BioNews Texas, by Charles Moore ~ December 1, 2014
Sickle cell anemia is the most common form of sickle cell disease among a group of inherited red blood cell disorders and the most common genetic disease in the U.S., afflicting an estimated 70,000-80,000 Americans according to the William E. Proudford Sickle Cell Fund Inc.
Normal red blood cells, which are shaped like doughnuts, contain an iron-rich, oxygen-carrying protein called hemoglobin, and travel easily through the body’s circulatory pathways delivering oxygen to cells. Sickle red blood cells, by contrast, become hard, sticky and shaped like sickles — crescent-shaped hand tools used to cut wheat and other crops prior to the mechanized agriculture era. As these cells hardened, pointed red cells move through small blood passageways, they clog the flow and break apart, causing pain and damage, the risk of infection, and a low blood count, or anemia. Sickle cells also contain abnormal hemoglobin called sickle hemoglobin or hemoglobin S. Sickle hemoglobin is what causes the cells to develop their characteristic crescent shape.
Sickle cell disease affects people of many ethnicities, including people whose ancestors were from Mediterranean countries such as Greece, Turkey, and Italy; the Arabian Peninsula; India; and Spanish-speaking regions in South America, Central America, and parts of the Caribbean. However, it disproportionately affects people of African descent. Approximately 1 out of 10-12 African Americans has sickle cell trait — a genetic predisposition to developing sickle cell anaemia, although not all will actually develop the disease.
According to a multi-institutional study that included researchers from The University of Texas Health Science Center at Houston (UTHealth), conclusive findings show that hydroxyurea therapy offers safe and effective disease management of sickle cell anemia and reduces the risk of stroke.
The promising data […]