--- title: "Cystic fibrosis: Symptoms & Treatment | HealthAnswers" description: "Cystic fibrosis — inherited lung and digestive disease, newborn screening, daily treatments, and NHS specialist centre care." url: https://healthanswers.co.uk/conditions/cystic-fibrosis/ robots: noindex --- # Cystic fibrosis: Symptoms & Treatment | HealthAnswers Health A–Z Cystic fibrosis — inherited lung and digestive disease, newborn screening, daily treatments, and NHS specialist centre care. Written by HealthAnswers editorial team Medically reviewed by [HealthAnswers UK doctor review panel](https://healthanswers.co.uk/our-doctors/) Reviewed 3 July 2026 ## Quick answer What is cystic fibrosis? Cystic fibrosis (CF) is an inherited condition causing thick sticky mucus — affecting lungs, pancreas, and other organs. Newborn heel prick screening in the UK detects most cases early. Daily treatment includes airway clearance physiotherapy, inhaled medicines, pancreatic enzyme capsules with meals, and high-calorie diet. CFTR modulator drugs (Kaftrio) transform life expectancy for many with eligible mutations. Specialist CF centres coordinate care from diagnosis through adulthood. See a GP if your child fails to thrive, has persistent chest infections, or salty skin — or if family history of CF with planning pregnancy (carrier testing). ## Who is this page for? This page is for people in the UK who want a plain-English overview of Cystic fibrosis, including when to seek help and what NHS care usually involves. It explains general information, not what is wrong with you personally. ## Who should skip this page? Skip this page if you need a diagnosis, a prescription, or emergency care. It cannot replace a GP, pharmacist, NHS 111 or 999. If you are in immediate danger, call 999; for urgent advice that is not life-threatening, call 111 in England, Scotland or Wales, or use your GP out-of-hours service in Northern Ireland. On this page 6 sections ## Key facts about cystic fibrosis - About 11,000 people in the UK have cystic fibrosis — median survival now mid-40s and rising with modulators. - Caused by CFTR gene mutations — most common F508del — autosomal recessive inheritance. - Lung disease is main cause of morbidity — chronic infection with Pseudomonas aeruginosa common. - Pancreatic insufficiency — greasy stools, poor weight gain — treated with Creon enzyme replacement. - Kaftrio (elexacaftor/tezacaftor/ivacaftor) treats underlying defect in ~90% of UK patients with eligible mutations. ## Cystic fibrosis — CFTR channelopathy **Cystic fibrosis (CF)** is **autosomal recessive CFTR mutation** → **defective chloride transport** → **thick dehydrated secretions** in **lungs, pancreas, bile ducts, reproductive tract**. **~11,000 UK CF population** — **life expectancy rising sharply** with **modulators** ## Organ involvement **Lungs:** - Mucus plugging - Chronic infection — Staph aureus → Pseudomonas → Burkholderia - Bronchiectasis - Haemoptysis - Pneumothorax **Pancreas:** - 90% exocrine insufficiency - CF-related diabetes — ~50% by age 30 **Other:** - Liver cirrhosis — focal biliary cirrhosis - Distal intestinal obstruction syndrome (DIOS) - Male infertility — congenital bilateral absence of vas deferens ## Newborn screening pathway **IRT on blood spot** → **repeat IRT or sweat test** → **genetics** → **CF centre within days** **Early Creon + physio** — **nutrition trajectory set in first year** ## Daily management **Airway clearance:** - PEP devices, autogenic drainage, percussion - Exercise as adjunct **Inhaled therapies:** - 7% hypertonic saline - Dornase alfa (Pulmozyme) - Bronchodilator before others **Antibiotics:** - Acute exacerbation — IV 14 days - Chronic Pseudomonas — inhaled colistin/tobramycin , oral azithromycin **Nutrition:** - Creon dosing — adjust to fat intake - Fat-soluble vitamin supplements ## CFTR modulators **Kaftrio (elexacaftor/tezacaftor/ivacaftor):** - Eligible ~90% UK mutation profile - FEV1 improvement , fewer IV antibiotics , weight gain **Access via NHS** — **specialist prescribing** ## Transition to adult care **Dedicated adult CF centres** **Mental health**, **employment**, **insurance** **Transplant referral** when **FEV1 <30%** or **rapid decline** CF is **chronic intensive self-care** — **specialist team + modulators** make **adulthood and parenthood realistic** for most born today. ## ! When to see a GP about cystic fibrosis CF is usually diagnosed via newborn screening — GP refers immediately to paediatric CF centre. See a GP if an older child has recurrent chest infections, failure to thrive, salty-tasting skin, or bowel obstruction (meconium ileus in neonates). Adults with late or mild CF may present with pancreatitis or infertility. Carrier testing before pregnancy if family history — GP or genetics referral. ## Common questions about cystic fibrosis What are the symptoms of cystic fibrosis? Babies — meconium ileus, failure to thrive. Children — persistent cough, wheeze, recurrent chest infections, bulky foul stools, poor growth. Adults — bronchiectasis, sinus disease, diabetes (CF-related diabetes), liver disease, infertility (men — absent vas deferens). Salty skin — classic clue. How is cystic fibrosis diagnosed? Newborn blood spot immunoreactive trypsinogen (IRT) raised — sweat chloride test over 60 mmol/L confirms. Genetic testing identifies CFTR mutations — guides modulator eligibility. Some variants cause mild or single-organ disease — pancreatitis-only presentations in adults. What is daily treatment for cystic fibrosis? Airway clearance physiotherapy twice daily minimum. Hypertonic saline or dornase alfa nebulised. Inhaled antibiotics for chronic Pseudomonas. Creon with all meals and snacks if pancreatic insufficient. High-calorie diet — 120–150% normal intake. Exercise encouraged. Annual flu vaccine, never smoke. What are CFTR modulators? Drugs correcting CFTR protein function — Kaftrio (Trikafta) for F508del and many other mutations — improves lung function, reduces exacerbations, helps weight gain. Ivacaftor alone for gating mutations. Not curative but transformative — lung transplant still needed for some advanced disease. Can people with cystic fibrosis have children? Yes — with planning. Most men have obstructive azoospermia — fertility via surgical sperm retrieval and IVF. Women often fertile but high-risk pregnancy — specialist obstetric care. Pre-implantation genetic diagnosis available for carrier couples. ## Sources - [NHS — Cystic fibrosis](https://www.nhs.uk/conditions/cystic-fibrosis/) - [Cystic Fibrosis Trust](https://www.cysticfibrosis.org.uk/) - [NICE — Cystic fibrosis](https://www.nice.org.uk/guidance/ng78)