Ivig For Small Fiber Neuropathy

[Tara Lynn]

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

Thanks

[spaceorca]

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.

[arizona girl]

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

[ajw4790]

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...

[ajw4790]

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.medscape.com/article/210367-overview

[arizona girl]

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.

[ajw4790]

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

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...

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!

[ramakentesh]

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.

[PotsMom]

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.

[arizona girl]

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.

[arizona girl]

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.

[Tara Lynn]

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

[PotsMom]

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.

[arizona girl]

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.

[dawson20]

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...

[juliegee]

Very interesting to hear of everyone's experiences. IVIG Therapy has been repeatedly recommended for me (by my allergist/immunologist) for an entirely different reason. My immune system is quite compromised. My IgG is consistently very low, around 500 (750-1,500 is normal.) So far I have resisted as I suspect I will "react" to the treatment negatively. Hearing all of the positive chatter makes me reconsider...

Thanks for sharing your experiences.

Julie

[PotsMom]

when my antibody titres came back negative (which they do 50% of the time I'm told) I was dx'd with 'seronegative AAG'.

Keely,

Thanks so much for sharing your very interesting story and I hope you will keep us updated in this thread as to your continued experience with the IVIG.

Do you know which antibody titres you had that you mentioned were negative?

Thanks again. Do keep us posted.

This is a very interesting thread...

[dawson20]

It was the Anticholinergic Autoantibodies - part of the Mayo clinics paraneoplastic profile - there was another name for it but i cant think of it offhand...

It is a specific antibody that is tested when AAG is suspected.

[arizona girl]

Oh my gosh, thank you so much for sharing your story. I do believe that they tested me for that gangliopathy and it was negative, so I thought that ruled it out. Apparently that is not the case. I'm so sorry to hear how severe yours got, before treatment worked. I'm so glad you are doing better, you give the rest of us hope.

I have symptoms also in all the areas you mentioned, just not as severe. I don't sweat, except nite sweats, I was getting fevers when I talk, they are not as high now, have had a colon rupture, digestion and motility issues, so far my BP/HR go up up up on standing, if I don't sit down or have an extra stressor like heat or motion I will get syncope, I have high levels of catechcolamines, which why I'm probably not hypotensive on standing. I have a lot of all over pain, my eyes, nose and girls stuff are very dry. My regular eye doc did the strip test and they were very dry, but this was before I found out about the neuropathy. Maybe I should have my eyes checked by optomologist, MD.

I have had slow improvements with the plasmapheresis. My supine HR is no longer in low 50's and hasn't gotten lower then 65. On standing it is now going higher then it was and feels more noticeable. My BP has also moved much lower, I was topping out as high as 170/140, now I'm mostly in the 130/90 range with a few spikes higher and a few below 100/for both systolic and dystolic. I also think I am eliminating more normally too and not getting full so fast. The pain levels have also decreased. I still feel unwell when I stand. The day I feel really good when I'm up right will be the day I know we have finally got over on this.

Thank you again, please keep letting us know how it goes.

[PotsMom]

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?

That's very interesting. My kids had the Q-SART testing. Son's results from that were very similar to results of your skin biopsy: "sweat production is decreased to less than 50% of minimum values at the distal leg indicative of length-dependent impairment of post-ganglionic, sympathetic-cholinergic nerve fibers." Daughter's Q-SART was normal except "the 'hung-up' pattern (persistent sweat production after the stimulus cessation) was observed at the foot which is observed in hyperalgesia secondary to neuropathies." Based on symptoms in her feet the neurologist in his report mentioned she has symptoms suggestive of small fiber neuropathy.

Based on these test results and our family history, the neuro recommended that they both have ganglionic acetyl choline receptor antibody testing. They did, and it was "normal" for both of them. However, regardless of the results of that test I am still convinced, due to their history and family history, that their dysautonomia has an autoimmune cause. I am also pretty convinced that the IVIG my daughter had helped to set things in motion for some improvement for her, and that too would seem to fit with the likely autoimmune cause. The more I think about this the more I think we should seriously thinkg about pursuing potential IVIG for my son.

[Tara Lynn]

Arizona girl,

I have a neurologist, cardiologist, endocrinologist, pcp. Currently - Im having my PCP direct medications/treatment plan at this point. Yes I also have autonomic dysfunction - POTS. Im not sure what the post gang involvment means or the importance of small fiber neuroppahty with this condition. I was suprised when I was told > 80% SFN w/post gang involvment...what does that mean?? I dont have the burning, pain or tingling in hands/feet only w/exstreme heat. Im so new at this.. Im not sure what causes what, what comes first?

[arizona girl]

Hi tara, While I'm not new to the illness, I too am new to the autoimmune small fiber autonomic neuropathy diagnoses. There are some other things that can cause SFN, I did test for as many of them as possible. The ANA did help us lean to an autoimmune antibody as the possible cause. So we are treating me under that protocol. I see from your meds that you are being treated for your symptoms with many of the meds that are common on this forum.

My next question, since I've had the chance to go before you, would be what other conditions have they tested you for and what have you ruled out or in? Next would be do you want to just treat symptoms or take a shot at treating cause? I had a bunch of blood tests to rule out a bunch of other rather scary causes. As I was already aware of these causes, it gave me peace of mind to know that I didn't have any of them. But, had I had one of them treatment protocol would have been first based on treating that primary condition that is causing the autonomic dysfunction, not just treating the autonomic symptoms.

I also am now trying to understand the gang and length involvement. I read a bit about it and the other post from the gal who was treated for AAG with IVIG. I am older then her and have not progressed to the severe dysfunction she has had. I'm grateful that mine appears to be a slow progression, but I can see that I am headed in the same direction, if I do nothing. I have some of the symptoms she has but not all of them. I do get syncope, but I'm usually hypertensive still on standing, which means my body is still able to compensate to some degree. Interesting that she had all the hallmarks for AAG and still tested seronegative, and then she responded to IVIG. BTW in your first post you asked about IVIG, have your docs suggested this to you?

As far as not having the burning symptoms of classic peripheral neuropathy, I did not really have that yet either when I was your age, it has only developed for me in recent years. Most of the studies on PN suggest that it starts at the fingers and toes and works up, then to autonomic dysfunction. In my case it is the exact opposite. I started having presyncope symptoms as a child. I bet if we did a poll people on the forum would see they had autonomic dysfunction long before any PN came on board. As for most of us here that is always the big questions what came first and what caused what? Even the researchers are still working on it, I don't know that there is an answer yet.

When you say you have autonomic symptoms, which ones do you have? This forum is such a wonderful resource to come and talk with each other and try and figure it out, I'm so glad you found us. Your story of symptoms, diagnosis and treatment will help the next person who comes looking. I look forward to hearing more about yours.

Arizona girl,

I have a neurologist, cardiologist, endocrinologist, pcp. Currently - Im having my PCP direct medications/treatment plan at this point. Yes I also have autonomic dysfunction - POTS. Im not sure what the post gang involvment means or the importance of small fiber neuroppahty with this condition. I was suprised when I was told > 80% SFN w/post gang involvment...what does that mean?? I dont have the burning, pain or tingling in hands/feet only w/exstreme heat. Im so new at this.. Im not sure what causes what, what comes first?

[Tara Lynn]

Arizona Girl,

Thank you for your response

When I was 25 years old, I went to bed one day healthy/normal and woke up unable to get out of bed. My resting heart rate was 90 - 100 but the moment I would sit, stand, talk, make any movement my heart rate would go up to 150 or higher. My blood pressure remained stable somewhat but I was exstremely light headed, dizzy and short of breath when I would stand - I also developed and continue to have vision difficulties, severe flushing w/eating, nausea, slow gut, insomnia, fatigue, weakness, swallowing difficulties, abdominal pain, light/noise sensativies, inability to regulate body temp, unable to concentrate (almost like brain fog), difficulties finding words or using the wrong words, so many symptoms to list. I was sent to multiple doctors (cardiologist, endocrinologist, GI) and they just looked at me with no idea what was going on.

They did an echo and stress test (which only last 3minutes because my heart rate went through the roof), electrical mapping of my heart (thinking it might be an abnormal pathway/arrthymia), adrenal function testing, GI emptying test, swallowing test, MRI of brain, CT of chest/abd). My GI emptying test - showed significant delay or gastroparesis and swallowing test showed dysphagia - other than that the work up was unremarkable besides the sky high heart rate w/movement. They just put me on BB, fluriinef, anxiety and insomnia meds and sent me home.

Over the last 10 years, I've had good days and bad ones. Last year I made a big mistake and had a hysterectomy and my symptoms doubled in severity. I finally switched MD's and was referred to UW autonomic testing center. They did the tilt table, QSART, breathing test. My tilt table confirmed POTS diagnosis and my QSART showed SFN w/post gang impairment (whatever that means . I started doing my own research because it seemed like these doctors dont have a clue, and read somewhere that SFN could be the cause of the autonomic dysfunction or POTS condition. I did have a positive ANA titer so I guess it could be an autoimmune related issue (??).

I feel helpless and hopeless at times I am 35 years old and cant even play sports w/my girls. I have to sit in a chair and watch from the side lines. I feel trapped in a body that doesnt belong to me.

I asked about IVIG - because if there is a cure for this, I want one All the meds I take - dont fix the problem, they just help me get through one day, so I can repeat the same thing the next day.

I have gotten so much out of this forum - all of the members here are incredible people facing daily struggles. For the first time since this started - I dont feel so alone and isolated. This disease or condition has taken away what it is to be normal, -just taking a shower makes me feel like I ran 20miles.

I am still learning a lot about this condition even though I have struggled w/it for 10 years, so bare w/me if I ask the wrong question or dont know what I'm talking about

I appreciate all the responses regarding this question - it has given me more information to go on. I see my MD on Weds and will see what she says. If I knew it would cure my POTS - I would pay anything or go in debit to obtain it.

Thanks again for everything I know I'm new here but being a part of this forum, has been a real blessing for me.

Thanks again,

Tara (sorry my response was so long)

[ramakentesh]

Just a note - autoimmune autonomic gangliopathies can include conditions where autoantibodies clog up or bind to a3 nicotinic receptors in the autonomic ganglia. There are also situations where the autoantibodies stimulate rather than clog up these receptors. Small Fiber neuropathy and AAG can occur independently - in AAG the receptors can be present but rendered non functional.