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Cystic fibrosis is the most common autosomal recessive genetic disease of white Indo-Europeans (Caucasians). Three main systems are usually affected by cystic fibrosis. These include the lungs and respiratory tract, the digestive tract (especially the pancreas and intestines) and the sweat glands. The lungs will normally have a thick mucus line them in cases of cystic fibrosis which requires physiotherapy to dislodge the mucus and create sputum. The digestive enzymes that would come from the pancreas are blocked by the thick mucus; thus the person afflicted with the disease has trouble digesting foods that are high in fat and protein. In cases that involve cystic fibrosis the salt that is lost during perspiration is much more than in "normal" situations.
The upper respiratory tract is normally lined with a little bit of mucus that is sent out of the lung by the constant movement of the cilia that line the respiratory tree. "It is clear from detailed research that poor mucus clearance is not due to uneven ciliary beating. However, there is no doubt that mucus is poorly cleared against gravity in the presence of bacterial infection" (Harris 13-14).
The pancreas itself secretes fluids that aid in the digestion and absorption of food in all of us. When cystic fibrosis is present these enzymes are not going where they are needed. Most of the time there is need for supplemental nutrients, supplemental minerals, and/or dietary management. In the case of dietary management there is a plan for seven to eight small meals throughout the day. "This meal pattern enables the patient to consume more food without feeling too full and enhances the utilization of nutrients" (Ekvall 391).
The sweat gland of a cystic fibrosis patient, when viewed under the microscope appears normal. The secretions that the gland gives off are where the abnormality occurs. "It is known that the basic defect in cystic fibrosis is expressed as an abnormal regulation of the movements of salt across the layer of cells that line certain specialized ducts such as the sweat gland duct" (Harris 26). This causes a great deal of salt loss and therefore requires most cystic fibrosis patients to ingest salt pills to compensate for the loss.
When all of the treatments are used together most patients of this fateful disease live long happy lives. The physiotherapy that they must endure is the most grueling for most of the patients. This involves coughing up as much of the sputum as possible while inhaling a moisturizing mist and getting pounded on the back to loosen the mucus deep within their lungs. This activity is especially frustrating in the adolescent years when resistance comes into play. Parents find that the teenage years are the most grueling because there is a rebellious stage that most teenagers go through anyway. Having this disease on top of that is almost asking for further trouble.
The well siblings of those who are afflicted with this disease do not see it as a problem. Early on in life they are observant of their parents\' frustrations, but later learn that this is just another facet of life that is dealt to certain individuals. They see that their parents do not treat them any differently than their sibling who has the disease. " \'We both have to do chores,\' \'She does the same amount,\' \'We get treated the same\' are not uncommon among those families with sick and well children" (Bluebond-Langner 201).
The life of the child with this debilitating disease is not always as easy as some of these previous books have lead one to believe. This disease affects the person who has it, the siblings in the household, anyone who comes into contact with the sick person, and especially the parents of the sick individual. Yes, the afflicted is the most directly influenced by cystic fibrosis, but it is the parents who must provide most of the care to the person afflicted. This is a disease that is primarily identified early in a child\'s life and treated from that day forward. As stated before special diets or extra vitamins can be required to help children digest their food properly, and antibiotics are given to fight lung infections. In addition to all of this is the rigorous task of physiotherapy. This must be
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Medicine, Clinical medicine, Health, RTT, Pancreas disorders, Cystic fibrosis, Pediatrics, Mucus, Sputum, Cystic fibrosis transmembrane conductance regulator, Ivacaftor
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