Sickle cell anemia is a red blood cell disorder, in which instead of being like normal round cells, affected blood becomes hard sticky, and shaped like sickles, clogging blood flow and breaking apart. Sickle cell anemia is inherited and can be expressed as either sickle cell trait when the gene is derived from only one parent, or as a full blown anemia, when both parents catty the recessive gene. (1) .
The genetic defect that causes sickle cell anemia affects hemoglobin, which is a component of red blood cells. Hemoglobin's job is to carry oxygen to all the cells and tissues of the body. Red blood cells that contain normal hemoglobin are soft and round. The soft texture enables them to squeeze through the body's small blood vessels. People with sickle cell anemia have a type of abnormal hemoglobin called hemoglobin S. Normal hemoglobin is called hemoglobin A. A genetic error makes the hemoglobin molecules stick together in long, rigid rods after they release oxygen. When these rods cause the red blood cells to become to hard and sickle shaped, it's like having mini-heart attacks throughout the entire body.
A heart attack is painful because the blood flow to the heart interrupts. In sickle cell anemia, the blood flow may interrupt any of the major organs, causing severe pains and organ damage at the site of the blood flow blockage.(2) Hemoglobin S trait can be found in 8 to 10% of the black population.(3).
Sickle cell is prevalent among many nationalities, including African Americans, Arabs, Greeks, Italians, Latin Americans, and East Indians. Caucasians also have sickle cell disease or trait. (5).
In the United States, sickle cell anemia is most common among African Americans. Every year, about one in four hundred African American infants are born with the disease after inheriting the genetic mutation from both parents.
All races are screened for this hemoglobin at birth. There are three common types of sickle cell diseases in the United States; ( Hemoglobin SS or sickle cell anemia, Hemoglobin SC disease, and sickle beta - thalassemia.
