hydroxyurea

hello everyone
Dr has advised us to give hydroxyurea to our daughter. But we have not started yet. we are going for transfusion on this saturday.
So when should it be started and what precautions should be taken before starting hydroxyurea.
waiting for earlier reply..
 

Just follow the doctor's instructions. A key value that should be monitored is the percentage of the total hemoglobin that is fetal hemoglobin, HbF. If this percentage shows a steady increase, it is an indication that the drug is working, as it affects HbF levels. I don't believe that there is anything you need to do in advance.

thanks Andy
what are the side effects of this drug and when should it be given?? 
When should We check her HbF level after starting the  dose??

Your doctor should guide you on the testing, but it should be at least every three months during the first year of use.

There has not been many reported side effects with the doses of hydroxyurea used in thalassemia.
http://www.ncbi.nlm.nih.gov/pubmed/21602718

Efficacy of hydroxyurea in providing transfusion independence in β-thalassemia.
Ansari SH, Shamsi TS, Ashraf M, Perveen K, Farzana T, Bohray M, Erum S, Mehboob T.
Source
Department of Pediatric Hematology, National Institute of Blood Diseases, Karachi, Pakistan. ansarisaqib@hotmail.com

Abstract
BACKGROUND:
Packed red blood cell (PRC) transfusion with iron chelation is the mainstay of treatment for β-thalassemia major. This prospective interventional trial serves as a follow up to our similar earlier study that evaluated the efficacy and safety of hydroxyurea (HU) in minimizing PRC transfusions in patients with β-thalassemia major.
METHODS:
One hundred fifty-two patients with β-thalassemia major received HU at a mean dose of 16 mg/kg/d. The results were analyzed at the end of 24 months. Transfusion requirement during the 6 months preceding the study was considered as the control.
RESULTS:
One hundred forty-six of 152 patients were evaluated after 24 months of follow up; 6 patients were either lost to follow-up or withdrew consent. Grade 1 myelosuppression was observed in 4 patients and diarrhea in 2 patients. Sixty children (41%) did not require any transfusion after using HU; 57 patients (39%) showed partial response with greater than 50% reduction in PRC transfusion; and 29 patients (20%) were nonresponders with less than 50% reduction in PRC transfusion. The mean volume of PRC transfused was reduced for all patients.
CONCLUSIONS:
HU was found to be safe in patients with β-thalassemia major, and resulted in the reduction of transfusion requirement and in an increase in the interval between transfusions.
PMID: 21602718 [PubMed - indexed for MEDLINE

hello everyone
Dr has advised us to give hydroxyurea to our daughter. But we have not started yet. we are going for transfusion on this saturday.
So when should it be started and what precautions should be taken before starting hydroxyurea.
waiting for earlier reply..
 

Dear Surendra,

At what age you are going to start to give hydroxyurea to your daughter ?
What type of mutation found in your daughters DNA studies ?
Please reply to my queries.
With regards,
sys

Dear Andy
I am very curious on this Hydroxyurea . If the medicine is so effective, why it is not mandated by CAF or other renowned doctors. Many Doctors treatng Thalk major syas that Hydroxyurea is only good for Thal Intermedia. is that correct?
Rgds

The patients in the quoted study were all thal majors. Most studies have been done in nations like Algeria and Iran, as they look for lower cost methods of treating thalassemia. Large drug companies are not interested in low cost treatments, so that may be one reason that this drug is not promoted more.

To,
Sys
my daughter is 27 mnths of age & yet to start hydroxyurea .we've not carried out any DNA studies kindly tell what benefits/outcome we are go'g to get with this.

doctor has advised 500 mg hydroxyurea to my daughter whose weight is 12 kg with 27 months age
is it advisable since we've to start the dose.

The normal dose of hydroxyurea used for thalassemia is 15-20 mg/kg. Talk to your doctor about this dose. He may be recommending a dose that is prescribed for uses other than with thal.

definitely Andy, I'll ask my doctor for prescribed dose in our next visit. As he is famous haematologist in our city(pune). Thanks for your kind concern and care.

I want to ask a question that should we go through for her DNA test now before starting hydroxyurea or start it now with the dose of 15-20mg/kg/d.

 

Andy...why do you think the major Thal centers such as CHOP and Cornell are not as informed with this treatment? Do you think it is because its cheap and less expensive as chronic transfusions?

I think US doctors have been slow to use hydroxyurea because they are comfortable with the system of transfusion and chelation. In addition, most of the long term studies on the drug's use in thal has been outside the western world. And of course, you're not going to have any big drug companies pushing for it, so it may be also a case of out of sight, out of mind. The results are also very hard to predict, so that may be another factor why doctors are hesitant.

hi Andy
we have started hydroxyurea since last 15 days (500mg every 3rd day). Now we have checked Hb and it is 8.7. We have planned to go transfusion on saturday. Should we wait for hydroxyurea to show its effects or go for the transfusion?
 

It takes months to even begin to judge the value of hydroxyurea, and this judgment is based on the percentage of HbF present. It can take more than two years to measure the full effect, but therapy will only continue that long if a steady rise in HbF percentage is seen. At this point, there may be little choice but to continue the transfusion regimen if the child suffers from the effects of low Hb. If the child is fine at a lower Hb, extending the gap between transfusions can be tried. Research has shown that adding L-carnitine and magnesium with hydroxyurea can improve the effects of the drug.