Ferriprox-exjade combination therapy

Hi Dey,

For elevated SGPT levels,try splitting the dose of Exjade in two,it helped in may daughter's case,her SGPT was also high.

Zaini.

hi every body,
i want to vote against combination bet ferriprox and exjade cause this will be a big load on  GIT, liver and kidney.
but i am with combination trials between exjade and desferal.
only i wanted to say my opinion in this issue

Hi Maria and welcome to the site

The doctor didn't encourge this combination cause there are no studies on using both chelators togather. Many doctors too are against this trail because both chelators have the same side effects so they think that you are doubling the side effects.

In spite of this, some patients try this on their own, but i don't know any except what Lena (our dear member on the site) reports about a young girl who use this combination ad she is doing well.

But i think you should follow your doctor advice because you  should be closely monitered in terms of white blood counts, kidney and liver functions.

Regarding your T2*, i should be honest and tell you that this is really alarming and you should act immediatly to stay above 20 in order to be in the safe zone. The best way to help change this reading, is by using IV desferal 24/7 for a while in combination with exjade or ferriprox. But ferriprox will be better since it is the best chelator for the heart

The MRI is a little elevated but definetly will be lowered by using the combination. What is important now is to concentrate on lowering the iron in the heart and i advice you too to follow up with a cardiologist to keep you monitered until the heart is clean from iron


Also I advise you to be on antioxidants to help you reduce the damge of the organs. IP6 is considered a natural chelator in addition of being an antioxidant. Also Vitamin E from a natural source (d-alpha tocopherol) is proven to reduce the oxidative stress on cells and studies showed that taking up to 600mg daily of vitamin E is very safe

Manal

Dear Manal,
Thank you for the answer and for the advices. 
I know why my doctor tell me "no" and I really do know that the best for me is ferriprox- desferal combination, but there are many things that make me to want ferriprox – exjade combination.
Yes, I’m little afraid about side effects, but I’m optimistic too. Will see … 

Hello Maria,

Welcome on the forum,I will totally agree with Manal,combination of ferriprox and Exjade should be taken under doctor's supervision,coz you'll have to monitor blood counts,serum creatinine and liver functions etc,and if you have had any liver problems already,exjade might not be the best choice.

What i don't understand is why was your ferritin elevated after IV desferal,were you not taking ferriprox along with desferal ?,and it seems like you are allergic to desferal? Was that only IV desferal or sub q also gives you these symptoms?

Zaini.

Hi Zaini,
I’m totally agreed with you and Manal, and I will have doctor’s supervision. I’m crazy, but not so mach
About desferal: nobody can tell why, just happened. I have problem with all kind of administration - no matter iv or sc, no matter of dose, no matter of 8 – 12 – 24 hours. I can accept the allergic reaction (somehow), but I can’t accept that my blood pressure goes down to 50/30… So the desferal is not my chelator.
In this moment my chelation therapy is Exjade - 31,25 mg/kg . I can go on only with Exjade, but I want to see some results this year, not after 2 or 5 years… So I think of only to combine Exjade and Ferriprox…

And thank you all for kind attention.

LactaCaramel,

I have just seen your mail and definitely I have a lot to advise you since I have been on the Ferriprox-exjade combination therapy for two months. Although my ferritin was low( around 100) I decided to give it a try as I wanted a break from the pump. When on ferriprox-exjade, I had not one serious side effect (no rush, no stomach problems except for slight stomachburn which was resolved with a stomach pill, no elevated creatinine, no side effect on liver or kidney whatsoever). Of course, I have been taking it for only 2 months together with ferriprox. The ferritin went down from 113 to 68 and then I decided to stop exjade (inspite of my doctor's advice to continue). I decided to stop it, as I estimated that the risk I was undertaking was too much considering the benefit.So I voluntarily completely stopped exjade.
As for you, although your MRI results are alarmingly low, I would be very reluctant to advise you in favour of ferriprox-exjade combination therapy. Others would say 'yes' to that, as your iron burden is too high. But as long as your doctor is against it, I am afraid you can do nothing about it.There is no protocol, no clinical trials for this combination, so it depends on the doctor to advise it. Most doctors do not.

There is only one thing I want to underline to you:

With exjade only, it will take you too long  to lower the MRI results and time is really pressing.
On the other hand ferriprox is necessary to you because it is cardio-protective.So you have to be on ferriprox. Ferriprox and desferal together is the ideal combination. Maybe you should re-try desferal and work with your doctor on your pressure problem.You may lower the desferal dosage and increase the frequency in a week.

Good luck,
Lena.

Excellent advice Lena. Thank you.

Maria,

I didn't have time to comment much earlier. As far as I know, within our group, only Lena and Panos have any actual experience using the Exjade/Ferriprox combination. I will say that both had positive results, but two people is not much to go by in terms of the safety of this combination in general. So this becomes a potentially risky undertaking, as everyone has pointed out, if not closely monitored by your doctor. Manal, Zaini and Lena have all provided good information and I hope you do listen to the advice about antioxidants also, as this is very important for protecting your organs in the face of a high iron load. Your ferritin is now under control and even though your T2* score is a serious eye opener, you should take some comfort in knowing that once the ferritin has been lowered, Exjade works much better at reducing the iron load (but not as well as Ferriprox in the heart). I don't know if a return to desferal would be advised, since your previous experience was quite negative. Honestly, my preference here if only one chelator is used would be Ferriprox. Your heart has a high iron load. Ferriprox is best for the heart. In addition to removing iron from the heart better than the other chelators, as Lena mentioned, it has a cardio-protective effect.

I will totally agree with Lena. You should be on Ferrirpox first.

I do have a question. Is your liver reading a T2* also, or was it an MRI? I ask because the scale for T2* and liver MRI are very different.

I am at a loss here, Andy.
Isn't T2* an MRI score? What do you mean by" liver  MRI"?

As for Maria's chelation scheme, returning to desferal would be a little painful, that's for sure.
However as much as I believe Ferriprox is the first to consider taking, I do not believe that a ferriprox monotherapy is suitable here. I have mentioned it before: after one year of ferrirprox monotherapy, my heart got much clearer, but my liver got a little more burdened with iron. No big deal, but this shows monotherapy is not enough. It needs something to add.

Lena.

A liver MRI, such as you with Ferriscan, will give an estimate of iron weight as mg per gram, just as a biopsy will. With this, the lower the weight, the better. A result under 7 is considered to be the safe region. With a liver T2*, which also uses MRI but a different measurement technique, the score is similar to that with the cardiac T2*, so the higher, the better.

The normal values for T2*
Heart 5216 ms,
liver 337 ms,
skeletal
muscle 305 ms,
spleen 5622 ms.

These are for normal subjects with no iron load. 52 may be a normal score for the heart, but above 20 is considered safe for thal patients. A liver T2* below 9 indicates iron overload.

I found this posted from the recent Berlin conference on the TIF site.Ferriprox produced more than double the rise in cardiac T2* score than desferal or Exjade. There is no question about its superiority in removing heart iron.

IMPROVEMENT IN THE GLOBAL HEART T2* IS SIGNIFICANTLY GREATER FOR DEFERIPRONE IN COMPARISON WITH DESFERRIOXAMINE  AND DEFERASIROX GROUP

It does affect the advice, so knowing what this low score represents is essential. A liver MRI of 2 is very good. A liver T2* of 2 is very bad.

Totally agree with Lena,and i would like to add,unless ferritin is within normal range,i don't think any kind of monotherapy is enough,Its been over an year Z started exjade,and we were able to reach her full dose just for a couple of months may be,because of her elevated SGPT levels,we lower the exjade dose,SGPT went down,we increased exjade,SGPT went up.after splitting the dose,her SGPT did go down in normal range,but then i increased the dose just by 100 mg and her SGPT has started to rise,not too much,it was 45 checked today,but this raises the question again if we'd be able to reach her max dose.So i was wondering how awesome would that be if she can take both ferriprox and exjade at the same time,ferriprox will not only help lower her ferritin,but it would also keep her heart safe.

Doctors here aren't ready to use desferal with exjade let alone ferriprox,and another problem that frustrates me is that doctors aren't ready to take her ferritin readings seriously,1100 doesn't seem to them too much,and i am unable to make them understand that i want my daughter's ferritin to be in normal ranges,this seems like impossible to them, .

There should be studies done on these combinations,or starch DFO would be great too.

Zaini,

I often think about you and little Z's case -  what with using exjade and all - and I would like to be frank about things but then I realise that you don't want to quit exjade and keep my mouth shut.

Anyway, your doctors seem to live in the previous thalassemia century and the fact they consider any ferritin round 1000 to be normal is very irritating. On the other hand, I don't know what I should do  if I were in your shoes...really. How to convince them??? I don't know which is the best way to tackle an ignorant doctor....Sorry for being blunt, it is irrititating to see a doctor blocking the patient's wellfare.It may  happen anywhere in the world.

I don't believe in monotherapy and especially exjade monotherapy. Even desferal monotherapy which literally saved us through years,  proved to be inadequate for heart protection. Let alone exjade which, day in day out, loses its credibility as a monotherapy potential drug in thalassemia.

Lena 

As some of you know I went back to deferiptone this week. Suddenly I understand why I quit with.it. Lena, how.many.of each did you take? does there exist amy data about this? Maybe is this the future I need...

Lena, I am very interested in this combi therapy. Do you have any more information availalble? For example how much of each or any idea how I can come in touch with your doctor? I would like to have more information about this before I want to ask my doctor about it.
As some of you may know I quitted Exjade last week, but once again I come to realise ferriprox is also not an answer for my ferritin issues. It's just a mess. My ferritine rised over 2000 already.