For thousands of Americans, living with sickle cell disease means a lifetime of painful episodes, blood transfusions and frequent trips to the hospital. These treatments can often cause complications that are just as bad or worse than the effects of the disease itself.
Sickle cell disease (SCD) is an inherited genetic disorder that affects red blood cells. The presence of Hemoglobin S, an abnormal hemoglobin, causes these blood cells to change into the shape of a sickle and makes it difficult for them to pass through small blood vessels. When these vessels become blocked, oxygen has trouble reaching the tissues since hemoglobin is an oxygen carrying molecule.Tissue that does not receive normal blood flow often becomes damaged, including tissue of the lungs, spleen, liver and kidneys.
Living with sickle cell disease is painful and can inhibit a person from living a normal life. Too much activity can cause a pain crisis and stroke. Currently the main treatments for SCD are blood transfusions, which can cause toxic build up of iron, and antibiotics to prevent infections caused or worsened by damage to the spleen, pain management, and surgery. Each of these treatments causes more complications. Even with multi-disciplinary treatments, the average life expectancy for someone who suffers from SCD is only about 40-to-45 years.