What is Cystic Fibrosis :

I would like to take this opportunity to tell you what Cystic Fibrosis (CF) is, to share with you what a child and their families go through, and to tell you how YOU can help these children and young adults.

 

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    Cystic Fibrosis is a genetic disease affecting approximately 30,000 children and adults in the United States.  One in 31 Americans (one in 28 Caucasians) - more than 10 million people - is an unknowing, symptomless carrier of the defective gene.  An 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.  CF causes the body to produce 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.  The thick CF mucus also obstructs the pancreas, preventing enzymes from reaching the intestines to help break down and digest food.


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    Symptoms of CF vary.  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 Sweat Test is the standard diagnostic test for cystic fibrosis. This simple and painless test measures the amount of salt in the sweat. A high salt level indicates that a person has CF.

 

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    Sweat Test  is a simple, diagnostic procedure that determines the amount of sodium and chloride (salt) in the sweat. In the first part of the test, a colorless, odorless chemical, known to cause sweating, is applied to a small area on the arm or leg. An electrode is then attached to stimulate a weak electrical current to the area. Individuals may feel a tingling sensation in the area, or a feeling of warmth. This part of the procedure lasts approximately five minutes. The second part of the test consists of cleaning the stimulated area and collecting the sweat on a piece of filter paper or in a plastic coil. Thirty minutes later, the collected sweat is sent to a hospital laboratory for analysis. The entire procedure takes approximately one hour.  Children and adults with CF have an increased amount of sodium and chloride (salt) in their sweat. In individuals who have CF, the sweat chloride test will be abnormal from birth. Once a test result is positive, it is always positive. Sweat test values do not change from positive to negative or negative to positive, as a person grows older. Sweat test values also do not vary when individuals have colds or other temporary illnesses.

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Last modified: 19 May, 2008