Sickle Cell Anemia is an inherited disorder that causes a person’s red blood cells (RBCs) to change their shape and go from being round to forming a C-shape. This change causes the cells to be more easily destroyed and also to clump together forming clots.
Clotting prevents the blood from flowing normally, caus- ing extreme pain, infection, and organ damage including strokes.
Sickle cell disease affects about 90-100,000 Americans. Forty years ago, the life expectancy for someone living with Sickle Cell Anemia was just 14 years.
Research has shown the inheritance patterns of sickle cell disease, including the gene mutations. As a result, all U.S. newborns are routinely screened for sickle cell disease.
Because of this research, a life-saving medication, hydroxyurea has been created and studied, which increases fetal hemoglobin to reduce the effects of sickle cell disease and helps the red blood cells to remain the correct shape and to be less fragile.
The use of prophylactic antibiotics and vaccines have also been studied and incorporated into the standard of care for a child with the disease, preventing important and often fatal illnesses, such as meningitis.
Bone marrow transplantation has been used as a cura- tive therapy, and more gentle means of transplantation are being developed that are effective.
Combining preventive care with standardized medical management of sickle cell crises have helped to increase the current life expectancy to more than 40 years of age.
Policy efforts to increase pediatric access to care, such as the Children’s Health Insurance Program (CHIP), have increased the reach of these best-practice algorithms.