Wish us luck !!

Thanks Bostonian

Hopefully her next ferritin reading will be even lower.

Zaini.

Zaini,

I wish that most parents were allowed to start on exjade/desferal combination as it is a quick method of lowering iron in the organs and plasma.  Once iron levels have been lowered, exjade can maintain it quite nicely.  I think that in lil Z's case combination therapy for 6 months would be enough to get her iron levels very low and then you would be able to maintain her nicely on exjade. 

The docs in Oakland feel that it is a very safe and extremely effective combination.  I hope that they will publish their data very soon and that more people will be able to try it.  I think it would be even better to have a combination of L1/exjade or
starch-desferal/exjade. 

Sharmin

Sharmin,

Yes you are right,and once this research is published and if i can get a hold of it,i might be able to convince her doctor,the problem is,the doctors here see patients with such high ferritin on regular basis,that 1200 doesn't seem much to them,and when i say that i want it to be below 500,they say 1200 isn't bad either,thats why they are not ready to take any new steps or measures,because according to them 1200 is just fine,which i know is not  .

Zaini.

Since Exjade was introduced, there have been doctors in the US who have prescribed the Exjade/desferal combo even though no testing had been done. The needs of the patients outweighed concerns about using the combo. As a result, I now get to see the wonderful updates on Facebook from Masuma, who is fully enjoying married life these days. A couple years ago she was in the ICU with congestive heart failure. Very aggressive chelation with Exjade and desferal brought her ferritin back down under 1000 and she is doing great.

If your doctor is willing to monitor the combination, I see no reason any doctor should not prescribe this combo in cases where it is needed. Please do not undertake this on your own. Have proper supervision and close monitoring. I will add that in NYC, I did hear some comment from a well known doctor about how many doctors will not wait for trials but will be proactive and trial it in patients themselves. This doctor nodded with approval of this practice. We can't always wait for trials. Sometimes, the needs of the patients are far more pressing.

Thats the thing Andy,her doctor is not willing to approve the combo and i don't wanna take this step on my own,so as for right now,we are just working with the exjade and hoping that it will lower her ferritin.

The thing that worries me is that we don't have T2* or ferriscan available,so we don't exactly know in what condition her organs are,its been 5 and a half years she has been transfusing and her ferritin has never gone higher then 2400,that seems like a good thing but we know that even with low ferritin readings you can end up with iron over load in organs,so as of right i can only pray that these technologies will be available here one day.

Zaini.

Zaini,

I wonder if your doctor would be willing to talk to the doctors in Oakland, and do the appropriate tests with lil Z.  In the past, levels between 1000 - 2000 were deemed acceptable for thals, and these thals, although they are growing up and living long lives are having some issues such as infertility, other endocrinology issues etc.  I truly believe that keeping a child's hemoglobin as close to normal (10) as possible and keeping the iron levels as close to normal as possible is the key to having them grow up normal and healthy.

I am confident that her organs are most likely free of iron because of the short duration that she has been transfused, and because she was on a very good chelation plan.  Especially, the L1/desferal - and now the exjade. 

Sharmin

Sharmin,

We are keeping her pre tx hb on 10,and working to lower down the ferritin .

Zaini.

Zaini,

why did they stop her from L1? because they turned her in  exjade? I tend to think that exjade alone is good only for low ferritin levels. 

Lena.

Yes Lena,They stopped L1 once she started exjade,and they are not allowing us to use any combo with exjade.

Zaini,

First of all they did well to stop Zainab from desferal--better exjade or ferriprox alone in her age. As to not allowing you to use the ferriprox-exjade combo...well, that's because there is no protocol yet and there is no way at all to convince them about that. It takes a little bravery from the doctor's part to dare do that. If it works, that would be the end of most of our problems. Of course one has to keep in mind that exjade was  promoted  as an "orphan drug", as monotherapy that is, and that's why there have not- up to now- been any clinical trials on the combo with ferriprox. But I wonder: how did the desferal-ferriprox combo start? Were there any clinical trials back then before they accept to apply it? Anyway, the only combo with exjade at the moment is that of desferal-exjade, both chelators made by the same company. Were there any clinical trials of desferal-exjade combo? I am not aware of any. If someone knows, please tell us. I am curious...

Lena.

Lena,

I am not aware of any clinical trials but Little A was part of the study done at Oakland on this combo,i hope Sharmin  would be abl;e to tell you more about it.

Zaini.

Dear Zaini,

It will come down InshaAllah. Both our boys ferritin peaked at 2500/2900 before it started to decline and that was just with desferal.  Lil Z is on a better chelation regime i feel.
I'm sure Allah Ta'ala will make things easy for your little Hafizah.....how is she getting on with hifz?...she looks beautiful by the way.

Sending heart felt love your way,
Emby 

Thank you so much Emby,

I am sure hopeful that it will come down,She is getting on fine with the hifz,she is on "Surah Yasin" (She started backwards),hopefully she'll finish it in couple of years.

Much Love ,

Zaini.

MASHAALLAH!!!!.....thats fantastic....I pray Allah make the memorisation easier and easier for her...and for you as parents the ability to help and support her all the way.

Lots of Love,
Emby

Thank you so much Emby

Prayers are our precious asset ,we definitely need them .

Zaini.