hydroxyurea , why not recommended ?

hi andy,

my name is lucky, from jakarat,indonesia. One of my child, youngest is thal intermedia, with double heterozigot mutation HbE and Condon 41-42. First I know for Hydroxyurea from Cure2Children Foundation, dr. Lawrence, by email, ask me to try this medicine, before go for BMT. Because this medicine need supervisor, so i consult to my doctor in jakarta, indonesia , Prof.Dr. Bondan. This doctor straightly refuse to use this drug. After that, i send email to Dr. Quah, at NUH Hospital in singapore. From email, dr.Quah agree if i tried this med first. So to make sure, i went to sing, to have a consultation. But again, after we meet, he is not agreed 100%. He said other country used this drug just for economy reason, cause blood transfusion is expensive.

My questions are :
1. Still bothering me, and still make me confuse, do i need to try this med ? cause i see from all article in internet, there is so many person, with thal intermedia, cure with this med.
2. Have any doctor in jakarta,indonesia ,can supervise my child while take this med ?

thanks before ,andy, all your done in this forum, is amazing. thanks to God, can know you, and you share information to us , without return.

thanks
lucky

Hi Lucky,

Is your child transfusing? What is the normal Hb of the child?

The doctors are wrong and wrong about hydroxyurea, but I need the above info to properly advise you.

Hi andy,

thanks for reply, now my child almost every 2 month need transfusion, start from february last year. Normal hb around 6.5 g/dl - 7.5 g/dl . She got her first transfusion at 2 year 7 month, now already 6 year old, and already 10 times got transfusion till now.

fyi, now i still find the doctor in jakarta, who can supervise me for take this drug,but not yet found. If cannot find the right doctor, may be I'll encourage my self to supervise my child. I can get the drug in here. And I read so many article that you post about hydroxyurea, but same term i don't understand. Is there simple way for me to do it , if finally i must supervise my child.
 
thanks
lucky

The phenotype is intermedia and your child is a good candidate for hydroxyurea. This drug is much more effective in patients who can maintain and Hb of at least 6-7 and the time between transfusions suggests that there is enough hemoglobin production already, that a boost from a hydroxyurea and wheatgrass combination might be enough to greatly reduce the need for transfusions.

I don't like to suggest patients take drugs without a doctor's knowledge, especially since the results need to be monitored, and any possible side effects, such as low white cells also need monitoring. I suggest that your doctor become familiar with Dr Vijay Ramanan of Pune, India and the work that he is currently doing using hydroxyurea in thal patients. Some patients have stopped transfusing completely under his care.

Continue to urge your doctor to allow the use of this drug, ideally at least for one year.

Hi andy,

thanks for your advise, it's very encourage me.... i will find the doctor who supervise my child. I have any update using this med, i will let you know. Don't get bored ,andy  :-) I'm very appreciate what you have done for thal person. Deep inside my heart , i so grateful to know you, contribution to thal person, without return. GBU

thanks
lucky

I may be very busy a lot of the time, but I am never bored. Persevere with your doctor. Your child deserves a chance to try this drug. If you are allowed to use, talk to me before starting. Wheatgrass should be taken with it. In fact, even if you can't get approval, wheatgrass tablets would be advised, anyway.

Hi andy,

sorry again,asking help. I just back from doctor, try to find the doctor who can supervise hydroxyurea.  I lost my paper which contain information what should we care in blood test during the treatment. I get the this website from this forum you post, but i want to print again, i cannot find it. Can u give that link again ?

tahnks
lucky

Hi Andy,

after see so many doctor in jakarta, indonesia. yesterday, i just make appointment with other hematologist doctor, more logical explanation. She tell me why hydroxyurea is not use in asia, cause pattern of dna , asian thal dna, is not same with india and europe. so useless to try. She said, my child is double heterozigot mutation HbE and Condon 41-42. Condon 41-42, it's mean cannot produce HbA. What is meaning of mutation of condon 41-42 ??

Is this true ? or any body , have mutation condon 41-42 try hydroxyurea and success ?

thanks
lucky

Codon 41-42 (-TTCT); TTCTTT) is the specific beta mutation.

Many HbE beta thal patients respond well to hydroxyurea. Every single study on hydroxyurea comes away with the same conclusion. The specific thal mutation present is not a good indicator of whether or not hydroxyurea will work on a patient and that in the end, the only way to tell is to try this very safe drug.

Your doctor is being arrogant and stubborn and is basing opposition to use of this drug on nothing more than arrogance. I do not know how you can change the mind of a doctor who prefers arrogant ignorance. There is not a single doctor on earth who can tell you that this drug won't work without having tried it.

hi andy,

thanks andy, i will find the doctor , who can minotor my child. fyi, next step , we will go to thailand , to make appointment with Dr. Suradaj, at Mahidol Hospital. I Will ask the doctor in thailand to try hydroxyurea

thanks
lucky

You can come to India for this purpose....If its possible for u

Codon 41-42 (-TTCT); TTCTTT) is the specific beta mutation.

Andy,
this codon 41-42 mutation is mild or severe mutation ?
Isha's mother has codon 41-42 & father's mutation was not detected . Transfusion was started at the age of 2 (thalassemia was diagnosed at 1 yr & 6 months of age) , and she is 26 now(regular 12-15 days transfusion now). I wonder whether isha is thal intermedia/major ?
Her Hb stayed lowest at 6.5-7 before 1st transfusion.

Codon 41-42 is a beat zero mutation. When combined with another codon 41-42, it is transfusion dependent thal major. When combined with other mutations, the outcome is not predictable, and can be anywhere from intermedia to transfusing major status.