Fetal Hemoglobin

Does anyone know how rare it is for a major to function off of fetal hgf for life?

Is it unusual that is lasts into adulthood???

Thanks,
Christine

Raised fetal hemoglobin levels are normally found in majors, but by definition, majors cannot survive without transfusions.The percentage of HbF found in majors varies but it is not enough for them to function. The only exception I know of is the few cases in trials where majors have had HbF stimulated by hydroxyurea and became transfusion independent. If the body makes enough hemoglobin to survive without transfusion, the diagnosis would be either intermedia or minor.

thanks Andy!~!

Makes perfect sense 

Regard my son we was in very bad situation when he born his FHB was 98% and Prof.jhon porter in UCH in london told me you son is thalass.intermidia and for 3years he didn't recive any blood trans. and always his HB between 8 and 10 .. but after this three years he be sick when i took him to the hospital i found out thy HB was just 4 so from that day they start transf. him every month until i made for him the BMT ..
                                                  khalifa
                                             state of kuwait

There is something here that i can't understand!!

We are supposed to take the hydroxyurea to increase the HB f levels in the body and therefore stop transfusions, so why Abdel Wahab for example couldn't though his HB F was 98%?? In intermedia thal, this works although genetically majors and intermedias are the same???

I understood from previous posts that adults can survive with HB F instead of the adult HB so why majors can't though they have a lot of HB F???

I am so confused

Manal

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.

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
                              khalifa
                       state of kuwait

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

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

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

Manal

Manal,

Actually the body can survive quite well using fetal hemoglobin just as you could make that bread from rye flour if you have enough rye flour. The purpose of the hydroxyurea is to stimulate the production of more fetal hemoglobin than what is normally present. Surprisingly, the increase in total Hb doesn't have to be a lot before positive results are shown. In Intermedias, as little as a one half point increase in Hb can result in freedom from transfusing.

So just as you can substitute another type of flour to make bread, you can substitute fetal hemoglobin for adult hemoglobin, and as long as you have enough flour or hemoglobin, you'll be ok.

One thing about this topic that I would like to clarify. The percentage of fetal hemoglobin present is not useful in determining the total volume of hemoglobin present. The reason it is mentioned is because fetal hemoglobin is one of the indicators of thalassemia and the higher the percentage of total hemoglobin that is HbF, the more likely the patient has thalassemia. 

Hi all,

Percentage is a relative quantity. Meaning that it depends on the part of a thing with respect to the whole stuff.

For example:

Your child has HB 6g/dl of TOTAL Haemoglobin; which everyone knows is not sufficient. Out of which 1g is HbA and 5g is HbF. => (1HbA+5HbF=6HB)
This means your child has:
¹/₆ X 100 = 16.66% of HbA
⁵/₆ X 100 = 83.33% of HbF

Now, although 83% seems a big number, but 83% of total 6g, which is 5g is still not sufficient for survival.

I hope I got this straight

Take care, Peace!

Thanks Andy a lot , it is more clear now

Thanks Sajid, but the last time i did HPLC for my son was in last July and the percentages were as follows:

81.5% HB A1
6.9% HB A2
11.6% HB F

so what do you think?

Manal

Hi Manal

In human being different stages of life there are different types of hemoglobin namely embryonic, fetal and adult hemoglobin. These switching of hemoglobin take place at different timing of life from embryo to adult.

How ever in certain cases such as Thalassemia due to mutations in globin chains there is imbalance in proper hemoglobin formation. For example in Beta Thalassemia major there may be no beta globin formation or less beta globin formation. That may lead to decrease in adult hemoglobin (alpha2 and beta2). Since there is mutation in beta globin formation gamma globin which has to be switch off by nature which is responsible for fetal hemoglobin formation remain in these patients (alpha 2 gamma2).

The question is what’s wrong with high fetal hemoglobin? The role of hemoglobin in the body is to carry oxygen and to deliver the tissues where it is need to produce energy to perform. Fetal hemoglobin has very high affinity for oxygen, but by nature it is not that good to deliver the oxygen to the tissues. Since there is imbalance in globin chains these cells tend to hemolyse quickly and that leads to severe anemia in these patients and they need regular transfusions.

But in Sickle cell anemia and thalassemia intermediate by giving hydroxyuria fetal hemoglobin is increased because both of this condition it helps to carry oxygen. Especially in sickle cell anemia, sickling is more when cells are in hypoxic condition. By having high fetal hemoglobin helps to increase oxygen level in these conditions.

Your question about HPLC results, High Performance Liquid Chromatography is technique to quantitate hemoglobin. Usually very little amount of dilute hemoglobin is fed in to this machine and the results are given as per retention time and these percentages are given. This is totally different from total hemoglobin levels we refer to estimate anemia.

I hope you got some idea about how hemoglobin works.

Thanks Sajid, but the last time i did HPLC for my son was in last July and the percentages were as follows:

81.5% HB A1
6.9% HB A2
11.6% HB F

so what do you think?

Manal

OK, even if there are 3 types of Hb, the sum of all the percentages will be 100% and even so, if the Total Hb. is low then any type of Hb will also be low no matter how high percent they show. It's just like Andy said. You want to bake a bread but you don't have enough flour. Let me try to re explain what Andy said.

Rye flour is HbF and Wheat flour is HbA

Consider it as you need four cups of wheat flour but you have only one cup of flour out of which only a spoon full is Wheat flour and rest is rye flour.

This makes rye flour 98% of 1 cup but you need 4 cups!!! So you can't bake bread!!

And if you had 4 cups of rye flour,(by inducing HbF with hydroxyurea or wheat-grass) the bread would bake but will not taste good as the Wheat flour bread



Take care, Peace!