Hydroxyurea therapy: improving the lives of patients with sickle cell disease

Abstract

Sickle cell disease anemia is an inherited blood disorder that affects many people of color in the U.S. Patients with sickle cell disease make abnormal blood cells that tend to clog and occlude blood vessels. Many sickle cell patients suffer from vaso-occlusive crisis and other complications leading to end-organ damage and failure. Hydroxyurea has been used to treat cancer patients for many years. Moreover, clinicians and researchers have determined that this medication increases fetal hemoglobin levels. Fetal hemoglobin does not interfere with sickle hemoglobin polymerization, yet prevents red blood cells from sickling. Hydroxyurea therapy significantly reduces the number of deaths from sickle cell complications. Additionally, hydroxyurea significantly reduces the number of hospitalizations, vaso-occlusive crisis, and acute chest problems; thereby reducing severity of the disease. Pediatric studies also have shown that hydroxyurea can be safely used in children.