What Is Sickle Cell Disorder?

This is a family of conditions, all related to malfunction of red blood cells. It is a genetic (inherited) set of disorders. The most common and best known conditions are sickle cell anaemia (especially common among people of African origin) and Thalassaemia (which is most prevalent among persons of Jewish and Mediterranean origin).

Having one gene for the sickle cell anaemia disorder (which makes you a carrier rather than a sufferer) provides some protection against malaria, which helps explain why sickle-cell disorders have persisted in human populations.

The manifestation of these conditions is occasional attacks when red blood cells stop carrying oxygen properly. This results in severe oxygen depletion in internal organs. Attacks are very painful and require emergency medical treatment, such as blood transfusions. Recurring attacks lead to long-term organ damage and (eventually) multiple organ failure. With good medical treatment, quality of life can be high for sufferers. But life expectancy is not high, and tends to be early middle age (about age 42 on average).

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