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Cystic FIbrosis

ood cells is approximately 1000 times larger in people with cystic fibrosis than it is in the normal lung. The long, viscous strands of DNA from these cells are the hardest things for the body to clear away. Realization of this situation resulted in the development of Genentechs Pulmozyme (or DNAse), the first drug made specifically to address the problems of the Cystic Fibrosis lung, and one of the first biotechnology drugs. However, one cannot expect DNAse/Pulmozyme to repair scarred tissue. The advantage is only in lowering the viscosity of the mucus and reducing future damage. There is some evidence that it works better on younger patients (Ramsey, B. W., 1999). Thick secretions may clog the pancreatic duct and block the transfer of enzymes from the pancreas to the intestine, leading to an enzyme deficiency. These enzymes break down food and supply the body with nutrients that are necessary for proper growth. The fats and proteins pass straight through the digestive system and produce greasy, smelly bowel movements. This condition is now correctable with pancreatic enzyme supplements. These pills, which are derived from animal sources (usually pigs) contain varying quantities of lipase, protease, and amylase, which are taken with meals and allow people with Cystic Fibrosis to eat the same foods as non-affected persons. In addition to enzyme supplements, oral supplements to augment total caloric intake may be needed as a result of decreased gastric absorption (Smyth, 1995). Cystic fibrosis also affects the reproductive organs, although it affects men and women differently. Males are usually infertile, and females may experience reduced fertility due to thick secretions in the reproductive tract. Women with cystic fibrosis can have children, although the health of the mother may limit this. Childbearing is an exhausting process and some women with CF who have decided to have children have reported that it has seve...

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