I am confused :( Help me

Hi Guys,,

how are u all?i am feeling really confused right now, , the thing is that,, my daughter six years old,, was diagnosed with thal major when she was three years old,,she looked a bit pale at that time,, and had some cold ,i went to our family doc and he suggested tseting her hb,, we did and it was 6 g/dl,,he refferred us to a peadiatrician and he prescribed her an iron syrup  yes an iron syrup,, she started it and her hb went to 7.6 g/dl,, but then he suggested hb electrophoresis,,we did it and it said she was a thal major,,the report showed Heamoglobin A = 10.2%,,,,,Heamoglobin A2 = 4.9%,,, and Heamoglobin F = 84.9% and concluded,,"HIGH F,,CONSISTENT WITH THAL MAJOR"  They started transfusing her ,,in the beginning,,the duration was 6 weeks and then 5 weeks and now it's four weeks,,and it's been three years she was first transfused,,last year i went to another heamo and he said that my daughter must be Thal intermedia,,, and he suggested hydrea,, but i didn't believe him, , i thought how come no doctor ever told me that,, and the report clearly says it's thal major,, he even suggeted hydrea but i refused,,,,now after joining this site,,, i am really confused  ,,is my daughter a major or an intermedia??? how can i determin that??and now after three years of tranfusing,, will hydrea work ?(if she is an intermedia),,was her diagnosis wrong ?? ( I'LL GO KILL HER DOCTOR  )Is there any other test to distinguish btw major and intermedia?what should i do now?is hydrea even successful?there are so many questions buzzing in my head ,, i feel so bad,, wt if she was an intermedia and we transfused her regularly with out any need,,,,plzzzzzzzzzz help me out,,, i am going nuts.

Take Care.

LOVE TO U ALL.

ZAINI.

Hi Zaini,

Good to know that you will be getting hydroxyurea, I think you should first try other natural HBF (Fetal Hb) inducers such as Wheat grass and/or Resveratrol. If it shows positive signs such as an increase between transfusion periods then it will be evident that your baby can produce enough HbF even now and HbA genes haven't taken over yet. Then ask your doctor about Hydroxyurea because treatment of intermedia with Hydroxyurea is still an uncharted territory here in Pakistan.

Take care, Allah Hafiz.

Hello Zaini

I totally agree with you, this is very frustrating.

To my knowledge and i am not a doctor, the need for transfusion in majors starts from early months to one year. The ability of your child to go on until the age of 3 without transfusion _to my knowlege - should be classified asa n intermedia. Actually the difference between major and intermedia lie in the ability to maintain a level of Hb between 6 to 7 and the overall clinical situation, though genetically they are the same.

At the same time, some children are classified as intermedias and the doctors prefer to go on regular transfusions. Their reason for this is that they believe that children shouldn't  have HB less than 9 in order to avoid growth problems. This point of view does exist among many doctors         ( though i don't recommend as long as the child is clinically fine )

So my advice to you is to find a REALLY good hematologist in thalassemia and have his opinion on whether your daughter can still produce and maintain her own Hb or those three years made her transfusion depended. I do ecourge you to start the hydroxyurea to practically see if it will makea difference or not.

Please Zaini keep us updated

Manal

Hi Zaini,

It does seem more likely that your daughter is intermedia and not major. An Hb of 6 without transfusions is more likely to be intermedia as a major's Hb would've been even lower and would never rise to 7 without transfusion. The time between transfusions also suggests intermedia, as does the increased frequency of transfusions as she has become transfusion dependent. I don't think the doctor necessarily made a wrong choice by transfusing. That depends on the development of the child and if there was any evidence of bone expansion or failure to thrive, then transfusion was the right thing to do. Hydroxyurea is sometimes an option in intermedia but not all doctors are aware of it.

Talk to the doctor about hydroxyurea. It is possible that it could be a solution instead of transfusions. It might be necessary to stop transfusing a couple months before hydroxyurea treatment begins so that the bone marrow starts to produce its own red blood cells again.

Hi Manal and Andy,

thanx for ur replies, i am happy that u can understand my feelings,,i have arranged an appointment with a heamotologist who is really doing research in the thal field,, not like other doctors who mostly follow books,,and i think though i am not sure that he is the one to introduce hydrea here in Pakistan, Dr Tahir Shamsee,, so let's see what he has to say on that matter,,,and Andy i knew that they ask to stop transfusion before starting hydrea,suggested hb level is 8,but won't it affect her bones?i hope u won't mind my silly questions,thanx again all of u,,and Manal give my love to ur son.

LOVE TO U ALL .

Zaini.