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A blood disorder, the result of an abnormalityof haemoglobin.Haemoglobin is the essential oxygen-carrying protein dissolved within red blood cells. It works by forming a biochemical nest trapping atoms of iron; haemoglobin takes up oxygen from the lungs and delivers it to the tissues. Sickle cell disease is a genetic abnormality, with the result that the haemoglobin molecule is less soluble. The consequence of this is that red blood cells are less flexible and adopt acurved shape, from which sickle cell disease gets its name. The abnormally shaped red cells pass less easily through the smallest blood vessels of the body called capillaries and are liable to stick and obstruct those capillaries.This tends to happen if the sufferer becomes cold or has some serious infection or is exposed to lack of oxygen e.g. during plane flights.
In these circumstances the haemoglobin molecules become even less soluble and therefore more cells become sickled and potentially obstructive. The sickle cells also disintegrate more easilyand so sufferers become anaemic.Despite all of the problems it causes, sickle cell disease is believed to give protection against acquiring malaria and it is thought that this is why it is so widespread in Africa and in people in other parts of the worldof African origin. It is also found in Asians andis some Middle East people.

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