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Lung Syndromes

Cystic Fibrosis

Cystic fibrosis is an inherited trait that causes mucus and sweat glands to work improperly. It is a lifelong disease. Cystic fibrosis is the most common fatal genetic disease among white children. It is less common among other racial groups. A parent may be a carrier of the trait without being affected.

People with cystic fibrosis have a defect in a protein that helps to transport salt across cells. The DEFECTIVE PROTEIN allows too much salt to leave the body through sweat. Very low salt levels in the body can cause heartbeat problems or shock. This happens most often in hot weather. MUCUS throughout the body does not contain enough water in cystic fibrosis patients. It becomes too thick and clogs the lungs and digestive tract. Breathing becomes difficult. Over time, there is permanent lung damage from recurring bronchitis and pneumonia.

Newborns with cystic fibrosis may often have a blocked intestine. Eventually, the thick mucus blocks the ducts of the pancreas. The pancreas produces several important ENZYMES for digestion. Without them, a person does not properly absorb fat, protein, and several vitamins. This leads to various problems in growth, development, and blood clotting. Liver disorders may also develop.

Cystic fibrosis is DIAGNOSED by testing sweat and by testing lung or pancreas function. There is now a genetic test that helps confirm the presence of the disease or if someone is a carrier.

At present, there is NO CURE for cystic fibrosis. The aim of TREATMENT is to help the person lead as normal a life as possible. To combat salt loss, the person should generously salt foods and take sodium supplements. Treatment also includes oral pancreatic enzymes. It is recommended that a person be on a high calorie diet with a liberal amount of fat. Vitamin and mineral supplements are also needed. Fluids in large amounts should be encouraged. These patients need to maintain a normal weight.

Management of lung problems includes physical therapy, breathing exercises, and aerosol sprays to clear mucous from the lungs. Some people undergo a lung transplant to reduce the effects of the disease. Also, research on aerosol gene therapy has shown early promise in reducing lung symptoms.

For more information, contact the Cystic Fibrosis Foundation at (800) 344-4823 or (800) FIGHT CF. Visit their website at www.cff.org.

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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