Cystic Fibrosis - Essay Example

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It occurs when both parents to a patient are carriers of the defective CFTR gene thereby contributing to the embryo. The carrier…
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Download file to see previous pages The CFTR protein is located in epithelial cells, which form a lining of major body organs. The CFTR protein in people without cystic Fibrosis controls the flow of salt and water through the epithelial walls. As a result, it retains mucus secreted thin and watery. The thin mucus is important since it protects the lining of the liver, lungs, pancreatic glands, reproductive organs and digestive organs. A Cystic Fibrosis patient who has a mutated CFTR gene, the passage of sodium chloride through the epithelial cells does not occur normally. The body lacks a healthy CFTR protein needed to maintain a balance of salt and water leading to a lot of salt in sweat and making the mucus produced by the mucous gland too thick and sticky. The thick mucus clogs and obstructs body organs, linings and passageways thus preventing the normal functioning of body organs (Bjorklund 15-16).
Cystic fibrosis causes acute respiratory problems; this is due to acute haemoptysis and pneumonia. The infecting organisms of patients who have pneumonia include Staphylococcus aureus and Pseudomonas aeruginosa. The body has a small amount of functional CFTR to bind with the infectious bacteria resulting in obstruction of the airways. As the obstruction increases, it becomes very hard for air to pass through during inhalation and exhalation. This leads to expansion of the alveoli leading to trapping of air in small tubes. Over time, this causes barrel-shaped chest leading to increased pulmonary artery pressure that in turn causes heart failure. In addition, thick and sticky mucus, which is the main symptom in cystic fibrosis patients, clogs the bronchioles and as a result, parts of the lungs become blocked off. The small air passage also becomes weak and loses the ability to work properly; this often develops to bronchiecstasis. The patient coughs a lot and wheezes while breathing. In addition, cystic ...Download file to see next pagesRead More
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