Respiratory
Medicines for Cystic fibrosis
An inherited condition that causes thick, sticky mucus to build up in the lungs and digestive system — managed with lifelong specialist care, and where new treatments have transformed the outlook.
Education and reference only. This explains which medicines are used and why, in plain language — it deliberately contains no doses and is not a substitute for advice from your doctor or pharmacist. Always discuss your own treatment with a qualified clinician, and check the BNF and the product labelling for prescribing detail.
Quick answer
What is Cystic fibrosis?
Cystic fibrosis (CF) is an inherited condition in which the body produces thick, sticky mucus that clogs the lungs and the tubes of the digestive system. It is caused by a faulty gene, and a child develops CF only if they inherit a copy from both parents (who are usually healthy carriers).
- How it is treated: CF is managed by specialist CF teams with a comprehensive, lifelong approach tailored to the individual.
- Self-care: Daily airway clearance and exercise, taking enzyme and other treatments consistently, a high-energy diet with nutritional support, keeping up with vaccinations, avoiding cross-infection with other CF patients, and attending specialist reviews all help people with CF stay well.
- When to seek help: CF is managed by specialist teams; people with CF should follow their plan and seek prompt advice for worsening chest symptoms or feeling unwell.
What it is
Cystic fibrosis (CF) is an inherited condition in which the body produces thick, sticky mucus that clogs the lungs and the tubes of the digestive system. It is caused by a faulty gene, and a child develops CF only if they inherit a copy from both parents (who are usually healthy carriers). In the lungs, the sticky mucus makes it hard to clear germs, leading to repeated chest infections and, over time, lung damage; in the digestive system, it can block the release of enzymes from the pancreas, causing problems digesting food and gaining weight. Symptoms often begin in early childhood, and in the UK it is usually picked up through newborn screening. It affects other parts of the body too, including increasing the risk of CF-related diabetes. Thanks to major advances in care and treatment, people with CF are living much longer, healthier lives.
How it is treated
CF is managed by specialist CF teams with a comprehensive, lifelong approach tailored to the individual. Care includes daily airway clearance (physiotherapy and exercises to clear mucus), treatments to thin mucus and open the airways, prompt and often preventive treatment of chest infections, and pancreatic enzyme replacement with meals plus nutritional support to maintain weight and growth. Regular monitoring watches lung function, nutrition, and complications such as CF-related diabetes and liver problems. A major advance has been a group of medicines (CFTR modulators) that target the underlying gene defect and have significantly improved health and outlook for many people with certain gene types. Vaccinations, avoiding cross-infection, exercise and psychological support are all part of care. The outlook has transformed, though CF remains a serious, lifelong condition needing intensive management.
For this condition, these medicines
Medicine classes used for Cystic fibrosis
Each links to a full, dose-free guide — what it is, how it works, who can and cannot use it, side effects, interactions and FAQs.
Beyond medication
Lifestyle and self-care
Daily airway clearance and exercise, taking enzyme and other treatments consistently, a high-energy diet with nutritional support, keeping up with vaccinations, avoiding cross-infection with other CF patients, and attending specialist reviews all help people with CF stay well.
When to get help
When to see a doctor
CF is managed by specialist teams; people with CF should follow their plan and seek prompt advice for worsening chest symptoms or feeling unwell. See a GP about a child with recurrent chest infections, poor weight gain or persistent digestive symptoms if CF has not been excluded.
Not sure how urgent it is? It is always OK to call NHS 111 for advice, day or night.
Answers
Cystic fibrosis: frequently asked questions
How do you get cystic fibrosis?
It is inherited — a child develops CF only if they inherit a faulty gene copy from both parents, who are usually healthy carriers. In the UK it is usually detected through newborn screening.
Has the outlook for cystic fibrosis improved?
Yes, dramatically. Advances in care and, more recently, medicines that target the underlying gene defect have significantly improved health and life expectancy, though CF remains a serious, lifelong condition needing intensive management.
Keep reading
Related articles
Sources
Where this is drawn from
- NHS — Cystic fibrosis
- Cystic Fibrosis Trust guidance
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