IVIG while having ET?

[George1976]

Hi everyone.

Has anyone with ET ever been treated with IVIG and if so did it help or hurt your symptoms?

Almost 3 years ago I stopped taking peg for my CALR ET due to a suspicion it was causing neuropathy in my feet and a constant sick dizzy flu like feeling in my head. I had been on peg for 4 years up until then and was happy with the way I felt. My last dose of peg caused intense pain in my legs so bad I swore I’d never take it again. I stopped peg and went with no treatment for 2 months then started HU and the neuropathy and the sick flu feeling has remained constant with few exceptions. The sick flu feeing is a daily occurrence often starting as soon as I open my eyes in the morning until I go to bed at night. Platelets are in the low 500k range and no improvement compared to when they’ve been as high as 850k during these past 3 years. CBC’s and bone marrow biopsy are yielding no clues either so it seems my blood is not contributing to this problem.

Many many doctors visits including neurologists and hematologist and rheumatologists have resulted in no clear understanding. One idea was to try IVIG.. Articles have been published about how pegulated interferons have caused CIDP when used for treatment in hepatitis and IVIG was used to resolve it. My neuropathy is not CIDP but I was told by a couple doctors to try IVIG anyway. Another doc said it could hurt my brain, although he did not say why he thought that just that my situation was very unusual and he would not risk it. If I had a JAK mutation I could see clotting being a risk but with CALR it appears I have a bleeding risk. At this point feeling so sick every single day is so hard to take I’m about to try it anyway.

[hunter5582]

It sounds like you are in a complex situation that does not have a clear answer. Sorry to hear about the problems with Pegasys. Unfortunately, hydroxyurea can also cause neuropathy and other symptoms you are experiencing. It raises the question of whether the adverse effects of cytoreduction outweigh the benefits in your case of CALR+ ET. It would be reasonable to consider other options like Jakafi or anagrelide if you do believe cytoreduction is needed. You are correct to think that it is possible that your greater risk may be for hemorrhage, particularly when your PLT levels are higher.

It seems like a reasonable thing to consider that you may be experiencing an immune-mediated neuropathy given that Pegasys can cause autoimmune disorders. Hydroxyurea may or may not be playing a role in sustaining the neuropathy. I would wonder about stopping all cytoreduction to see if the neuropathy improves. That presumes that the risks of stopping cytoreduction are acceptable. I do not know what to suggest in that regard. I would suggest that feeling so sick every day is not an acceptable outcome of treatment.

I believe that IVIG has shown efficacy in the treatment of immune-mediated neuropathies as well as CIDP and other types of neuropathies. Given that you may be experiencing an immune-mediated neuropathy, IVIG seems like a reasonable consideration. Like everything else you may choose, IVIG comes with risks as well as benefits. Given your struggles with past medication use, I can understand where you would have a lot of reservations about IVIG. There is not a clear answer. This is certainly not an easy decision.

Wishing you all the best as you move forward. Please do let us know how you get on.

[George1976 in reply to hunter5582]

Thanks Hunter. Great points. It may be that the meds damaged my immune system which is mostly in our guts. Initially when my ET was diagnosed in 2014 I felt fine. I had some curious gut issues that felt like painful gas that would come and go, some minor excessive bleeding and a minor dull headache but generally felt great. No weakness or numbness.

First med for it was anagrelide and it made me feel sick as a dog with palpitations and numbness. That first docs insisted I take enough anagrelide to get my platelets down to normal for first few months. I eased up a bit after seeing Dr. Spivak and let my platelets hover around 1m. After 2 years of that I switched to peg and felt better except for the day of the injection. Four years of that and I began to feel sicker for 3 or 4 days after injection and foot weakness came on too but I didn’t feel really bad or have numbness until I began to take my peg shots every 2 or 3 or 4 weeks. That’s when my gut seems to have taken a major hit and the numbness and intense flu feeling in my head came on within days.

After my last dose of peg i took nothing for two months and my platelets slowly rose from about 600k to 900k. During this time my sick head was the worst. I also had major gerd symptoms during this time which caused me to severely limit foods I ate. Things got a little better after starting HU but not drastically.

Last spring I tried Rux but didn’t like it. My platelets were going up and needed more and more of it. If I missed a dose I felt agitated like withdrawal symptoms so I switched back to HU after two months. I think Rux may just not work as well for CALR. Same for peg. I think it works better for JAK mutations. On peg my platelets got to normal but my allele burden rose in the last few months of being on it.

Looking back over the past 3 years now the most noticeable change is that my gerd is much improved. I can eat many things that caused great pain 2.5 years ago.

And yes I do think I need to try backing off the Hu a bit more to see if that helps. I’ve eased up on it a little in the past couple weeks. Even though I have some really bad days there have been a few more days that are better than most. Perhaps this all started when I took anagrelide. Then going straight to peg may not have allowed my body to heal. And then now the HU is preventing more healing.

[hunter5582 in reply to George1976]

I agree that the gut microbiome is critical to health. Disruption can cause a host of issues. I have had my own series of interesting and unique health learning opportunities with GERD, esophagitis, hiatal hernia, and one incident of gastroparesis. What is interesting is that the worst GI-system issues I experienced occured at the same time that the ET progressed to PV. I have come to understand that our bodies are integrated systems where many parts need to work in harmony to maintain health.

You have certainly been through the mill with trying all of the treatment options. There is no way to be sure what caused what at this point. I expect all you can do is try to deal with what you have now.

I had forgotten that you saw Dr. Spivak, who used to be my MPN Specialist too. He was a great doc with a unique perspective on MPNs. It is unfortunate that he is no longer in active practice, though he did deserve the chance to retire. Regarding ET treatment, he famously said "Leave the patient alone!" I expect you heard something to this effect from him too. As you know, he was not a proponent of HU in most situations. HU may make the most sense for you at this point if you need cytoreduction. Hopefully, we will soon have other options.

The next steps are certainly a conundrum. Wishing you all the best in sorting it out.

[EPguy]

I'm still here checking on any autoimmune concerns. You can see posts on my extensive ordeal with a severe autoimmune reaction to a flu vax and Besremi.

It's possible we share that fateful "one last IFN dose". Like you I had various warnings including that dizzy feeling etc, but I took a last (reduced) dose anyway.

I post to help others here be on the lookout for this rare event. This risk is in the labels, (from the hepatitis experiences) but who would think "that will be me"?

IVIG can be very effective for neurological and related rhuem conditions. Note there is also more convenient SCIG, administered similar to PEG/Bes. Details matter and either one is strong medicine so it does need close monitoring and skilled Drs to design the formulation.

My neuro suggested Alpha-lipoic acid supplement. Might help some, I'm intolerant to it as to near all rhuem meds, and anyway my suffering is not of a neuro sort now.

I relate to your openness to anything that might work. Sleeping is the main time we feel normal. Traditional meds all blew up on me. I'm starting TCM (Chinese med) from a top such Dr, might be helping.

My MPN is nice and boring in the background.

[George1976 in reply to EPguy]

Thanks EPguy. This kind of experience is likely why some MPN docs suggest taking just enough meds to feel better and not so much to hit “normal” levels.

I’ve had many theories over the past 3 years but a couple points have stayed constant. Mainly my gut microbiome was blown out at the start literally a couple days before the serious numbness started. As that has improved there have been some hopeful signs that I am getting back to MPN as my only problem baseline which never really made me feel bad even when my platelets were 1700k. When my gut is unhappy my neuro issues flare up sometimes really badly. So I’ve been trying to exercise as much as possible and repopulate my gut with as many healthy herbs and spices and varieties of healthy foods as I can and hopefully I will improve. Occasionally I have a really bad day and I begin thinking about treatment’s like IVIG again so thanks for the suggestions.