Sickle Cell Anemia is a blood disease that most often affects African-Americans. Sickle cell disease is an inherited blood disease that causes red blood cells to become deformed (sickle-shaped). The red blood cells deform because they contain an abnormal type of hemoglobin, called hemoglobin S, instead of the normal hemoglobin, called hemoglobin A.

 

Sickled blood cells are destroyed by the body faster than normal blood cells, which can lead to the body receiving an inadequate supply of oxygen; this condition is called sickle cell anemia. Also, sickled blood cells can become trapped in blood vessels, reducing or blocking blood flow. This can damage organs, muscles, and bones and may lead to life-threatening conditions. Sickle cell disease is an autosomal recessive disease. This means that to have the disease, a person must inherit a gene for the disease from both parents. Each person inherits two chromosomes (one from each parent).

 

As a result, a person may have:

· Two chromosomes that produce normal hemoglobin (hemoglobin A). These people have normal red blood cells (unless they have some other disease).

· One chromosome that produces hemoglobin A and one that produces hemoglobin S. These people carry the sickle cell trait (and so are called "carriers"), but they do not have sickle cell disease. Sickle cell trait is usually a harmless condition.

· Two chromosomes that produce hemoglobin S. These people have sickle cell disease. Both parents either carry the sickle cell trait or have the disease. Sickled red blood cells often cause recurring health problems (called sickle cell crises).

· One chromosome that produces hemoglobin S and one that produces some other abnormal type of hemoglobin. Depending on the other type of abnormal hemoglobin, these people may not have the disease or may they have mild or severe sickle cell disease.

 

Various forms of sickle cell disorder may occur when a person inherits one sickle cell gene and one defective hemoglobin gene of another type. This test checks for the presence of hemoglobin S. This test may also detect the presence of other rare blood diseases, such as Bart's, CGeorgetown, Alexandra, CHarlem, Porto Alegre, MemphisS, CZiguinchor and STravis, though it should not be used for that purpose because it was designed for Sickle Cell Anemia.

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