Cystic Fibrosis

Cystic Fibrosis

Dr. KennedyCystic fibrosis (CF) is the most common fatal genetic disease in the United States today. It affects approximately 30,000 children and young adults and occurs in approximately one of every 3,300 live births. Approximately 1,000 new cases are diagnosed each year, usually by the age of three. The median age of survival for a person with CF is 31 years.

CF causes the body to produce an abnormally thick, sticky mucus. This abnormal mucus clogs the lungs and leads to fatal infections. The thick CF mucus also obstructs the pancreas, preventing enzymes from reaching the intestines to 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, foul-smelling stools.

The basic defect in CF cells is the faulty transport of sodium and chloride (salt) within epithelial cells — which line organs such as the lungs and pancreas — to their outer surfaces.

One in 29 Americans — more than 10 million people — is an unknowing, symptomless 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.

The sweat test is currently 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.

Treatment depends upon the stage of the disease and which organs are involved. One means of treatment, postural drainage (also called 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.

Since the defective CF gene was discovered in 1989, the pace of CF research has greatly accelerated. In 1990, scientists successfully made copies of the normal gene, and added them to CF cells in laboratory dishes, which corrected the defective cells. The next major step was achieved in early 1993 when the first experimental gene therapy treatment was given to a patient with CF. CF researchers modified a common cold virus to act as a delivery vehicle — carrying the genes to the CF cells in the airways. Continuing studies are underway, but as in so many other areas, in the area of delivery on the promise, there is much more promise than delivery.

This is one disease which should be treated both conventionally and alternatively. Antibiotics have made it possible for patients to survive their teens which, until antibiotics came on the scene, was rare. Mainstays of complementary medicine treatment of CF is detoxification and dietary changes. Also, certain oral nutrients are known to thin the mucus secretion making it easier for the patient to expel the mucus. For best results this should be done under the supervision of a doctor experienced in nutritional medicine.

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