World Sickle Cell Day, created by a United Nations resolution, is recognized globally as a day to spread awareness of Sickle Cell Disease (SCD). SCD is the most commonly known genetic disease in the world today. While it is seen most frequently in people originating from Africa, the Mediterranean and parts of India, it affects many other races and ethnicities.
SCD is a group of red blood cell disorders where an abnormal form of hemoglobin is made by the red blood cells. The abnormal hemoglobin causes the red blood cells to become rigid and block blood vessels in the body resulting in pain and damage. The red blood cells are also destroyed quickly leading to anemia.
The symptoms of SCD are not always consistent, and can appear differently in each patient living with the condition. However, experts believe that as many as 25 percent of patients with SCD are living the most severe form of the condition, which can cause episodes of intense pain, acute chest syndrome, and even the potential for a stroke during childhood.
Approximately 90 percent of children with SCD in the US will survive into adulthood. However, extensive organ failure can significantly shorten a US patient’s lifespan to end in the 40s. SCD specialists say the rate of adult longevity is even less for children in developing countries.
Currently, the only cure for SCD is a bone marrow transplant. Preferably, the donor for the transplant is a matched sibling and bone marrow, mobilized peripheral blood or cord blood can be used for the transplant.
Bone marrow transplants at this time are not routinely performed for SCD because of the risks associated with the transplant procedure and many patients do not have a suitable donor. Researchers, however, continue to investigate new methods to improve bone marrow transplants to increase the donor pool and to improve outcomes.
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