UNDERSTANDING THE SICKLE CELL DISEASE IN CHILDREN (PART I)

Sickle cell anemia, which is also called sickle cell disease, is an inherited blood disorder that affects red blood cells of affected individuals. It is common among people of African, Middle-East and Mediterranean descent. In Africa, where children are indispensable factor in a successful marriage, many women have lost their marriages as a result of the death of their children because of this blood disease.

There is a substance called hemoglobin, which is found in the red blood cells. Its primary function is to help red blood cells to transport oxygen from air in our lungs to all parts of the body. This hemoglobin is of two types i.e. A and S types. People with sickle cell disease have double dose of the S hemoglobin (SS), each of which they inherited from each of the two parents. As a result of this defect, their red blood cells are sickle shaped (that was how the disease got its name), and therefore have difficulty in passing through small vessels because they are stiff. Also, red blood cells containing mainly S hemoglobin do not live as long as those with A hemoglobin. On the average, such cells live for about two to three weeks, in contrast to the red blood cells that have A hemoglobin, which may live for as long as one hundred and twenty days before there is a need for replacement. In other words, the red blood cells need replacement too frequently in sickle cell disease patients. This is the reason why these individuals are frequently anemic. For now, there is no cure for the disease except through divine intervention.

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