Sickle Cell It's Your Choice	What is Sickle Cell? Sickle is a type of hemoglobin. Hemoglobin is the substance that carries oxygen in the blood and gives blood its red color. Hemoglobin type is not the same thing as blood type. The type of hemoglobin we have is determined by inheritance. The majority of individuals have only the normal type of hemoglobin (A). However, there are a variety of other hemoglobin types. Sickle is one of these types. Two Forms of Sickle Cell Sickle cell occurs in two forms: Sickle cell trait is not a disease. Sickle cell anemia is a disease. Sickle Cell Trait Sickle cell trait is found primarily in African Americans. People from areas around the Mediterranean Sea and from islands in the Caribbean may also carry sickle genes. Sickle cell trait occurs when a person inherits one sickle cell gene from one parent and one normal hemoglobin gene from the other parent. A person with sickle cell trait is well and usually unaware that he or she has the condition. Since sickle cell trait is inherited, a person can pass it on to one's children. If one parent has sickle cell trait and the other parent has the normal type of hemoglobin, there is a 50% (1 in 2) chance with EACH pregnancy that the baby will be born with sickle cell trait.
When ONE parent has sickle cell trait, the child may inherit two normal hemoglobin genes or one normal hemoglobin gene and one sickle cell gene. With EACH pregnancy there is a:	Sickle Cell Anemia Sickle cell anemia occurs when a person inherits two sickle cell genes, one from each parent. Sickle cell anemia is a severe disease. If both parents have sickle cell trait, there is a 25% (1 in 4) chance with EACH pregnancy that the baby will have sickle cell anemia. The child with sickle cell anemia appears normal at birth. However, later in infancy, anemia develops and the child tires easily. A second problem is attacks of pain called crises. These crises can occur without warning, affect any part of the body, and last hours or days. A third problem is frequent infections and unexplained fevers. Crises and frequent infections lead to significant numbers of absences from school or work. Daily penicillin and folic acid are required. Infections and crises may require hospitalizations and administration of antibiotics and medicine for pain. Sickle cell anemia does not cause mental retardation, but is a severe physical handicap. In sickle cell anemia, the red blood cells have an abnormal appearance: NORMAL SICKLE CELL ANEMIA
When BOTH parents have sickle cell trait, the child may inherit two normal hemoglobin genes or one normal hemoglobin gene and one sickle cell gene or two sickle cell genes With EACH pregnancy there is a:	Other Types of Sickle Cell Disease Other types of sickle cell disease are sickle-hemoglobin C disease and sickle-beta-thalassemia. Individuals with these conditions have inherited a sickle gene from one parent and a gene for either hemoglobin C or beta-thalassemia from the other parent. How Can I Find Out If I Have Sickle Cell Trait? There is a simple and accurate test for sickle cell trait. It requires a sample of blood taken from a vein. However, this test is not done routinely and must be requested. The test is called hemoglobin electrophoresis. Why is it Important to Know if I Have Sickle Cell Trait? If both you and your partner have sickle cell trait, you are at risk for having a child with sickle cell anemia, a serious disease without satisfactory treatment.
What Can I Do If I Have Sickle Cell Trait? If you have sickle cell trait, you need not be concerned for your own health. However, you may be at risk for having a child with sickle cell anemia. If your partner is tested and has normal hemoglobin test results, then no child born to the two of you can have sickle cell disease. If your partner also has sickle cell trait, you are at risk for having a child with sickle cell disease. Your physician can discuss the options available to you. Even if you already have had children who do not have sickle cell disease, you might still consider a test for your partner. If both parents have sickle cell trait, the risk of a sickle cell anemia child is the same for each pregnancy no matter how many children with normal hemoglobin or sickle cell trait you already have. Wouldn't it reassure you to know that you are free of the risk of having a child with sickle cell anemia?
Prevalence of Sickle Cell Sickle hemoglobin is found in people whose ancestors come from Africa, the Arabian States, South India and from countries around the Caribbean Sea, such as Puerto Rico, Cuba, Haiti and Jamaica, and also from countries around the Mediterranean Sea, such as Italy, Cyprus, Greece, Turkey, and Syria. Sickle cell trait occurs in approximately 1 in 12 (8%) African-Americans. Sickle cell disease occurs in apprroximately 1 in 375 African-Americans and affects more than 50,000 Americans. For further information ask your health care provider or call or write to us: Division of Genetics, Box 641 University of Rochester Medical Center 601 Elmwood Avenue Rochester, New York 14642 (716) 275-4602 Visit our website: www.urmc.rochester.edu/genetics

Comments/Suggestions to: Mary_True@urmc.rochester.edu