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Ivig For Small Fiber Neuropathy


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#1 Tara Lynn

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Posted 26 May 2010 - 10:15 PM

How many members have tried IVIG for small fiber neuropahty and was it successful?? What type of response did you notice if any?

Thanks

#2 spaceorca

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Posted 26 May 2010 - 10:35 PM

I am curious about this as well. I have pestered various doctors to try IVIg with my son--numerous medical reports suggest that it helps some patients. But the treatment is very expensive, and doctors have to complete extra paperwork to order it. That seems to make them reluctant, even when I've said we would pay for the treatment ourselves rather than seeking insurance reimbursement. I was never able to get a doctor to order it.

Our son's current neurologist said last year that he probably would have ordered IVIg if he had treated Dan earlier in the course of his disease, but that he didn't think it would be useful now: most reports suggest it is most useful when used within the first few years after the disease starts. I checked the medical literature and, sadly, it seemed that this might be true. So we have been focusing on other treatments instead. But I have always wondered what IVIg might do, so am curious to hear from others here.

#3 arizona girl

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Posted 26 May 2010 - 11:32 PM

Well my neuro did order this for me after he diagnosed small fiber neuropathy. My insurance united health care denied saying it was experimental for my form of neuropathy. I guess it is not yet FDA approved for small fiber. However all treatments for small fiber are experimental as treatment and research are still very young. Ivig is used for other forms like polyneuropathy. He believes these neuropathies are autoimmune based and seems to be treating small fiber with the same course of treatment as other neuropathies. IVIG is expensive about $30,000 per treatment.

After ins denial we started plasmapheresis which pulls the antibodies out of your blood. I had some mild improvements and things did change, my response confirmed an autoimmune cause, and he has now added in cellcept. If this does not work then he will probably go back at insurance for IVIG. If none of these treatments work then I will back to trying to treat symptoms and hope it doesn't get worse.

He said that it takes six months to a year for the nerves to regenerate once the antibodies are over come. From what I understand small fiber and c nerves can regenerate. So, I don't think with small fiber, length of time you've had it is an issue. Of course, the sooner any of these neuropathies are found and treated the better chance for recovery. Unfortunately, I think if it is autoimmune, once you stop treatment it will return. There is no cure yet.

I'm curious Tara have you been diagnosed with small fiber and has your doctor suggested treatment with IVIG? My best guess at getting a doctor to treat in this manner, is to try and find a neurologist that specializes in autoimmune neuropathies and treats with ivig, the centers that do skin biopsy for small fiber would also be another source. In phoenix, Phoenix neurological associates do all of this and have an informative webpage if you want to go look

#4 ajw4055

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Posted 26 May 2010 - 11:51 PM

Hi,

I thought I would jump in even though I do not know that much on the issue... I do have small fiber neuropathy and no dr. has ever mentioned to me ivIg as a treatment option. But, I have seen it here on the board and in research. I really have always had the feeling that it is used very little for dysautonomia. Likely due to the cost, not knowing enough about dysautonomia to really know that it would be a good choice of treatment, and also I though there was a high risk of relatively severe side effects from the treatment (or am I confused with something else?)?

I think that when it is used for someone with dysautonomia they will often have at least one other condition that helps them decide that it would be an appropriate treatment. So, if someone has another condition that also can be treated with ivIg, they are more likely to try it. It may not then actually be for only treating that persons dysautonomia. Not sure if that makes any sense???

But, from what I have seen and heard it seems to be one of the very last treatments they try and symptoms have to be severe enough for them to want to use it.

Also, there are many forms of dysautonomia and I think that ivIg would only be a possible treatment for a few of the types. Then you of course get into whose definitions of the types of dysautonomia you are talking about and what causes them etc.

I think that I know of only 2 or 3 people on the board that have tried it.

Also, there are likely many drs./hospitals/areas of the world that for various reasons would not want to or legally be able to use it for particular diagnoses. Larger hospitals may be the best places to find drs. comfortable enough with prescribing it.

That probably doesn't help, but thought I would throw my 2 cents in... :)

#5 ajw4055

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Posted 27 May 2010 - 12:08 AM

Ok, so I did decide to go back and look it up. As, far as the side effects I must be confusing it with something else because it does not sound like they are that serious very often with ivIg treatments. :)

Also, when I looked at what conditions they use it to treat... it mentions ACUTE Idiopathic Dysautonomia, CIDP, MG, Lupus, etc. When it is talking about neuropathies it looks like they mainly treat LARGE fiber neuropathy with ivIg, but small fiber not as much. It seems to be used more for demyelinating forms of neuropathy. So, for most of us it does not sound like it would be that helpful or less you had another condition that indicated its use, or you have a lot of signs of an autoimmune type disorder, but no clear diagnosis.

What do others know or think?

Forgot my link: http://emedicine.med...210367-overview

#6 arizona girl

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Posted 27 May 2010 - 12:13 AM

Yes, you are right aj, this treatment we are discussing is specifically for autoimmune small fiber neuropathy, not dysautonomia's in general. Dysautonomia is the umbrella name for all these orthostatic intolerances. If you say had EDS as your cause, then IVIG would be useless. These treatments are directed at pots patients who have confirmed autoimmunity, even small fiber neuropathy can have other causes then autoimmunity. IVIG is FDA approved for several forms of autoimmune neuropathy, small fiber is not one of them yet. It is however being used off label to treat it. I don't think we used the term dysautonomia while discussing this question, just small fiber.

If there were other IVIG pots patients on this board I'd love to talk with them.

#7 ajw4055

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Posted 27 May 2010 - 12:50 AM

Oh my, I am struggling to try to put my thoughts into words and then to type them tonight. :D

Yes, so you have been talking about ivIg in regards to small fiber neuropathy and not the umbrella term of dysautonomia. I think what I was trying to describe or talk about was dysautonomia that is caused by small fiber neuropathy. Because small fiber neuropathy can present in a multitude of ways and not always as dysautonomia. Because this is a board for dysautonomia, I guess I was just trying to clarify or look at it purely from a dysautonomia viewpoint.

As far as discussing it in terms of autoimmune neuropathy, I guess I was just not assuming that the original poster was discussing purely autoimmune neuropathy. I did see that you were discussing it in regards to that though.

The thing I am still not sure about is that there must be a reason that ivIg is used mainly in large myelinated fiber neuropathies and not as often in PURE small fiber neuropathies. It would be different though if you had large and small fiber involvement due to an autoimmune cause.

I keep confusing myself tonight, so I am not sure I make sense anymore... :D

I think though what I am trying to say is that I am not sure how much it would be used for someone who has purely small fiber neuropathy and is presenting with no other identifiable conditions than dysautonomia. If they also have an autoimmune disorder that ivIg would be indicated as a treatment option, that would be different. You can be determined to have autoimmune small fiber neuropathy by a blood test and not necessarily have any obvious signs of an autoimmune disorder.

Not sure if that makes any more sense? I think I am giving up on my brain tonight. It has decided to not cooperate! Uggh! :)

#8 ramakentesh

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Posted 27 May 2010 - 05:34 AM

Yes, you are right aj, this treatment we are discussing is specifically for autoimmune small fiber neuropathy, not dysautonomia's in general. Dysautonomia is the umbrella name for all these orthostatic intolerances. If you say had EDS as your cause, then IVIG would be useless. These treatments are directed at pots patients who have confirmed autoimmunity, even small fiber neuropathy can have other causes then autoimmunity.


Autoimmunity is implicated in more forms of orthostatic intolerance than just small fiber neuropathy. IVIG may assist in any autoimmune disorder and is sometimes used experimentally to assess a possible autoimmune basis.

#9 PotsMom

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Posted 27 May 2010 - 08:50 AM

I will add our experience to this thread. When my teen daughter was quite severe with POTS four-plus years ago, she was put in the hospital for a few days for observation and one of the things they tried while she was there was some IVIG infusions. We were told that the benefits, if any, would not necessarily be immediate, but it was worth a try for anything at that point as she was completely debilitated at 13. We will never be able to say for sure if the IVIG had impact, but interestingly about two months after the infusion she began to show the first flickering signs that things were changing. She had been down for almost exactly a year at that point. She was miserable and unable to do anything. I will never forget the day I saw her get off the couch and sit on the floor and play with the dogs for about five to 10 minutes. That may sound ridiculous, but I was ecstatic. Though seemingly miniscule, it was a major change relatively speaking. For a year she had been horizontal on the couch and was in agony when we tried to force her to walk around a bit, etc. Now for the first time she was voluntarily sitting on the floor doing an activity. That was the very tiny beginning of her improvement. She has always told me that she felt like something switched in her body at that point and that it was ready to begin recovery. It was very, very slow progress over a very long period of time, but that was the marker that we always look back to as to when things started to change. Did the IVIG have something to do with that? I don't know, but you really have to wonder and she has always thought that it did. There have certainly been other factors that have impacted her progress over time as well, but I will always think that the IVIG had something to do with setting things in motion, though we of course cannot know that for sure. She is still not completely recovered but she is night and day now from where she started. I have often wondered if we should give it a try with my son, 16, who is still struggling greatly with dysautonomia and has not had the progress that she has had.

We have extensive autoimmune issues on the maternal side of my family including myself. I am absolutely convinced that the dysautonomia with my kids is tied into that. If IVIG is helpful with some autoimmune issues, then that would be additional support that the IVIG likely did in fact have an impact on my daughter.

#10 arizona girl

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Posted 27 May 2010 - 12:17 PM

Thank you rama! You always put things so clearly and to the point. :)

I did have a positive ANA for years. My neuro also tested me for all the other known auto antibodies so far other than the ana I am not positive for the other known ones. He tested me for a bunch of other stuff too, all normal. He then called it idiopathic, which is how most of the small fibers are classified right now.

But you are right while he couldn't use IVIG because insurance denied, he ordered the plasmapheresis as a diagnostic tool as well as a treatment. When I responded as he expected, it confirmed his leanings toward autoimmune cause. Whether this will work long term, jury still out as far as I'm concerned.

AJ, even according to the article you posted IVIG is used to treat many conditions that are not large fiber nerve based. Many of the neuropathies like CIPD involve both small and large. IVIG for autoimmune small fiber isn't FDA approved yet, doesn't mean it won't be. As it is used off label with success, it probably will be approved. My neurologist confirmed to me that all treatments for autoimmune based small fiber are experimental, as diagnosis and treatment are still very early for this nerve disorder. The skin biopsy itself is only about ten years old. As more people get diagnosed and treated, treatment course will change. This is probably the main reason you aren't seeing it associated with small fiber more often, it is still in the research stages.

I am getting treated at a large hospital in phoenix. I am just lucky that there is a neurologist that specializes in autoimmune neuropathies in the phoenix area. Dysautonomia is such a general term to classify symptoms, there are so many causes and many people come here to see where they fit in all this. This dysautonomia forum is very broad. I think most of us pick and choose which posts apply to our underlying cause. I only post about what relates to me. I wouldn't know what to suggest to an EDS or anemia person, but they and many others post here too.

#11 arizona girl

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Posted 27 May 2010 - 12:42 PM

Thank you so much for posting this. I have yet to fight for this treatment, we are trying other things first. From what you are saying here though it seems it might really help. How she responded is very similar to how my neuro said it would work. He was going to do two ivig back to back days and then once a month for 3 months. He would continue if symptoms improved and revisit IVIG if they returned. He said that we would start to see results by the second month. Which sounds like what happened with your daughter. You know there is a familial dysautonomia, have you looked into that? Your right it is now being used with a lot of autoimmune diseases. It is nice to know that this could be an option for me, thank you for sharing your experience, you are the first one that has posted about using it. Do you mind if I ask which autoimmune conditions your family deals with?

I will add our experience to this thread. When my teen daughter was quite severe with POTS four-plus years ago, she was put in the hospital for a few days for observation and one of the things they tried while she was there was some IVIG infusions. We were told that the benefits, if any, would not necessarily be immediate, but it was worth a try for anything at that point as she was completely debilitated at 13. We will never be able to say for sure if the IVIG had impact, but interestingly about two months after the infusion she began to show the first flickering signs that things were changing. She had been down for almost exactly a year at that point. She was miserable and unable to do anything. I will never forget the day I saw her get off the couch and sit on the floor and play with the dogs for about five to 10 minutes. That may sound ridiculous, but I was ecstatic. Though seemingly miniscule, it was a major change relatively speaking. For a year she had been horizontal on the couch and was in agony when we tried to force her to walk around a bit, etc. Now for the first time she was voluntarily sitting on the floor doing an activity. That was the very tiny beginning of her improvement. She has always told me that she felt like something switched in her body at that point and that it was ready to begin recovery. It was very, very slow progress over a very long period of time, but that was the marker that we always look back to as to when things started to change. Did the IVIG have something to do with that? I don't know, but you really have to wonder and she has always thought that it did. There have certainly been other factors that have impacted her progress over time as well, but I will always think that the IVIG had something to do with setting things in motion, though we of course cannot know that for sure. She is still not completely recovered but she is night and day now from where she started. I have often wondered if we should give it a try with my son, 16, who is still struggling greatly with dysautonomia and has not had the progress that she has had.

We have extensive autoimmune issues on the maternal side of my family including myself. I am absolutely convinced that the dysautonomia with my kids is tied into that. If IVIG is helpful with some autoimmune issues, then that would be additional support that the IVIG likely did in fact have an impact on my daughter.



#12 Tara Lynn

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Posted 27 May 2010 - 06:08 PM

Thanks for all your input. My QSART showed > 80% small fiber neuropathy indicating postgang involvement. Im not sure if the SFN is the cause of POTS or as a result. Do all POTS patients have SFN or just some? I also had a positive ANA titer - but was told just because you test positive doesnt indicate anything. It depends on the level (??) to indicate anything important. Im going back to my physician in a couple of weeks and wasnt sure if it was worth bringing up.

Tara

#13 PotsMom

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Posted 27 May 2010 - 07:02 PM

arizona girl, That's interesting that the timeframe we saw is similar to what your neuro had said. The doctor who ordered this for my daughter had talked about a similar timeframe, but he felt it wasn't worth doing more of it as the changes that we saw were so small and thus not conclusively as due to the IVIG. However, in retrospect I wonder if some additional treatments at intervals similar to what your neuro has recommended would not have been very helpful. This discussion is making me rethink looking into this possibility for my son. Both son and daughter have had testing that indicated they have a "length-dependent neuropathy" with signs of small fiber neuropathy. Very interesting in light of this discussion.

As for autoimmune in my family, severe Sjogrens x 1 and very likely x two more, arthritis, Hashimoto's, Parkinson's disease, colitis, and some very highly elevated autoimmune panels. There is also severe fibromyalgia and chronic fatigue syndrome which I am not sure are technically autoimmune but I have found it very interesting in talking with others that it is not uncommon for patients with dysautonomia to have these in their family history.

#14 arizona girl

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Posted 02 June 2010 - 07:06 PM

Hi tara, sorry missed your question. In my case I do think that an autoimmune antibody is attacking my small fiber autonomic nerves and thus causing the symptoms of dysautonmia which are symptoms of autonomic dysfunction in my case.

A positive ANA at the very least is an indication that there is some sort of autoimmunity going on. I had a very high ANA for years, diagnosed by a rheumy. I was told back then that it was an indicator and as you that it wasn't specific enough, to know what it meant. I think that they are now finding that a lot of people with the neuropathies also have a positive ANA, so maybe it is a more specific indicator then was thought in the past, and maybe more likely to be associated with neurological autoimmune conditions then rheumatic ones.

80% SFN is pretty high, do you have autonomic dysfunction as well as the burning neuropathy in the arms and legs? Are you being tested and treated by a neuro or a cardio? Do you know what they meant by the postgang involvement? I think it is worth bringing up. If you want to read up or get a referral to a doctor in your area, perhaps you can take a look at my docs website: "phoenix neurological associates". He might be able to suggest someone in your area that deals with autoimmune neuropathies.

Potsmom, I still don't completely understand the length dependent differences in neuropathy. My biopsy results were: "abnormal nerve fiber density at the distal site with normal findings at the proximal site. This pattern is consistent with a length-dependent neuropathy affecting small nerve fibers." I have biopsy sample pictures too! So, it seems your kids and I have the same kind of findings. Did they do skin biopsy or something else to determine this?

Also a while back I posted all the tests that Dr. Levine ran on me to rule out other causes, if you want to see what he tested for.

#15 dawson20

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Posted 02 June 2010 - 08:14 PM

Just throwing my experience out there...

I was diagnosed with POTS initially in '03, then NCS, then 'progressive dysautonomia' and later 'progressive severe autonomic failure'.... all basically a description and not a true dx... all they could do was say I was broken, and getting worse.

Last summer I was dx'd in Hamilton ON with Autoimmune Autonomic Ganglionopathy (clincally - by last summer I had all the hallmarks - severe orthostatic intolerace in spite of massive doses of Florinef, midodrine, NaCL, and beta blockers, severe global gut dysmotility (very nearly wound up on TPN), hypohydrosis (didnt sweat), and pupil/optic nerve dysfunction) - when my antibody titres came back negative (which they do 50% of the time I'm told) I was dx'd with 'seronegative AAG'.

That was March of this year.

At that point I was waiting to see the Hemo department for assesment as far as a trial of IVIG therapy as the handful of case studies out there suggest that seronegative AAG patients who fit the clinical profile well (dysmotility, OH, pupillary dysfunction, hypohydrosis) do indeed respond to IVIG and immunotherapy.


Come April the motility stuff hit a crisis point and I was admitted for 3 weeks for malnutrition/hypoglycemia and they started talking about TPN and small bowel feeding tubes etc.

Everyone agreed we should trial the high dose IVIG first on the off chance it did actually work.


It worked.


within 10 days my blood pressure did not drop AT ALL when standing (this was mind blowing - prior to this, for the past 7 years, I couldnt stand without it dropping significantly, and for the past year, it has consistently always dropped to 60's and 70's systolic... by 10 days post IVIG my systolic BP was RISING when I stood....)

My motility picked up, my pupils started responding appropriately, and my heart rate stopped spiking when I stood.

I even ended up dropping the dosage on my florinef and beta blocker as my BP was getting a bit too HIGH...

It was phenomenal. The improvement continued over the following month. every week I was a bit stronger, my numbers were a bit better, my medication doses were a bit lower.

Once I finished the first round of high dose daily therapy for 5 days, they dropped me to the 'maitenance' dose every 3 weeks and unfortunately as of this week I officially am 'relapsing' as my pressures are not as good, heart rate is higher, and other symptoms are returning - they have started me on a high dose daily regimen again this week, and I will be put on a higher/more frequent maitenance dose from here out.

The plan for now is to continue this maitenance until October and reasses then.


IVIG is not necessarily approved for but is used frequently off-lable for many autoimmune neuropathies - some very obscure and unheard of (like AAG)...
Immune therapies of this nature (immunosupressants, plasmaphoresis, IVIG) have been reccomended for me various times by various doctors over the past decade - this is the first time we have actually taken that step and trialled one though.

I am in Canada however so I know the process of getting approval/coverage is different and easier.


Just my experience...