About Cystic Fibrosis

Cystic Fibrosis (CF) is a genetic disease affecting approximately 30,000 children and adults in the United States.

CF causes the body to produce an abnormally thick, sticky mucus due to the faulty transport of sodium and chloride (salt) within cells lining organs such as the lungs and pancreas, to their outer surfaces. This abnormal mucus clogs the lungs and leads to life-threatening infections. The thick CF mucus also obstructs the pancreas, preventing enzymes from reaching the intestines to help break down and digest food.

CF has a variety of symptoms. The most common are: very salty-tasting skin; persistent coughing, wheezing or pneumonia; excessive appetite but poor weight gain; and bulky stools. The sweat test is the standard diagnostic test for cystic fibrosis. This simple, painless test measures the amount of salt in the sweat. A high level of salt indicates that a person has CF.

The treatment of CF depends upon the stage of the disease and which organs are involved. One means of treatment, chest physical therapy, requires vigorous percussion (by using cupped hands) on the back and chest to dislodge the thick mucus from the lungs. Antibiotics are also used to treat lung infections and are administered intravenously, via pills, and/or medicated vapors which are inhaled to open up clogged airways. When CF affects the digestive system, the body does not absorb enough nutrients. Therefore, people with CF may need to eat an enriched diet and take both replacement vitamins and enzymes.

CF occurs in approximately one of every 3,200 live Caucasian births (in one of every 3,900 live births of all Americans). There are about 1,000 new cases of CF diagnosed each year. Most individuals are diagnosed by the age of three; however, nearly 8 percent of all newly diagnosed cases are 18 or older. According to the CF Foundation's National Patient Registry, one half of all individuals with CF live to the age of 31; however, one half do not. One in 31 Americans (one in 28 Caucasians) more than 10 million people is an unknowing, symptom less carrier of the defective gene.

A individual must inherit a defective copy of the CF gene one from each parent to have cystic fibrosis. Each time two carriers conceive a child, there is a 25 percent chance that the child will have CF; a 50 percent chance that the child will be a carrier; and a 25 percent chance that the child will be a non-carrier.