Is there any treatment for beta thalassemia major?

Hello,

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Beta thalassemia major is a hereditary disorder and is due to a genetic deficiency of beta globin chains of hemoglobin. It is transfusion-dependent anemia. Management of beta thalassemia major includes: chronic transfusion to maintain hemoglobin in the range of 9-10 gm/dL, iron chelation therapy to avoid iron overload, surgical splenectomy to decrease the number of transfusions, but it is risky, and cholecystectomy for gallbladder stones. The above therapy is for symptomatic treatment only and not definitive.

Long-term cure is possible by allogenic bone marrow transplantation with an HLA (human leukocyte antigen match to the individual. With regard to G6PD - glucose-6-phosphatase dehydrogenase deficiency, the prevention of oxidizing drugs, such as fava beans, is required. G6PD deficiency is very rare in females as it is X-linked recessive.

If it is so, you should also be having the same, as it is asymptomatic and usually neglected. If your daughter has G6PD deficiency disorder, your wife should be a carrier, and you should be evaluated with a quantitative assay for G6PD enzyme analysis, as the same avoidance of oxidizing drugs is needed for you, too.

I hope this helps.

Thank you.