Thalassemia Patients and Friends
Discussion Forums => The Spotlight => Topic started by: teddyjas on October 14, 2008, 02:33:20 AM
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Hi,
I am 30 yrs old thal major residing in Singapore.
I stopped blood transfussion since 27 yrs old as my doc here asked me to do so :) and I just started on ferriprox this week.
Any one else from Singapore here?
cheers...
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Hi Teddy!!
I am a thal major too but not from Singapore. I'm from India. Your post has stunned me!! You being a thal major have not taken blood transfusions from past 3 years, how is that possible? What kind of treatment you are on? Have you got your splenectomy done? If yes, then when?
I would really love to know how did you get off transfusions.
\peace/
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Hi Teddy
I am from S'pore. Were you at the conference.
My son has thal intermedia.
Tell us more abt yourself.
Take care
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Hi! Welcome to the group........Teddy....... I'm also a Beta thal patient and just like you I stopped having blood transfusion 5 years ago. Although I had a transfusion a year ago because I got sick and my hb got really low. Anyhow, please tell us a little more about you........ :welcomewagon :welcome
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Lyanne,
I never knew you weren't transfusing :noway really how do you maintain your hb without transfusing if you are a beta thal major? You haven't been transfusing since 5 years? it's hard to believe,,then you must be iron free?
Here i thought i know every one on the site :huh .
Zaini.
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Teddyjas,
My son is a thal major, and we live in Canada. Welcome to our site.
I am also interested in hearing how you guys are transfusion free - that is awesome!!
Sharmin
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WOW .
unbelievable but true ,,,,
are you people on any medicine to maitain HB .??
please share something about your medicines and treatment with us ....
its realy nice to hear something like this .... please have some words about
how it happen .. and complete treatment and medicine details .. if you can ..
Take Care
Umair
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Hi! Zaini . What can I say!It's amazing but true :grin I just maintain vitimins E, B complex, Folic acid and as for me being iron free nah I do have at least twice a month of chelation :nurse to keep my iron low......I did have my splenectomy when I was 15 and my transfusion needs became less often and gradually noticed that my hb count got stable to a certain number. :yahoo of course, I'm sure our fervent prayers :pray were finally answered. :pray.
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That is so great Lyanne! I am so happy for you! :yaaaaaay
Sharmin
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Lyanne and her sis have always fascinated me because they both stopped taking regular transfusions years ago. This brings up the gray area between intermedia and major. Intermedia is such a vague classification so maybe it applies to the sisters in some way. I think many intermedias have been transfused when they were young in order to counter slow development and failure to thrive. Their Hb levels may be closer to an intermedia range than major but if normal development is not taking place, it makes sense to transfuse. However, once grown and development has finished, patients may be able to become transfusion free, as their Hb maintains high enough to manage without taking blood. Lyanne is also taking some very important supplements and I am sure they help her produce blood and keep her energy level up. This should be an important part of all thals' health programs.
I think this is a lesson for parents of children who are on the borderline. If your child is not thriving, it makes sense to transfuse. It may not result in lifetime dependency but only during the growing years and normal development is of extreme importance because it cannot be made up later. Once the child becomes an adult, a review should be done to see if reducing or totally eliminating transfusions is a possibility. One young patient that comes to mind is Kathleen's child, Olivia. She may need to transfuse regularly or sporadically as she grows but as an adult it may be worthwhile to explore the option of reducing or eliminating transfusions altogether.
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wow this is awesome, I had to pinch myself that i was reading correctly.
Lyanne - this is really good news. What is the hb you are maintaining. Have u ever got the DNA done? if we get the mutaitons maybe we wil have a better idea. I am ecstatic this is like major becoming an intermedia. I have heard intermedia becoming major but this is the other way round.
Andy - this is why I guess Doctors keep on insisting tht intermedia's are so complex and is based on clinical study of individual patients and no two patients will be the same. Also yes it is important to transfuse theintermedia's as and when their body needs but only one concern that what ever bone marrow function happen;s will that shut down or not. May be sporadic transfusions can be the key.
Also in lyannes case it must be the spleenectomy which would have helped along with continuous vitamins.
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Puja,
You said it perfectly.
...intermedia's are so complex and is based on clinical study of individual patients and no two patients will be the same.
Intermedia ia a vague classification, but actually we have seen that within all classifications there are great differences. This is why it is so important to design treatment specifically for each patient, as is done in the comprehensive care centers.
And yes, the splenectomy most likely has some effect. It doesn't help all intermedia patients long term but many are able to remain transfusion independent for long periods.
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Hi! Sharmin and poo gill.Thanks! Even the doctors here are amaze on how we stopped having transfusion since I have been worked up and diagnosed as beta thalassemia but I guess As Andy said there are some vague classifications of intermedia that applies to me and my sister..... or maybe because we had an early treatment of desferal ? is that applies Andy? Anyhow, my hb is always on the 9 levels but whenever I get sick it's the first thing that go down.......as for DNA I guess not, but when I was 2 years old I had a lot of laboratory test including the test confirms that I am a thalassemic and a Major at that......... and since then it's like a routine after a month my hb would go low 6-8 then I have to be transfused. :wah hmm...... thinking of it I miss that routine...... LOL! but I would not want to do it again, it's really tiring and stressful :noway
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May be we'll be able to do the same with my daughter when she grows up,but what worries me is the duration between her transfusion which has been decreasing since she started transfusing,we started with 6 weeks and now she is on 3 weeks.Last time doc gave her appointmenet for 4 weeks but i feel like her hb is low as she is looking a bit pale.
Zaini.
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Zaini,
Your daughter is an Intermedia, why don't you try Hydroxyurea?
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Sahil,
Once a doctor also advised us,but frankly i am scared to try anything new,plus we haven't seen any big difference in users of hydro oxy urea,and i want to maintain my daughter's hb on 10,while with hydrea people got as much high hb as 8,as far as i remember.
Zaini.
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Zaini,
I'll tell you something.
Where I take my blood transfusions, there are 5 thal Intermedia patients, and all of them are off blood transfusions since 2 years. All are 20+ years old and their hemoglobin is maintained between 10-11.
Two of them are females out of which one got married this year or last year not sure.
You can surely give it a try.
PS- Before getting on hydroxyurea, all of them transfused every 15-20 days. Two of them even got their splenectomy done.
\peace/
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what is hydroxyurea???
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Hydro Oxy Urea is hb F inducer,it increases hb sometimes and keeps it stable so one can be transfusion free,but mostly it is used by thal intermedias,i don't know if any thal major has tried it.
Zaini.
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Hi Zaini,
I thought of Zainab immediately when I read this post, because she did not present as a major right away and did not need regular tx until she was 3 (am I right?). But she is looking and developing so well right now that I think it is best for her to continue to tx until she is full grown. At that time, she will probably be one of the ones who doesn't need to transfuse anymore - I'll pray for that :pray
What do you guys think?
Sharmin
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my brother didn't needed blood transfusion untill he was 2 years old, then something happened and parents were advice to transfused him
a doctor few years ago said that we brother and sister had intermedia and someone misdiagnosed us...
how can one know what he/she was? major or intermedia after having transfusion for 20 years?
we had an elder brother too, elder than me and Salman and he was Thalassemia Major, so the doctor thought each of us will be Thal. Major? if we have been misdiagnosed then whom should we blame?
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Don't blame anyone. Having intermedia type hemoglobin levels does not mean a child can thrive without transfusions. It has to be judged by the condition of each child. If normal development is significantly stunted, then transfusions are recommended. Once fully grown, it may be possible to re-examine this and see if a patient can get by with none or minimal transfusions.
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Andy
Can the bone marrow start producing the small quantity of good blood (in case of intermedias ) after 20 years of transfusion?? I thought that if the bone marrow shuts down the body becomes dependent on transfusion for ever. Please advice
manal
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The bone marrow activity actually never even drops to normal unless the hemoglobin level is kept at around 11 or higher and never allowed to fall below. At the normal level of 9-10, bone marrow is 2-3 times more active than normal. Lowering the Hb more will increase the bone marrow activity. It may take a few months but the bone marrow will resume its previous level of activity, but it will resume.
One reason it has been hard to find majors to test hydroxyurea on, is because transfusions have to be ceased for several months in order to stimulate enough bone marrow activity so that hydroxyurea can work. Majors generally have little interest in intentionally dropping their Hb low enough to take part in the trials. This is one reason the main study using hydroxyurea in majors was conducted in Algeria. It is much easier to find under-transfused majors in countries without good blood facilities. The conclusion of the trial was that hydroxyurea might be a good, cheap treatment option for majors who have trouble finding adequate blood supplies, especially in poorer countries.
There is also an irony with hydroxyurea. It is used in higher doses as a chemotherapy because it has an ability to suppress bone marrow activity. Obviously, this is counter to what patients need if at the same time it is supposed to help produce HbF, but the balance is in favor of HbF, so it is used as a therapy for thals. But its affect is also somewhat limited and this is why there is more promise with coming drugs that are based on fatty acids.
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Zaini,
I'll tell you something.
Where I take my blood transfusions, there are 5 thal Intermedia patients, and all of them are off blood transfusions since 2 years. All are 20+ years old and their hemoglobin is maintained between 10-11.
Two of them are females out of which one got married this year or last year not sure.
You can surely give it a try.
PS- Before getting on hydroxyurea, all of them transfused every 15-20 days. Two of them even got their splenectomy done.
\peace/
Sahil,
They must be the lucky ones,maintaining hb of 10-11 with hydrea,really we haven't seen it here,i met a girl here in my city who was on hydrea,and wasn't transfusing,but unfortunately her facial bones were so deformed that just seeing her face brought tears to my eyes,and her mom told me that she was 13 yeras old,well she didn't look much old then my own daughter,so these are the things i am worried about,i don't wanna disturb my daughter's growth cycle and i want her to have normal facial features,so it might not add to her problems,if you have seen her pics in the gallery ,you'll see she doesn't much look like a thal major,may be because she was diagnosed at the right time and her hemo started transfusions immediately.I am happy with the way things are going Alhumdulillah,only i'd love if her transfusion intervals can be increased a little bit somehow,but i don't want her bone marrow to over work and expand or weaken the bones.
Sharmin,
Yes you are absolutely right she was diagnosed at age of three and didn't need any transfusions till then,and she was growing very normally,there was no stunted growth,she just looked pale and we did her hb which came back 6,then doctor tried a little bit iron,it did increase her hb from 6 to 7.7,then he asked us to get electrophoresis,i've repeated the story so many times,but it still horrifies me,what we went through at that time,my husband didn't even know what thal major was,and what i remember was that one of my cousins died at age of 20 due to thal major in 1986.
May be when se grows up,she'll decide if she want to drop transfusions and try any hb inducer,right now i just don't wannt put her in any kind of stress,as i've seen her miserable with low hb.Or may be we'll see a cure even before she grows up,INSHALLAH.
Zaini.