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Thalassemia Patients and Friends and thalpal Ā© A. Battaglia 2019





55248 Posts in 5898 Topics by 6188 Members
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A message for all  parents who are thals. Keeping your iron load under control is an absolute obligation to your children.
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Author Topic: hydroxy urea in transfused patients  (Read 24668 times)
Andy Battaglia
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« Reply #45 on: February 15, 2011, 12:25:10 AM »

Hi Zahra,

In two months the Hb jumped two points while on hydroxyurea. The HbF also rose. I would say you should start again. Hydroxurea reduces ineffective erythropoiesis, which means there are fewer bad red blood cells floating around in the blood, which in turn leads to less destruction of the good blood cells, and then the good red cells live longer, causing an increase in Hb, along with the increased HbF. I would really go six months more and then evaluate. I know parents worry about the side effects of the drug, but there is a silent danger within the blood of thalassemics, and it is caused by a combination of bad red blood cells and unmatched alpha globin chains, that are in excess due to the deficient beta globin production. We should not underestimate the damage that this does to thals. Even thals with no iron load suffer from a shrinking pituitary gland that eventually disappears altogether. It is not just iron that is the danger. The same thing goes for the heart and pulmonary artery. The damage is not solely iron related. When you consider this, hydroxyurea becomes a much safer option in relative terms because of this ability to reduce the ineffective erythropoiesis. Results are also seen in reduced spleen size, lower bone marrow activity, which means less stress on the bones from expansion, and more normal growth. I have yet to see a study that did not show a positive effect on the majority of thals who used hydroxyurea. In fact, I have often found a tone of surprise in some reports, because they really did not expect it to work so well. Even with a low increase in Hb, these other things are taking place. As I have advised with Manal's son and found that Dr Marwaha concurred, we have to look at the child. If growth is in the normal range and the child is healthy and has normal activity, then we have to keep doing what we are doing. If you see noticeable changes in activity level, increased lethargy and worsened health, then transfusion may be necessary, but as long as a child is healthy, transfusion isn't an ideal choice.

Doctors are really pushing BMT these days because of increased success rates. You may want to investigate if a mother to child transplant is possible. I don't think there's a good way to screen. Only siblings would have a good chance of being a perfect match. I doubt there is any better chance within a set of relatives than there is in the general public. I would suggest contacting Eileen at CAF to see if she can give you information on the bone marrow registry.
Patient Services Manager
Eileen Scott
eileen.s@cooleysanemia.org
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Andy

All we are saying is give thals a chance.
zahra
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« Reply #46 on: February 15, 2011, 04:25:15 AM »

Hi Andy,
thank you as always for your expert advice. I should have made it clearer that my son has been continually transfused during this time. More often than before which was 4 weeks.During use of hydroxy urea it was once at two weeks and the rest at 3 weeks. The doctors stopped the hydroxy urea b/c of no increase in transfusion intervals. Even with more frequent transfusions his HB was less until they upped the amount transfused from 250 to 280 ml. This last report of Hb 12.7 is from 5 days after transfusing 280ml of blood. So the only positive thing is the slight increase in HbF. Hb has not gone up b/c of hydroxyurea and neither have transfusion intervals increased at least not yet. Is it still your opinion that hydroxy urea should be restarted?
Thanks again.
Zahra
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Andy Battaglia
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« Reply #47 on: February 15, 2011, 12:03:33 PM »

Hi Zahra,

I feel that the trial has to be six months minimum for any patient on hydroxyurea. The full effect is often not see for two years or more, so it definitely is a gradual change. What I would look at now is was there any change in retic count or bilirubin levels, as positive changes in these would indicate that hydroxyurea is cutting down the amount of ineffective erythropoiesis. I don't know how much change you might see after a short time on hydroxyurea, but any lowering of either value would have to be considered as a positive development.
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zahra
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« Reply #48 on: February 15, 2011, 01:16:47 PM »

Hi Andy,
i feel really lucky to get two answers in one day. They havent done any retic tests since he started transfusing. Ther are bilirubin results though. Before starting hydroxyurea on sept 23
bilirubin total 19 and bilirubin direct 2.6  Sept 21
BT 23 and BD 3.0  Oct 20
BT 27 and BD 3.0 Oct 27
BT 29 and BD 2.0 Nov 23
BT 21 and BD  4.1 Dec 8

and I dont have any later reports back from insurance yet....................
Will try to get them tomorrow and post them then.
Thank you for all your help once again.
Zahra
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zahra
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« Reply #49 on: February 19, 2011, 07:27:48 AM »

Hi

i spoke to the head doctor this visit and he said the change in fetal Hb really isnt useful unless transfusion intervals increase. He also said bone marrow transplant is not recommended for thalassemia patients unless they have a 100% matched sibling.This is to limit the chances for rejection so it is of no use to look for a match in further relatives or bone marrow registry.
The only option since both of my other kids don't match is to go for PGD-HLA. That is such a loaded option. So many things to wrestle with.
I wish I could ask what to do but I know no one really would have the answer to this one.
The doctors parting words think about it and pray and see what you are comfortable with. May Allah guide us correctly.
Zahra
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