Improving the diagnosis and treatment of CF lung infections and maintaining lung health

CF lung infections need to be detected and diagnosed as quickly and accurately as possible. This is important for new infections and for flare ups of long-term infections (exacerbations). Sputum samples are commonly used to detect lung infections. However, improved lung health from the use of CFTR modulators such as Kaftrio mean that sputum is much harder to produce. We are supporting research to find different ways of diagnosing infections and managing exacerbations. 

Can we detect lung infections from saliva samples?

We are co-funding a PhD student in the lab of Dr Rian Griffiths at the University of Nottingham through our Venture and Innovation Award scheme. The aim of the project is to develop a rapid and highly sensitive method for detecting infections from saliva. This will mean infections can be found earlier and treated more effectively. 

Lung infections caused by Pseudomonas aeruginosa (P. aeruginosa) are common in people with CF and can be difficult to treat due to antimicrobial resistance. Researchers will focus on detecting P. aeruginosa infections by measuring signalling chemicals the bacteria produce. 

Other bacteria and fungi grow alongside the bacteria P. aeruginosa in the lungs of people with CF. These other bugs also produce signalling chemicals. So, it is important to check the methods for detecting P. aeruginosa infection still work in the presence of other infections and are unique for this bacteria.

Diagnostic Target Product Profiles

We are a managing partner in the CF Antimicrobial Resistance Syndicate  (CF AMR Syndicate for short) alongside Medicines Discovery Catapult and LifeArc. The Syndicate is a cross-sector initiative that brings together leading experts in CF or antimicrobial resistance (AMR) from industry, academia, and the clinic, as well as people with CF, to accelerate the translation of CF antimicrobials to the clinic to bring new and effective treatment options to people with CF. The Syndicate’s patient focussed Target Product Profile on developing new diagnostics is an important guidance document to support industry-based researchers.

Read more about the Diagnostic TPPs

Understanding exacerbations (flare ups of poor lung health)

Understanding more about flare ups of long term infections (known as exacerbations) is a focus of several of the Innovation Hubs within our Translational Innovation Hub Network on lung health and lung infection in CF. This Network is funded in partnership with LifeArc. Programmes of workinclude finding triggers that cause flare ups, working out how to predict early signs of a flare up, and understanding how individuals with CF experience them. 

Find out more about our Translational Innovation Hub Network 

Bugs that cause CF lung infections change over time and can become resistant to antimicrobial medicines, meaning that medicines such as antibiotics don’t work as well or no longer work at all. This is known as antimicrobial resistance (AMR). AMR is a problem for treating many different infections around the world. We are funding research to find new medicines that are so urgently needed to treat CF lung infections.

Testing promising new antimicrobial medicines in the lab

After researchers have found that a chemical or compound could have potential benefits as a treatment for lung infections, the next step is converting that promising chemical into a new medicine. This is done by doing lots of rigorous tests, known as the ‘pre-clinical phase’ of development. If the results of the testing are positive, the medicine will then be tested in clinical trials. 

The CF AMR Syndicate’s patient focussed Therapeutic Target Product Profiles provide a guide to innovators developing new medicines to treat lung infections for people with CF. It provides insight into the needs of people with CF in developing new medicines, and support in how to test their effectiveness in the lab and in clinical trials. 

The aim of PIPE-CF Strategic Research Centre, co-funded by the Trust and Cystic Fibrosis Foundation is to create and check new pre-clinical methods to test the effectiveness of new CF antibiotic medicines. Professor Jo Fothergill at the University of Liverpool is leading a multidisciplinary team of researchers to advance our knowledge in this area. The research will make it easier for researchers to develop new antibiotics specifically designed to treat CF lung infections and is supported by the CF AMR Syndicate.

Find out more about PIPE CF Strategic Research Centre

Repurposing existing medicines as treatments for CF lung infections

Many different approaches are being studied to treat CF infections. Designing new antimicrobial medicines is one way to do this. Another is to use existing medicines that reduce resistance and improve the effectiveness of existing medicines. 

Previous Trust-funded research led by Professor Jane Daves at Imperial College London in collaboration with researchers at Aarhus University in Denmark has shown that an existing drug called Glatiramer Acetate (GA), approved for use to treat multiple sclerosis (MS), improves the effectiveness of medicines used to treat Pseudomonas aeruginosa lung infections in the lab. This work is being further funded by a grant from LifeArc.

We’ve recently awarded Prof Davies a Venture and Innovation Award to expand these studies to investigate if GA is effective at improving treatments for other CF lung infections.

Learning more about having different lung infections at the same time

The lungs of people with CF usually contain a mixture of many different bugs. These are known as polymicrobial infections. Researchers are beginning to understand that a bug’s ‘neighbours’ can change how it causes infection and how it responds to treatment. It’s important to find out more about this, to be able to treat CF lung infections as effectively as possible and ensure people with CF have as healthy lungs as possible.

We’re co-funding a VIA award in partnership with Cambridge University to fund a PhD student in Professor Martin Welch’s research group who is using innovative new methods to study polymicrobial infections in the lab. In particular, they are studying the effects of hard-to-treat strains of Pseudomonas aeruginosa and the effects of this infection on other bugs growing in the lungs.

Research within the  Translational Innovation Hub Network is also focusing on understanding the effects of polymicrobial infections on lung health.

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

More accurate and sensitive measures of lung health are needed, particularly for young children and those on CFTR modulator medicines, who may be experiencing subtle changes in lung health that current methods cannot detect. 

Using new MRI techniques to understand lung health in people with CF

Led by Prof Jim Wild and Dr Laurie Smith at the University of Sheffield, researchers in our ‘Pulmonary Magnetic Resonance Imaging for Cystic Fibrosis or MAGNIFY SRC’, are investigating an exciting new type of magnetic resonance imaging (MRI), known as hyperpolarised Xenon-MRI, that could be used to track subtle changes in lung health over time. 

Their studies include how their MRI approaches can improve knowledge of lung health in people with CF and the practicalities of introducing the techniques into more hospitals, to exploring software to interpret the results and the best way to support people with CF and their families to understand this new information.

Read more about MAGNIFY SRC

Could existing lung scanning methods be helpful in CF care? 

With funding from the Medical Research Council (MRC) and with additional funding from a Trust VIA award, clinical fellow Dr Mina King is investigating whether a type of lung scan known as oxygen-MRI can be used to provide more detailed information on the lung health of people with CF. Oxygen MRI scans are routinely available in hospitals across the country, but they are not used in CF care. Dr King hopes that these scans could help detect earlier signs of lung damage than other ways to monitor lung health in people with CF.