What causes the distortion in shape seen in Sickle cell anemia?

The distortion in shape seen in sickle cell anemia is primarily caused by a change in the structure of the red blood cells. In this condition, the normal hemoglobin (HbA) is replaced with an abnormal form known as hemoglobin S (HbS). Under low oxygen conditions, hemoglobin S becomes less soluble, leading to the characteristic sickle shape of the red blood cells. This sickle shape restricts the cells' flexibility, making it difficult for them to pass through small blood vessels. As a result, the distorted cells can cause blockages, leading to pain and potential organ damage. The unique structural change of the hemoglobin molecule itself is what directly leads to the altered morphology of the red blood cells, making this the central factor in the pathology of sickle cell anemia.