For thalassemia major, when would my son require his first blood transfusion?

Question

Hello doctor,

My son is 15 months old and has been diagnosed with thalassemia major. My only question is when he would require his first blood transfusion.

Typically, people feel strong symptoms of anemia and need medical help when Hb goes below 6. But my child has Hb of 3.7, probably for over a month, and two weeks have passed till the last testing, and he looks normal.

I want to know how he is surviving at this low Hb. He plays well, sleeps well, and walks in a walker, and from today, he started taking a few steps on his own. Is this because of HbF, which is 96%?

If so, then till what age will HbF be produced in his body?

I have read about treatments for people with thalassemia in which medicines are given to increase HbF production, and by this, many people do not have to go for BT. Is this a viable option, or should I go for BT for my child?

Please help.

Thank you.

Answer 1

Hi,

Welcome to icliniq.com.

I can understand your concerns.

The definitive management of thalassemia is a bone marrow transplant.

For this, HLA (human leukocyte antigen) matching is done, and if possible, this is the best treatment available right now. The second one is a regular blood transfusion. We usually keep hemoglobin levels between 9.5 and 10.5 g/dL. It is necessary to maintain hemoglobin levels at these levels so that complications like bone changes, endocrine changes, and effects on the heart are minimized.

Along with this, iron chelator drugs are given. Hemoglobin of 3.7 g/dL requires an immediate blood transfusion. Third, there are some new drugs that increase HbF levels and decrease blood transfusion requirements. But this is in the experimental stage.

I suggest an HbF transfusion for your child immediately.

I hope it helps.

Thank you.