Is beta thalassemia major a concern in children?

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If you are having thalassemia minor and your wife is thalassemia negative, then there is no chance of acquiring thalassemia major in your child. So your wife must also have thalassemia minor if your child is thalassemia major. Your child's attached report is suggestive of thalassemia major (attachment removed to protect patient identity).

The only treatment in thalassemia major is either bone marrow transplantation or regular blood transfusion to maintain hemoglobin level within range. So your child might need a routine blood transfusion to maintain hemoglobin within range. She can be given an iron tablet, but it will not work in your child's case as she has thalassemia major. I am sorry to say, but your child will need a blood transfusion for the treatment of low hemoglobin.

You can consult a nearby good hematologist or the place where a bone marrow transplantation facility is available, which is a curative treatment for thalassemia major. But it is a costly treatment.

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Hello,

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The success rate can vary for different people. The overall success rate of BMT (bone marrow transplant) for thalassemia major is around 85 to 90 %. The HLA (human leukocyte antigen) matching is also required for bone marrow transplantation. The cost also varies in different hospitals.

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Hello,

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Thalassemia major usually becomes symptomatic after one year of age. Sometimes the clinical picture might be delayed. But the attached report states a case of thalassemia major. She might have thalassemia intermedia, a condition in which the patient might not need a regular blood transfusion. She has enlarged spleen as well, which is suggestive of hemolytic anemia, such as thalassemia.

You need to consult a good nearby hematologist for a detailed physical examination and get a prescription accordingly. Initially, the iron injection can be prescribed, and if no improvement than blood transfusion is given. You can repeat her testing for thalassemia major if your wife is normal without thalassemia. You can attach all reports so that I can check them. Kindly attach your and your wife's reports as well if available.

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Hello,

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I have read your questions, and below are the answers to your questions.

1. In alpha, thalassemia alpha chain deletion occurs. It means the reduced alpha chain is the defect. In beta thalassemia, beta chain synthesis is reduced, so an error is in the beta chain. The hemoglobin contains two alpha and two beta chains. According to the reduction in the alpha or beta chain, the thalassemia is classified.

2. In thalassemia, major blood transfusions need to be given at 20- to 30-day intervals regularly.

3. If her blood group is AB positive, then she needs to be given only the AB blood group. Before transfusion, cross-match testing and an indirect Coombs test should be done to check the compatibility of donor and recipient.

4. Hemoglobin level below 7 is considered severe anemia. However, it is advisable always to keep the hemoglobin level above 11 gm per dl.

5. If she has beta thalassemia minor or intermedia, then she can live normally. Or else in beta-thalassemia major, the average life span is 25 to 30 years.

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Hello,

Welcome back to icliniq.com.

The attached HPLC (high-pressure liquid chromatography) report of the mother does not show beta thalassemia (attachment removed to protect patient identity). So, as per this report, beta thalassemia is not present in the mother. However, iron deficiency anemia can give a false negative result for beta thalassemia. In such a case, after anemia correction, repeat testing is necessary.

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Thank you.

Hello,

Welcome back to icliniq.com.

If bone marrow transplantation fails and is unsuccessful, like graft rejection occurs, then medication can be given for that. The second time, bone marrow transplantation is risky if rejection occurred the first time. However, the decision can be individualized depending on examination and detailed evaluation. The donor should be HLA-matched, and the hospital can arrange a suitable donor for you.

You can discuss all these regarding transplantation at the concerned hospital where such bone marrow transplantation is done regularly.

I hope I have answered your question.

Let me know if I can assist you further.

Thank you.