Research we're funding into understanding and treating symptoms of CF

Cystic fibrosis (CF) affects a range of different tissues and organs throughout the body, leading to symptoms such as CF-related diabetes (CFRD) and GI symptoms such as abdominal pain, bloating and diarrhoea. The Trust is funding research to reduce the impact of these symptoms. This includes a better understanding of the cause of the symptoms, improvements in their treatment and more effective ways to monitor them.

  • Cystic fibrosis and the lungs

    The main organ affected by CF is the lungs. The lining of the lungs builds up with thick, sticky mucus that can be hard to clear, leading to long-lasting and hard to treat infections. In CF the body also has a more active than usual immune system. This means that during lung infections the body’s immune system overcompensates and inflammation is triggered, which in turn leads to permanent lung damage.

    We are funding a large portfolio of research to understand, diagnose and treat lung infections, covered here. More detail on the research we’re funding to understand the underlying biology of lung symptoms is covered in our information on tackling the underlying cause of cystic fibrosis.

    Improving how the lung health of people with CF is monitored is also important area of research for the early detection of lung infections and assessing the effectiveness of new CF treatments.

    Improving how lung health is monitored

    New, more accurate and sensitive measures of lung health are needed, particularly for young children and those on CFTR modulator medicines such as Kaftrio who may be experiencing subtle changes that current methods cannot detect. This information could be used to manage their condition from day to day, for example spotting flare ups of lung infection. Monitoring lung health is also important to find out the effectiveness of new treatments being tested in CF clinical trials.

    We’re funding a VIA study in improve the accuracy of Lung Clearance Index meaurements that are increasingly being used in CF clinics and in testing the effectiveness of new medicines.

    We are also funding the ‘Pulmonary Magnetic Resonance Imaging for Cystic Fibrosis (MAGNIFY SRC) led by Prof Jim Wild at University of Sheffield, where researchers will investigate whether an exciting new type of magnetic resonance imaging (MRI) could be used to track subtle changes in lung health over time.

    Read more about research we’re funding on this topic, through our strategic research centre (SRC) and Venture and Innovation Award (VIA) funding schemes:

    Read more about MAGNIFY SRC

    VIAs on new approaches to monitor lung function

    Understanding and treating infections section of the website

    Tackling the underlying cause of CF section of the website

  • Cystic fibrosis diabetes

    According to the 2020 UK CF Registry report, more than one in three adults will develop CF diabetes (CFD) requiring those affected to manage their blood sugar levels through careful dietary monitoring and regular insulin injections. This is in addition to an already time-consuming daily treatment regime to manage other symptoms of cystic fibrosis.

    Blood sugar levels are controlled by the release of insulin from a specific part of the pancreas. In CFD not enough insulin reaches the blood. Professor James Shaw at the University of Newcastle is leading a Strategic Research Centre to investigate how signals from the rest of the pancreas affect how and whether CFD develops. Understanding more about these signals could lead to entirely new approaches to treating CF diabetes, avoiding the need for insulin injections.

    Read more about research we’re funding on this topic, through our Strategic Research Centre (SRC) and Venture and Innovation Award (VIA) funding schemes:

    Research in focus report on CF diabetes (CFD)

    SRC on how disease in the digestive-juice-producing part of the pancreas leads to CFD

  • Gastrointestinal (gut) symptoms

    The CF community told us that the question “How can we relieve gastro-intestinal (GI) symptoms, such as stomach pain, bloating and nausea?” is an important research priority for scientists and clinicians to tackle. 

    Treatments for gut symptoms of CF are often ineffective because doctors do not fully understand why symptoms occur. By recruiting people with CF with gut symptoms in our GRAMPUS CF SRC, the researchers aim to link specific gut symptoms to changes seen within the intestines. When these links have been made, other colleagues within the SRC will conduct lab studies to understand what triggers these changes to take place and investigate possible symptom-specific medicines that may be effective to treat them. The SRC will be led by Professor Alan Smyth at the University of Nottingham and a multidisciplinary team of researchers.

    We have funded a number of other studies in this area, which are outlined in our Research in focus report on gut symptoms, including better ways to monitor gut symptoms and laboratory-based studies investigating better treatments for distal intestinal obstruction syndrome or DIOS.

    Read more about research we’re funding on this topic, through our strategic research centre (SRC) and Venture and Innovation Award (VIA) funding schemes:

    Research in focus report on gut symptoms

    GRAMPUS CF SRC to develop more symptom-specific treatments for CF gut symptoms

  • Cancer and cystic fibrosis

    As the general population get older, they start to become affected by a range of different diseases and conditions, such as cancer. The same will be true for people with CF; however, they will likely be affected by conditions like cancer in different ways. It is important to explore these differences, in order to reduce their impact.

    There is some research to suggest that people with CF may be more at risk of developing cancer than the general population. More information about this can be found on the cystic fibrosis complications page of our website.

    While more evidence about the chances of people with CF developing cancer are being explored, biomedical scientists within an SRC programme led by Professor Stephen Renshaw at the University of Sheffield Medical School, are investigating whether or how disruption to the normal workings of the CFTR protein might directly or indirectly lead to the development of bowel cancer.

    Alongside this, the SRC team will explore whether a class of existing and well used drugs could be beneficial in preventing any damaging effects of the mutated CF protein.

    Read more about this topic:

    Q&A with Professor Daniel Peckham on risk of people with CF developing cancer

    More about bowel cancer

    SRC Understanding and preventing bowel cancer in cystic fibrosis

  • Digital health

    There are so many factors that contribute to an individual’s experience of the condition that ultimately the best treatment is likely to be tailored to that person. Our digital health research programme is exploring how this might be realised. This includes: investigating the feasibility and psychological aspects of collecting sufficient timely information about day-to-day changes in someone’s health, to which are the most important measures of health to monitor, and whether changes to current models of care are safe and acceptable.

    Read more about our Digital Health research programme.

Research we fund

We fund research to tackle some of the most pressing issues in CF today. Find out how your donations are making a difference.

What is CF?

Cystic fibrosis, or CF, affects the lungs, digestive system and other organs. There are around 11,000 people living with it in the UK.

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