Cystic fibrosis

Cystic fibrosis is a life-threatening condition that causes the glands that produce mucus, tears, sweat, saliva, and digestive juices to work improperly. Usually, secretions from these glands are thin and slippery. In cystic fibrosis, a defective gene causes the secretions to become thick and sticky. Instead of acting as a lubricant, the secretions can plug up the passageways, especially in the pancreas and lungs. The most dangerous consequence is respiratory failure.

Cystic fibrosis can affect all races and ethnic groups, but occurs mostly in whites. It is less common in black Americans, Native Americans, and Asian-Americans. There is no cure for cystic fibrosis. Those who have it may live a lifespan of 30 years. A parent may be a carrier of the trait without being affected. If both parents come from families with cystic fibrosis, there is a one in four chance that their children will have it.

Newborns with cystic fibrosis may develop a blocked intestine the first day or two after birth. Other signs in newborns include failure to grow, bulky foul-smelling greasy stools, and frequent respiratory infections.

Common symptoms of cystic fibrosis in children and young adults include salty-tasting skin, blockage in the bowels, foul-smelling greasy stools, thick sputum, chronic coughing or wheezing, frequent chest and sinus infections, and nasal growths. Additional signs may include delayed growth, enlargement or rounding of the fingertips and toes, cirrhosis of the liver, chronic diarrhea, protrusion of part of the rectum through the anus, and collapsed lung.

Diagnosis may be made by a sweat test to measure the amount of sodium and chloride in a person's sweat or a genetic analysis of a blood sample.

The goal of treatment is to help the person lead as normal a life as possible and slow down the progress of the disease. Treatment may include:

• oral antibiotics to prevent lung infections
• bronchodilators (drugs that open the airways)
• mucus-thinning drugs
• bronchial airway drainage with manual or mechanical percussion, clapping on the chest or back 2 or more times during the day and
• better nutrition (including special vitamins, oral pancreatic enzymes, and high-calorie nutrition)

Self-care may include a variety of strategies as recommended by your healthcare provider. Children need to be up-to-date on their immunizations. It is important to encourage your child to lead as normal and active a life as possible. Exercise is essential because it can loosen mucus and make the heart and lungs stronger. Avoiding any kind of smoke is crucial. Second-hand smoke is extremely harmful to people with cystic fibrosis. Drinking plenty of fluids is essential to loosening mucus. Good and frequent hand washing is important, too, because it can help stop the spread of infection.

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Disclaimer: This content is reviewed periodically and is subject to change as new health information becomes available. The information provided is intended to be informative and educational and is not a replacement for professional medical evaluation, advice, diagnosis or treatment by a healthcare professional.

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