Critical Care Nursing: Sickle Cell Anemia

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Critical Care Nursing: Sickle Cell Anemia

Critical Care Nursing

10/15/2009

Sickle cell anemia affects millions of people worldwide. Sickle cell anemia is a disease in which your body produces abnormally shaped red blood cells. They don't last as long as normal, round red blood cells, which leads to anemia. Sickle cells contain abnormal hemoglobin that causes the cells to have a sickle shape. Sickle-shaped cells don’t move easily through your blood vessels. They’re stiff and sticky and tend to form clumps and get stuck in the blood vessels. The sickle cells also get stuck in blood vessels, blocking blood flow. This can cause pain and organ damage. Sickle cell disease is caused by a mutation in the beta-globin chain of the hemoglobin molecule. Sickle hemoglobin, the result of this mutation, has the singular property of polymerizing when deoxygenated. In sickle cell anemia, a lower-than-normal number of red blood cells occur because sickle cells don’t last very long. Sickle cells usually die after only about 10 to 20 days. The bone marrow can’t make new red blood cells fast enough to replace the dying ones. The disease has no widely available cure. In this paper I will review the test for detecting this disorder, the treatments available and nursing interventions for patients with Sickle Cell Anemia. Despite genetic identity at the site of the sickle hemoglobin mutation, all patients with sickle cell anemia are not affected equally by this disease. People with the disease are born with two sickle cell genes, one from each parent. If you only have one sickle cell gene, it's called sickle cell trait. About 1 in 12 African Americans has sickle cell trait. A DNA analysis can show if you have the trait or anemia. This test is used to investigate alterations and mutations in the genes that produce hemoglobin components. It can determine whether a patient has one copy of the hemoglobin S gene, two copies, or copies of different hemoglobin variants. Genetic counseling is strongly encouraged if a positive sickle screen from one or both parents is determined. Family studies can be done to identify sickle cell trait in other family members. Most states test newborn babies as part of their newborn screening programs (Mann-Jiles & Morris 2009). Newborn screening is the practice of testing every newborn for certain harmful (like SCA) or other potentially fatal disorders that aren't otherwise apparent at birth. There are treatments for the symptoms and complications of the disease, but no actual cure. Sickle cell anemia varies from person to person. Some people who have the disease have chronic pain or fatigue (tiredness). With proper care and treatment many people who have the disease can have improved quality of life and reasonable health much of the time. The signs and symptoms of sickle cell anemia vary. Some people have mild symptoms. Others have very severe symptoms and often are hospitalized for treatment. Sickle cell anemia is present at birth, but many infants don't show any signs until after 4 months of age. The most common signs and symptoms are linked to anemia and pain. Other signs and symptoms are linked to the disease's complications. Common symptoms include: Attacks of abdominal pain, bone pain, breathlessness, delayed growth and puberty, fatigue, fever, jaundice, rapid heart rate, ulcers on the lower legs (in adolescents and adults). Other symptoms include: chest pain, excessive thirst, and frequent urination, a painful and prolonged erection (priapism - occurs in 10 - 40% of men with the disease), poor eyesight/blindness, strokes, and skin ulcers (Mann-Jiles & Morris 2009). Sudden pain throughout the body is a common symptom of sickle cell anemia. This pain is called a "sickle cell crisis." Sickle cell crises often affect the bones, lungs, abdomen, and joints. A sickle cell crisis occurs when sickled red blood cells form clumps in the bloodstream. These clumps of cells block...
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