hydroxy urea in transfused patients

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Offline Zaini

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Re: hydroxy urea
« Reply #15 on: September 27, 2010, 03:36:30 PM »
Hi Syaida,

Welcome on the forum,we have a member Cherrianne who is a thal major and she uses hydroxyurea,it didn't make her transfusions stop totally but it helped her to prolong intervals between her transfusions,i believe she goes for transfusion every six weeks and she is a grown woman .

Just though you would like to know :)

Zaini.
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Offline Syaida Lee

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Re: hydroxy urea
« Reply #16 on: September 29, 2010, 03:07:00 AM »
Zaini,
Thanks for your note. Where is Cherrianne from? My doctor just replied to me after reading some of the articles that I "linked" her on Hydroxyurea and she said it depends on the mutation. It may work on middle eastern mutation of beta-globin gene but in her experience the south east asian patients that she has doesn't respond to it. However, we are exploring it, I understand my son, adel's inherits a copy from his father which is usually by Mediteranean descent and one from me, which is from South East Asian descent. She is also worried about the long term side effects (after 10 years) which is unknown, apparently cancer causing?  What do you say?

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Offline Manal

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Re: hydroxy urea
« Reply #17 on: September 29, 2010, 05:37:39 AM »
 Hi Syaida Lee


Our member is from Newzeland.

As for the usage of hydroxy according to mutation. This is correct and there is a test called Xmn polymorphism is made. If the result is positive,it means you will benefit and if it is negative this means you won't, BUT practically doctors found that some times patients with the negative result react very good to hydroxurea and their hb rises to the extent that they eliminate transfusion and their explanation to this is that sometimes there are other unknown factors that help the body to react positively and this resulted in that many doctors try hydroxurea any way  because each patient is completely a different case.
 

As for the safty of the drug, there is a study done by an Iranian group that proved the drug safty on a long term usage on low doses which is between 10mg per kg until 20mg per kg.

And in my opinion other drugs will definetly be available for patients before those 10 years that would do the same jo as hydroxy (hopefully)

Please make sure from the doctor that Adel's issues concerning his lungs does not contradict with the usage of hydroxy

Take care
manal


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Offline zahra

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Re: hydroxy urea
« Reply #18 on: October 03, 2010, 07:07:49 AM »
Hi everyone,
Yesterday on the 8th day of hydroxy urea 20mg/kg my son got a slight fever. This morning he was ok but now he seems feverish again. He is sleeping so I haven't seen how much exactly. The doctor had said if W.B.C. go low there will be fever and infection but not what to do if there is a fever and what kind of fever needs action. Should I wait and see ................or go to the ER?????
Advice wanted.
Zahra
PS: Others in the family have a cold and he did complain of throat pain yesterday.

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Offline Manal

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Re: hydroxy urea
« Reply #19 on: October 03, 2010, 08:29:52 AM »
Zahra,

Most probably he is having a cold or an infection specially that others in the family complain too. Take him to the doctor and check especially if there are other symptoms like runny nose,cough,...


But i must also note that i know a family whose son's body had a reacton with hyrea that was continous high fever (almost reached 41 c and throat infection and after several ons and offs and lowering the dose, it was discovered that his body does not tolerate hydrea and it was stopped but this is very very rare, even the doctor said that he is the only case during her whole medical life)


But in the case of your son, having other members in the family ill makes more sense that this does not have to do with hydrea. Keep monitering and give him th suitable medication according to his infection and keep us informed please

manal

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Offline zahra

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Re: hydroxy urea
« Reply #20 on: October 03, 2010, 11:32:08 AM »
Dear Manal,
thank you so much for your prompt reply. It means the world. My sons fever was gone by the time he woke up so I didnt take him to the doctor. I hope it is over but if it comes again I will take him to the doctor. The fever was very low and he has stayed active and happy throughout.
Zahra

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Offline Manal

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Re: hydroxy urea
« Reply #21 on: October 03, 2010, 12:56:08 PM »
You are welcome Zahra and i am sure he will be fine :hugfriend

Please keep in mind that we can not avoid the common colds or infections that our children are exposed to like any other children. Hydrea can be questioned if only things don't go the way it was and the frequency of infections increased, fevers with no reason...thiings that you are not used to or not common. Also bare in mind that these days the weather is changing and all of us are prone to infections

take care
manal

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Offline zahra

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Re: hydroxy urea
« Reply #22 on: October 24, 2010, 02:07:29 PM »
Hi everyone,
 4 weeks after starting hydroxy urea my sons Hb was 9.2 but he was not transfused. Next visit will be in a weeks time. The prescribing doctor had recommended letting the Hb drop to 8.5 before transfusing to give the hydroxyurea a chance to work but the 4 weeks after doctor said they will let it go down even below 7 if he is active and has no spleen or bone issues. Which is the right way? I couldnt see any real changes in his blood reports yet.
Zahra

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Offline Manal

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Re: hydroxy urea
« Reply #23 on: October 24, 2010, 02:18:05 PM »
Zahra

I can not get your question.... but if you mean when to transfuse him, i think that since he is on hydrea, you should not transfuse him as long as he is active and doing and eating well. You should be monitering his spleen and body activity

Did you check his hb after using hydroxy or not yet?? Also did you check his fetal Hb before starting hydrea??

manal
« Last Edit: October 24, 2010, 02:40:55 PM by Manal »

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Offline zahra

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Re: hydroxy urea
« Reply #24 on: October 24, 2010, 05:42:03 PM »
Dear Manal,
I understand your point of not transfusing now that he is on hydroxy urea so that the hydroxy urea would get a chance to work but the primary doctor looking after my son here had told me that initially they wont be trying for transfusion independence but spacing out the transfusions. He has been on regular transfusions a long time now  so it is scary all over again to watch him get pale and weak. I really hope to get to transfusion independance but perhaps it will take quite a while to get there. The last time his fetal Hb was checked was before his first transfusion at age 6 months which is about two years ago. Since then he has been living on donated blood. Maybe they will check again once the last tranfused blood is ending its life cycle. I dont know how long that is but I suspect its more  than the 4 weeks at the last check up.Till then I doubt there would be much fetal Hb. I worry also about the idea in some of the posts here that once the bone marrow has been surpressed so long via transfusions it wont start producing RBC's again. I hope that is wrong as hydroxy urea has been shown to benefit even Thal major patients sometimes and they must have been transfused long term.
His Hb after 4 weeks on hydroxy urea was just a little less than it usually is 4 weeks post transfusion at 9.2 but that was to be expected b/c of the fever he had anyway so no information there yet either. I guess its really just pray and wait for now.
Zahra

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Offline Manal

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Re: hydroxy urea in transfused patients
« Reply #25 on: October 24, 2010, 09:45:26 PM »
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

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Offline Andy Battaglia

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Re: hydroxy urea in transfused patients
« Reply #26 on: October 24, 2010, 10:20:18 PM »
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.
Andy

All we are saying is give thals a chance.

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Offline zahra

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Re: hydroxy urea in transfused patients
« Reply #27 on: October 25, 2010, 09:11:56 AM »
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
 

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Offline Andy Battaglia

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Re: hydroxy urea in transfused patients
« Reply #28 on: October 26, 2010, 07:07:37 AM »
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.
Andy

All we are saying is give thals a chance.

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Offline zahra

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Re: hydroxy urea in transfused patients
« Reply #29 on: October 26, 2010, 10:16:24 AM »
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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