Love your lungs: what it’s like to have cystic fibrosis and lung infections

Dr Lucy Allen

Director of Research and Healthcare Data, Cystic Fibrosis Trust

Cystic fibrosis (CF) is an inherited genetic condition that affects over 10,900 people in the UK. The condition is caused by a faulty gene which controls the movement of salt and water in and out of your cells.

Cystic fibrosis affects many organs and tissues within the body including the lungs, which become clogged with thick, sticky mucus, making it hard to breathe and clear away infection-causing bugs and particulates in the air.

People with cystic fibrosis are extremely vulnerable to lung infections, which can be hard to treat, hard to detect and resistant to antimicrobial medicines. These infections can be picked up from the environment or by coming into contact with other people with the condition. While these infection-causing bugs are usually harmless to people who don’t have CF, they can cause permanent lung damage for those who do.

Living with long-term lung infections

The bacterium Pseudomonas aeruginosa (P. aeruginosa) causes serious, long-term infections in the lungs of some people with CF. The bacterium is found in many natural and domestic environments including plants, soils and surface water — especially warm, moist environments. Environments such as hydrotherapy pools and jacuzzis have been reported as a risk for people with cystic fibrosis.

Within the lungs of people with CF, P.aeruginosa infection can sometimes be kept under control by antibiotics. However, if it takes root, it can become resistant to antibiotics and cause serious problems.

People with cystic fibrosis are extremely vulnerable to lung infections, which can be hard to treat, hard to detect and resistant to antimicrobial medicines.

Candice King, involvement manager at Cystic Fibrosis Trust, shares her thoughts and experience living with the condition: “Having chronic pseudomonas lung infections means that, over the years, I’ve exhausted a number of antibiotics. Despite advances being made with modulator drugs — meaning many people with CF are living healthier lives — there are still plenty of people who can’t benefit from these, and lung infections can still cause serious lung damage. It’s important that research continues to make new advances and ensures everyone with CF can live a life unlimited.”

What are we doing about it?

Cystic Fibrosis Trustis funding several multidisciplinary research programmes to investigate how P. aeruginosa becomes so well adapted to live in the lungs of people with CF. With this knowledge, we hope researchers in the future will be able to develop better ways to detect and treat it. Research also is taking place to investigate the impact of pollution and air quality on the lungs of people with cystic fibrosis and their risk of infection.