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What did the project achieve?
“Our work has highlighted certain mutations that can arise in Pseudomonas bacteria living in the nose which may help them to survive in the lungs,” says Professor Jo Fothergill of the University of Liverpool. “Understanding how these bacteria can adapt and evolve to living in the lungs could ultimately lead to effective new ways to prevent or treat long-term infections, which would hugely improve the outlook for children with cystic fibrosis.”
Cystic fibrosis (CF) is a genetic condition affecting over 11,300 people in the UK, including 3,800 children and babies.1 Sadly, two people will lose their lives to the disease every week.2 A major cause of lung damage and reduced life expectancy is persistent chest infection with a bacterium called Pseudomonas aeruginosa. If these infections are diagnosed quickly, it’s possible to clear the lungs using intensive antibiotics – but if a long-term infection sets in, it’s almost impossible to eliminate these bacteria from the lungs.
Prof Fothergill’s team examined the genetic code of Pseudomonas bacteria living in the nose, throat and lungs of a laboratory model of long-term infection – to look for gene mutations that may help to adapt and evolve during the early stages of infection in ways that increase their ability to spread through the lungs, persist there and become resistant to antibiotics.
“We found two mutations in Pseudomonas bacteria that improved their ability to survive in the lungs,” says Professor Fothergill. “We subsequently found these changes are commonly found in bacteria from the lungs of children with CF with long-term infections.”
The researchers then carried out experiments to explore how these mutations may alter the biological characteristics of the Pseudomonas bacteria.
“We identified two key processes which are disrupted in Pseudomonas bacteria, both of which may contribute to their increased potential to infect the lungs,” says Professor Fothergill. “One is how they interact with molecules in the body – and the other affects how the bacteria acquire iron, an element known to be essential for the survival of bacteria during infections.”
Previously, both of these genes had been linked with antibiotic resistance – but these new results suggest that the situation is more complicated.
“These mutations may be a marker of adaptation – offering potential opportunities to spot dangerous Pseudomonas infections early on so that children can have treatments to clear the infection before it becomes established,” says Professor Fothergill. “They could also offer new clues about how to prevent the bacteria from adapting to living in the lung and the development of chronic infection.”
References
- Cystic Fibrosis Registry 2023 Annual Data Report (2024), Cystic Fibrosis Trust. London https://www.cysticfibrosis.org.uk/sites/default/files/2024-11/CFT_2023_Annual_Data_Report_Oct2024%201.pdf.[Accessed March 2025]
- British Lung Foundation. Cystic Fibrosis statistics: https://statistics.blf.org.uk/cystic-fibrosis [Website accessed 09 May 2022]
This research was completed on
Around 10,800 people in the UK – including over 4,000 children – have an inherited lung condition called cystic fibrosis (CF).1 Children with CF are susceptible to chest infections which can be difficult to treat, often persisting for the rest of a child’s life and reducing life expectancy. Dr Jo Fothergill, of the University of Liverpool, is investigating what makes these 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.
Action Medical Research and the Cystic Fibrosis Trust are together funding this study.
How are children’s lives affected now?
One in 2,500 babies born in the UK has CF, a serious condition with no cure that’s typically diagnosed when babies are just 26 days old.1,2 Symptoms of CF include a persistent cough, shortness of breath and recurrent chest infections, with some people eventually needing a lung transplant.
Around one third of children with cystic fibrosis (CF) in the UK develop a long-term lung infection with the bacterium Pseudomonas aeruginosa by their late teens.1
“Long-term lung infections with Pseudomonas bacteria are a major cause of ill health in children with CF,” says Dr Fothergill. “Sadly, these infections have been linked to a more rapid decline in lung function, more frequent periods of ill health and a shorter life expectancy. Families and patients have described these infections as ‘devastating’.”
“If Pseudomonas infections are diagnosed quickly, it’s possible to get rid of them using intensive antibiotic treatments,” adds Dr Fothergill. “However, if a long-term infection sets in, it’s almost impossible to eliminate Pseudomonas from the lungs.”
How could this research help?
Researchers are investigating why Pseudomonas infections, which are usually harmless and short-lived for people with healthy lungs, can become so difficult to treat and long-lasting for children with CF.
“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 that 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 CF. If so, taking regular nasal swabs may enable doctors to spot potentially dangerous Pseudomonas infections sooner, so children can have treatments that clear the infection before it becomes established.
Long-term infections with Pseudomonas bacteria are key concerns for children with CF and their families. Finding ways to prevent them would greatly improve the outlook for children with CF.
References
1. Cystic Fibrosis Trust. UK Cystic Fibrosis Registry. 2015 Annual Data Report. Published August 2016. https://www.cysticfibrosis.org.uk/the-work-we-do/uk-cf-registry/reporting-and-resources Website accessed 10 October 2016.
2. NHS Choices. Cystic fibrosis. http://www.nhs.uk/conditions/Cystic-fibrosis/Pages/Introduction.aspx Website accessed 12 October 2016.
| Project Leader | Dr Jo L Fothergill BSc (Hons) PhD |
| Project Team | Dr Dan R Neill MA PhDProfessor Craig Winstanley BSc PhDProfessor Aras Kadioglu BSc (Hons) PhDDr Kevin W Southern PhD FRCPCH MBChB |
| Project Location | Department of Clinical Infection, Microbiology and Immunology, Institute of Infection and Global Health, University of Liverpool |
| Project Location Other | Department of Women's and Children's Health, Alder Hey Children's Hospital, Liverpool |
| Project duration | 30 months |
| Date awarded | 6 September 2016 |
| Project start date | 1 August 2017 |
| Project end date | 15 September 2021 |
| Grant amount | £185,269 |
| Grant code | GN2444 |
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