almost 1 year on exjade

Little A has been on the full dose of exjade and completely off of desferal for one three weeks now and his ferritin is 533.   I hope that everyone will be patient with exjade, it takes time but it does work!

Best of luck to all,

Sharmin               

Sharmin
i have a question abt the study of exjade + Desferal combination therapy ...
this study was in trials a few months ago , i wana ask that , is this study is now approved and published now ... i m asking this bcoze doctors who told me that they won't to try anything on me, thay is not approved yet,  ...... those doctors are now practicing this .. that is the reason why i m asking this ..

umair

Umair,

From what I was told, the combination has been very safe and effective because both drugs were required in small doses.  The study will be published shortly from what I have been told.  I will post as soon as I know that it has been published. 

Sharmin

An interesting point about the combination of Exjade and desferal is that since shortly after the approval of Exjade, some doctors have been using the desferal/Exjade combo in their iron overloaded patients. Masuma reversed congestive heart failure with the combination and Danielle has also used it. Even with a lack of trials to measure the efficacy and safety of the combination, doctors have been prescribing this for some patients, so I am not surprised that doctors in Pakistan are also doing so. Frankly, when a doctor knows that the patient's best chance of survival is intensive chelation, there's a good chance that this may be tried. Every case I have heard about has had good success with this combination. As long as proper monitoring is in place, I would suspect they will have good results. The doctors in Pakistan deserve great credit for advancing thalassemia care so much in the past few years, and they are doing a good job keeping up on what's new. We have had many doctors from Pakistan frequent our site. This tells me they are quite active in gathering information to help their patients and I wish this was true of doctors everywhere.

Sharmin, that's great news for little A. Exjade is very effective for chelation when iron load is low. As you observed, Exjade is very good at lowering iron load when combined with desferal. I think this therapy is probably a better approach than just switching to Exjade when the iron load is high. Once the iron load is under control, a switch to Exjade alone will be right for many patients. This is something that could be of much help to those who find themselves in a dangerous situation with their iron load, and I am wondering if we should start suggesting it in extreme cases, as long as it can be done under a doctor's regular supervision.

Thank you Sharmin and Andy for a helpful & detailed reply, i was wondering that the doctor who said me that she won't to do experiment on me and she will wait for the result of study of Desferal & Exjade's combination therapy, bfore starting this therapy here on her patients ....


Umair

Andy,

Little A is currently on an exjade dose of 32mg/kg.  His last ferritins have been 900, 866 and 533 respectively.  His transfusions are 3 weeks apart, and his pre tx hg is around 100 each time.  Do you think that his exjade dose should be reduced at some point, ie. if his ferritin is 300?  Or should we base it on his tranfusion requirement over the next few months?  I am wondering, if his ferritin declines drastically if it may be useful for us to fly to Oakland for another SQUID sooner than 6 months.  We are still waiting to see if Blue Cross has changed their minds after our appeal - about covering exjade. 

Thank you for you help,

Sharmin

Hi Sharmin,

Not mean to interject since the question is addressed to Andy. However, just for information that Little Miss A was off of Exjade for three months after her ferritin dropped to 500. The optimum ferritin for thalassemia is 700 (neither too high nor too low) rather just enough to keep chelating. Little Miss A has started the exjade again and her last reading was 1000.

Thank you Canadian Family,

Your responses have been very helpful to us, we would have been lost without your advice - thank you so much.

Sharmin

I'll participate in this discussion about ferritin levels, even though the question was addressed to Andy. There is an essential point here as to which are the optimal levels of ferritin that will enable a thal to lower chelation-even for a while.

Time and again in this site have I observed thals to express the will to lower or even stop the dosage when achieving a good level of ferritin. Is this right? considering that transfusions do not cease to take place, I wonder. I know a lot of thals here who are not allowed to stop or lower dosage when their ferritin reaches 100. At a time when my ferritin was 45 I continued to chelate non-stop. Was this wrong?I do not know. The only thing I know is that every time I relax and follow the easy path, my ferritin goes up. 

Don't you think it is time for thal experts to reset levels for thals who are "clean"?
More and more thals -now that we have in our disposal a variety of chelation drugs- get low ferritin levels and maybe it is time for doctors to examine at which point the drugs become toxic and set the new levels. Maybe a question should be raised in New York next October in the TAG conference (for those who will attend.)

I have much more to say on this subject and a lot of examples, too, but I'll stop here.

Lena.


 

Lena's suggestion to bring this issue up at the October conference is an excellent one. While patients do continue to transfuse and add iron to their bodies, it does not necessarily mean that chelation should continue at the same level. Since we will have the top doctors available, let's do find out what is considered the optimum ferritin level for thals and if this varies depending on which chelator they use, and at what point should the dose be reduced.

Sharmin, for now I'll say that once little A's ferritin drops below 500, you should ask Oakland if a dosage reduction would be appropriate. All chelators do have side effects, so we do want to minimize the dose to only what is truly necessary. It's a fine line but with current research, we should be able to get some answers.

Thank you Andy, I will send an email to the docs in Oakland after we get his next ferritin level. 

Lena,
We will be going to the conference in New York so we will address the questions that you have posted.  Hopefully we can bring back useful information for everyone. 

Thanks again,

Sharmin

Thanks Sharmin, thanks Andy

Yes, it would be very useful to address these questions to the specialists and see what they have to say. Let's say at which point do drugs become toxic? That, of course, may depend on each individual case.


Lena.

Lil A's ferritin November 3rd was 476. He is still on the high dose of exjade.  His hemoglobin 1 1/2 weeks post last week was 130.  I think that I will watch his hemoglobin over the next few months to determin if it may be a good idea to put him on a maintenance dose of exjade.  Perhaps I should let his ferritin drop to normal levels before deciding that.  It took us 16 months on exjade to reach this point, but it feels good to be here.  I hope that we can keep his iron levels low from now on, especially during his teen years. 

Because his ferritin is so low, a part of me does not want to take him to Oakland in February for all of the tests - I would rather just go in the summer.  I don't think that having so many tests and appointments is good for him.  I think he would enjoy a vacation elsewhere to get a break from all of this.  I will see what they have to say in Oakland about this.  Stomach issues are quite significant, so I hope that exjade with food will be an option soon. 

I think that exjade has been good for him so far, other than stomach upset.  He has grown from 131.5 to 134 cm within 2 months after switching to exjade, his ferritin has come down considerably and he has a lot more freedom from needles and the pump.  

I will keep updating, thanks to everyone for your ongoing support - we would not have made the progress we have without your love and support, 

Sharmin

Great report! My fe is still dating 1000. 

Well done! 

I might not be the right person to give an opinion, but I think it will be good to do all those tests and appt's now. I mean the ferritine in the blood is low, but maybe not yet in the organs..?

Personally I have my doubts about the 30 minutes wait after Exjade. I have such feeling that this is very outdated. I rarely follow that law. Both options didnt help with my stomach issues, so I dismissed that law.

Sharmin,

Thats great my friend i hope Lil Z's ferritin will be down soon, lil Z has grown just 4 cms in the last year,i don't know if i should be worried,there is a lot to worry about already,doc says she is ok in height for her age,i don't know if  she means it or she just says it to get me off her back  she is 127 cms .