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A PUBLIC AWARENESS INFORMATION PROVIDED BY THE INSTITUTE OF GENETIC CHEMISTRY AND LABORATORY MEDICINE.
What is Sickle Cell Disease?
Just as we inherit color of our eye and our hair from our parents, some diseases can also be inherited. Sickle cell disease is an inherited disorder or the hemoglobin in the red blood cells. Characteristics of our hemoglobin are also inherited, or passed on through the genes, from our parents.
Normal red blood cells are disc-shaped and flexible. The hemoglobin of individuals with normal red blood cells is called Hemoglobin (Hb) a. individuals with sickle cell anemia have inherited genes, that cause their red blood cell to take on the shape of a crescent or sickle when they lose oxygen to the body tissues.
Their hemoglobin is called S.
Sickle-shaped cells cannot pass easily through the small blood vessels and may result in blockage in the blood vessels. This blockage often results in painful crises and may lead to many other serious complications such as bone damage and leg ulcers’. Once the cells are sickled they become more fragile and tend to be more easily destroyed than normal cells. This means that individuals with sickle cell disease have fewer red blood cells than normal an hence have low blood level of anemia. Sickle cell disease patients may have recurrent episodes of painful crisis characterized by unbearable pains in the extremities, back, chest and abdomen. The inter-crisis period is highly variable in a given individual as well as among individuals.
What is the Sickle Cell Trait or heterozygosis?
Sickle cell trait is often confused with sickle cell anemia .In a sickle cell trait, both normal (Hb A)and sickle (Hb S) hemoglobin are present in the same red blood cells. People are the said to have “Hb AS” (an S gene is provided by one parent and A gene by the other). Approximately one in four Nigerians have sickle trait. Such person is not sick; does not have sickle cell disease and suffers from no anemia but he or she can pass the sickle cell gene on to his or her children. In population of 140 million people in Nigeria, 28 millions are carrying probable the sickle gene that are capable of passing the sickle cell genes to their children.
Sickle cell disease occurs in about 1-2% of the population so in a population of 140 million people between one to two million people would be suffering from the diseases. The diseases, as well as the control of the frequency of the disease, should be a major health undertaken in our country