e/beta thal -new member

hellow everybody! i m from west bengal,india,36 yrs old. my only child Aayan (13m) recently diagnosed with e/beta thalassemia.we just compleated 2nd transfusion 3 days ago.after 30 days from 1st one.before first transfusion his hb was 6.8.  his hplc: 
A=13.2
A2+E=42.4  and F=44.4   and Ferritin=71   before 2nd trafsn hb was 8.1 and post transfusin 12.5.   he is taking a multivitamin without iron and folic acid .Dr advice me to see hb level after 3 weeks.

anyone can help me here and suggest for future course of action plse.

Hi Sahidul,

You are at the right place. you will get ample of support and correct advises.

My daughter was diagonised with thal major at the age of just 3 months and her 1st transfusion was done when she just completed 4 months. And its a long journey. But first we have to aknowledge it. ok, fine if God has given this, he will also see us thru. first forget about - why us, why my baby?  now just concentrate on the best possible care you can give to him.

= pls ensure pre-transfusion HB remains around 9. (It generally fluctuates. My daughter's HB sometimes comes to 8.5 or 9 or even 8 at the exact 4 weeks interval. thats fine. If his last pre-trfn HB comes to 8 or less than 8 at 4 weeks interval, then try at 3 weeks interval the next time. FOr some transfusions you need to vary the frequency and then you will get idea at what interval her pre- trfn HB remains around 9.

= pls dont unnecessarily poke her second time to check post-transfusion HB. thats not needed. why to make him suffer when its not at all needed.

= as of now, only folic acid is enough for him.

= need to start chelation when ferritin reaches 1000 (will take around a year or so)

= B4 transfusion give him more liquid and wash his hands with warm (bearable hot) water. That helps to get vein easily.

For thal major - only 2 things are of utmost important.

1) keeping the HB around 9
2) iron chelation tablets to control iron overload.

rest, he will be normal like any other child. Take care and do ask any more information u need. We have gone thru all.
Take care and lots of love to aayan.

Angel's mom

thank you angel's mom,

is desferal or tablets is better for iron cheleton.

if it is needed after 3 to 4 transfusion?

Hi,

Welcome to Thalpal, nice to meet you. Relax and don't worry, everything will seem normal once the routine starts.

Well for your recent doubt, most people are advised to start transfusion once Ferritin reaches 800-1000. Desferal is good, but I would like to stress the point that if you plan to use Desferal, use Kelfar (Defriprone) with it as well as a combination (very effective and used by many thals). As you are in India, there also is a Thalassemia magazine which you can subscribe to which will send you issues about their activity and what's new on timely manner.

Combination of Desferal & Kelfar, Desferal removes iron in Liver while Kelfar is effective in removing iron from heart, so both works in a complementing manner to each other. I personally take Asunra (Exjade) and many people from here take just it and their FE levels are maintained just fine.

I'm from Gujarat by the way. And don't worry, all will be good once a routine is developed, you'll recognize your child is no different than a normal one.

-P.

thank u pratik

for ur advice.
u suggest kelfer but why u take asunra?
plse clarify this

Hi,

There are some points which I want to make clear.

1. I never took desferal, but only Defriprone (Kelfar) since my childhood till May 2008, when there was no availability of Asunra (Exjade). But Asunra was introduced in India in May 2008, hence I made a switch to it.

2. If I was to be using Kelfer + Desferal combination, I wouldn't have switched.

And don't worry, Exjade alone also gives good results. I just made a point that if you or your doctor plans to use Kelfer or desferal, don't take any of them alone, but do the combination therapy as it's more useful and effective.

-P.

thanks a lot!

you clarified correctly.i have no idea about that.it will help me
when i will meet  dr. and plannig for cheleton.

wish your good health in coming festive season.

one more qstn:
if frequency of transfusion are same(3-4 weels)
in case of e/beta tha major  amd beta thal major.

Dear Sahidul,

My apologies. I thought your son is thal major. thats y i said thal major needs transfusion every 3 to 4 weeks.

I am not aware of e-beta thal.

Other members : pls guide sahidul.

For clarification purposes.

In HbE beta thal, one gene is a beta mutation and the other is called the HbE mutation. The outcome or phenotype, of this combination can range from non-transfusing intermedia to a phenotype of thal major, which requires regular transfusions. In this case, we need to look at this as similar to thal major. Our long time member, Poirot is also in this category.

I would also favor Asunra over Kelfer for very young children. When starting, please follow this advice. Take one third to one half dose to begin, so the body can get acclimated to the new drug. Doing this for the first two weeks before raising to the full dose will greatly reduce associated side effects. Splitting the full dose into two and taking twice daily will also help to reduce side effects and actually will chelate better.

Sahidul,
Just saw in your post that your child is 13 month old. Do not worry about ferritin and Iron at this time. As per Hemo in Boston, Chelation should not start till the child is atleast 2 years old and ferritin above 1000.

Thanks to
Andy,Angel"s Mom,Bostonian-4

I feel lucky to join here.
Plse advice me for planning chelaton of my son.

I am also looking for e/beta thal patient/parent here for advice/discussispecial thanks to andy for his contribution here.

Generally chelation does not begin until a minimum of 10 transfusions, up to the 20th, and not before age two. These days Asunra or Exjade is the chelation drug of choice for young children, as it is the easiest drug to begin with. When the time comes we can give you more specific instructions.