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Cystic Fibrosis: Defective System

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Cystic fibrosis is a disease that is continually affecting children and adults in the United States. This is an inherited and life-threatening disease which affects many organs in the body. According to the Center for Disease Control, there are an estimated 30,000 people affected by this disease today. There are also approximately 2,500 babies born each year with Cystic fibrosis and unfortunately there are millions of people unaware they even carry the gene for this disease.
Cystic fibrosis is an inherited, chronic disease of the secretory glands which directly affects the lungs and digestive system (NLM, 2015). To look more closely at a cellular level, there is a defective gene in which their protein product that regulates movement of sodium …show more content…

The signs and symptoms seen in each patient is different and vary greatly depending on the severity and stage of the disease. The respiratory system creates thick mucus that clogs the tubes blocking for the lungs ability to carry air in and out. Signs and symptoms possibly seen include a persistent cough capable of producing thick mucus, wheezing, chronic lung infections, inflamed nasal passages and decreased ability to exercise or complete any tasks that require stamina for long periods of time. The digestive system is also greatly affected by the thick mucus that develops. This mucus inhibits the tubes that carry the necessary digestive enzymes from the pancreas to the small intestines. These enzymes are important in aiding in nutritional absorption. This in turn can produce greasy, foul-smelling stool, poor weight gain, poor growth and development, chronic constipation and intestinal blockage (particularly in seen in newborns). More frequent in children, rectal prolapse can occur as there is frequent straining which causes the rectum to protrude outside the …show more content…

The main goal of any treatment for someone who has cystic fibrosis is to prevent and/or control lung infections, loosen and remove the mucus build up in the lungs, prevent and treat intestinal blockage and ensuring proper and adequate nutrition. If the treatment includes medications, antibiotics would be used to treat and/or prevent lung infections. To help get the mucus up and out of the lungs, mucus-thinning drugs will be used to improve lung function. By relaxing the muscles around the bronchial tubes, bronchodilators can help keep the airway open. Lastly are oral pancreas enzymes which help the digestive tract absorb nutrients. Non-medication treatments include chest physical therapy which helps the thick mucus loosen and in turn makes it easier to cough up. There is also something available called pulmonary rehabilitation which is something that can help improve lung function in an outpatient setting and this may include exercise training, nutritional education, breathing techniques to conserve energy and counseling and/or support groups. There are also several surgical procedures that can improve quality of life for patients with cystic fibrosis. These in removal of any nasal polys to help breathing, oxygen therapy to help prevent pulmonary hypertension or high blood pressure in the lungs, endoscopy and lavage treatments to

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