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HbF Gene Variant Linked to Moderated symptoms of beta-thalassemia
Manal:
Hi Hopefulmommy and welcome
I know that in holistic medicne, breast feeding the baby from another mum ( not the real mum) has the ability to alter or affect the genetic coding .I know that this sounds crazy, but many holistic medicne doctors admit it
manal
Andy Battaglia:
Hi Hopefulmommy,
The chances of you and your husband having an HbE beta thal are the same as if two thal minors have a child, 25% with each pregnancy. As you have already experienced, the odds are meaningless in the real world. Do not believe that because you already have one HbE beta thal, that you won't have another. I know quite a few families that have produced three thal majors, so odds are something to be chanced only in Las Vegas. Genetic counseling is very important and In Vitro Fertilization should be considered.
I have never heard of any case of HbE beta thal that is worse than thal major. Generally when they are transfusion dependent, the transfusions are not quite as frequent as with thal major. HbE beta thal zero does not produce any hemoglobin A because no beta globin is being produced to combine with the alpha globin to make normal hemoglobin. If the beta hemoglobin mutation is plus, it means some beta globin is being produced so you would see some HbA. However, even with HbE thal beta 0, it is not possible to predict the clinical outcome. The ability to produce HbF will be one factor that moderates the condition, but there are other factors that are not understood which can also moderate HbE beta thal. Even though it seems that combining HbE with beta 0 should lead to the most severe condition, only about half of those affected have a condition as severe as thal major.
http://asheducationbook.hematologylibrary.org/cgi/content/full/2007/1/79
--- Quote ---The most serious Hb E syndrome is Hb E ß0-thalassemia. The compound heterozygote state of Hb E ß-thalassemia results in a variable phenotype ranging from a complete lack of symptoms to transfusion dependency. In review of 378 patients with Hb E-ß0-thalassemia from Thailand, the hemoglobin concentrations ranged from 3 to 13 g/dL, with an average of 7.7 g/dl. Approximately one-half of the patients are phenotypically similar to patients with thalassemia major who require regular transfusion therapy, and the other half resembles thalassemia intermedia.
--- End quote ---
Wheatgrass is safe for any age. For a child as young as yours, you might want to try a small dose of the extract daily. I don't think a child that young would willingly take wheatgrass juice since it does not taste good at all. The course of HbE beta tha can be significantly altered in many cases through the use of HbF inducers, so it may provide some help in maintaining an acceptable hemoglobin level.
Because the expertise of the lab can come into play when analyzing hemoglobins, I would suggest another hemoglobin electrophoresis after six months to both verify that there is no HbA and also to see what course the HbF levels have taken.
InGreece:
About Hb fetal...
In our daughter's treatment (Lepore, 2 and a half years old) we were told that one of the -admittedly secondary- goals of the transfusion regiment was to reduce her high Hb fetal which when she started transfusions (18 months old) was extremely high (70-80%).
This is now down to 7-8% which seems to have our doctors happy.
I was told that lowering this percentage means that her own bone marrow is suppresed and that has the benefit of avoiding bone deformations.
From what I understand some individuals have such a high percentage of Hbf (which is if antyhing more efficient in oxygen transportation) that they can avoid or minimise transfusion.
Should we have looked into this?
Doesn't having a high fetal hemoglobin percentage mean that bone marrow is working overtime?
Thanks and God bless
Andy Battaglia:
Hi InGreece,
When your daughter's fetal Hb was 70-80% what was her total hemoglobin level? I assume fairly low if transfusion was started.
I don't understand what the concern was about the high HbF percentage unless it was being used as a test of bone marrow activity. If the HbF wasn't enough to raise the total Hb to acceptable levels, I don't think it could cause any harm but it would be an indicator that there was excess bone marrow activity. Of course, transfusion will both dilute this high HbF % and also suppress bone marrow activity. Whether you should have followed the HbF induction route depends on her clinical picture. If her Hb was in the typical range for majors, HbF induction would not be sufficient to avoid transfusion and there would be excess bone marrow activity. This may very well change in time as new HbF inducers are developed. The work of Dr Susan Perrine is of specific interest because of its potential to raise Hb levels several points.
The goal in fetal hemoglobin induction is raising hemoglobin levels which in turn, helps regulate bone marrow activity. Rather than increase it, the rising hemoglobin level will adjust the bone marrow activity closer to normal, depending on how high the Hb can be raised.
Manal:
Hi Andy
This issue of fetal HB is always confusing me because i don't get it as why in majors we decrease it as it is an indication of high bone marrow activity and in intermedias we tend to increase it and increasing it in intermedias means that their bone marrow is being suppressed (that is what hydrea does)????
Why an increase in fetal Hb is sometimes not reflected on the total Hb?????
manal
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