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Cystic Fibrosis

Cystic fibrosis (CF) is a genetic disease affecting approximately 30,000 children and adults in the United States. A defective gene causes the body to produce an abnormally thick, sticky mucus that clogs the lungsand leads to life-threatening lung infections. These thick secretions also obstruct the pancreas, preventing digestive enzymes from reaching the intestines to help break down and absorb food.

More than 10 million Americans are unknowing, symptomless carriers of the defective CF gene. An individual must inherit two defective CF genes ¾ one from each parent ¾ to have CF. Each time two carriers conceive, there is a 25 percent chance that their child will have CF; a 50 percent chance that the child will be a carrier of the CF gene; and a 25 percent chance that the child will be a non-carrier.

CF occurs in approximately one of every 3,200 live Caucasian births (in one of every 3,900 live births of all Americans). About 1,000 new cases of CF are diagnosed each year. More than 80 percent of patients arediagnosed by age three; however, nearly 10 percent of newly diagnosed cases are age 18 or older.

People with CF have a variety of symptoms including: very salty-tasting skin; persistent coughing, at times with phlegm; wheezing or shortness of breath; an excessive appetite but poor weight gain; and greasy, bulky stools. Symptoms vary from person to person due, in part, to the more than 1,000 mutations of the CF gene. The sweat test is the standard diagnostic test for CF. A sweat test should be performed at a CF Foundation-accredited care center where strict guidelines are followed to ensure accurate results. This simple andpainless procedure measures the amount of salt in the sweat. A high salt level indicates CF.

The treatment of CF depends upon the stage of the disease and the organs involved. Clearing mucus from the lungs is an important part of the daily CF treatment regimen. Chest physical therapy is a form of airwayclearance done by vigorous clapping on the back and chest to dislodge the thick mucus from the lungs. Other types of treatments include:TOBI® (tobramycin solution for inhalation), an aerosolized antibiotic used to treat lung infections; Pulmozyme®, a mucus-thinning drug shown to reduce the number of lung infections and improve lung function; and azithromycin, an antibiotic recently proven to be effective in people with CF whose lungs are chronically infected with the common Pseudomonas aeruginosa bacteria.

When CF affects the pancreas, as it does in the majority of patients, the body does not absorb sufficient nutrients needed to grow and to thrive. Most people with CF take daily vitamin supplements, eat a high-caloriediet, and take pancreatic enzyme replacements with every meal to help them digest their food.

According to the CF Foundation’s National Patient Registry, the median age of survival for a person with CF is 33.4 years. As more advances have been made in the treatment of CF, the number of adults with CFhas steadily grown. Today, nearly 40 percent of the CF population is age 18 and older. Adults, however, may experience additional health challenges including CF-related diabetes and osteoporosis. CF also can cause reproductive problems — more than 95 percent of men with CF are sterile. But, with new technologies, some are becoming fathers. Although many women with CF are able to conceive, limited lung function and otherhealth factors may make it difficult to carry a child to term.

There are more than 117 CF Foundation-accredited care centers across the United States that specialize in the diagnosis of CF and provide care to people with the disease. Staff at a CF care center includes physicians,nurses, nutritionists, respiratory therapists, social workers, genetics counselors, and other medical professionals.

The mission of the CF Foundation is to assure the development of the means to cure and control CF and to improve the quality of life for those with the disease. It funds medical research and care programs which areimproving the length and quality of life for people with CF.For more information about the CF Foundation and the programs and services available to people with the disease, or to learn how you can volunteer and help make a difference, please visit www.cff.org or call (800) FIGHT CF.