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hydroxy urea in transfused patients

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zahra:
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

Andy Battaglia:
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.

zahra:
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

Andy Battaglia:
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.

Manal:

--- Quote ---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.
--- End quote ---

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

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