Sickle Cell Disease is common throughout the world, and people of all races should be screened to determine if they are a carrier of the inherited sickle cell trait or if they have the disease. A simple and painless blood test is all that is needed.
Although "sickle cell disease" and "sickle cell anemia" are sometimes used interchangeably, the general term, "sickle cell disease," refers to a group of inherited disorders that affect the red blood cells. Persons are usually diagnosed as newborns, though the disease can also be diagnosed in a fetus in the early stage of pregnancy.
Sickle Cell Disease is caused by an inherited form of hemoglobin, a protein that enables red blood cells to carry oxygen from the lungs, via the bloodstream, to all parts of the body. Red blood cells with normal hemoglobin, visible under a microscope, are smooth and round, and glide easily through blood vessels. In the bodies of people with sickle cell disease, after the carrier cells release oxygen they assume a curved or "sickle" shape and become hard and sticky, blocking blood flow and causing damage to organs. Other complications include episodes of intense pain, swelling, fatigue, jaundice, stroke and anemia.
Treatments for Sickle Cell disease include antibiotic therapy, supplemental oxygen, transfusions and bone marrow transplantation, a technique more successful in younger people. The drug hydroxyurea has been found to reduce the principal symptoms of sickle cell disease, apparently by activating a gene that triggers the body’s production of fetal hemoglobin.