Hello All Again! Some good news

InGreece,

Thank you very much for sharing this information with us, it will be useful to all of our members.
I am glad that you were able to have this discussion with your doctor. 

Best of luck,

Sharmin

Hi ingreece ,
 :cograts for the new baby in your family , may God bless the new baby and his family with best in everything ..

Best Regards for you all
Take care
Umair

There are a lot of interesting things in this post, but this is something I would like to know more about and wonder if Sharmin can ask Dr Vichinsy what he thinks about the pros and cons of stimulating fetal hemoglobin as an alternative to transfusions. Can fetal hemoglobin deliver sufficient oxygen to ensure normal growth in thals? We know that fetal hemoglobin has a high affinity for oxygen, which means it does attract oxygen quite well, but it also doesn't let go of oxygen as easily as adult hemoglobin does.

They argued that the quality of Hbf is extremely low (Hbf -fetal hemoglobin, that Sophia's type has very high) is quite inefficient and that, in their department's experience (they regularly manage 450 thal children) relying on it leads to several problems including cardiological ones. Obviously, if a child has sufficiently high Hbf and no stress on her bones they might monitor and start transfusions later (we had to start because bone indications showed that we should) but I got the feeling that in the past, children with high Fetal hemoglobin relied on the Hbf  too much to their detriment.

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I also want to point out, that the decision to transfuse does have to be made based on the physical development and bone growth of the child. Ultimately, it all comes down to that, regardless of hemoglobin levels. For parents of intermedias, HbE beta thals, and beta thal Lepore, this is very important to understand. Predicting whether transfusions will be necessary is not exact and observation of the child's growth and pace of development, must be the determining factors.

Andy,

I will add this to my list of questions. 

Sharmin

what he thinks about the pros and cons of stimulating fetal hemoglobin as an alternative to transfusions

Andy, i thought is issue is being finalized... or what is the point of giving hydroxyurea. Remember in Singapore when they discussed the case of the 60 year old man who has mutation in codon 39 and he has all his blood 100% fetal and never got the adult hb. But again we can not count on only one case


Sharmin it will be good to ask him too if possible whether switching on gamma genes to produce fetal Hb has proved to switch on other unwanted genes or not ?/

manal

Manal,

This is correct. Many thals do well because of fetal Hb. But, is it the case that not all fetal Hb is so useful, and should not be relied on because it will lead to poor growth? Ingreece has been told that fetal Hb is not sufficient. Is this belief shared in other treatment centers. I hope Dr Vichinsky can elaborate on this subject.

Andy, Manal,

I will add to my list of questions:

1)  pros and cons of switching on fetal hg as an alternative to transfusions, can fetal hg deliver sufficient hg to ensure growth because
     fetal hg has a higher affinity for oxygen and is therefore less likely to release it.
2)  do techniques that switch on gamma genes to produce fetal hg tend to switch on unwanted genes as well. 

I will add to this conversation that Dr. Vichinsky had told us that it is preferable in our case to at least attempt to switch on fetal hg.  He also mentioned that there were techniques that he has been investigating that may be quite promising in this regard.  He told us of a case in which a young boy had strong reactions to blood and was therefore un transfuseable.  In this case Dr. Vichinsky, with the help of fetal hg inducers was able to have him produce his own blood. 

I wonder if in these specific cases (where antibodies are involved) fetal hg is preferred over possible transfusion reactions or the hemolytic anemia that little A was experiencing.  Perhaps, if fetal hg is less efficient, he felt that in certain cases this con may outweigh the complications of transfusions and iron overload.  I am speculating at this time - but I will try to get some answers during our visit. 

Sharmin

Thanks Sharmin

manal

What is fetal hg 

Fetal hemoglobin is the hemoglobin a baby uses in the womb, before birth.  It uses globin produced by the gamma globin gene rather than the beta globin gene. This disappears for the most part by 6 months of age, and is replaced by adult hemoglobin. In thalassemia, the level of fetal hemoglobin varies but it is higher than the trace amounts found in non-thals. Because some thals have a great ability to produce fetal hemoglobin, methods are often used to induce its production. Some of these methods are drugs like hydroxyurea and butyrate, and natural supplements like wheatgrass and resveratrol. With the use of these inducers, some patients have a reduction in transfusion needs or complete elimination of transfusion dependence.

Has anyone heard anything else about Hemaquest?  The concept sounds so promising.....

Sharni, they didn't start yet, don't know what is making them late. If i receive any thing, i will update you all

manal

Thanks Manal 
Sharmin

I see that the subject still has some legs. :-)

What Andy and Manal have brought up is quite interesting especially in light of the fact that Hydroxyurea is more or less based upon increasing fetal hemoglobin.

I tend to agree with Andy.  It comes down to judgement calls based on an overall picture.

The conversation was with regards to Fetal Hemoglobin in a child patient.  I got the impression that their decision in the past to allow children to get by using their Fetal Hemoglobin was not the correct one but that does not not mean necessarily the same as far as adults are concerned.

Certainly hearing from another medical center would be great.

I'm also wondering about Hemaquest.