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Cystic fibrosis

Life-threatening lung infections

Cystic fibrosis is a serious, inherited lung condition with no cure. It makes children very vulnerable to chest infections and around one third will develop a long-term lung infection with Pseudomonas aeruginosa bacteria by their late teens.

In people with healthy lungs this is usually a harmless and shortlived infection, but for those with cystic fibrosis it’s a major threat which can cause long-term damage and reduce life expectancy. Families and patients have described these infections as ‘devastating’.

We’re currently funding two research projects that hope to find new ways to prevent and destroy Pseudomonas infections to help improve the outlook for children with cystic fibrosis.

Illustration of biofilm containing Pseudomonas aeruginosa
Illustration of biofilm containing Pseudomonas aeruginosa

Improving treatments

Identifying new ways to wipe out these bacteria

Pseudomonas aeruginosa bacteria are a particular problem for people with cystic fibrosis as they form a biofilm that coats the lining of the airways, acting as a barrier that stops antibiotics and immune cells from destroying the bacteria.

Dr Bharat and his team at the University of Oxford are investigating substances made and released by these bacteria to understand how they help them to form biofilms. They will also test specific small biological molecules to see if they can disrupt or prevent biofilms from forming, which they hope will improve the success of antibiotic treatment.

“Our work could open the door for enabling treatments to destroy these bacteria, helping children with cystic fibrosis overcome serious and potentially fatal infections,” says Dr Bharat.

This research is jointly funded by Action Medical Research and the Cystic Fibrosis Trust.

“If we could identify new ways to wipe out these bacteria, it would help children with cystic fibrosis live longer, better lives.” Dr Bharat

Illustration of bacterium Pseudomonas aeruginosa
Illustration of bacterium Pseudomonas aeruginosa

Preventing infections

Improving the outlook for children with cystic fibrosis

Dr Fothergill, of the University of Liverpool, is investigating what makes Pseudomonas infections so difficult to treat, and how to diagnose them sooner, with the ultimate aim of protecting children from lifelong infections and improving their outlook.

“We’re conducting laboratory studies into the possibility that Pseudomonas bacteria living in the nose and throat during the early stages of infection adapt in ways that increase their ability to spread through the lungs, persist there and become resistant to antibiotics,” says Dr Fothergill.

The team aims to find out whether bacteria, which have adapted to life in the lungs, have changes in their genetic code that can also be detected in nasal swabs taken from children with cystic fibrosis. If so, taking regular swabs may enable doctors to spot Pseudomonas infections sooner, so children can have treatments that clear the infection before it becomes established.

This research is jointly funded by Action Medical Research and the Cystic Fibrosis Trust.