Cystic fibrosis: protecting children from life-changing infections

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.