Planning to try Hydroxyurea. Have few questions.

Hi Andy,

I just heard from one of our Indian Thalassemia groups on whatsapp (mobile app), that the person is now free from transfusion and has just completed an year without requiring transfusions. He is major and has his genetic testing done several times to confirm that he is thal major.

I was very surprised as well as impressed by this and I want to start hydroxyurea that's what.

But I Have few questions:

1. Is hydroxyurea safe or taken to be under anyone's administration? Any special precautions to take?
2. What should I dose it? My weight is 46-47 kg.
3. How long should I wait to see results or to conclude or to go to any any decision?

4. Just in case if it works, is there something like some point where I can stop and the Hb would be produced on it's own or I'd have to take it for life long?

Thank you,

-P.

I know the patient and I do not believe that he is technically a major, as he did not transfuse until he was 4 years old. I have asked him this question about his status twice and he has managed to not answer it. I am convinced that he is thal intermedia. I think hydroxyurea would have very little value for you and most majors.

I know the patient and I do not believe that he is technically a major, as he did not transfuse until he was 4 years old. I have asked him this question about his status twice and he has managed to not answer it. I am convinced that he is thal intermedia. I think hydroxyurea would have very little value for you and most majors.

I'm talking about Jatin, is he the one that you're talking about?

He told me that genetic test was also done multiple times to see and it came out as thal major only. Although 4 years old and not transfusing sound much like intermedia but from old links here on thalpal and findings such as:

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

Makes me think it is worth a try.

Could you answer the 4 questions that I have? Would help very much.

And I also read somewhere that long term usage might convert it to carcinogens and that it also may affect fertility.

Still worth a try? What do you think?

Best,

-P.

Pratik,

Yes, it is Jatin. Majors cannot go to 4 years old without transfusion. Regardless of Jatin's genotype, his phenotype is not major. Even under the best circumstances, hydroxyurea will not raise Hb more than 1-2 points. Do the math. An intermedia can maintain an Hb of 6-10. Majors are always below 6 and most likely below 3 without transfusions. I suspect that Jatin has a genetic modifier that has not been found that helps to keep his Hb level high enough where hydroxyurea can provide some benefit. I have heard from many majors who have tried hydroxyurea and have had no worthwhile results. Oakland Children's has determined that hydroxyurea does not provide enough benefit to recommend it to majors.

Hydroxyurea is fairly safe but it can have a negative effect on fertility while it's being used. This is reversible. Dosage for thal is 15-20 mg/kg. Higher doses have more side effects and do not produce a better result. Results should include electrophoresis because if hydroxyurea is working, the HbF level will rise as a total percentage of Hb. Even if the Hb does not rise, hydroxyurea is working if the HbF rises considerably. However, this would be insufficient for majors.

I have to agree with the studies that have recommended hydroxyurea use by majors in countries so poor that transfusions create a burden financially or there is a lack of blood. But, I find it hard to recommend its use for true majors, as it cannot help enough to free a major from transfusion. Keep in mind that thal intermedia is actually a vague classification and many intermedias have been classified as majors because they do require transfusions.

Do I think it's worth trying for you? No. From your history, I think you are a true major and would see little benefit if any.

And for those where hydroxyurea does work, it will only work as long as they continue the drug. I feel we have much more hope with the newer HbF inducing drugs that are in development.

Jatin has not transfused for one year. I think this is something to celebrate but the results should not be used to give false hope to majors. It can have real value to intermedia patients and those with HbE beta thal, but has proven to be of little value to most majors.

Thanks Andy.

But what I was thinking that even if it does not eliminate transfusion completely, even if it gives me 15-30 days extra boost, I can have one set of transfusion less and therefore fast ferritin reduction as well.

I got this thought from the posts of old members such as Priya whose tx period rose to 45 days from 30.

Keeping fertility and other factors in mind, do you think it's worth to try this at all or I should just pass over this?

What do you think?

Your opinion and advise truly values and matters to me!

Best,

-P.

Especially because of the fertility issues, I would recommend against it, at least until your physical development is judged to be normal.

This trial done on Hydroxyurea in B thals..

http://www.ncbi.nlm.nih.gov/pubmed/21602718

Hi Pratik,

I am on and off hydroxyurea since 2001. It did not increase my Hb level and as it has fertility issues, it was stopped.

Last year on I am again on it- this time not for HB but for my bad back. Will go for a review next month. Will get to know the exact position then.
But it has not improved my Hb or the transfusion gap. I am an intermedia.

Thanks for the feedback Rashmi.

I actually decided not to take it due to the same issues.

-P.

Dear Andy & Pratik,

My son in 3 years 2 months old. He has been diagnosed with Thal Major, but till now he has been behaving as a Thal intermediate. He has maintaining a healthy Hb level of 8+ without transfusion till May 2013. However, his Hb level has fallen to 7.0 in June and further to 6.9 in July end. My doctor has advised to use Hydroxyurea. I have a few questions regarding this :

1. He is 3 years old and has never been transfused, is it safe for him.
2. What are the side effects on his health and growth
3. He is 13 Kgs now, if I use what is the dosage and amount that should be given.

Please advice

Regards
Benjamin D
Kolkata

Hi Benjamin,

1) Yes, it is safe even for infants.
2) The side effects are usually mild and temporary. They may include nausea and a rash.
3) He should begin on low dose of 10 mg/kg to adjust the body to the drug. Once he is comfortable with the new drug, the dose can be raised to 15 mg/kg. 20 mg/kg should be the maximum dose.

I would suggest that wheatgrass tabs are taken with the drug.

Thanks Andy,

Is this a liquid?
Should I give it to him once a day?
Any specific name or brand of wheatgrass tablets I should use?
What is the wheatgrass dosage?

Thanks
Benjamin

Hydroxyurea is provided as capsules. It can be taken mixed with water or juice, but precautions have to be taken so there is no contact with skin. See the post at http://www.thalassemiapatientsandfriends.com/index.php/topic,4983.msg47523.html#msg47523
It is preferable that it be taken as a capsule. Your doctor should guide you in the drug's use.

Wheatgrass is available from several sources in India. I don't know anything about any specific brands. They're usually in 500 mg tabs. Take 1 to 2 daily.

you can use this site for wheatgrass.
http://www.goodlife.com/search.aspx?q=wheatgrass

HI,

My son is 1 year 7 months old and his HB remains between 7 to 8. He has been transfused twice and last transfusion 4 months back. Doctor is advising to start Hydroxyurea.

I am little concerned about side effects of taking hydroxyurea. Do you know who is taking it from long time?


Thanks.