What is sickle cell disease?

Abnormal blood cells: Sickle cell disease occurs when there is an abnormal type of hemoglobin in blood cells. Thought to be a survivor advantage against malaria, when these cells have too little oxygen they collapse and get stuck in small vessels. This cuts off the blood supply to organs and causes severe pain. Sickle cell traits is when only half the genes code for the abnormal hemoglobin, usually not symptomatic.

A diseae of blood: Sickle cell disease is a disease cause by an abnormal hemoglobin protein carriedninside red cells. The protein becomes insoluble and causesthe red cells to stick in the blood stream and blocks blood flow.

Common & inherited: Sickle cell disease is a very common inherited condition affecting hemoglobin & thus red blood cells, often leading to anemia as well as complications resulting from abnormal cell shape. Each (carrier) parent must have at least one gene for this condition in order for affected person to receive both to have actual disease. Check out http://ghr.Nlm.Nih.Gov/condition/sickle-cell-disease for info.

Red blood disorder: Autosomal recessive disorder when rbc's become rigid and cannot carry oxygen. The hemoglobin is abnormal. Also know as hbss vs hbas or trait. With low oxygen tension the beta chain of the hemoglobin molecule cannot carry oxygen properly and takes a different form-becomes sickle shaped, inelastic. They cannot pass through capillaries and lead to occlusion and ischemia. Rx transfusions, fluids.