Sickle Cell Anemia

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Sickle cell anemia is a genetic blood disorder which is inherited from both parents, that causes red blood cells in patients to be sickle-shaped. This causes the red blood cells to clump together, and be unable to retain oxygen. Sickle cell anemia was first noted in 1910, and is thought to have evolved as a way for the body to naturally fight malaria. It is most prevalent in Africa, India, the West Indies and the Mediterranean, places where malaria is more common. In this country, it is most prevalent in African Americans, affecting approximately 1 in 400.

The most common method of diagnosing sickle cell anemia is through DNA analysis. because the disease is inherited from both parents, most states will do a mandatory screen for sickle cell at birth. If both parents carry the gene, the child will get the disease. If only one parent carries the gene, the child will inherit the trait, but the disease itself will not manifest.

Sickledex Hgb S is a preliminary blood test that can detect sickle-shaped cells. If cells are detected, the doctor will move to hemoglobin electrophoresis. This test identifies abnormal forms of hemoglobin, and will help the doctor determine which form of the disease the patient has, along with the severity.

Arguably the most severe complication of sickle cell anemia is what is known as the sickling episode. The pain that results from such an episode has been described as, “more severe than post-operative pain, and as intense as cancer pain.” when the patient is tired, stressed, dehydrated, been exposed to temperature extremes, or has been smoking or exposed to cigarette smoke, a sickling episode can be triggered. When this happens, the deoxygenated cells become sickle-shaped, clumping together due to a blood vessel wall protein called thrombospondon. These clumps are unable to pass through the blood vessels, which have also constricted. The shape of the blood cells causes them to stab at the walls of the vessels, causing great pain,...
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