Cystic fibrosis (CF) is a recessive hereditary disease that causes the mucous glands in the lungs and digestive tract to secrete unusually thick and sticky mucus. Instead of acting as a lubricant, these secretions clog the lungs and prohibit the pancreas from working efficiently, resulting in a number of secondary illness characteristics. CF affects approximately 30,000 people in the United States, and an additional 10 million are unaware that they are symptomless carriers of the disease. Because CF is an autosomal-recessive disorder, two recessive genes for the disease (one from each parent) are required for symptoms to develop.
Symptoms of CF include coughing and wheezing, poor digestive ability, salty-tasting skin, nose polyps, reduced growth, and infertility. There are thousands of mutations of the CF gene, which results in varied symptoms within the CF population. Adult CF patients also deal with health issues such as related diabetes and osteoporosis.
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Treatment of CF varies with the range of symptoms and the stage of the disease. Mucus clearing of the lungs is a common, daily treatment for CF patients. Other treatments include antibiotics for lung infections, drugs that thin mucus for better lung functioning and fewer infections, and pancreatic enzyme supplements. Individuals with CF are also advised to be physically active and to adhere to a nutritious (often high-calorie) diet. People who have been diagnosed with CF have a mean survival age of 33.4 years. However, recent advances in available treatments have resulted in an increased life span for those suffering with CF.
CF patients may suffer from psychological issues related to the disease. For instance, patients of all ages can have difficulty coping with living with their disease. CF patients may have problems sleeping and may withdraw socially. Other psychological problems could be present because of anxiety-provoking medical visits and prolonged hospital stays related to the illness. Social issues that arise surround the problems or stigma that might shroud the CF patient because of coughing, wheezing, and medication noticed by peers.
There may be special psychological issues related to children diagnosed with CF. Children may have difficulty understanding their diagnosis. Many children with CF may also have problems with developmental tasks because of the nature of the illness and the treatment regimens. In addition, there are often problems in adherence to treatment regimens, which can possibly lead to decreased pulmonary functioning. Some children with CF also exhibit behavioral problems that may be related to their condition and management of their disease. Many children face problems with their education due to illness and hospitalization resulting in missed schoolwork and fewer social interactions with children. In adolescence, CF patients may face increased anxiety and apprehension as their responsibility for their treatment increases. There are also possible issues with independence, feeling accepted by peer groups, and issues with planning realistically for the future. Infertility becomes an issue as individuals with CF age. Men with CF can become sterile, and women often find it difficult to carry a fetus full term because of limited lung capacity and other related problems. The CF patient may also battle with the decision of having children and possibly passing on the CF gene.
Early intervention is important so that CF patients can have the appropriate support during various times of need from the onset of their illness. It is crucial that early intervention is provided by individuals knowledgeable in the emotional and social aspects of living with CF.
There is currently no cure for CF, although medical advances have allowed those with CF to live longer and more comfortable lives. Quality of life for CF patients continues to improve, making the diagnosis of CF less inhibiting than in previous years. Although more research needs to be done from a medical and psychological aspect of CF, the outlook of the illness continues to look more and more positive.
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