hydroxy urea in transfused patients

Can anyone suggest where I can do the xmn polymorphism test? The last I checked with my son's doctor that it is not available in Singapore. I was told by her the nearest could be Thailand for me. Can anyone link me to a hospital or doctor contact so I can air flown Adel's blood overseas from Singapore/Malaysia?
I need to have a back-up plan should we need to start HU. Thanks in advance.

hiiii all ,
     Today after reading all the posts on this topic i was very disturbed thinking that some of u are so lucky that atleast ur doctors guide u in using l-carnitine/hydroxyurea/magnesium.....but here in my case m hearing all these names for the first time even after being wth thal from past 7 years
       I know that the doctors will never be proactive and let u knpowbut here i had asked ma doctor about this and he told me not to give attention to all these things and he is a renowed haemat..........its been high time that i think we should change the dr who also is concerned wth the patients and not jst wth the earnings........m really dissapointed and angry at the same trime as even we are not finanacially tht good that we could meet wth all the expenses but atleast if we know that there are ways to help our child may be we can atleast try for that and if possible
          can anyone pls suggest me good haemat in mumbai........................pls i really need one for my daughter m literally in tears ...some of u may think that i m so negative and rude in my remarks but the kind of approach i ve got is horrible just cant say nything else here...............so pls help me out

Hi pleasance

I really can understand what you feel and i definetly agree that this is annoying to any parent. But unfortunatly not all doctors are fully aware of the management of thal and to some extent in chronic disease parents have to search and search for any thing that can be of a benefit.  But the good thing is that it is never too late

Definetly you will see anyof our friends who live in mumbai will guide you to where to go

Wish you all the luck

manal

Dear Pleasance,

I can understand your pain and frustration.  To be honest, most doctors, even here in Canada are not aware of these things.   We also had to research these things on our own in order to give them to our son.  My son was 8 or 9 when we began giving him these supplements.  To be honest, thalpal is where I found out about most of them.  Were it not for Andy and our great friends here I don't know where we would be now.  4 years ago when I came to this site my son's antibodies were out of control, we were not on any of these supplements and his iron levels were beginning to increase. 

With Andy's advice, we took him to Oakland, had him assessed and began treating his antibodies and reducing his iron levels.  Thankfully, his iron is now well below 1000 and he is on many different supplements.  You have come to the right place, we will all help you here and we will make sure your daughter does well regardless of what your doctor is educated about. 

Don't worry Manit, as Manal has said - it's not too late.  Your little girl is precious to all of us - and we are here to support you.

Sharmin

i thank all of u here m so lucky to be a prt of this site and if there is any one in mumbai who is on all these supplements pls lemme know tht as tht wld be of gr8 help

Hi,
Today it is 9 weeks since starting hydroxy urea and I am feeling depressed. At yesterdays appointment the doctor suggested stopping it. My sons Hb was 7.5 yesterday at 4 weeks post transfusion and was 8.2 four weeks ago. These are pretty much the lowest values since he started transfusing. I would have said it wasnt fair b/c he had been ill in a period starting a week before and lasting at least a  week after the 8.2 and thus affecting the HB both times but he had more  reasons. He said twice the urea levels had been high so it seems it is affecting his kidney functions. The first time they had thought it may be b/c he had been refusing to eat and seemed dehydrated and the urea went down from 8.8 to 6.4 with IV fluids. Yesterday the urea was 8.3 again. In the end we decided to give it one more month  with great care towards keeping him hydrated.
I do have questions after looking at the old reports. His Urea was 9 in July which was before they even started hydroxy urea??? Could it be  the exjade that is causing this. Perhaps this is why the doctor said to stick with a lower dose of exjade for now.  How concerned should I be?  Am I doing the right thing going on with hydroxy urea?
Zahra

Hi Zahra,

Do you have electrophoresis tests before and after starting hydroxyurea? This is far more important for determining whether or not any benefit is being derived from hydroxyurea than Hb alone. What is hoped is that the percentage of HbF is rising even if the hemoglobin level is staying in the same range. If hydroxyurea has little or no effect on HbF, there is much less chance of it working, but if there is a transition to  more HbF, this is a good sign and shows the intended changes are happening. Hydroxyurea will somewhat suppress the bone marrow, so the amount of bad hemoglobin being produced will drop, while the HbF level will rise. If this occurs but the hemoglobin  level doesn't move much, it is still a positive gain, so then physical observations may become more important in deciding whether or not to continue hydroxyurea.

I would also say,it hasn't been long enough to make a determination, as it takes one to two years to see the total effect. Also, since he was sick, this can affect everything, so patience is required.

Hydrate, hydrate, hydrate. All thals should listen to this. Chelators affect the kidneys and liver and you need to stay hydrated at all times, especially when using oral chelators.

Hi Andy,
Thank you for replying so quickly. You are amazing. I am feeling so guilty about not getting the elctrophoresis done. When they didnt do it at his hospitak I took him to see our regular family paediatrician and asked there but they said their machine was out of order and to call back in a few days. I still cant believe I forgot . Would it still be useful if I got it done now? do you think the elevated urea is b/c of exjade? He does show symptoms of dehydration like chronic constipation. I'm working harder to get him to drink water now. If the urea is elevated b/c of hydroxy urea what would be the level of urea at which discontinuation should be considered. I have to admit I was shocked by the Hb of 7.5 b/c he was behaving normally and his appetite was improving.
Zahra

Hi Zahra,

Yes, get an electrophoresis done. Even now, it is important to note the HbF level. As time goes by, you want to see this percentage of HbF be a significant portion of the total Hb and hopefully rise. You need some point of reference, so it is not too late.

The high urea is undoubtedly connected to being dehydrated. Encourage him to drink plenty of water daily. As you have already seen when his urea dropped to 6.4. he gets within an acceptable level once properly hydrated. With Exjade, hydration becomes even more important. Correcting this is an area where the parent makes much more difference than the doctor can. Establish good hydration habits when he is still young.

The physical description you gave of your son shows what is most important. Normal behavior and a good appetite. What we have seen with Manal's son is that his Hb has not risen, but is fairly stable. However, his HbF percentage rose over time and his growth and activity level are in normal ranges. I feel that this is more important than an elevation in total Hb. If the child's Hb stays fairly level and his health and energy are good, and he shows some ability to produce HbF, then I feel hydroxyurea should be continued.

The physical description you gave of your son shows what is most important. Normal behavior and a good appetite. What we have seen with Manal's son is that his Hb has not risen, but is fairly stable. However, his HbF percentage rose over time and his growth and activity level are in normal ranges. I feel that this is more important than an elevation in total Hb. If the child's Hb stays fairly level and his health and energy are good, and he shows some ability to produce HbF, then I feel hydroxyurea should be continued.

I totally agree Zahra.

From my expierence, hydrea started giving some rise to my son's hb after the fifth month so it is too early to decide especially that he was sick as you mentioned.

One more thing is the tuning of the dose as it may affect the result.

When you get the result of the HPLC, you should do another one after 3 month to compare the percentage of fetal Hb

Good luck
manal

Hi Andy and Manal,
I will get the electrophoresis done as soon as the weekend is over. I had some success getting him to drink extra water by pretended it was petrol for his tricycle so he kept coming back for refills. I hope it continues to work. I have tothink of how to convince the doctor to let us continue with hydroxy urea. Perhaps if the urea decreased by hydrating I wont need to. Wish me luck.
Zahra

Hi Andy
My son has been on hydroxy urea for almost four months now and the doctors are saying it is time to decide if it is working or not. They think it isnt. He has had quite low Hb durng this time despite being transfused more frequently. Even after 2 or 3 weeks his Hb would be around 8. Last time they increased the amount of blood transfused from 250 to 280 ml (weight 13.9 kg). This seems to have helped as his Hb after 3 weeks was 10.2. The plan that they have outlined now is to let his Hb drop to even 7 before transfusing. They will have him come in weekly to keep tabs on where his Hb is during this time. The doctor said that if his Hb does drop below 8 it means hydroxy urea isnt really helping him and should be discontinued. He said hydroxy urea has potential side effects even if they arent visible at the moment and the child should not be exposed to those risks if it is not helping him to increase transfusion intervals. I dont know how to convince him to try for longer as I have read on the forum that it can take upto a year for hydroxy urea to take effect.
I was really hoping hydroxy urea would help and am feeling depressed that it isnt. What do I look forward to now?
On the flip side he gained in both height and weight (0.4 kg in 3 weeks) once they got the Hb up. He hadn't really been gaining weight for a long time now. Is it ok to let him be on such a low Hb that he cant grow properly?
I am so confused about what to do .
I had the Hb electrophoresis done a little late . His fetal Hb in it was 2.1 % (not very hopeful is it?) and Hb 10.7 on 29 November. Would it be useful to do a repeat test with less than 2 months gap?
How can i convince the doctor to allow us to try hydroxy urea for a longer time? Also should I try to convince him of this??
Looking to you for help again.
Zahra

I think another electrophoresis should be done before stopping hydroxyurea, just to double check that there is no real movement in the HbF. The percentage is so low that I am not optimistic, but you don't want it lingering in your mind about whether or not there was any sign of improvement. If the HbF remains low, I would have to agree that the hydroxyurea is having little if any effect.

thank you Andy Your advice is invaluable as usual. I will try to get them to give me some time at tomorrows appointment while I get the Hb electrophoresis done elsewhere.
Zahra

Hi Andy,
I have got the Hb electrophoresis report. On 29/11/2010 Hb was 10.7 HbF was 2.1%. This comes to 0.2247g/100ml of HbF(2.1% of 10.7).
On 31/01/2011 Hb was 12.7 and HbF was 3.7%. This comes to 0.4669 g/100ml of Hb F (3.7% of 12.7).
This is  almost double but its still a very small amount. Do you think it warrants a request for trying hydroxyurea for longer?
This would mean restarting it as it had been discontinued on 26th Jan. which is about 3 weeks now.

The doctor at that appointment told us we should get serious about a bone marrow transplant when he turns 3. My other two children, my husband and I all are only 50% matches but he said sometimes you can get matches from extended family. Is there anyway to decide who to test? He has 14 first cousins  and 7 aunts and uncles. Someone suggested to first try those with the same blood group but I am doubtful b/c they tested me and my bloodgroup is different. The doctor also mentioned a bone marrow registry in the US. Is it likely to find matches in strangers?

Tomorrow is his next appointment. I have so many questions and am so confused.

Zahra