Sickle Cell Anemia

Sickle cell anemia is a hereditary disorder in which the body can not produce normal hemoglobin (iron-containing protein). Sickle cell anemia is a disease in which the body can not produce normal hemoglobin (iron-containing protein). Abnormal hemoglobin may change cells and may get stuck in thin blood vessels and prevent oxygen from reaching organs and tissues. Tissues without normal blood flow will eventually be damaged. This is a problem that causes sickle cell disease.

In humans, sickle cell anemia is a hereditary disease in which blood cells have mutants of oxygen binding protein hemoglobin. Because sickle cell anemia is caused by a single amino acid mutation of hemoglobin, it is considered as a disease of primary structure of protein. 13). Normal blood cells are spherical, but those with this mutation make sickle shaped red blood cells. Normal blood cells are elastic and can flow freely through veins, but sickle cells are very hard and often can not move with vein branches. This occlusion causes the downstream oxygen tissue to become poor, causing many medical problems including a decrease in life expectancy. Once the body is stuck and destroyed, the body recognizes them. Healthy red blood cells usually survive between 90 and 120 days, but sickle cells have a life span of 10 to 20 days. 14)

Sickle cell anemia is a blood disorder affecting erythrocytes. Normal red blood cells are round. In people with sickle cell anemia, substances in hemoglobin - erythrocytes - become incomplete, changing the shape of erythrocytes. Defective hemoglobin called hemoglobin S (HgbS) replaces normal hemoglobin called hemoglobin A (HgbA). As time goes on, red blood cells become hard, shaped like crescent shaped satellites and sickles. In the United States, the disease is most common among African Americans (about 1 in 400 African Americans) and Caribbean Hispanics (1 in 1,000 to 1,400 Hispanics). The disease is seen all over the world in the Arab, Greek, Italian, Sardinian, Turkish, Maltese, South Asian breeds.