Discussion Forums > Thalassemia Major

Please Advise me!!

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Andy Battaglia:
IVS 1-1 (G>T) and IVS 1-5 (G>T) are the two beta thalassemia mutations that are present. They are 2 of the 6 most common thal mutations in the Indian sub-continent. IVS 1-5 (G>T) is a severe mutation often found in thal majors. IVS 1-1 (G>T) is a less severe mutation and is often found in thal intermedia. The two combined are likely to result in thal major, but would also account for why she has not needed transfusions earlier.

vampirebaby:
Thanks Andy :) That makes sense now!

vampirebaby:
Hi Andy,

Hope you're well. I need some help from you again if that's okay.

Eva had her 1st ever transfusion about 3 weeks ago. We're gonna have her Hb tested again this week. What should her pre-transfusion Hb be? If it's sitting at 9-10, would she still require a transfusion?

I understand that the Hb needs to be maintained above 9-10 but what if it's around 7-8 before her next transfusion? Does that mean she requires a transfusion sooner than 3 weekly?? Is it harmful to drop to 7ish briefly before a Tx??

Thanks alot in advance,

Regards
Sameet

Andy Battaglia:
Sameet,

Hb should not be allowed to drop much below 9, as it will have to be made up at some time with more frequent transfusions. Generally, you get around one point of Hb increase per unit of blood, so transfusing at 9 means less blood is needed. If you let it drop to 7-8, the child will not be as healthy and will need more blood during transfusions. Transfusing blood stresses the heart, so it's best to establish a routine that does not require more blood at one transfusion. Sometimes the blood is affected by antibody reactions and does not last as long. During those times, transfusions become more frequent. Outside of that and during illness, the Hb should not drop too fast. 3-4 weeks is pretty standard for that age, and a pattern will be established by how long she can stay above 9. If the test is above 9.5 transfusion may be put off for a week.

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