Cystic fibrosis

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Cystic fibrosis

Cystic fibrosis is a chronic and progressive disease, usually diagnosed in childhood, that causes mucus to become thick and sticky. The mucus builds up and clogs passages in the lungs, pancreas, and many other organs in the body.

• In the lungs, cystic fibrosis causes respiratory problems when thicker-than-normal mucus forms in the airways and lungs. Children who have cystic fibrosis almost always have breathing problems and frequent lung infections.
• In the pancreas, the mucus blockage can interfere with normal digestive processes and increase the risk of infection. Babies and children who have cystic fibrosis may not be able to absorb nutrients from food and may have below-normal growth and development. Weight loss and difficulty gaining or maintaining weight are common problems for people of all ages who have cystic fibrosis.

Early symptoms of cystic fibrosis include abnormally salty sweat or skin and a failure to thrive, which includes a poor appetite, lack of energy, and weight loss during infancy. Some babies who have cystic fibrosis are born with a blocked small intestine. Later symptoms include coughing up mucus and a lack of energy. Adults who have cystic fibrosis may have fertility problems.

There is no cure for cystic fibrosis. Management of the disease varies from person to person and generally focuses on treating respiratory and digestive problems to prevent infection and other complications. Treatment usually involves a combination of medicines and home treatment methods, such as respiratory and nutritional therapies.

Credits

Last updated:	May 25, 2007
Author:	Caroline Rea, RN, BS, MS
Reviewed By:	Kathleen Romito, MD - Family Medicine
Editors:	Susan Van Houten, RN, BSN, MBA, Pat Truman