A Study On Cystic Fibrosis

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You’re the Expert: Cystic Fibrosis Cystic fibrosis is an inherited genetic disease that currently affects approximately 30,000 Americans and 70,000 people worldwide. The isolated gene malformation affects the exocrine glands that results in alterations in the physiologic functioning of the respiratory, gastrointestinal, and reproductive systems (Ball and Bindler, 2014). The name cystic fibrosis refers to the characteristic cysts and fibrosis that occurs within the pancreas. It is the most common autosomal recessive disorder amongst Caucasians and is present at birth, hence its importance when studying pediatric nursing (Litin, 2003). The modern history of cystic fibrosis, or CF begins in 1938 when Dr. Dorothy Anderson, a pediatric…show more content…
The CTFR gene expression can be found in the apical membrane of skin cells in places such as the respiratory and gastrointestinal tracts. In the respiratory tract, a mutation in this gene causes excess secretions to be produced by the seromucous glands which in turn bog down the hair-like cilia, making the lungs unable to clear the excess fluid, bacteria or debris. This mutation can also cause this liquid-like layer to be thinner resulting in inefficient cilia. There is also an increased inflammatory response in patients suffering from CF, although the reasoning for this symptom is not yet quite understood. Altogether, these malfunctions lead to myriad of lung and respiratory issues including infection, a decrease in lung functioning, and eventually advanced lung disease (Davies and Bush, 2011). Typically in the gastrointestinal system, enzymes are produced by the pancreas in order to aid in digestion. These enzymes must travel through small channels to reach the pancreatic duct and the small intestine where it can digest food. In CF patients, these channels become blocked, typically due to impairment in the lubrication of the endothelial cells that line the gastrointestinal organs. When this occurs, decreased amounts of enzymes reach the intestine resulting in pancreatic insufficiency. These patients must ingest enzymes every time they eat to aid in the digestion

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