It currently serves 2,000 patients, about 1,200 of whom are treated on a regular basis. Sickle cell screening for newborns is now a statewide program. About 90 percent of children born with sickle cell anemia now survive to at least age 20. About 80,000 Americans have sickle cell disease. Until the mid 1970s, one-fourth of children born with sickle cell anemia died of overwhelming infections before age 5.
Anemia associated with sickle cell causes fatigue and a susceptibility to infections.
Red blood cells carrying the abnormal molecule known as hemoglobin S travel through the arterial circulation until they are deoxygenated. Hemoglobin S molecules often form long, rigid rods, causing the normally circular cells to stiffen and distort into a sickle shape. These sickle cells have difficulty moving through small capillaries. As blood flow slows, endothelial cells lining the vessel wall become sticky, attracting the sickle cells and causing congestion in the vessel. The spleen traps and destroys many of the abnormal sickle cells, resulting in rapid turnover of red blood cells and chronic hemolytic anemia.