Discussion Forums > Thalassemia Major

Fetal Hemoglobin

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Andy Battaglia:
Hi Manal,

When the Hb is described as 98% HbF it means that 98% of the total hemoglobin is fetal hemoglobin, but it is not a measurement of the total quantity of hemoglobin. The total hemoglobin in this case is actually very low, but what hemoglobin is there is mostly HbF. An analogy would be, suppose you are baking bread and need 4 cups of wheat flour and you discover you only have a pinch of wheat flour but you have half a cup of rye flour. 98% of the flour you have is rye flour. You have flour, but not enough to bake your bread. This is the same thing. Of the hemoglobin that Abdulwahab had, 98 % was HbF, but the total Hb wasn't enough to "bake the bread" of survival.

KHALIFA:
Dear Manal
   my son when he born until he was three years old never ever use any DRUGS because the Dr in kuwait and  also Prof.jhon Porter they want to see if he can be indebendent transfusion or no they want to know if he can be thalass. intermidia for ever or just for some years and after that he will be thalass. major and don't forget the thalassi. inter. also he need some kind of transfusion ones or two times a year .. actually that what i understand from the Drs everywhere :dunno :huh
                              khalifa
                       state of kuwait

KHALIFA:
OK... Andy you got the right explaination to us  :thumbsup
            i think  :huh we can open a beakery store  :rotfl lolup
        i love cake too much  :food
                          khalifa
                      state of kuwait

Canadian_Family:
Hello,

Once the child TOTAL HEMOGLOBIN is deducted low, he/she should be given three months at the most to see if total hemoglobin in not dropping. In such case, transfusion regimen should start immediately. However, there are cases where a child has survived upto six years without transfusion. Genetic testing these days immediately determine if Beta chains are failing or not.

Christine27 daughter is a classic case where the total hemoglobin so far is holding good as a result of Fetal hemoglobin production, once the baby grows the fetal portion does not provide enough hemoglobin and the body reacts to compensate for the deficiency. The HBF % is always high in thalassemia major but like Andy said total hemoglobin can be low.

Regards

Manal:
Thanks Andy, Khalifa and Canadian family.

But Andy, so why do we give hydroxyurea then? . What i understood from  your answer is that what matters is the total quantity of HB. So giving the hydroxyurea will induce the production of the F HB which is not '' the kind of flour i need '' , so why give it and make the body has a big percentage of it since it can not help the patient to survive in thal.

Sorry i feel really stupid that i don't get it :huh :huh

Manal

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