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hydroxy urea in transfused patients

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Manal:
Dear Zahra

I totally understand your fear about the idea of not transfusing, but all what i meant is to monitor very closely in a way that will not make him reach a stage of ''weakness and palness''. The purpose for this is to know how long hydrea will keep him away from transfusion.

It is as if you were transfusing for the first time, definetly you kept monitering until you knew for example that he transfuses every 4 weeks.

If you just followed your normal schedule of transfusion, how will you know if you will benefit from hydrea in increasing the gaps between transfuions, hope you get my point :wink

As for the fetal HB, there is no fetal Hb in transfused blood so any fetal Hb measured in his body will be only his. Usually measuring fetal Hb is done before starting hydrea in order to compare before and after usage  to see if hydrea has caused any increase or not

Wish you all the luck :biggrin

manal

Andy Battaglia:
Hi Zahra,

As Manal mentioned, you should have a baseline HbF reading for later comparison. If the HbF rises while using hydroxyurea, it is a sign that the hydroxyurea has a better chance of working. Don't worry about the bone marrow not being able to start producing again. Every thal can tell you about bone pain when the Hb is low, a sign that the bone marrow regularly does try to start to produce red blood cells when the Hb gets low. Of course, it is mostly useless activity in majors, but it does readily occur as soon as the Hb drops.
I suggest patience, as it does take 1-2 years to adequately assess if hydroxyurea is having a real impact. Hopefully, you will see some change in the percentage of HbF present.

zahra:
Dear Manal,
Thank you for your support. You are right that it is like starting all over again. There was a sense of calm in the knowledge that his Hb will stay in the safe range for 4 weeks at least after transfusion. Less worry I mean. But of course the danger of transfusiona nd iron overload is greater that way. If hydroxy urea works it is the best way  I guess b/c it will eliminate transfusion risk, reduce iron overload and reduce the clutter of unused excess alpha chains. That is why we must try.
Andy ,
Thanks for your post. It makes me wonder if the body pain my son has been complaining about is from his cold or from the resumption of bone marrow activity. He especially complains of pain in his hands. ??? As you mentioned we need a baseline fetal Hb to compare with but I was just thinking that when tranfused there is no bone marrow activity and hence no fetal Hb and that will come into the picture when transfusion is stopped and bone marrow is active again. Maybe I am wrong and need to be corrected. Is there high fetal Hb in a transfused thal patient or is it normal as long as transfused? What I mean is when do do the test to get his fetal Hb before the effect of hydroxy urea but after he starts making his own blood again so there is a baseline Hb. If we test while he transfused wont it give a false impression of increased fetal Hb due to hydroxy urea when it is actually due to going back to his own pattern of high fetal Hb due to thal. I hope I am making sense. Also is the fetal Hb at 6 months of any value in making this assessment of baseline Hb F?
Thank you both again.
Zahra
 

Andy Battaglia:
Zahra,

The 6 month fetal Hb percentage isn't good for a baseline, but it might give a hint at his ability to produce HbF by what its percentage was at that time. It doesn't matter how much HbF is currently present, but the level now gives a reference for future measurements. What you hope to see is even if the Hb level doesn't rise significantly, but remains somewhat stable, that the percentage of this Hb that is HbF will rise. When this happens, we know that the bad red blood cells are being suppressed while the red cells with higher amounts of fetal hemoglobin are becoming more prominent. This is far more important than it sounds because it means there is less bad hemoglobin produced, so fewer defective red cells and less "debris" in the bloodstream.

One thing to remember with hydroxyurea is the seeming paradox where bone marrow is suppressed to reduce the amount of defective beta globin produced, but the drug also can basically turn back on the gene that produced the gamma globin found in HbF. I really think this needs to be emphasized, as the benefits of hydroxyurea are more than a stabilization or rise in Hb. Reducing the amount of defective globins produced is one of the main goals of treatment for thalassemia. This is somewhat accomplished by keeping the Hb high through transfusions, and it is also accomplished by use of hydroxyurea. Both reduce bone marrow activity. Use of hydroxyurea in transfusing patients has worked in trials and some patients have become transfusion independent, while others have reduced their transfusion frequency. Every patient is unique, but the majority of patients in trails have found some benefit from hydroxyurea use. I also want to add that better drugs are in trials, which will make this approach to treatment much more productive.

zahra:
Hi Andy,
Thank you. I will ask for a Hb electrophoresis but I remembered on looking for his 6 month report that this lab doesnt give fetal Hb amount. just greater  than 40%. I will ask but will probably have to take him elsewhere to find the correct amount of fetal Hb . That will be sometime after his appointment tomorrow then.
Zahra

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