Everyone has two copies of the HBB gene, which codes for a building block of hemoglobin known as beta-globin. Individuals with sickle cell anemia have a change known as hemoglobin S (Hb S) in both of their copies of this gene. When both copies of the beta-globin gene have the Hb S change, the hemoglobin made by that individual is abnormal. This abnormal hemoglobin causes the red blood cells to be abnormally shaped, leading to the various symptoms of sickle cell anemia.

