Cancer and CF Q&A

As people with cystic fibrosis (CF) live longer, we expect to see new trends in health data, with many positive developments but also new health challenges such as cancer. Access to national and international registries and increasing global collaboration mean we can identify trends and emerging issues more quickly and ensure we respond in a timely and effective way. We spoke to Professor Daniel Peckham, Director of the Adult CF Centre in Leeds, to find out more about the topic of cancer and CF, and work that is underway to further our knowledge in this important area.

  • What is the incidence of cancer in the CF population and are the rates of cancer in people with CF going up? If so, why may that be?

    As the number of people living with CF is growing, and people are now living longer, more cases of cancer are likely to occur. We don’t know at the moment the exact figures, but we are beginning to collect this data to give us a more accurate and very specific cancer-related picture. In collaboration with the Cystic Fibrosis Trust, we are presently analysing the national cancer registry data and will be reporting the results later this year. Therefore, we will be able to answer these important questions very soon.

  • Are there particular types of cancer that may be more prevalent?

    International Registry data has shown an increased rate of bowel cancer, which is occurring earlier in people with cystic fibrosis. It has also shown an increased risk of other types of cancer, like skin and lymphoma, in people who have had a transplant.  

  • How does having a transplant affect your risk of cancer?

    Immunosuppression is used to stop transplant rejection, which increases the risk of certain types of cancers such as non-melanoma skin cancers, especially squamous cell carcinoma and post-transplant lymphoproliferative disorder. There is also an increase in the risk of other cancers such as bowel cancer.

  • How can I reduce my risk of bowel cancer? Will Kaftrio reduce my risk of bowel cancer?

    We do not know why there is an increase in bowel cancer. Modern Western life, including diet, are associated with an increased risk of bowel cancer in the general population – around 40 per cent of cancers are caused by lifestyle factors. Dietary changes may help lower risk in the longer-term; however, more research is needed to understand this.

    Treatment with Kaftrio or other CFTR modulators may reduce certain cancer rates by correcting the underlying inflammation and cellular functions. There is lots of ongoing research to investigate the effects of CFTR modulators and we will start to understand the precise mechanisms soon. The good thing is that they have powerful local and systemic (general) anti-inflammatory properties and it is possible that they may be protective.

  • What do we know about how the CFTR protein may increase the risk of bowel cancer?

    As part of a Trust-funded SRC (Strategic Research Centre) programme, an international, multidisciplinary team of researchers led by Professor Stephen Renshaw at Sheffield University, will be investigating a possible role for the CF protein, CFTR, in causing CF-related bowel cancer. Biomedical scientists within this SRC programme are investigating whether or how disruption to the normal workings of the CFTR protein might directly or indirectly lead to the development of cancer. Find out more about this SRC here.

  • Why do some centres screen people with CF age 30 and over for bowel cancer, and others don’t?

    This is an area where there has been much debate. My personal opinion is that screening is important to help prevent and diagnose bowel cancer at an early stage, though there is no consensus yet on the best age to start screening.  This is something to discuss with your local CF centre.

  • Should I get screened for bowel cancer?

    When you are invited for screening, please attend as early diagnosis is crucial –  the sooner cancer is diagnosed, the easier it is to treat. It’s important to remember it is highly likely that the test will be negative.

  • Is treatment for cancer different if you have cystic fibrosis?

    No, you would receive the same treatment.

  • Is there anything I can do to reduce my risk of developing cancer?

    It is important that you:

    1. Attend all national screening programmes irrespective of CF, including cervical, breast and bowel screening. They are there to pick things up early and avoid disease progression.
    2. Follow national guidelines for human papillomavirus (HPV) vaccination.
    3. Stay safe in the sun.

Bowel cancer and CF

There is some research to suggest that there may be an increased risk of bowel (or colorectal) cancer in people with cystic fibrosis. Find out more about bowel cancer, the risks in people with CF and how it is screened for.

Research into bowel cancer

While more evidence about the chances of people with CF developing bowel cancer are being explored, biomedical scientists within our SRC programme are investigating whether or how disruption to the normal workings of the CFTR protein might directly or indirectly lead to the development of cancer.

Complications and symptoms of CF

Learn about the symptoms that people with CF can have and find out more about the complications that can arise as a result of the condition.

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