Thalassemia Patients and Friends
Discussion Forums => Working Towards a Cure => Topic started by: surendra on May 10, 2012, 04:56:38 AM
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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..
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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.
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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??
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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
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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
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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
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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.
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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.
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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.
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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.
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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.
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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?
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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.
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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????
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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.
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At this time she is fine, active and eating properly so we have postponed her transfusion next week. But I am little afraid that if Hb level will drop to low level. Anyways hope for the best. On what low Hb level we should worry?????
Thanks for reply
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Testing below 7 on two consecutive tests is the threshold for transfusion.
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sorry Andy I have seen your post right now. So should we wait that her Hb will come to level 7. Actually We have checked her Hb yesterday and it was 8.1. we have decided to go for transfusion today.
I want to tell u that she is as active as a week before but only problem is that her diet became low not very low. what should we do???
seeking for advice.
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Transfusion should improve the appetite. Observation of the child is the most important factor when deciding if transfusions should begin. If growth is slow, energy is low or appetite drops with Hb (which will result in slower growth), then transfusion is recommended. I would suggest getting her transfused and seeing how long her Hb maintains post-transfusion.
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Thanks Andy.
Actually due to some reasons(donor was not available) her transfusion has been postponed. She is playing good, fine and energetic but only problem is her apetite which became little low. one thing I want to tell that small tiny swelling on her body still appearing and also disappears without doing anything. Should we concern about it ?????
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Andy,
My son is having E-Beta Thal, got detected in 6months of age. He is now 2.5 years, under regular transfusion of around every 4 weeks. We have checked with our Dr in Bangalore about starting HU on him. Dr is not very eager to start it as his experience of HU on E-Beta thal is not great. I want to check your thought about this drug on E-Beta.
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Hi Andy
my daughter is taking hydroxyurea (500mg alternate day) since last 3 months. Last week on 30th august her Hb was 9.2 and today on 7th september it was 7.1. How can it be possible??? And this is not the one time, The same thing happened in the last two months also.
In the last month her Hb was 10.1 at the time and after two weeks it was 6.9. We couldn't understand. In these days she has no fever and no any kind of infection. She is active and eating properly. The main problem is fast decrease of Hb level without any reason.
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Surendra,
Has she had any transfusions during this period?
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Yes Andy
when her Hb level was below 7 she had transfusions. She is getting transfusion every month after using hydroxyurea.
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Hb decreases fast in last few days(2 points in a week). Is this normal when using hydroxyurea????
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I would think the drop in Hb is related to antibody reactions and has nothing to do with hydroxyurea.
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Thanks Andy
why this antibody reaction happened every month(specially in last week) ?? and we are using leukocyte filter during transfusion every month. Could u please tell me the reason of this reaction???
What I observed that first three weeks after transfusion her hb drops only 1-2 points and after this it drops very quickly (almost 2 points in a week).
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If it only happens at the end of the transfusion cycle, it is simply the transfused blood breaking down. This is normal. The only thing that may make it last a bit longer is using fresh blood, which in most cases means arranging a group of regular donors, so that the blood is always newly drawn from the donor. Electrophoresis should be done to see if the HbF level is increasing, as it is the method to determine if the hydroxyurea is having any impact.
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Yes Andy We have arranged some donors who donate blood a day before transfusion which means it is always fresh blood to give her. It is continue since a year. So that antibody reactions should minimize.
Definitely we will do Hb electrophoresis test in next month before transfusion.
I want to share one thing that her Hb goes down but her activities become normal. We can't recognize that her Hb is so low.
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Hi Surendra,
Hope you are still continuing with HU for your little one. Just wanted to know your experience with HU and if there is any positive indications (raise in Hb-F in total Hb) and increase in transfusion intervals.
Wanted to check this as my little one is just over 2.5 yrs and was looking into the possibility of starting HU for her. Also, wanted to know other's experience as well with HU.
Kindly advice, thanks!!