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Sickle Cell Anemia

Sickle cell anemia is an autonomic recessive disorder in which abnormal hemoglobin leads to chronic hemolytic anemia along with periodic episodes of pain. The mutation in hemoglobin sickle cell anemia is a blood condition primarily affecting people of African ancestry. It has been researched and proven that the error in the hemoglobin gene results from a genetic mutation that occurred many thousands of years ago in people in parts of Africa, the Mediterranean basin, the Middle East, and India. It is associated with being immune to malaria, a parasitic blood disease that is carried and contracted by infected mosquitoes. Since malaria is mostly found in hot, moist areas in West Africa and the Mediterranean, people originating from that area produced an immunity to towards malaria, but with consequences (Bloom). The individual will not contract malaria because the mutation in his/her genes quickly fight off the disease by changing the shape and composition make-up of the blood cell so the parasites will not be able to survive in it (Van Cleve, Lemerond). The consequence of this is that because the individual’s blood cells mutate and become misshaped, becoming “moon crescent” or “sickle-shaped”, the blood cells then gr


-Both children and adults are more vulnerable to infections and have a harder time fighting them off. This is the result of damage to the spleen from the sickled red cells, which prevents the spleen from destroying bacteria in the blood. -Infants and young children, especially, are susceptible to bacteria (infections that can kill them in as little as 9 hours from onset of fever).

But patients can’t wait for these drugs to be approved or for gene therapy to finally reach its final stage. Basic treatment of painful crises relies heavily on pain-killing drugs and oral and intravenous fluids to reduce pain and prevent complications. Blood transfusions are used to treat and to prevent some of the complications of sickle cell anemia. Transfusions correct anemia by increasing the number of normal red blood cells in circulation. Transfusions are used to treat spleen enlargement in children before the condition becomes life threatening. Regular transfusion therapy also can help prevent recurring strokes in children at high risk of crippling nervous system complications. Giving young children with sickle cell anemia oral penicillin twice a day, beginning when the child is about 2 months old and continuing until the child is at least 5 years old, can prevent pneumococcal infection and early death in these children. Recently, however, several new strains of pneumonia bacteria that are resistant to penicillin have been reported. Since the vaccines for these bacteria are ineffective in young children, studies are being planned to test new vaccines.

-If both parents have sickle cell trait (HbAS) there is a one in four (25%) chance that any given child could be born with sickle cell anemia. There is also a one in four chance that any given child could be completely unaffected. There is a one in two (50%) chance that any given child will get the sickle cell trait.

The clinical course of sickle cell anemia does not follow a single pattern; some patients have, mild symptoms, and some have very severe symptoms. However, the basic problem is the same, the sickle-shaped red blood cells tend to get stuck in narrow blood vessels, blocking the flow of blood. This results in the following conditions/problems:

Some topics in this essay:
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Approximate Word count = 2447
Approximate Pages = 10 (250 words per page double spaced)


  

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