Taking a Look at Cystic Fibrosis

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INTRODUCTION Cystic fibrosis (CF) is an inherited autosomal recessive disorder that affects the lungs and digestive system most often. In the United States some 30,000 children and adults have CF. There are approximately 1,000 new cases of cystic fibrosis diagnosed each year in the US with 70% of patients diagnosed with CF by the age of two, 40% of patients with CF are 18 or older. In the 1950's most children with CF did not survive to attend elementary school, but in 2006 the median age of survival was 37 years (Cystic Fibrosis Foundation, 2007). The overarching effect of cystic fibrosis is the production of a thick mucus from the epithelial layer of the respiratory, digestive and reproductive systems. In the lungs, a thick layer of mucus accumulates and makes it difficult for individuals with cystic fibrosis to breath. In the pancreas, the ducts are clogged with mucus and digestive enzymes are unable to reach the small intestine. Failure to secrete digestive enzymes leads to malnutrition, failure of growth and late development in children. In the intestines, both small and large, the accumulation of the abnormal mucus can lead to bowl obstructions. In the male reproductive system the vas deferens is often clogged by the thick mucus and damaged leaving most males infertile when they reach puberty. Although 97% of men with cystic fibrosis are infertile they are not sterile. Some women have fertility difficulties due to thickened cervical mucus or
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