ORAL L GLUTAMINE THERAPY FOR SICKLE CELL ANEMIA

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The sickle cell diseases are a group of hereditary hemoglobin disorders characterized by presence of abnormal red cells. The complications of this disease may start early in life, such as fetal deformity, brain blood vessel accidents, infection and vaso-oclusion painful crises. Current therapy (with the exception of bone marrow transplantation) is usually limited to the prevention of infections and management of complications (pain relief, prevention of dehydration, treatment of infections, blood transfusions). The search for effective treatments for sickle cell disease has been generally disappointing. While certain new approaches, such as gene therapy, are promising, they are still in early stages of development. The utility of bone marrow transplantation in sickle cell disease has to be assessed for risk/benefit issues. While cure is possible when this treatment modality is used, the morbidity and mortality of the procedure is still very high. Clearly, development of new treatment modalities exploiting novel mechanisms of action is necessary. In order to relieve those who are affected by this disease, which is a relatively large population among people of African descent, an agent or method of therapy is urgently needed to prevent morbidity and mortality of sickle cell disease. L-glutamine is an amino acid that is utilized in the formation of NAD, a substance that protects red blood cells from oxidative damage. Previous data showed that sickle cell anemia patients may benefit by supplementation with the amino acid, L-glutamine. Therefore, the purpose of this research is to evaluate the efficacy of this amino acid in treatment of sickle cell anemia.