The key to management of G6PD deficiency is prevention of hemolysis by prompt treatment of infections and avoidance of drug and household triggers. The most important measure is to avoid drugs and food that causes hemolysis. Vaccination against some common pathogens may prevent infection induced attacks. Patients presenting with severe anemia and hemolysis may require resuscitation and erythrocyte transfusion, in the acute phase of hemolysis the blood transfusion might be necessary or even dialysis in acute renal failure. Blood transfusion is an important symptomatic measure as the transfused RBCs are generally non G6PD deficient and some patients are benefited from splenectomy as this is an important site of red cell destruction. Folic acid and vitamin E should be used in disorder featuring a high red cell turnover. Babies with neonatal jaundice tend to respond well to standard therapies like hydration, light therapy and exchange transfusion. Thus, if a neonate is found to be G6PD deficient, counseling for the parents and an establishment of breastfeeding practices might be excellent prophylactic measures in preventing hemolytic crises later in life. Undoubtedly, a good knowledge of chemotherapeutic drugs, household agents and other environmental triggers of hemolytic anemia in G6PD deficiency are vital for all physicians to not only reduce the incidence of hemolytic events but also augment awareness amongst at-risk individuals.
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