Chelation question

Poirot,


what is the generic to Ferriprox? Maybe Kelfer? Is it still in use? and how much does it cost now? Do you still use it in India? does it have any considerable side effects? and if someone wants to order it from India how does one proceed?

Lots of questions - thanks in advance for your answers.


Lena

Lena - Yes - Kelfer (also known as L1) is the generic for Ferriprox. It is still in use in India. Thousands of thal's lives were saved with the use of Kelfer (L1) until Asunra (Exjade) was launched in India. Similar to any drug, there are side effects to Kelfer and the trick is to start with a lower dose and work it to the required dose.

As far I know, without a doctor's prescription, Kelfer (L1) is not available.

Thanks a lot, Narendra for your information.

Poirot,


what is the generic to Ferriprox? Maybe Kelfer? Is it still in use? and how much does it cost now? Do you still use it in India? does it have any considerable side effects? and if someone wants to order it from India how does one proceed?

Lots of questions - thanks in advance for your answers.


Lena

Lena,

Kelfer, Ferriprox, L1 are all the same drug - the generic name is Deferiprone.

Kelfer + Desferal is the chelation drug mix of choice in India - this is primarily because Kelfer is extremely effective in taking out iron from hearts (usually the main cause of mortality in thals) and Desferal is better at taking out iron from the liver. This combo therapy or just Kelfer are/is very much the dominant chelation therapy in India.

Our experience with Exjade or Asunra/Desirox has been more mixed in terms of price vs performance - not many haematologists want to go with Exjade as their first line of chelation treatment, especially in thals with already high Ferritin levels/compromised livers.

Cost of Kelfer = Rs350($7) per 50 capsules of 500 mg each. Standard dosage = 60-75mg/kg of body weight per day - so, you can calculate the dosage cost per day/per month. It is available via doctor's prescription only and has to be ordered directly from key distributors (ie not available at pharmacies) - but, generally a friendly Thal in India can buy the drug for you and mail it out to you. The strict monitoring of usage ceased a long time ago, as the drug has now been in use for over 20 years.

The main side effect of Kelfer reported by nearly 30% of Thals is joint pains - knees, and in severe cases, elbows and ankles. However, practice has again shown us that the best way to beat this is to increase dosage over a period of time (4-6 months). This allows the body to adjust to the drug, and usually even if there are joint pains at the starting, they disappear over time, even as dosage is increased. Other than this, different Thals have reported initial comments of nausea, stomach upsets and dizziness - but generally, these side effects disappear within a month.

A severe side effect, reported in about 2% of Thals, is neutropenia - depletion of WBCs. On starting Kelfer, one should monitor WBCs monthly for any change, and one can gradually reduce this monitoring over time. However, given that pre-transfusion CBC is done at most centres, this is automatically monitored. Kelfer should be stopped if WBCs fall below 3,000. Then, restart with a significantly lower dose, and see if the condition persists. Roughly, this condition persists in about 40-50% of those Thals who initially report neutropenia.

Hope this is useful.

Cheers

Poirot

Thanks, Poirot, your info was very valuable and enlightening.
Thanks a lot for your time,

Lena

Hi...I know that Ferriprox is good for targeting the heart and Desfaral for the liver for excessive Iron...so which organ/s does the Exjade work better on?  

Has anyone heard anything more on the new single does Ferriprox?

Exjade does clear iron from the liver and heart over time, but not as quickly as the other chelators. It has its best success when the iron load is moderate to low. For high iron loads, combination therapy using desferal and deferiprone is still regarded as the best method, although small trials have shown good results when one of the combination is replaced by Exjade, but more trial is needed. Deferiprone is still unsurpassed for the heart, because in addition to removing the iron, it also has a cardioprotective effect, not shared by the other chelators. I have not yet seen any information that indicates that Exjade works better in one organ than another.

My opinion is that Exjade is a good chelator for lower iron loads, but is not necessarily the best method for attacking a high iron load. A compilation of results from Exjade users around the world does show Exjade to be a capable chelator, with similar long term results to the other chelators. I will also mention that a dose of 32.6 mg/kg was required for iron balance, so the 30 mg/kg may be slightly lower than required. Doses of 40 mg/kg are used when iron load is high. As with all chelators, full compliance is essential.

I found your post very useful, Andy, as I believe I am in a turning point in my chelation now. The reason is desferal has become more or less toxic (my guess) to me as it makes my heartbeat rise to 90-100 while when on ferripox day my heartbeat is up to 70-80. My scheme is 13 ferriprox per day and 6 desferal four times a week (6X4). The last, unfortunately, leads to a problem, so I am contemplating now to turn into a ferriprox/exjade chelation scheme. Perhaps you remember I  tried this for 1-2 months two years ago and voluntarily dropped the scheme by myself. You see, I am a little afraid as, back then, I had stomachburn and took a gastroprotective Nexium 20 pill every morning.
Forgot to mention my ferritin is 150 and last check might prove to be even lower (i will have the resutls in a week), so I am in the "lower iron loads" that you mentioned. Same thing, last MRI was clear for heart and liver and LIC - all within normal range.

During the last years, I have come to conclusion that lower ferritins must be closely monitored by doctors - I mean the chelation scheme, considering that chelators might be toxic when body is more or less clear from iron load.


Lena

Thank you Andy.

We are waiting for the boys to have the MRI Scan for this year to decide which combi if at all to go ahead with as BB's liver showed moderate Iron level and perhaps to change LB's chelator from Ferriprox to Exjade. Both their ferritin level are good bordering at 500.

Lena I think you have managed all this for yourself brilliantly. Even with an intensive programme with chelation with your ferritin being low, your body has probably always signaled to you that this is right for you but now your body is starting to 'moan' a bit, you know you have to adjust your chelation programme. I feel you have a very strong mind because you know what works for you then you stick to that rather than do what some doctor say as what they say is guidlines and yes they do think about safety but you yourself know what is good for you (along with alot of backup research)....I hope I don't sound rude but I do admire you and hope InshaAllah, God Willing, my boys would manage things similarly.
   

Emby,

thank you so much for your kind words. I hope your boys and every thal for that matter, will manage their thal and make the best out of it. I try hard, that is true and always "regulate" my doctor's orders to fit my personal case.
Now I have some great news which made me happy: my last ferritin came out and it has reached 45 now. My doctor proposed to stop desferal or bring it to once-a-week scheme and continue only with ferriprox. That because, as I wrote in another thread, it has become toxic on me, it drops my pressure to 6 and my heartbeat to 90-100, which are normal otherwise This is a sign that, as my ferritin has dropped to 45, I need to lower my chelation rate and of course, monitor things. In my Thal Unit, we check our ferritins every month and do all other check ups periodically, so it is easy to monitor and stay safe as for your ferritin levels.

Thanks again, Emby.

Lena  

Wow 45...that is an achievement! The normal range is between 23 and 540 I think. If our boys reach that point (or about 300) then their doctors would not give them anymore chelation med for sure...at that point the boys may consider taking a break completely or I prefer that they take even 1 tablet, or 1 of their 3 daily dose, every day or every other day for about 6-12 weeks  just so that some chelation is still going on in their bodies for some of the 24 hours every day. Lena would you consider this? I dunno..I feel a bit insecure about stopping all together and I dont even take those meds...I'll be thinking 'what if..what if' but if they want to take a break then then I mustn't be 'iffy' about it, it's just for a few weeks then check ferritin level then decide again what to do. Would you consider this Lena? You have been chelating far longer than them and I think for you it would give your body a 'rest'. They also have more units of blood, 4, for transfusions every 4 weeks. You have always been a few steps ahead and you have managed brilliantly so it would work in your favour I think. I am not trying to tell you what to do I'm just asking for your advice because you have far more experience dear Lena.

Emby
       

My dear Emby,

I wish the best for your boys and as long as you ask my advice I would reply this to you:

Never-ever- stop chelating. This is, according to my opinion and my doctor's too,  a mistake. Lower your chelation dosage, yes - but never stop altogether. That is because, you see, you never stop transfusion and every blood bag brings the ferritin 200 up. No, I have been chelating since 1976 and have never stopped completely. Re-arrange the dosage every now and then, but have not stopped altogether. Do you  stop any other medication like anti-oxidants, for example? No. Then why the chelators? Iron tends to pile up in the organs so quickly and before you know it, you will find yourself in the position to try to drop your ferritin level and perhaps with greater effort. Why not maintain it in a certain level with less effort before it starts going up again?
And another thing for your boys: why 4 bags in 4 weeks and not 2 bags every 2 weeks? I try to manage 1 bag every 2 weeks. What I mean, is that it is much easier for the body to maintain a steady Hb and not going so up and so down or take so much blood once a moth.
Of course, I may not know all your personal constraints and see it from my point of view.

Lena.

Lena,

I may not completely agree with you. In some cases, stopping completely is useful, specially in children. What works for one may not work for others.

Canadian family,

you may be right as to children, I do not know.

In adults' case however, which I was talking about, things go the way I mentioned. I know some doctors stop even with 500 or 300, but this is not right. Our doctor says not to judge by the ferritin level because deep down inside the organs may be overburdened with iron despite low ferritin and this, I have witnessed with many thals in our Unit.

I repeat that I am not aware if there are any special constraints regarding children (though in our hospital children do not have a break, too). Allow me though, to have witnessed a lot as I have been chelating and researching chelation for more than 30 years...

Lena

Lena..I get what you are saying that is why I wouldn't want them to stop either...but I'm worried what my oldest sons Dr. might do because she said she would like him to stop in case of toxicity. Actually he told me that one person was told by the same Dr. to stop chelation and the reading for ferritin was up by 2000 after 6 months! You would think she realised this and would not advice this in future.
....But what if, with your heart and liver readings being normal and your ferritin at 45, which is quite excellent, you stopped for two weeks then after transfusion carry on with the same dosage with chelation that you are on (minus desferal or reduced because of it's affect on you), look at the ferritin level  and when it drops as low as this then take another 2 weeks off. It's a bit 'yo-yoing' but could this be a safer method? Could this work or not?

Both the boys prefer 4 weekly because they don't want to spend more time than they have to at the hospital and their hb maintains quite well usually above 9.2 pre transfusion.