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What is sickle cell syndrome?

Sickle cell syndrome is a group of inherited diseases caused by a mutation in a blood protein called haemoglobin. The mutation leads to changes in the shape and behaviour of red blood cells. They become hard, sticky and shaped in the form of a sickle (the sharp hooks used to cut the wheat). This causes small blood clots that block blood flow, and cause repeated painful episodes, called crisis. In the long run, this causes severe damage to the organs in the body. The clinical picture of patients with sickle cell syndrome can be remarkably different. Some patients remain virtually without complaints, while others suffer repeated crises requiring admission to hospital from early childhood. The prototype disease in the sickle cell syndrome group, is the sickle cell anaemia, which is at the same time the most common and severe in the group.