Sickle Cell Disease Research Papers

Sickle Cell Disease (SCD) is a group of inherited red blood cell disorders. People with Sickle Cell Disease have abnormal hemoglobin, called hemoglobin S or sickle hemoglobin, in their red blood cells. People with SCD inherit two abnormal hemoglobin genes, one from each of their parents. One of these abnormal hemoglobin genes causes the production of hemoglobin S in the body.1 When a person has two hemoglobin S genes, Hemoglobin SS, this disease is called Sickle Cell Anemia.1 This is the most common and most severe case of Sickle Cell Disease. In the United States, approximately 70,000-100,000 people have sickle cell anemia and this disease is most commonly seen in African-Americans. Sickle hemoglobin is not like normal hemoglobin. It can
The life expectancy in the U.S. is 40-60 years.2 The only cure for SCD is bone marrow transplant, but finding a donor is extremely difficult and many health risks accompany this procedure, including death.3 There are many treatments to help manage the symptoms and pain that are experienced with sickle cell anemia. People with sickle cell anemia are supposed to make regular visits with their doctor to check red blood cell count and to receive medications. Medications help to relieve the pain associated with the crises and to prevent complications. Blood transfusions are used to help replenish lost red blood cells and this relieves anemia. Newer treatments include stem cell transplant, also known as bone marrow transplant, gene therapy, fetal hemoglobin boosting drugs, and nitric oxide.3 Bone marrow transplant involves replacing bone marrow affected by sickle cell anemia with healthy bone marrow from a donor. Because of the risks associated with a stem cell transplant, the procedure is recommended only for people who have significant symptoms and problems from sickle cell anemia.2 Sickle cell anemia is caused by a defective gene, so scientist are looking into if inserting a normal gene into the bone marrow will produce normal