Starting ivig - concerns

[Gingergrrl]

Thank you for clarifying. However, the very treatments that helped you with your non-ME/CFS also have the potential to help those of us with ME/CFS, so I fear that in separating your case from all of ours, you unfortunately put people off from what might be useful treatment options

I apologize for the long delay in my reply! I definitely do not mean to put anyone off from the treatments that helped me and that would never be my intention. I try to answer every question that I am ever asked about my treatments, both on the public board and via PM. I just always clarify that in the end, ME/CFS did not turn out to be my diagnosis b/c I feel this is helpful information and I do not want to confuse anyone or mislead anyone. I had severe progressive muscle weakness that affected my ability to breathe and to walk but I did not have PEM.

I don't understand though - how can there be ME/CFS once we have enough information? Unless it is literally the IDO mutation trap, in which case it would still probably be renamed to IDO syndrome or something..

That's a very good point.. I've been in and out of the IDI trap and it didn't cure me, though it likely helped my fatigue a bit.

What does this mean (above) re: IDO Syndrome or IDI trap?

ME/CFS is a useful label for when we don't understand what is happening, and it captures the collection of symptoms that emerge from whatever overwhelming issues our bodies have. But as soon as we figure out the core issue(s), then we will rename each one and that subset of people will fall way into their own disorder.

Do you mean that ME/CFS is not a distinct diagnosis in and of itself? I am not sure if I understand. Are you saying that everyone with an ME/CFS diagnosis was misdiagnosed like I was? Or is that not what you meant?

I think you are on to something, but then we have the problem of everyone advocating for their own set of issues rather than all of us together fighting ME/CFS... so there might be some resistance to that notion

I don't think everyone is fighting for their own set of issues but that there are many people (like myself) who were initially misdiagnosed and never had ME/CFS vs. something else like Lambert Eaton Syndrome, or Autoimmune Encephalitis (or literally any number of other diagnoses). I think my experience is helpful b/c if people can be properly diagnosed, it will help not only the individual person but it will also help with screening people for ME/CFS research studies.

Apologies again @Deebeewaldo

Likewise, I apologize as well and if you are still around @Deebeewaldo and have more questions re: high dose IVIG, please let us know!

 

[GlassCannonLife]

Do you mean that ME/CFS is not a distinct diagnosis in and of itself? I am not sure if I understand. Are you saying that everyone with an ME/CFS diagnosis was misdiagnosed like I was? Or is that not what you meant?

I'll respond to this but I think we should make a separate thread if you want to continue chatting about this.

What I mean is that currently there is no diagnostic test for ME/CFS, and there is no known cause. There is only a number of leading hypotheses, and a collection of symptoms.

I expect that as we either find potentially various diagnostic tests, or determine the cause (it could be a number of different causes leading to slightly varied dysfunctions with shared symptoms), we will start calling them different things.

Eg if we find that there is a subset of patients that are ill because of chronic enterovirus infection that is clearly diagnosed (not absolutely clear atm) and can be reproducibly and successfully treated (not the case yet), then we will probably break that group off and they will be labelled as having a "chronic enterovirus infection" or they will be kept within ME/CFS but with this sub-label.

Ideally, this type of thing can happen over and over as we discover more, with each understanding leaving a subgroup of people diagnosable and treatable.

So in some ways it is the same as you (ME/CFS symptoms) but with a known cause (autoimmune and other issues). I would still say that you had ME/CFS, whatever that ultimately means, as you met the criteria, but you just happened to find your cause was among conditions that have already been characterised.

 

[Deebeewaldo]

@Gingergrrl @Learner1 @GlassCannonLife

Thank you all for your kind and detailed responses - I really appreciate it. And apologies for my delayed repsonse, the fatigue and other symptoms has been hitting me hard the past few days unfortunately
I will be having a short Zoom consultation with my doctor on Friday morning, so I will put my questions to him then regarding dosing amounts and frequency. Your responses have given me the confidence and knowledge to do this - so I am very grateful for your responses.

@Gingergrrl thanks for suggesting the FB groups - great suggestions. I too am generally not a fan, but since getting sick I've found a few of the groups to be invaluable in terms of sharing information, finding good doctors and learning about treatment options. I found the doctors that are willing to treat me via FB, so in a bizarre way I am very grateful for it! I am joined to a few IVIG groups now, thanks.

It did put me into remission but I have to give two caveats. The first was that ME/CFS turned out not to be my diagnosis (vs. several other autoimmune diseases) and the second was that it put me into remission in conjunction with Rituximab. I did a total of three years of infusions and the first year was IVIG alone, the second year was both IVIG & Rituximab, and the third year was Rituximab alone. There were specific reasons why we did it this way (in my case) that I can also explain if it would be helpful.

This is very interesting - and I'm glad it worked for you! It would be interesting to understand the rationale for this regimen if you can share it (but no worries if not too)?
Rituximab has been mentioned by a few doctors to me, especially as I get skin lesions suggestive of a form of vasculitis, but they are inclined to 'keep it in their back pocket', in part due to the ongoing pandemic.
I personally suspect I have Sjogren's (bouts of dry mouth, POTs, MCAS, burning feet) and/or a form of lupus (there is a family history of both in two maternal uncles), but apart from positive ANA, TPO Abs, mild low complement, and highly positive celltrend antibodies my bloods are more or less normal, so I've been dubbed with Post viral fatigue (EBV) and fibromyalgia by doctors in my country, neither of which fully fit my symptoms anyway, and I just have a niggling gut-feeling. I will be self-funding the IVIG, so long-term continuous use will not be an option for me, but it seems like the lowest risk of the options that are on the table for me right now.

Just in case it is helpful for the original poster, my pre-meds for IVIG were Atarax, Tylenol & Pepcid (all in pill form). I am allergic to many dyes so my meds are all dye-free (but that is in general and not specific to IVIG pre-meds).

This is very helpful - thank you for sharing. In general, do people get pre-meds even with the very first infusion, or do doctors tend to wait and see how it goes, and only had in pre-meds if they are deemed necessary? This approach does not appeal to me, and I will be saying this to my doctor on Friday, but I'm also not super keen on steroids as pre-med, if I can avoid them (I get very bad rebound inflammation as I come off of them, no matter how slow I taper).

If there are any other general tips or suggestions, please feel free to share them - I am immensely grateful for any experience you can share. Asking for the doctor to write out the rate limit seems to be a major one, and keeping that relatively low first time in particular (< 100ml/hr?)

 

[Gingergrrl]

I'll respond to this but I think we should make a separate thread if you want to continue chatting about this.

Thanks and I totally agree that this should be a separate thread (if we decide that we want to continue this conversation). If you do end up making a separate thread, can you tag me so I will see it? But if you don't, that is totally okay, too!

So in some ways it is the same as you (ME/CFS symptoms) but with a known cause (autoimmune and other issues). I would still say that you had ME/CFS, whatever that ultimately means, as you met the criteria, but you just happened to find your cause was among conditions that have already been characterised.

I wanted to reply to this one part (b/c I thought it was important) and then I'll reply to the OP's questions re: IVIG in my next post.

I have to respectfully disagree w/you that I still had ME/CFS once the true cause of my illness was found (and my doctors would disagree w/you as well). I had multiple separate diagnoses (all of an autoimmune cause) and to me, the differential diagnosis is very important b/c once someone tests positive for a paraneoplastic autoantibody (in my case the LEMS autoantibodies), then it is very important to be screened for the type of cancer that often correlates to these autoantibodies, and to do the screenings for several years in a row to rule out cancer as the cause vs. autoimmunity.

Having the proper diagnosis is very important and (unless I am misunderstanding you?), LEMS and ME/CFS are truly two different illnesses. I never had the core symptoms of ME/CFS (lack of ability to create or maintain energy, fatigue, brain fog, or the #1 symptom of PEM). Misdiagnosis is very common and I do not fault the doctors who originally gave me a "CFS" diagnosis (the term used in the US) but in my case, it was an incorrect diagnosis. I hope this makes sense

 

[Gingergrrl]

Thank you all for your kind and detailed responses - I really appreciate it. And apologies for my delayed repsonse, the fatigue and other symptoms has been hitting me hard the past few days unfortunately

No apology needed, and I am glad that we can all help, and am sorry that you are feeling so poorly right now.

I will be having a short Zoom consultation with my doctor on Friday morning, so I will put my questions to him then regarding dosing amounts and frequency. Your responses have given me the confidence and knowledge to do this - so I am very grateful for your responses.

I'm glad that you will have a Zoom consult w/your doctor and that you will get to talk to him re: the IVIG before starting.

@Gingergrrl thanks for suggesting the FB groups - great suggestions. I too am generally not a fan, but since getting sick I've found a few of the groups to be invaluable in terms of sharing information, finding good doctors and learning about treatment options. I found the doctors that are willing to treat me via FB, so in a bizarre way I am very grateful for it! I am joined to a few IVIG groups now, thanks.

I share your feelings re: Facebook but the private medical groups on FB (like the IVIG groups) were extremely helpful for me during the 2-years that I was doing IVIG. They really had some valuable info that I was not able to find other places and I think that you will get some good feedback there.

This is very interesting - and I'm glad it worked for you! It would be interesting to understand the rationale for this regimen if you can share it (but no worries if not too)?

In my case, the high-dose IVIG started to reduce my symptoms (and almost immediately put my MCAS into remission) but it was like a band-aid in the sense that it would have needed to be done forever and this was not sustainable. What I mean by band-aid is that it was like a stop-gap measure that helped to reduce the fully grown autoantibodies but it did nothing to stop my B-cells from creating NEW autoantibodies.

So once I had done a full year of high-dose IVIG, my doctor had me start the Rituximab to kill my B-cells. So then we overlapped the IVIG & Rituximab so that BOTH the fully grown autoantibodies were addressed, but in addition, without B-cells, no new autoantibodies could be created (so the autoantibodies were stopped at the production level).

Once I was on the Rituximab for about 9-months, I went into remission and I was able to walk without the wheelchair and breathe normally and was able to start PT to try to regain muscle strength after 4-years of using wheelchair, etc. So at the point that I had done 2-years of IVIG, we stopped the IVIG b/c it was no longer necessary for the autoimmunity and the Rituximab alone ensured that no new pathogenic autoantibodies were being created (b/c I had no B-cells).

Let me know if this makes sense!

Rituximab has been mentioned by a few doctors to me, especially as I get skin lesions suggestive of a form of vasculitis, but they are inclined to 'keep it in their back pocket', in part due to the ongoing pandemic.

I completely understand and in my case, I did the Rituximab from 2017 to 2019 (pre-pandemic). I had planned to continue maintenance infusions of Rituximab 2x per year (in 2020) but my doctor had me stop Rituximab completely in light of Covid. I had to do a super strict quarantine for 3-4 months in 2020 until blood tests showed that my B-cells were fully back (both in number & function) b/c of the risk of being exposed to Covid while not having any B-cells.

I was extremely fortunate that my new B-cells grew back healthy and I was able to maintain the remission without Rituximab (which we could not predict until it happened but I could not take the risk in light of Covid). My last Ritux infusion was Nov 2019 and I did not continue it in 2020 b/c of Covid. Luckily this turned out okay but it was kind of a no-win situation at the time (either risk getting Covid with no B-cells or risk ending my remission by stopping Ritux).

I personally suspect I have Sjogren's (bouts of dry mouth, POTs, MCAS, burning feet) and/or a form of lupus (there is a family history of both in two maternal uncles), but apart from positive ANA, TPO Abs, mild low complement, and highly positive celltrend antibodies my bloods are more or less normal

To me what you described (positive ANA, TPO Abs, low complement, high positive celltrend abs, etc), is NOT "normal blood tests"! Especially not with a family history of autoimmunity. I am sorry that your doctors are dismissing clear autoimmune results (in my opinion). I also had a positive ANA, both Hashimoto's Abs, very high positive cell trends abs (in addition to the other stuff I mentioned).

I will be self-funding the IVIG, so long-term continuous use will not be an option for me, but it seems like the lowest risk of the options that are on the table for me right now.

I am so sorry that you are having to self-fund and I am hoping that it costs less outside of the US (as it is cost prohibitive here to self fund). I was very lucky that my insurance covered it although they literally put me through hell. I had no problem getting my insurance to cover Rituximab but it was a monthly epic battle for them to cover the IVIG (in my case).

This is very helpful - thank you for sharing. In general, do people get pre-meds even with the very first infusion, or do doctors tend to wait and see how it goes, and only had in pre-meds if they are deemed necessary?

In general, everyone who gets IVIG would have pre-meds starting with the first infusion. There is a high risk of allergic reaction or anaphylaxis from IVIG so you would want to have Benadryl or Atarax or a strong H1 Blocker as a pre-med (even if you do not have MCAS). The Infusion Center should also have Epi available in case of anaphylaxis from the IVIG.

Each individual's pre-meds are different but it is usually an H1 Blocker (like Benadryl), an H2 Blocker (like Pepcid) and Tylenol. Some people also require steroids as part of their pre-meds but in my case, I didn't take any steroid with IVIG.

This approach does not appeal to me, and I will be saying this to my doctor on Friday, but I'm also not super keen on steroids as pre-med, if I can avoid them (I get very bad rebound inflammation as I come off of them, no matter how slow I taper).

I would discuss this with your doctor.

If there are any other general tips or suggestions, please feel free to share them - I am immensely grateful for any experience you can share. Asking for the doctor to write out the rate limit seems to be a major one, and keeping that relatively low first time in particular (< 100ml/hr?)

I would definitely do a very slow infusion speed for your first IVIG b/c this can reduce the risk of allergic reaction or anaphylaxis and also reduce the risk of aseptic meningitis, blood clots, and other IVIG side effects. IVIG is a very thick substance and the slower infusion speed also reduced the severity of the headache that IVIG causes (in my case).

I was not able to tolerate over a speed of 40 ml/hr b/c I had such a high risk of third spacing/edema of the fluid (due to MCAS). But others tolerate a higher speed with no problem! I hope this helps.

Edited to add: The brand of IVIG that I did for the entire 2-years was Gamunex b/c my MCAS specialist felt that it was the one that I was most likely to tolerate without an allergic reaction (and he was correct). I wanted to mention this in case it was helpful.

 

[GlassCannonLife]

I never had the core symptoms of ME/CFS (lack of ability to create or maintain energy, fatigue, brain fog, or the #1 symptom of PEM).

Ah ok, I didn't realise you didn't meet a number of the criteria.! In that case I fully agree.

 

[Deebeewaldo]

Hi all,

I just wanted to update you on my first IVIG treatment. Unfortunately it did not go so well, but also not so bad.

I was premedicated with Tylenol, famotidine and cetirizine, and I drank buckets of water and electrolytes in the 3 days beforehand. I had 500ml IV saline pre-IVIG.

The IVIG started at a rate of 15ml/hr. This seemed okay, no major issues. It was then upped to 25 ml/hr. I started to feel a heavy fatigue, like I experience when in a flare/after a flare, so I wasn't too worried about this. By the end of this 30 minutes I was starting to get flu-ey aches and pains, a temperature, fever-ish, but nothing too severe. The rate was upped to 35 ml/hr, and all these symptoms worsened, along with my abdomen starting to swell hugely! Almost like fluid retention. I also started to get diarrhea at this stage. Most worryingly it worsened what I call my nerve symptoms. I get a lot of what I would call nerve pains since getting sick- sharp pulsing pains, in the back of my shoulder blade, around my left hip and down my leg, with sharp pains coming out through my tendons. I also get a nerve pain in by my sternum and it caused all of these to rile up.

I was getting a bit out of it at this stage too from the fever, so asked for the infusion to be paused. We started again at the lowest rate, but all the symptoms returned. A doctor was called and gave me an IV shot of hydrocortisone. Then we paused for two hours and some of my symptoms subsided, my temperature went slowly down, and my stomach swelling eased off a small bit. I was urinating, although not as much as I would have expected given all the fluids. I had gained 1.5 kg in weight during this attempt at infusing (only about 5 g of IVIG at this stage). They did a bladder ultrasound which was normal.

We restarted after two hours, but again I only made it to the 35ml/hr rate before we had to abort again for recurrence of the symptoms. So overall I had 8g of IVIG. I had a burning achy spine, bad chills and generally felt acutely unwell that night. However it was manageable. My abdomen swelling went down completely in the next day or so. I didn't get a very bad headache the next day, although my IBS-type symptoms (which are usually constipation) was IBS-D instead, and I only managed to eat some toast for the two days after it.

Unfortunately it also seems to have worsened some symptoms significantly, or lowered my overall baseline since the infusion. E.g. I have the worst bladder pain and cystitis I've had in over a year (since starting low dose amitriptyline, which helped hugely). I have much worse chills, nausea and burning pains all over too, particularly in my tendons, and my morning POT-sie issues have gone back to what they were like before amitriptyline (super shaky, nausea, bowel urgency, generally feeling awful). My erythromelalgia has also worsened notably.

I now suspect this has all triggered an MCAS flare-up, although not super-typical maybe?

Has anyone else experienced this type of reaction to IVIG?

I'm thinking maybe I need to try get my MCAS under better control before I try IVIG again? Does anyone have any thoughts or suggestions or been through anything similar? I'd be extremely grateful to hear if so. My doctor isn't totally against trying a different brand, as he saw that I rebounded in terms of the typical side effects reasonably quickly, and I didn't have an allergic/anaphylaxis reaction, but I don't think he gets the MCAS thing at all, and I'm now terrified of lowering my baseline further with more IVIG, as I'm in more pain than I've been in for a long time.

Thanks for reading if you got this far

D

 

[Gingergrrl]

I just wanted to update you on my first IVIG treatment. Unfortunately it did not go so well, but also not so bad.

Thank you for updating us and I had wondered if you started the IVIG and how it went.

I was premedicated with Tylenol, famotidine and cetirizine, and I drank buckets of water and electrolytes in the 3 days beforehand. I had 500ml IV saline pre-IVIG.

Was there a reason that your doctor had you take Cetirizine as your H1 blocker pre-med vs. something stronger like IV Benadryl (diphenhydramine)?

I was also curious, have you ever had a problem with getting that much fluid in an IV infusion before (even just plain IV saline)? I had problems with third spacing of fluid from MCAS (prior to ever trying IVIG) and it would have been physically impossible at my first IVIG infusion to get 500 ml of saline in addition to the IVIG. I realize that this might not pertain to you whatsoever but just wanted to mention it just in case.

The rate was upped to 35 ml/hr, and all these symptoms worsened, along with my abdomen starting to swell hugely!

The maximum speed that I could safely tolerate for IVIG was 40 ml per hour (although I usually stayed with 35 ml per hour). I am wondering for your first ever IVIG infusion if even this slow speed was too fast for your body to tolerate?

I was getting a bit out of it at this stage too from the fever, so asked for the infusion to be paused. We started again at the lowest rate, but all the symptoms returned. A doctor was called and gave me an IV shot of hydrocortisone.

You definitely did the right thing to pause the infusion! At any point did they give you IV Benadryl?

I had gained 1.5 kg in weight during this attempt at infusing (only about 5 g of IVIG at this stage). They did a bladder ultrasound which was normal.

Did they check if you had third spacing of fluid in your abdomen or lungs? Were you getting the IVIG in the hospital or at an outpatient infusion center?

We restarted after two hours, but again I only made it to the 35ml/hr rate before we had to abort again for recurrence of the symptoms. So overall I had 8g of IVIG. I had a burning achy spine, bad chills and generally felt acutely unwell that night. However it was manageable. My abdomen swelling went down completely in the next day or so. I didn't get a very bad headache the next day, although my IBS-type symptoms (which are usually constipation) was IBS-D instead, and I only managed to eat some toast for the two days after it.

I am so sorry to hear that you had such a bad experience. My very first infusion of IVIG was a nightmare (b/c they ignored my doctor's written order and infused it way too fast). He had written not to go above 35 ml per hour under any circumstances but they pushed it up to around 100 ml per hour (but I did not know). I had the most excruciating headache and intracranial pressure of my life and after 10 days, I went to the ER.

After that, my doctor made it clear to the infusion center that each infusion must take 8 hours and not go above 35 ml per hour and I never had that problem again (and I did IVIG for 2 yrs). I always got a headache from the IVIG, and this was unavoidable, but it was manageable. And it was never to the severity of that first IVIG headache (which I now suspect in hindsight was aseptic meningitis).

I now suspect this has all triggered an MCAS flare-up, although not super-typical maybe?

What are your normal MCAS symptoms like (prior to IVIG)? In my case, my MCAS went into remission from IVIG but we are all so different.

Does anyone have any thoughts or suggestions or been through anything similar?

Do you know what brand of IVIG you got? My MCAS specialist (at that time) felt that the brand that I would tolerate best was Gamunex. If I remember correctly, you are not in the US so I am not sure if Gamunex is an option (but if so, I would try to switch to it).

I would also try a slower infusion speed. Even if it sounds ridiculous, and your next infusion is only 10 or 15 ml per hour, if that is what you need, then that is okay!

Last, I would ask if you can have IV Benadryl as your pre-med and if needed, to get a 2nd infusion of IV Benadryl half-way through the infusion. This can tamp down the allergic symptoms before they even start. Everyone uses different pre-meds (and I actually used Atarax as my H1 blocker b/c it worked better for me). Sometimes getting the right pre-meds also takes a lot of trial and error.

Actually I have another thought... were you ever tested for the anti-phospholipid autoantibodies or blood clot risk (since IVIG is such a thick substance)?