Cystic fibrosis

Also called CF

A genetic condition causing thick mucus in the lungs and digestive system. Treatment has transformed the outlook in a decade.

What it is

Around 11,000 people in the UK have cystic fibrosis. It is caused by a faulty gene affecting the movement of salt and water in and out of cells, which makes mucus thick and sticky.

In the lungs, that mucus traps bacteria and causes repeated infection and progressive damage. In the pancreas, it blocks the enzymes needed to digest food, so people cannot absorb nutrition without replacement enzymes.

The last decade has changed the picture profoundly. Modulator therapies such as Kaftrio treat the underlying protein defect rather than the symptoms, and for the majority of people with CF they have substantially improved lung function and quality of life.

Signs you might notice

Diagnosed in almost all UK cases by newborn screening, so this section is about recognising deterioration rather than onset.

Increased cough, more sputum, or a change in its colour.

Breathlessness on less exertion than usual. Reduced lung function on home spirometry.

Weight loss, or worsening digestive symptoms.

Fever and feeling unwell, which means an exacerbation needing treatment now.

How it can affect day-to-day life

The treatment burden is enormous and rarely acknowledged: airway clearance twice daily, nebulisers, enzymes with every meal and snack, vitamins, and often several hours a day in total.

Cross-infection rules mean people with CF cannot meet each other, which removes the peer support every other long-term condition relies on. Online community is not an optional extra here, it is the only route available.

Nutrition is treatment. A high-calorie diet and correct enzyme dosing directly affect lung function and survival.

CF-related diabetes and reduced bone density are common as people live longer, and need active monitoring.

Supporting someone well

Protect the treatment time. Airway clearance skipped for a week costs lung function that does not come back.

Get enzyme dosing right, with every meal and every snack containing fat. Under-dosing is common and shows up as weight loss and abdominal pain.

Maintain strict infection control, and understand why two people with CF must not be in the same room.

Treat any exacerbation early and fully. Delayed treatment costs lung function permanently.

Support the mental health side. Depression and anxiety are significantly commoner in CF and in CF carers, and screening for them is now part of good practice.

Ask about modulator eligibility if the person is not on one. Access has widened repeatedly.

Where to get help

Care is from a specialist CF centre, with a multidisciplinary team. Nothing here should be managed by a GP alone.

Cystic Fibrosis Trust run a helpline on 0300 373 1000 and provide financial and benefits support.

Coughing blood, sudden chest pain or breathlessness needs emergency assessment.