mutations in the bowel cancer population is higher than would be expected. 

Further work is needed to determine whether people with CF would benefit from a tailored screening programme and what form it should take. More research is needed to understand the role of CFTR gene mutations in the development of GI cancer, for people with CF and those without.

What is the Trust doing to support research into CF and cancer?

Scientists and clinicians are working together to understand why people with CF may be at an increased risk of developing cancer on a cellular level, and what could be done to reduce the chances of someone developing it. The Trust has already played an active role in bringing together a wide range of researchers to look into this topic, and in January 2021 we began funding a Strategic Research Centre (SRC) proposal led by Professor Stephen Renshaw. Professor Renshaw and an international, multidisciplinary team of researchers are investigating why people with CF are at a higher risk than expected of developing cancer.

We know that many in our community may feel worried about this research. There is more information on our website about understanding the risk of cancer, about bowel cancer screening and about our lab-based SRC mentioned above. You can also speak to our Helpline, or do raise any questions or concerns with your CF team.

We will keep our community updated on the progress of this vital research – and we won’t stop until everyone with CF can truly live a life unlimited.

You can read more about the research here.

Find out more about cancer and CF here.

Cystic fibrosis (CF) is a genetic condition which causes sticky mucus to build up in the lungs and digestive system. It affects more than 10,800 people in the UK. One in 25 of us carries the faulty gene that causes it, usually without knowing. 

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