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Cystic Fibrosis Research Paper

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Cystic fibrosis is known to be one of the most common and deadly diseases in Caucasians, affecting 1 in 2500 children. This percentage results in 30,000 individuals within the United States to be diagnosed with CF. There are over 1900 mutations of this gene that cause a wide variety of severities within this disease. (McCance, Huether, Brashers, & Rote, 2010) Due to its complex mutation and unknown cause, only treating the symptoms of CF have been the main treatment protocol to this disease. Current treatments are cumbersome and expensive providing patients with life expectancy only into their twenties, but usually younger in most cases. There has been specific progress towards a cure involving gene therapy providing hope for a cure to …show more content…

CF is broken into 6 classes of the disease. Class 1 through 3 appears to be more severe than the latter 4 through 6; which mainly result in only pancreatic deficiency. The rate of deaths due to cystic fibrosis is dependent on the class of disease and age of diagnosis which can range from 6 months old to 10 years old. The average age of survival, with treatment, in the United States is 25 years old. Abnormal secretions cause respiratory, digestive and reproductive obstructions. Respiratory failure is caused by mucous impaction of airways due to plugging, chronic inflammation and infection. Respiratory infections are the main cause of death. A person with cystic fibrosis has a persistent cough or wheeze resulting in some sputum production and reoccurring pneumonia. This excess mucus creates a chronic bronchiectasis within the lungs resulting in cyst and peripheral bullae formation. (McCance et al., 2010) Excessive coughing called coughing fits normally allow a person to clear their airways and remove the excess fluid from their lungs. However, in CF when a person has a coughing fit they produce very little mucus excretion and do not have any symptom relief. This could cause a rupture of one of the many peripheral bullae leading to pneumothorax, a collapsed lung. (McCance et al.,

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