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Sickle-cell anemia - Definition


Sickle cell disease is a hereditary genetic disorder also called sickle cell anemia. It is a disease of the blood, especially of the hemoglobin whose function is to transport oxygen in the blood. Red blood cells are deformed, sickle-shaped, and have difficulty circulating and can get stuck in the blood vessels. The disease is suspected in chronic anemia identified in blood tests and confirmed by hemoglobin electrophoresis, an examination to demonstrate a high level of hemoglobin S, involved in the disease. Patients have occasional bouts of pain in the extremities or the abdomen called vaso-occlusive attacks. When the spleen is affected, individuals are also more susceptible to infections.