UK’s first cystic fibrosis innovation hub network

The UK’s first network of cystic fibrosis innovation hubs is being launched to accelerate the development of new diagnostic tools and therapeutic approaches to detect, treat and manage lung infections.

The not-for-profit medical research charity LifeArc and the Cystic Fibrosis Trust are jointly investing £15 million in the Translational Innovation Hub Network for Lung Health and Infection. The network consists of four Innovation Hubs, based at the universities of Cambridge, Liverpool, Manchester, and Imperial College London. Guided by the insights and experiences of people with cystic fibrosis, the network will address areas of unmet medical need and improve the way that lung health is managed in the UK.

The network is the first of its kind to be dedicated to improving lung health in cystic fibrosis. It is supported by LifeArc as part of its Chronic Respiratory Infection Translational Challenge, which focuses on progressing new medical and scientific discoveries that will improve the lives of patients with cystic fibrosis and bronchiectasis.

Dr Catherine Kettleborough, Head of Chronic Respiratory Infections at LifeArc says:

Even with the development of new treatments like Kaftrio, people with cystic fibrosis still face many challenges which impact their quality of life and life expectancy. The Innovation Hub Network is a unique approach to addressing some of these problems, using shared knowledge, partnerships and investment to accelerate new tests and treatments for people living with CF.

The Innovation Hub Network builds on a programme of work funded by the Cystic Fibrosis Trust as part of its CF Innovation Hub at the University of Cambridge. Dr Lucy Allen, Director of Research and Healthcare Data at the Cystic Fibrosis Trust explains:

We’re thrilled to be partnering with LifeArc and expanding our Innovation Hub programme, combining our expertise and exploring exciting areas of research to maximise the impact for people with CF.

Those with the condition are particularly susceptible to lung infections, meaning they often have to spend time in hospital having IV antibiotic treatments and this has a huge impact on all areas of their life. These new Innovation Hubs will help transform our understanding and lead the way to new ways to test and treat lung infections.

Cystic fibrosis is one of the UK's most common life-limiting inherited diseases, affecting over 11,000 people here and nearly 200,000 people worldwide. Despite recent advances in research, there is still no known cure and the median age of death is just 33. The condition is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, which results in mucus build-up in the internal organs, especially the lungs and digestive system. This leads to chronic chest infections, lung inflammation, and other complications such as digestive problems. For many people, managing their health involves a rigorous daily treatment regime including physiotherapy and oral, nebulised and occasionally intravenous (IV) antibiotics.

CF lung infections can cause breathlessness and difficulty breathing. They can cause major disruptions to day-to-day life and may lead to permanent lung damage. Quick and accurate detection and diagnosis is important for new infections and for flare-ups of long-term infections (exacerbations). Bugs that cause CF lung infections change over time and can become resistant to antimicrobial medicines. Further research into the lung health of people with CF including diagnosis and effective treatment of infections is urgently needed.

The Cambridge Innovation Hub will build on the success of the original CF Innovation Hub, using machine learning to help forecast future pulmonary exacerbations. Breath, cough, sputum and blood samples will be analysed using a range of analytical and “omic” approaches. Researchers will also use machine learning to predict the best combination of existing antibiotics for each individual with cystic fibrosis.

The team at the Imperial College London Innovation Hub will bring together microbiologists, healthcare professionals, breath scientists, cell and animal biologists, physiotherapists and even sniffer dogs to detect the pathogens, such as bacteria, that are often present in the lungs of people with cystic fibrosis. They will explore how these pathogens are affected by other bacteria and fungi. The aim will be to develop new predictive diagnostic tools for lung infections.

At the University of Liverpool, Innovation Hub scientists will use phage therapy to reduce the negative effects of antibiotics, including antibiotic resistance, in people with cystic fibrosis. The team will develop new combinations of phage, test the best way of giving them, and ensure that the UK can produce phage to medical standards. They will also use the latest technology to find the microbiological causes of pulmonary exacerbations and pave the way for other new treatment approaches. This hub will also train early career clinicians and scientists to deliver high quality research in the future.

Researchers at the University of Manchester Innovation Hub will study the cause of pulmonary exacerbations for people with cystic fibrosis by analysing blood, saliva, sputum and sweat samples. They will also gather spirometry results and record symptoms via a smartphone app. Researchers will then explore why individual patients sometimes respond in different ways to IV treatments for exacerbations and understand who will respond best to antibiotics. This research will hopefully lead to clinical trials testing different methods for preventing pulmonary exacerbations.

Sarah Sharp, 24 from London has cystic fibrosis. She experienced her first lung infection when she was a baby and has spent a lot of time in hospital over the years. Sarah explains:

This condition affects everything about my life, every single day. I have to adjust my life around my symptoms, in everything from work to friendships. The dream, for me, is to have less of a treatment burden and more time feeling like a valuable member of society. These new Hubs give me hope because improving research into the cause of exacerbations and the development of potential new treatments takes away a lot of my fears around what my health is going to look like in the future.