Update and questions!

Hi all!

I apologize that I have not been on this board for a very long time!

We are a family in the US who adopted a little one with Beta Thal major at 9.5 months  old in Dec. 2006.
Then in April 2008, we were finally allowed to adopt a little 4 year old with Beta Thal major too.  Both girls are from China and they are just so precious to us!  We also have 2 boys that are not adopted.

Everything with the girls is going fairly well, however I do have some concerns about their ferritin levels.
They are both getting transfused every 3 weeks.  The last few times they have each been over 11 hgb, but their ferritin levels keep increasing steadily.  The older girl had a high ferritin when we brought her home.  I think it was over 4000.  Now, it is approaching 6000.  The little one's ferritin is about 1600.  They are both on ExJade, but it does not seem to be working very well, especially for the older girl. 

My questions are:

1.  Would it be better to wait longer for the transfusions to try to get the iron levels down?

2.  Should we try desferal for the older girl?

3.  How much does diet really factor in?  We have been told that the iron in the blood contributes so much that the diet is really not a bog deal.  However, these girls DO have a low iron diet.  They never eat red meat or green leafy veggies.  The highest iron content food they are allowed to eat is bread at 10% iron.  I always try to give them milk, yogurt and other things with calcium to combat the iron intake. 

4. What is going on with ferriprox?  Is it an option yet?  If EJade isn't working, could ferriprox work? 

I am so fortunate to be a mommy to these precious girls and I want to do the best for both of them! 

At ages 3 and 5, are there vitamins and/or supplements that would help them a lot with their iron levels? 

We had a hard time adopting our 2nd daughter because the Chinese adoption officials changed the regulations and we had not been home with our first daughter very long.  Andy and others were instrumental in advocating for us and it worked!  So, I just wanted to say a public "Thank you!". 

I will try to post a picture!

 

Hi EMommy,

It's so good to hear from you! Can you provide a bit more info. What is the weight of each girl and what is their Exjade dose? The diet would not be a factor in the iron load, as they are on a low iron diet. Often the problem with young ones on Exjade is that a cautious approach is taken to the dosage while their bodies acclimate to the drug, so it may just be a matter of dose adjustment and patience. Ferriprox will probably be approved in the US during the next year.

Hi Andy!
Our 3 year old is 25.5 pounds as of yesterday.  We struggle with keeping her weight up because she is a light eater.  She is on 375mg of exJade and they are going to increase her soon.

Our 5 year old is 41 pounds.  She is on 750mg just recently.

Is there any time of day that is better to take the ExJade or does anyone have any tips to make it absorb better?

The girls get their medicine in the mornings and mixed with juice.  Mornings are a rush and sometimes the 5 year old does not get breakfast because she drinks her juice too slow.  She eats lunch at school at 10:15 am though, so it's not like she is starving.  After drinking so much fluids neither of them are really hungry right away in the mornings. 

Thanks for any input and/or suggestions!

I was able to add a couple pics of these beautiful girls!

~EMommy

Hi EMommy,

The dosages are correct for their weights, so it may be just a matter of time before you see results. We often hear reports that indicate that Exjade is removing organ and tissue iron before removing it from the blood, so the ferritin level may not drop at first. I would suggest just giving it some time before making any decision on changing chelators. I have talked to many people who have actually seen their ferritin rise during the first year of use of Exjade and then see a dramatic reduction in ferritin levels during the second year of use. There are some patients who do not get a good chelation effect from Exjade, but for your girls, it is too early to make this assessment.

Some advice: Never use any metal container or utensil for Exjade. The metal will react with Exjade and partially neutralize it. Also, avoid any antacids that contain aluminum. Make sure that you mix a bit of liquid with any residue left in the container and have them drink that so they get the full dose. You can experiment with another time of day if mornings prove difficult. The main thing is that it is taken on an empty stomach at least 30 minutes before eating. Some people have switched to taking it later in the day and this has helped with the nausea that some experience. It will not reduce the effect as long as it is taken on an empty stomach.

Patience is often needed when starting with Exjade and I feel many parents have quit Exjade before giving it a long enough period to show results.

Thanks so much!

I will stick with it and hopefully the ferritin will decrease.  It makes sense what you stated about the iron being removed from the organs first. 

The girls have only recently started at these dosages too, so I'm sure it will take some time. 

Take care!

It's not true that green leafy veggies contains lots of iron. That's a lie from the industry. Green leafy veggies are very important to get our daily intake of vitamine K.

As for the Exjade: it took me more than 8 months before I saw the FIRST drop in my ferritin. Be patience & give it a try!

Warm wishes, Dore
p.s. my heart is full of joy that there exist a family who adopted even 2 thal majors. [see here nice lauging smilies and a heart]

I'll second what Dore has said.

You are surely one of a kind! One in a million!
Your  babies are so lucky!

Lena.

Yes i agree

Emommy, the pictures in the gallery are so cute, may God bless you and your family

manal

 

Emommy, you are a very special family  

Sharmin

Words are not enough to describe what i feel .

E Mommy....You know that saying about God being unable to be everywhere and so He made mothers...I guess He is most present in YOU!
Hats off to your heart, your spirit, your courage, your being in this world...those who are able to be showered by your love are the closest to the Almighty!
Madhavi

Everyone's comments are so kind! 
But honestly, these daughters of mine are such a HUGE blessing to me! 
They have taught me a lot about life.  My only wish is to see them live long healthy lives themselves.
Whatever we can all do in order to make life better for all people living with Thal is what we must do!

I am wondering if any Thal patients have had the H1N1 (Swine Flu) and recovered from it?

My 2 sons have had this virus and we have been trying very hard to make sure the girls do not get it.

So far, so good.  The girls do not show any signs of the virus yet.  I spoke to someone at CAF and they said they would get back to me about any reported cases.  If so, I will share any info I receive here. 

Thanks!

Please do. This would be very important.

Hope the boys are doing well now

manal