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 difficulty breathing, long-term lung infections, CF-related diabetes (CFRD), GI symptoms such as abdominal pain, bloating and diarrhoea, and joint pain. 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.

Monitoring the lung health of people with CF is important. This includes checking for the presence of infections, understanding how well the lungs are working and looking for the presence or extent of any lung damage. It is monitored as part of someone’s usual care and it is also used to measure the effectiveness of potential new medicines in clinical trials. However, new, more accurate and sensitive measures of lung health are needed, particularly for young children who may be experiencing subtle changes that current methods cannot detect. We’re co-funding collaborative research projects to develop two new approaches to monitoring lung function .

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

Cystic fibrosis-related diabetes

According to the 2019 UK CF Registry report, one in three adults will develop CF-related diabetes (CFRD) 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 CFRD not enough insulin reaches the blood. A Strategic Research Centre (SRC) led by Professor James Shaw in Newcastle is investigating why this might be happening. Prof Shaw and colleagues have found that there is no CF protein in the parts of the pancreas involved in insulin release. This is a really important finding as it has allowed the SRC team and other researchers in this field to eliminate this line of enquiry from future studies. This is also a call to action to researchers to look at the consequences of what’s happening in the rest of the pancreas to truly understand what’s causing CFRD.

Following on from this success, Professor Shaw will lead a second SRC, to investigate how signals from the rest of the pancreas affect how and whether CFRD develops. Understanding more about these signals could lead to entirely new approaches to treating diabetes, avoiding the need for insulin injections.

People with CFRD need to carefully monitor their diet to help control their blood sugar levels. As people with CF are advised to consume a calorie-rich diet it can be hard to balance this with keeping their blood sugars at the right level to manage their CFRD. Research dietician Laura Birch at Bristol University is looking at the feasibility of new ways to maintain this delicate balance.

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

Gastrointestinal symptoms

In 2017 the CF community told us that the question “How can we relieve gastro-intestinal (GI) symptoms, such as stomach pain, bloating and nausea?” was the second most important research priority for scientists and clinicians to tackle. Later that year, following a research workshop we announced funding for a programme of research investigating GI symptoms in more detail.

People with CF experience blockages in their guts such as constipation in their large intestine and Distal Intestinal Obstruction Syndrome (DIOS) if the blockage is caused in their small intestine. Existing treatments for DIOS are limited in their success, so more effective treatments are needed. Liverpool-based Professor Soraya Shirazi-Beechey is leading an SRC to understand more about what causes DIOS on a cellular level and the potential of a new type of drug to treat it.

Current methods to assess DIOS symptoms in people with CF are uncomfortable and require exposure to X-rays. A safer, less invasive method of examining symptoms using MRI has been used for other GI conditions such as irritable bowel syndrome. Professor Alan Smyth and colleagues at the University of Nottingham are investigating the feasibility of using MRI to assess DIOS  to help manage the condition, and the Trust is co-funding this research. As well as improving the management of DIOS in the clinic, the results will complement the SRC research in furthering our knowledge of this CF-related complication.

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

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:

Joint pain

More than one in ten people with CF will develop joint pain. Joint pain can be caused by a number of different factors around the joint, and it requires specialist understanding to correctly identify its source. One source of joint pain in people with CF is inflammation around the joints, known as CF-related arthropathy. As well as being found in the joints, inflammation contributes to CF-related lung symptoms. Within their SRC investigating joint pain, Professors Michael McDermott and Daniel Peckham and colleagues are researching improved clinical management and treatment for joint pain and a detailed investigation of the biology of inflammation in people with cystic fibrosis.

Living with CF and its symptoms

Treating the symptoms of CF isn’t always about receiving a drug treatment, it also requires people to adopt a particular lifestyle to stay healthy, for example being physically active. Getting enough physical exercise is important for people with CF as it helps them to keep fit and improve the clearance of mucus from their lungs. However, questions remain about the best way for people with CF to exercise, and how to develop an exercise plan that fits with daily life and keeps them motivated. Research programmes within our physical exercise SRC are seeking answers to these important questions.

Analysis of registry data, such as data from the UK Cystic Fibrosis Registry can identify new lifestyle environmental and socioeconomic factors that affect people with CF, and can also be used to understand more about factors that researchers are already aware of. Researchers within our CF-EpiNet SRC  are enhancing the ability of researchers to conduct this type of research, and conducting their own studies using the Registry data. Topics under investigation include looking at the quality of life of people with CF, the long-term effects of treatments, how socioeconomic factors affect health, and how health in childhood affects outcomes in later life. Findings from these studies can lead to ways to improve care and quality of life. The Trust is supporting two PhD students in the lab of Dr Fred Piel at Imperial College to understand more about the effects of environmental exposures on cystic fibrosis.

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

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.

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, and there are over 10,600 people living with it in the UK.

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