Ivig Experience - Post Viral Pots/autonomic Neuropathy

[teagirl]

Hello to all,

After a very bad flu virus in August I struggled with acute onset of POTS (although nobody called it that at the time). I was diagnosed in November by Mayo Clinic with "post infectious autonomic neuropathy manifesting as POTS". My tests also showed reduction in postganglionic sympathetic sudomotor function with mild cardiovascular adrenergic impairment and postural tachycardia on head tilt-up (TTT). My hematology, chemistry, endocrine, and immunology test results were all within normal limits, with the exception of slightly low potassium. Worth noting my ANA antibodies measured positive at 0.4, although within normal reference range of <1.1 according to the results.

I'm still trying to wrap my head around all this. Mean time my symptoms are full blown orthostatic intolerance (can't be on feet for more than a few minutes), nausea, insomnia, and erratic blood pressure/pulse even in sitting position. I also get bad shakes and tremors sometimes, especially if coming off a bad night's sleep. I am on .05 mg fludrocortisone, between 5-10 mg midodrine 3x per day, and compression stockings, plus increased salt intake.

Mayo suggested IVIG with the caveat that it might not do anything, but worth a try. I just completed 5 consecutive days of Gamunex-C (10% / 22gm / 220 ml). (Side note - unlikely that insurance will cover this (!!)...but still appealing). Side effects were mostly bad headaches and fatigue, but that's improving now that I am 7 days clear of the last infusion.

So far I don't feel any improvement as a result of IVIG. I can't tell if this is because it's too early, or simply because my main problem is not auto-immune related. Anyone have any thoughts on this based on my profile? Obviously I'll follow up with Mayo in a few weeks but wanted to see if anyone has been down this path.

In the mean time my husband and I are also researching means to help with autonomic neuropathy. As many have pointed out in this forum, there are supplements like Alpha Lipoic Acid, Benfotiamine, and Acetyl-L Carnitine that have shown promise in studies. So we're looking to work one or more of those in depending on what Mayo advises in our followup.

I know this is a rambling post but I'd be interested in viewpoints on the auto-immune angle, in light of my results above, and likewise any other suggestions or words of wisdom. Like many, I am struggling both physically and emotionally but want to be as proactive as possible with my treatment plan.

Best to all.

Teagirl

[arizona girl]

Hi teagirl,

No worries about a rambling post. We've all done that. I've been on a different brand of IVIG for a couple of years now. I have autonomic small fiber neuropathy documented by skin biopsy, I also had very abnormal ttt and am hyperandrenergic with a significant rise in norepi on standing. Like you my body was on a roller coaster ride. I also have 3 autoimmune diseases that didn't completely show by antibody testing. Biopsies proved two of them and blood test antibodies for hashimoto were finally caught. I have had a known high ANA for years. I qualified for IVIG when we found out my total quantitative immunoglobulins IGG and IGM were low. Just wanted you to have a point of reference about my path. I hope it works out with your insurance. If you tested positive for any of Mayo's dysautonomia antibodies that is a covered diagnostic code. The small fiber autonomic neuropathy isn't always covered, but it is being used off label. If you have a polyneuropathy diagnostic code it might be covered that way. I think Mayo will try and work with you and the insurance company to get it covered. It is very expensive without insurance. The insurance is also billed for way more then they actually pay. The going insurance rate that is paid is $4-5 thousand per monthly infusion lower if you have less infused.

Sorry about the headaches and fatigue they do happen, but there are things that can be done to hold them off. From my experience the first infusion course didn't work, we actually had to switch to privigen after 4 months my side effects were so bad on carimune.

It took probably another four months on privigen before I started noticing improvements and I have had slow improvement since then. After we added in plaquinel and cytomel for autoimmune complex, I had further improvement. I'm not cured by any means and withdrawal of any part of my treatment plan triggers a flare up. For me for now at least we are managing it. I still don't travel well and can't do as much as I would like, but I'm better then I was.

It has been documented that many autoimmune disease trigger autonomic neuropathy, so it certainly can be a consideration. There must have been enough cause with you for Mayo to offer it to you. Many never get that opportunity even try it. I've never tried any of the sups so I can't speak if they help or not. I'd go with the guidance of my doctors on that one.

I remember how difficult the diagnostic process was and am so grateful to be on the other side of it. It still takes strength though to manage the specialists.

Since it is probably to soon to know if the ivig is beneficial for you. Have you taken a closer look at the other meds, since starting those. Though they work well for some, others get worse and they can't stay on them. Did you notice an improvement on those meds?

Know you are not alone, there are many here that understand what you are going through. We eventually learn a new normal and find ways to not let the illness rob all our Joy. Take care!

[looneymom]

Hi Teagirl,

Did they check you for any autoimmune antibodies? My son was diagnosied with POTS 3 years ago but has not responded to medications for POTS. After illnesses with infections, his body goes down hill. Recently, a test has showed autoantibodies that are high in the brain. These are the antibodies that have been found in PANDAS children. My son is going to be seen by an immunologist this coming Friday. Other testing that has been done indicates this is something immune related. My son also has a low IgA. I was wondering if you had a headache before you started the IVIG treatment. If so, did the IVIG treament make it worse before it got better? I am trying to prepare myself for the worse. Our cardiologist feels certain that my son is probably going to be treated with IVIG or plasma exchange. Please keep posting and hopefully everything will indeed get better for you.

Rachel

[E Soskis]

I started with IVIG (privigen) and it worked almost immediately - over 2 years the benefits began to dwindle until there were more side effects than benefits. I then switched to plasma exchange and have been on that for almost 3 years. I have a permanent necklace graft and go for exchanges anywhere between 3 weeks to 8 weeks - just depends upon how bad my symptoms get and how quickly I deteriorate. I remember the loading dose of IVIG was very rough - severe headaches, aseptic meningitis, fevers, very low blood pressure, nausea and vomiting - I was quite ill. Yet, I also remember it was the only treatment at that time that provided any sort of relief. I was near death when I was started on IVIG so, I figured I would have severe symptoms just because my own condition was so unstable. I have autoimmune disease so, produce "bad" antibodies. I don' know how IVIG would work on anything but autoimmune antibodies except maybe in those who don't have enough antibodies of their own. I hope you get your insurance to assist with the cost - my loading dose consisted of 2 IV bags that ran over 12 hours each bag - 20,000 dollars a bag......

[kim5204]

I have been having ivig since October and will do it for 6 months, I keep catching infections so cant tell if the ivig is helping yet. Havi ng j tube changed this week thinking that what was making se sicker but who knows now. Goodluck.

[teagirl]

Thanks everyone for the helpful replies! Today marks day 8 since my IVIG round was completed. I am just flat out fatigued, and while still early, I haven't seen any noticeable improvements. I'll be patient though. Today I see my Primary doc and I will ask for a CBC blood panel to check on my red blood cells and platelets post IVIG, as I want to see if there was any hemolytic anemia impact (which occasionally can happen). I also notice a mild skin rash on my face and neck that is a new development.

To answer some of the questions:

My ANA results (test for auto immune antibodies) registered at 0.4 when tested last month. That is within the normal range according to Mayo (<=.12), and as such they did not note any abnormal auto-immune features in my write-ups.

I had headaches during the infusions (common side effect) but those seem to have subsided. Ibuprofen helped me deal with that. I also had some itching sensations (again, common side effect) and Benadryl helped me deal with that during the infusions.

I'll keep monitoring things over the next week or 2 to see if I show improvements as a result of the IVIG. If nothing else it was worth a try...and perhaps it will help rule out any auto-immune drivers for the POTS. (After all...if auto-immune related, I should benefit from IVIG, right?)

Follow-up with Mayo coming in 2 weeks to sort all this out. Will report back then, if not sooner.

BTW - still working w/ insurance and Mayo, but good chance this comes out of my pocket. I actually had to pre-pay it since it was time critical (post viral). Total price tag for my treatment as described = $15,000. I only offer this as a reference point for anyone who may benefit from the knowledge.

Also, I hope nobody gets discouraged on IVIG if it so happens my results are not stellar. The nurse mentioned countless examples of people who have shown immediate/dramatic improvement.

Best to all.

teagirl

[looneymom]

Hoping for the best. Sometimes it takes more than one treatment for this to work. Keep us posted.

Rachel

[kitt]

Am just wondering if anyone in the U.S. has had insurance pay for IVIG?

Thanks for sharing,

K

[ramakentesh]

I havent tried IVIG but do have small fiber autonomic neuropathy. its hard to get in Australia.

[ramakentesh]

In most autoimmune diseases there are usually a percentage of patients that are 'seronegative' - that is having the disease but without measurable antibodies.

The specific autoantibodies that cause autoimmune small fiber autonomic POTS (neuropathic POTS with patchy abnormal QSART) havent been identified other than in the gangliopathy (and these autoantibodies werent found by other researchers in any POTS patients). Recent studies in POTS also identified autoantibodies to specific alpha 1 adrenoreceptors and other strange locations. In some conditions small fiber neuropathy was caused by inflammation from the cytokine TNF alpha rather than antibodies.

What's the point of my post? Well QSART or skin biospy abnormalities with an acute onset may be indicative of autoimmune POTS even where no autoantibodies are identified.

[looneymom]

I wish that MAYO and some of these other medical centers would start looking at other antibodies that could affect the nervous system. Such as the antibodies found in PANDAS. If a child's immune system does not mature and these antibodies hid in the cells. Then the cells keep replicating themselves and the antibodies. If this is not caught during childhood, I would think by adulthood this would be mess.

I know the antibodies that were found in my son are not what they would look for in a POTS patient. However, from what I am understanding these antibodies do affect the nervous system. Currently, I am waiting on another test that shows elevated antibodies that have affected his heart. This is a test being done in the PANDAS study. If anyone suffers from vocal or motor tics, I highly recommend the Cunningham Panel. This test is not just for children. These antibodies have showed up in adults.

Since these antibodies are affecting Tyler's blood pressure and heart. Our cardiologist has started talking to our insurance company about IVIG and plasma exchange.

Rachel