L1 or exjade? really worried.

HI to all
I havent been here for a while. Sara is 5 now and has started school. She is still taking exjade.Its been 3 years since she is taking exjade. The thing is now exjade is giving her swollen face  and iam really worried. she has got puffy face from exjade  few times before and it went away, but this time its not going away i have read  side effects of exjade. It is not really clear to me what problems  would sara will be  having to have such swollen face.I hope she doese not end up with big problem.
I dont really wanto start her with the liquid chelater as i dont think she can handle it and i dont know what else to do.  Her urine and blood test doese not show anyting major. Can any one plz tell me what other test we can do for her to show whats happning with her. ONE  more thing the doctors told me the other day that L1 will be funded here soon but iam scared to start with that as i heared its only work for certain part of the body. Can any one plz tell me if they or their kids on L1 and if its works good. How diffrent would L1 then exjade. The doctors told me sara would be taking it 3 times a day if i decided to start her with it. Sara ferriten is now 2500 and been high for quite some time now. Its goese down a little then go back up again. Can anyone plz help some of these qestions thanks.

Alia,

Are you regularly monitoring Sara's SGPT and creatinine? Are these level with in ranges? whats her dose and whats her weight?

Zaini.

salams sis zaini
No i dont really know that but i will ask her doctor. She is taking 620mg.
of exjade in 20ml of water.

Alia,

I wasn't aware that exjade could cause a swollen face - usually steroids cause such a reaction. 
Ferritin is often not a good indicator of iron levels - but 2500 is high and we should work to get that number down.  In North America doctors have become quite comfortable with combination chelation - exjade and desferal until iron levels become low enough to maintain on exjade alone.  This was the case for my son. 

As Zaini says - it would be a good idea to monitor her SGPT (ALT) and creatinine levels.  It may also be a good idea to monitor her ESR levels to see if she is experiencing any inflammation in the body.  More importantly however - liver and kidney function needs to be monitored if you see puffiness.

What is your daughter's weight?  If exjade is not causing any problems it may be a good idea to give her 30mg/kg of exjade for a while in order to reduce her iron levels a little more aggressively. 

Best,

Sharmin

Hi ,
I dont know anything about the comparision of exjade and L1 or the cause of swollen face but could exjade in only 20 ml of water be causing this. My son was initially told to take his 375 mg in 200 ml . Later they said 100 ml was ok b/c he couldnt keep 200ml down on an empty stomach.
Zahra

Yes, for that amount of Exjade, 100 ml liquid would be recommended. When the dose is more than one gram, 200 ml is suggested.

Ferriprox (L1) is a good chelator and gives the most protection to the heart. However, to get the ferritin down, I think it would take a combination with desferal to make good progress. Swelling in the face could be an allergic reaction to Exjade and should be closely monitored. If there is any sign of swelling in the mouth or the tongue, stop Exjade immediately and contact your doctor. I would hesitate to take a higher dose until the swelling is resolved. If the swelling dissipates and is no longer an issue, it may just be a matter of time before you see a drop in ferritin. If the swelling does continue, serious consideration to switching chelators should be given. In terms of effectiveness, the best combination of chelators is L1 and desferal. As with all chelators, tolerance of the drug must be established for long term use. Try raising the amount of liquid to 100 ml, and see if there is less reaction. If the swelling does clear up, make sure her dosage is no less than 30 mg/kg body weight.

Thanks Andy sara is taking 34mg per kg of her weight. I told her doctor about exjade to put in 100ml instead of 20. We will try and see if it will work on her now. Thanks for all the replies. Iam just very angry with her doctor about not telling us the right info regasrds to ex jade.
thanks

It has to be very difficult to take Exjade with so little liquid. It just seems this would make the stomach reactions even worse.

Thanks Andy
As exjade is expensive medicine, and its not funded in a lot of countries i think doctors should give the right direction to Pationts about it. Every  time i saw Saras doctor and told her my concern about exjade on how gives her some problems, she always told me there is nothing i should be worried about.