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Overview of Cystic Fibrosis

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Cystic Fibrosis is a heredity disease that causes severe damage to one’s digestive system and lungs, as it greatly affects the cells that produce mucus, sweat, and digestive juices.  Cystic Fibrosis causes these secretions to plug up tubes and ducts, including those in the lungs and pancreas. It is caused by a mutation in a recessive gene, therefore both parents must be carriers of the recessive trait to pass a risk of Cystic Fibrosis onto offspring.

Cystic Fibrosis is diagnosed through newborn screening, which checks for higher-than-normal levels of the chemical IRT, which is released by the pancreas. Two further tests are available, as well.  One is a sweat test, usually done on a one-month-old infant, which analyzes the sweat to determine if it is saltier than normal.  Secondly, genetic testing can be done on DNA samples—blood or saliva—to test for the specific defects of the gene responsible for Cystic Fibrosis.  Tests can also be done on older children if they exhibit an array of symptoms common with the disease.  Tests are also done to help a patient maintain their condition, including imaging tests to monitor damage to lungs and intestines, lung function tests, tests analyzing spit for bacteria, and blood tests to determine the health of the pancreas and liver.  Additionally, patients over the age of ten are also regularly screened for diabetes.

Symptoms vary greatly between patients and even during the lifetime of one individual.  Because of the thick mucus associated with the disease, a persistent cough, repeated lung infections, and inflamed nasal passages are common.  Because this mucus can also block the tube from the pancreas that secrete digestive enzymes to the small intestine, intestinal blockage, severe constipation, and poor weight gain are also frequently seen in patients. Foul-smelling stools are digestive signs of the presence of the disease.  In infants and young children, if any arise, parents are to contact a doctor.

Because there is no cure for cystic fibrosis, the goal of treatment is to focus on lung infections, mucus buildup in the lungs, intestinal blockage, and nutrition.  There are an array of options for medication, including antibiotics for lung infections, mucus-thinning drugs to help improve lung function, bronchodilators to relax muscles around bronchial tubes to help keep airways open, and oral pancreatic enzymes to be taken at meal times to help absorb nutrients.  Furthermore, physical therapy can be focused on the chest to help loosen mucus buildup in the lungs, including an inflatable vibrating vest and breathing devices. Pulmonary rehabilitation for long-term care is also used.

In advanced cases, surgery is also utilized.  These include removal of nasal polyps, feeding tubes to aid in digestive problems, and removal of bowel blockage.  If life-threatening lung complications arise and persist, a lung transplant is an option.  Typically, both lungs need to be replaced as they are both affected by the disease.  However, this option also comes with controversy because complications are common, and many question whether it enhances a patient’s quality of life.



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