Use of Hydroxyurea in Thal Major

I've often heard that many thalassemia intermedias use hydroxyurea to boost hemoglobin levels, but does anyone know of it being used in majors? I just ran across this article from 2003 and majors were treated with hydroxyurea and some were able to completely discontinue transfusions after being treated.

Has anyone heard more about this being tried in majors? Is there any reason it isn't being done?


http://www.scienceblog.com/community/older/2003/C/20031073.html

In the study, seven transfusion-dependent beta-thalassemic children in Algeria were treated daily with hydroxyurea at the maximum levels...The total fetal hemoglobin level increased in all patients, as early as the first month of treatment. Mean hemoglobin levels increased from 6.5 to 10.5 g/dl in two children with thalassemia intermedia, and from 4.5 ±0.9 to 7.0 ±0.8 g/dl in the five children with thalassemia major. The increase enabled five patients to stop undergoing transfusions.

I'd love to try this one out. Where can I get this stuff and in form is it available?

Andy,

If I remember correctly some of the stuff that I have read on this topic, hydroxy urea only works on a small proportion of people by stimulating an increase in Hb F. But, it has to be administered as a drip intravenously, so you are just substituting one drip for another, and it has its own side effects.

Let me see if I can dig up more data on this.

Cheers

Poirot

Andy

My wife was on Hydroxy Urea in India and in the United States. She had good results with it. Because it is a also a chemotherapy drug, the side effects were her nails started to turn bluish. But it helped with the hemoglobin and also helped to reduce her  platelets. My wife is Thal Intermedia.

Bharat

Poirot,
Based on the limited knowledge that I have, Hydroxyurea treatment is administered by taking few tablets orally. One possible side effect of the treatment is reduction in total WBC count. 
It's kind of slow chemotherapy, but the amount of dose is very small so it doesn't hurt that much(in terms of side effects) if it suits you. Also once started, patient has to be on it lifelong.

It does not work for all patients. Not sure why exactly.

--Hallu

well, I guess then this stuff is not for me as I have a history of losing Platelets and this stuff could stimulate the process 

Thanks, Hallu.

My knowledge is a bit dated on this one. Have searched for more data on it, but there does not seem to have been any scientific trails conducted for this drug on thal major. Only a few anecdotal cases, like Bharat's earlier in this topic.

Poirot

Hello,

I had a bit of a search too.... it seems there is a lot more info on thal intermedia and hydroxyurea.  I did find one study however, that looked at the use of hydroxyurea on beta-thalassaemia majors... it can be accessed for free at:  http://www.haematologica.org/journal/2004/891172.pdf

So go and have a read!  I haven't read it in detail, so have no idea whether it is a good study or not but the results look promising... would need to have further studies done to confirm the results.

Cheerio!
SalD.

Hello,

I had a bit of a search too.... it seems there is a lot more info on thal intermedia and hydroxyurea.  I did find one study however, that looked at the use of hydroxyurea on beta-thalassaemia majors... it can be accessed for free at:  http://www.haematologica.org/journal/2004/891172.pdf

So go and have a read!  I haven't read it in detail, so have no idea whether it is a good study or not but the results look promising... would need to have further studies done to confirm the results.

Cheerio!
SalD.

I visited the site . If the study is correct then it is a positive thing and can be helpful to the patients of Thal Maj.

Hello. I was prescibed hydroxyurea to lower my platlette count. For fear of blood clots. This was given to me 2 weeks after my spleen was removed, due to my platlette count skyrocketing to 1866. Hmg count at that blood test was 9.7. I get more blood done next week and will posts those results.