SICKLE CELL

ABOUT SICKLE CELL

Sickle cell anaemia is a disorder of the blood and it is commonly (but not predominantly associated) with the black race. It is a hereditary disorder and is derived when both parents have the sickle cell trait, AS. Generally, anaemia is defined as a deficiency in the number of red blood cells or in their haemoglobin content, resulting in pallor (paleness) and lack of energy.


It is an inherited haemoglobin disorder comprising sickle cell anaemia (Hb Sc) and some less prevalent but related considerations such as sickle haemoglobin C disorder (Hb SC) and sickle beta thalassaemia (Hb SC). The genes for these conditions arose as a result of mutation from in where there is or there was a high incidence of a type of malaria called falciparium malaria, the most lethal strain of malaria known to man.


People with sickle cell trait (Hb AS) are thus protected from sickle cell symptoms and complications by the presence of Hb A. They are mostly protected from death caused by malaria, especially in early childhood, by the presence of Hb S. They enjoy the best of the world and are naturally better adapted to malaria infested environment than their Hb counterpart, who is more liable to childhood death from malaria, or the sickle cell affected one with Hb SS in whom malaria easily induces a lethal degree of anaemia.


Generally anaemia is a deficiency in the number of red blood cells or in the number in their haemoglobin content, resulting in paleness and lack of energy. In the case of Sickle Cell Anaemia, the name is derived from the sickle shape the blood cells take, which then makes it difficult for these cells to receive adequate oxygen the time the blood needs to be renewed in the body. As a result, the red blood cells soon die off, leading to moderately severe anaemia. The resultant effect is the attacks of pains in the bones, joints, and abdomen etc, usually referred to as “crises”. These attacks could last for hours, days or even weeks and could lead to complications such as leg ulcers, osteomyelities (bone infection), jaundice, gall bladder among others. There is still no documented cure for this disease although various drugs have been discovered which reduce the frequency in crises attack like Hydroyurea, Ciklavite, Nicosan, however some drugs like Hydroyurea should be use with caution as a result of side effects it brings. Research is still going on in finding a safe curative solution.