Help required please..! Thal screening report

Hello Everyone,

My son 2-1/2 year is Thal major as doctor told us. My Son color was little pale since early childhood, but we observed major color pale at his age of 2-1/4 yr. So we consulted doctor and he made some some blood tests from a local lab in Saudi Arabia, His HB was 5.5 so the doctor recommended for Thal screening, After seeing the screening report the doctor declared him Thal major. So blood transfusion was done at that time and his Hb came around 12. The report is as under.

Hb A = 13.11% , HB F = 84.50%, Hb A2 = 2.39%

After one and half month we decided to do another Thal screening  from a foreign (German) Lab just to re-confirm the first report. High performance liquid chromatography was done. The report of this second test is as under.

Hb A = 63.7% , HB F = 33.6%, Hb A2 = 2.7% 

This second report says that it is not Thal Major.

We shared this report with our doctor and he told that He cannot rely on this second report as the second Thal screening was done after blood transfusion. So may be this report is not correct.

We are confused here what to do. We did this second Thal screening after 1-1/2 month of blood transfusion. So I just want to get the opinion from some experts here on this forum. Is this true that this 2nd screening is not reliable? Or we should monitor the Hb of son on monthly basis? Please advise, appreciate any help. Thanks

Best Regards
Saqib

The second screening does reflect the transfused blood. The first screening shows thal intermedia, not major, but since the Hb was so low and transfusing was required, it may not make much difference. Has the child continued to transfuse?

Yes transfusion was done three times.

3 April - Hb was 5.5, transfusion was done and Hb raised to 12
17 May - Hb was 9.5, transfusion was done and Hb raised to 12
14 June - Hb was 10.6, transfusion was done and hb raised to 13

Doctor has advised us for monthly transfusion, regardless the Hb was 10.6 last time.

What you suggest Andy?

Thanks

It definitely looks like regular transfusions are required. The current guidelines recommend never letting the Hb drop below 9.5-10. I fully agree with this and considering the age of the child and the desire that he grow normally, I would advise not allowing the Hb to drop below 10 before transfusions. Many of the growth and development issues in thals can be avoided by maintaining a high Hb during the growth years. A good nutritional program, with an emphasis on antioxidants is also advised.