Sickle Cell Anemia

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Sickle Cell Anemia

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31/5/13

Sickle Cell Anemia

A severe hereditary form of anemia in which a mutated form of hemoglobin distorts the red blood cells into a crescent shape at low oxygen levels.

Red Blood Cell

A biconcave disk shaped cell without a nucleus that contains hemoglobin.

Hemo- A protein with the globin function of carrying

oxygen from the lungs to the rest of the body An abnormal red blood cell with a crescent shape containing an abnormal form of hemoglobin by a deficiency of red blood cells or hemoglobin in the blood resulting in pallor and weariness

Sickle Cell

Anemia A condition marked

(Fig.4)

Clinical Description

According to the Sickle Cell Society (SCS), sickle cell anemia is a disorder that affects red blood cells, which contain hemoglobin (Hb) whose function is to carry oxygen from the lungs to the rest of the body. SCS goes on to say that people affected with the disorder have sickle hemoglobin (HbS) rather than normal hemoglobin (HbA). Further, they note that people with sickle cell anemia can suffer from severe pain attacks called crises, experience damage to organs such as liver, kidney, lungs, and heart, or even die as a result. This is because cells containing HbS do not live the normal 120 days, which results in a chronic state of anemia. William E. Proudford Sickle Cell Fund (WEP) states that normal red blood cells are round like doughnuts, and they move through small blood tubes in the body to deliver oxygen (fig.1). They go on to say, sickle red blood cells become hard, sticky and shaped like sickles used to cut wheat. (fig.2). Furthermore, that when these hard and pointed red cells go through the small blood tube, they clog the flow and break apart causing pain, damage, and anemia (low blood count) (fig.3). The disorder is chromosomal affecting chromosome 11 and is a recessive gene (SCS). Fig. 5 shows that the gene is recessive because two of the children are carriers and only a recessive trait...