Discussion Forums > Thalassemia Major

My Son Rudra

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Mukta:
Hello Andy Sir and rest of the folks on this group ,

My son Rudra (3.5 mths old) has been diagnosed to have thalassemia major . In last 21 days he has already gone through 2 transfusions.
At 2 mths and 15 days (4.2 kgs at that time) his hemoglobin had dropped to 5.5 g/dl after which the doc did a transfusion of 100 ml blood. His HB shot up to 12.4 g/dl after this transfusion. At 21st day after his 1st transfusion we went for HB test again at which his HB was at 8.8 g/dl . Since he had HB dropped below 10 the doc advised us for the 2nd transfusion of 50 ml (As per his weight of 4.9 Kgs) . His HB after transfusion is 12.1 g/dl.
In these days we got his Hemoglobin electrophoresis and DNA study test done which more of less confirmed his case to be of thal major .

His DNA study report had the following observations :
Child : Mutation type : IVS 1-5 (G-->C) B-Thalassemia trait Homozygous.
Both Parents Mutation type : IVS 1-5 (G-->C) B-Thalassemia trait Heterozygous.

Pre - Transfusion HB electrophoresis report
HBF : 90.9 , Hb A0 : 6.9 , Hb A2 : 0.7

We are getting him treated in Jupiter Hospital , Thane (Pretty close to Mumbai/Bombay) , India under a pediatric and hematologist. 

I have been following and reading a lot in this group from last 20 days and the group post have helped me immensely to understand this disorder much better and in a large extent given me strength to understand how we can manage this disorder and what are my options of treating him. Thanks to everyone in this group for putting up such a great forum.

My questions to the group to start with :

1) Want to know some good doc in Bombay , India for second opinion and more guidance on how we can manage this disorder and if anything we can do more for such a small child.
2) The 2 transfusion he had to undergo the doc advised us to go for irradiated blood which will help in future for BMT. Is there anything else I should take care w.r.t the blood transfusion
3) Should I worry so soon about the iron accumulation .
4) It is normal for such a small baby to drop his HB in like 20 days. From what I had read for small babies it takes usually 50 - 60 days cycle.

This is my first post to the group . Sorry for long post but wanted to provide all info.

Canadian_Family:
1) Want to know some good doc in Bombay , India for second opinion and more guidance on how we can manage this disorder and if anything we can do more for such a small child.

Sorry, can't help you with respect to this question. Perhaps our members from India can help.

2) The 2 transfusion he had to undergo the doc advised us to go for irradiated blood which will help in future for BMT. Is there anything else I should take care w.r.t the blood transfusion

I don't see how irradiated blood can help BMT in future (two different concepts). Irradiated blood is sometimes used for neonatal and paediatric patients to prevent Transfusion Associated Graft-Versus Host Disease (TA-GVHD). You should try to maintain 10g/dl. We never used irradiation for our daughter.

3) Should I worry so soon about the iron accumulation .

It is too early to worry about iron accumulation. Iron chelation usually starts after 12 to 15 transfusions. Children as young as 2 can start exjade.

4) It is normal for such a small baby to drop his HB in like 20 days. From what I had read for small babies it takes usually 50 - 60 days cycle.

It is possible that the HB drop in 20 days with the use of irradiated blood. Irradiation reduces the storage-life of red cells and whole blood.

Himanshu Kumar:
Irradiation of blood is given to prevent transfusion related graft vs host disease. There are few hospitals in India which provides irradiated blood for transfusion. You must follow Dr's advice and I don't see any reason for not using irradiated blood if your hospital is providing one. Since you are planning for transplant, you should also opt for blood filters for Leuko depletion of blood and also to prevent transfusion associated complication like CMV virus. CMV is one of the big complication that arises after bone marrow transplant and patients exposed to CMV virus before transplant are more susceptible to develop CMV post transplant.

You can find details of blood filters from the link below.
http://www.pall.in/main/medical/product.page?id=48145

You can order Pall filter from the below link at at subsidized rate. They charge a nominal membership fee. For bulk order the price per filter will be 50% lesser than the market price. 
http://www.thalassemicsindia.org/what-is-thalassaemia.php

Since your child's HB had dropped to a very low level 5.5, it will take a few transfusions (2-3) before your child's HB will start stabilising. Once your start maintaining a HB of 9+, you will find the transfusion cycle to be more between 5 - 6 weeks for the first 2-3 years.


Regards,
Himanshu

Lokkhi maa:

DearHimanshu,

After 2/3 yrs the duration of week will be reduce or not? I mean more transfusion will need?

Nomad:
Dear Lokkhi maa, more transfusions or more blood bags on every transfusion will be needed as your son will grow up and gain more weight. Which on the other hand is important so a child can grow up healthy and most of all with healthy bones...

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