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Cystic Fibrosis
Reviewed by Medicinsk Expert•Last updated: 2026-10-22•Lungs Breathing

Cystic Fibrosis

Cystic fibrosis is an inherited disease that causes thick, sticky mucus to clog the lungs and other organs, leading to chronic infections and breathing difficulties.

Overview

Cystic fibrosis (CF) is an inherited disease of the mucus and sweat glands. It primarily affects the lungs, pancreas, liver, intestines, sinuses, and sex organs. The condition causes the body to produce thick, sticky mucus that clogs the lungs, resulting in breathing problems and creating an environment where bacteria grow easily, leading to repeated infections and lung damage.

Symptoms & Diagnosis

The severity of CF varies significantly. Some individuals experience serious complications from birth, while others have a milder form that does not manifest until the teenage years or early adulthood. Symptoms may fluctuate over time.

Diagnosis is confirmed through various medical evaluations, including gene tests, blood tests, and sweat tests.

Treatment & Management

There is no cure for cystic fibrosis, but advancements in treatment have significantly improved life expectancy, with many patients now living into their forties, fifties, or older. Management strategies include:

  • Chest physical therapy
  • Nutritional therapies
  • Respiratory therapies
  • Medicines
  • Exercise

Frequently Asked Questions

irway clearance techniques, such as chest physiotherapy, percussion, or the use of oscillatory positive expiratory pressure devices, are essential for mobilizing and expelling the thick mucus that accumulates in the lungs. Most CF care teams recommend performing these techniques at least twice a day, often before and after meals, and before bedtime. Consistent daily practice helps prevent mucus plugging, reduces the frequency of infections, and maintains better lung function over time.
Cystic fibrosis is an autosomal recessive disorder, meaning a child must inherit two defective copies of the CFTR gene, one from each parent, to have the disease. If both parents are carriers (having one normal and one defective gene), there is a 25 percent chance with each pregnancy that the child will have CF, a 50 percent chance the child will be a carrier, and a 25 percent chance the child will have two normal genes. Carrier testing is recommended for couples planning a family, especially if there is a family history of CF.
Common signs of a CF exacerbation include a sudden increase in coughing, a change in the color or thickness of mucus (often becoming more yellow, green, or bloody), a drop in weight, increased fatigue, and a decrease in lung function measured by spirometry. Patients should also monitor for new fevers or chills. It is critical to contact a CF care team immediately if these symptoms appear, as early intervention with antibiotics and increased airway clearance can prevent severe lung damage.
Because CF affects the pancreas, the body often cannot digest fats and proteins efficiently, leading to malabsorption and poor weight gain. A CF diet is high in calories, fat, and protein to support growth and maintain muscle mass, which is vital for breathing mechanics. Patients typically need to take pancreatic enzyme replacement therapy with every meal and snack to help break down food. Unlike general healthy eating guidelines, CF patients are encouraged to consume more fat and calories than the average person to maintain a healthy body mass index.
Source: MedlinePlus, National Library of Medicine

MedlinePlus brings together authoritative health information from the National Library of Medicine (NLM), the National Institutes of Health (NIH), and other government agencies and health-related organizations.

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