Cystic Fibrosis




What is Cystic Fibrosis?

Cystic fibrosis (CF) is the most common fatal genetic disease in the United States today. CF affects approximately 30,000 children and young adults, but it involves some hundreds of thousands more.A handful of these people are represented in the photos throughout these pages. Some are friends or family and others have the disease themselves. All of them are waiting for a cure. Approximately 1,000 new cases are diagnosed each year, usually before three years of age.

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.McDR 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. 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. The treatment of CF depends upon the stage of the disease and which organs are involved. One means of treatment, postural drainage (also called chest physical therapy [CPT]), 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.

Cystic Fibrosis is not contagious but it is fatal .The median age of survival for a person with CF is 31 years. The defective CF gene was discovered in 1989, since that time CF research has grown with leaps and bounds. "In 1990, scientists successfully made copies of the normal gene, and added them toCF 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 patientwith CF. CF researchers modified a common cold virus to act as a delivery vehicle carrying thegenes to the CF cells in the airways. Several Foundation-supported studies are underway to test new versions of the cold virus, and other cutting-edge technology, such as fat capsules and synthetic vectors." this according to the CFF. For more information on CF gene therapy, see the CFF Fact Sheet entitled,Gene Therapy and CF.

 All information regarding this disease was found on the Cystic Fibrosis Foundation's Web Page. Please visit this site. It has some of the most up to date information about the disease.


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