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Sickle cell anaemia

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This is a problem stemming from sickle cell disease which is found among inhabitants of Africa (25% carry the gene), the Middle East and Mediterranean countries.

It comes about because of a complicated genetic inheritance which cause formation of an abnormal type of haemoglobin, which is necessary to the function of red blood cells. Under the microscope the cell is sickle shaped, giving rise to its name.

Symptoms vary from mild anaemia to severe haemolysis (abnormal destruction of red blood cells) resulting in a 'sickle cell crisis'. Most patients do not have symptoms of anaemia because tissue oxygen delivery is normal owing to a hyperdynamic circulation and the fact that Hbs releases oxygen more easily than normal Hb.

A rapid fall in haemoglobin may produce a sickle cell crisis, sometimes due to a previous infection or ingestion of drugs. Extreme pain is felt due to necrosis (dying off) of the bone marrow. This is felt mainly in the long bones, ribs and spine. A hospital admission for pain control is often necessary and complications include splonic atrophy (shrinking of the spleen), retinal ischaemia (loss of blood to the back of the eye) and even closure of the vessels supplying the brain leading to fits.

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