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A to Z: Cystic Fibrosis (CF)

Cystic fibrosis (SIS-tik fy-BRO-sus) is an inherited disease that primarily affects the lungs and digestive system.

More to Know

Cystic fibrosis (CF) is caused by a defective gene that changes the nature of bodily secretions. Instead of being thin and lubricating, mucus is thick and sticky, especially in the lungs and pancreas. This leads to breathing problems, lung infections, and difficulty digesting food properly. CF also can affect the liver, the sweat glands, and the reproductive organs.

Signs and symptoms of cystic fibrosis include frequent lung infections or pneumonia; persistent wheezing; persistent cough with thick mucus; bulky, light-colored, foul-smelling bowel movements or diarrhea; and failure to gain weight despite a good appetite. Someone with CF may also have very salty sweat, poor height growth, nasal polyps (small growths of tissue inside the nose), frequent sinus infections, and fatigue. Symptoms can be mild or severe.

Treatment for someone with CF usually includes pulmonary therapy (treatments to maintain lung function) and nutritional therapy (a high-calorie, high-fat diet with vitamin supplements). Pancreatic enzymes can be prescribed for better food digestion. People with CF also may need antibiotics to treat lung infections and mucolytic medication (a mucus-thinning drug) to keep mucus thin and flowing.

Keep in Mind

People with CF have to take special care of themselves, but thanks to a better understanding of CF and improved treatment, they can participate in school, work, and leisure activities. Meanwhile, researchers are searching for a cure and working on even better treatments to extend life expectancy and improve quality of life.

All A to Z dictionary entries are regularly reviewed by KidsHealth medical experts.

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