Understanding and treating symptoms of CF VIAs

The Trust is funding research to reduce the impact of a range of different symptoms and complications of cystic fibrosis (CF). These include better ways to diagnose, monitor and understand GI symptoms, refining and improving ways to monitor lung symptoms, and managing CF-related diabetes through the diet.

CF and the lungs

Quantifying bronchiectasis severity in cystic fibrosis

Dr Joseph Jacob, UCL

Our contribution: £50,000
Partner contribution: UCL, £50,000

Performing CT (computerised tomography) scans can help assess lung damage in cystic fibrosis. Currently, scans are analysed manually and it’s difficult to spot small changes within the lungs or monitor changes that happen over time. We’re co-funding a PhD student to develop a method to identify more subtle signs of lung damage using machine learning. This faster, more detailed analysis could assist doctors in assessing lung function as part of day-to-day care. It may also be used by researchers to assess the benefits of drugs in future clinical trials.

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The idealised LCI (i-LCI): tuning in on the ‘silent years’ of paediatric CF

Professor Jane Davies, Imperial College London

Our contribution: £98,600
Partner contributions: Engineering and Physical Sciences Research Council (EPSRC), £500,000, Imperial College London, £50,000

 

Standard tests of lung function are not sensitive enough to pick up lung damage in young children (aged between two and six years old). Professor Davies is collaborating with researchers at the University of Oxford to develop a new, more sensitive version of the lung clearance index (LCI). Over three years, it will be tested in gradually younger people with cystic fibrosis. If successful it has the potential to distinguish between permanent lung damage (such has lung scarring) and temporary narrowing (caused by excess mucus).

CF-related diabetes

Dietary manipulation to improve glycaemic control

Ms Laura Birch, University of Bristol

Our contribution: £25,000
Partner contributions: NIHR, £269,498

People with CF-related diabetes (CFRD) need to closely monitor their diet to control their blood sugar levels and ensure they’re eating enough calories. It is a difficult balancing act to achieve both and there is little evidence to guide practice on how best to achieve this. Research dietician Laura Birch is investigating whether following a low glycaemic index diet is feasible for people with CF, and whether this is a useful and acceptable way for them to manage their diabetes. The findings of this study will inform the design of future, large-scale studies.

Gastrointestinal symptoms

Gut Imaging for Function & Transit in CF - The GIFT CF study

Professor Alan Smyth, University of Nottingham

Our contribution: £35,000
Partner contributions: CF Foundation and Vertex Pharmaceuticals, £266,000
 

To understand the best way to treat GI tract complications, it’s important to understand how the gut functions normally, and how it’s different in cystic fibrosis. The current ways to study gut function involve passing tubes into the gut or the use of Xx-rays. Professor Smyth is investigating whether MRI is a safer and less invasive alternative to these methods. If successful, he will extend the study to look at whether taking the CFTR modifying drug Orkambi has any effect on GI movement.

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Organization and function of the gut microbiota in cystic fibrosis

Professor Chris van der Gast, Manchester Metropolitan University

Our contribution: £47,000
Partner contribution: Manchester Metropolitan University, £47,000
 

Everyone has a range of bugs living in their intestines that contribute to keeping them healthy. In people with CF it is likely that how these bugs live and grow is different compared to people who don’t have the condition. The aim of this PhD research is to gain a better understanding of how the natural bugs are affected in cystic fibrosis. This could lead to more effective management of GI symptoms of CF such as DIOS.

Living with CF and its symptoms

Studying the effects of environmental factors on cystic fibrosis

Dr Fred Piel, Imperial College London

Our contribution: £85,000
Partner contribution: Imperial College London, £85,000
 

More information is needed about how environmental factors affect cystic fibrosis. 

We are funding two PhD students to develop this information. They have access to many detailed environmental datasets that can be overlaid with information about cases of CF, to identify any links between them. The factors they’re investigating include air pollution and geographical patterns of lung infection. This information can be used in the future to develop lifestyle advice and to campaign for changes in practices to reduce these factors.