Sickle Cell Disease
Sickle cell disease, an inherited disease affecting mainly Afro-Caribbeans, is so-called because the red blood cells become crescent - or sickle - shaped. This is due to abnormal haemoglobin which, when it gives up its oxygen, becomes straight rather than folded. The red cell becomes rigid when it adopts the sickle shape, and can’t pass through tiny capillaries, which become blocked, causing tissue damage. Being abnormal, the red cells are also friable, and subject to breaking up. Besides anaemia, the patients may suffer painful crises precipitated by infection, dehydration or cold weather. These can affect the bones, the skin or the internal organs - particularly the spleen in early childhood.
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