Sickle Cell Anemia

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Introduction Sickle cell disease (SCD) is an autosomal-recessive inheritance that results from a alteration in the gene responsible for the hemoglobin production. A healthy hemoglobin A is produced under normal circumstances, but for people with SCD they produce a sickle hemoglobin S. (Gill, V., 2010) Unlike normal hemoglobin A, hemoglobin S has a tendency to agglutinate, or clump together, when the oxygen supply is decreased. As a result, the red blood cells take on a rigid or “sickle” shape causing the diameter of the cells to increase relative to the size of normal red blood cells. These sickled cells block the blood vessels causing compromised blood supply to the tissues and bones, resulting in a vaso-occlusive crisis. (Jisieike, C., 2007) This crisis causes acute episodes of severe pain, leg ulcers and avascular necrosis. Although vaso-occlusive pain itself is not directly life-threatening, inappropriate or inadequate treatment can lead to unnecessary suffering and potentially fatal complications related to the disease. SCD is a long-term condition, so people with the disease have to learn to live with and manage it for many years. (Wright, K., & Adeosun, O., 2009) Assessment Managing vaso-occlusive pain appears to be one of the difficulties with SCD due to the various ways that it can manifest in different individuals, making it hard for health-care providers to manage it efficiently. Pain episodes can vary greatly in the rate at which they occur and the severity depending on the individual. Some patients develop agonizing severe pain for as little as fifteen minutes, while others have pain that gradually escalates over hours to days. The location and the type of pain is also patient-spe... ... middle of paper ... ...osocial aspect of SCD. They all explained the vaso-occlusive crisis, what pain management is involved, and the psychosocial problems a SCD patient might experience. They were very informative on what exact medications a patient should take, and what other alternatives the patient has to relieve their pain. The articles talked about what psychosocial problems a patient may experience, and how the patient can enhance their quality of life in dealing with those problems. References Gill, V. (2010). Managing Sickle Cell Disease. Nursing Made Incredibly Easy, (November/ December), 24-31. Jisieike, C. (2007). Sickle cell disease: psychosocial perspective. West African Journal of Nursing, 18(2), 138-141. Wright, K., & Adeosun, O. (2009). Barriers to effective pain management in sickle cell disease. British Journal of Nursing (BJN), 18(3), 158-161.

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