2018 Dysautonomia International Conference: SFN, POTS, MCAS, Vagus Nerve Stimulation & CSF Leak

[Gingergrrl]

I searched if this was already posted and did not see it (moderators, please merge if there is already an existing thread)! These are the links to @Cort's two-part blog from the 2018 Dysautonomia International Conference in Nashville. Below the links are a few highlights (but there is much more and definitely worth reading)! I would LOVE to be able to attend this conference in the future.

https://www.healthrising.org/blog/2...omia-international-pots-sfn-mcas-vagus-nerve/

https://www.healthrising.org/blog/2...l-spinal-fluid-leak-pots-me-cfs-fibromyalgia/

The first speaker, Blair Grubb, MD, was perfect to kick off the conference. A dynamic and at time hilarious speaker with years of experience under his belt – Grubb participated in the first tilt-table studies ever – Grubb was full of hoary stories about the bad old days.

Grubb is professor of Medicine and Pediatrics at the University of Toledo. He authored the interesting “Quest for an Underlying Cause of POTS“, found on Dysautonomia International’s website (which features a hilarious slide) – and showed that in addition to about ten other conditions (diabetes, joint hypermobility syndrome, chemotherapy, amyloidosis, sarcoidosis, Lyme disease, Parkinson’s, MCAS), mitochondrial problems, traumatic brain injury and multiple sclerosis can all cause or be associated with POTS... Grubb noted how extraordinarily interconnected the ANS system is: you can’t change anything, he said, without changing everything

The destruction of the small nerve fibers in the skin (small fiber neuropathy or SFN) is a big deal in POTS and fibromyalgia research right now (and with Drs. Kaufman and Systrom reporting similar findings in their ME/CFS cohorts... SFN can also be caused by a wide variety of factors, but the good news is that if you can stop the nerve damaging process, those nerves can quickly regrow. The bad news is that it’s not easy to stop that process... If the small nerve fibers in the rest of the body (gut, blood vessels and more) are damaged – which Grubb and it seems everybody believes is happening in POTS, FM and ME/CFS – then watch out. SFN issues could conceivably account for almost every symptom and problem – from fatigue to problems standing to poor energy production – found in these diseases.

The big news in POTS has been the discovery of autoantibodies that are whacking a receptor (adrenergic-1 receptor) in the legs that causes the veins in our legs to narrow or vasconstrict as we stand. The knock on the past autoantibody POTS studies has been their size, but Grubb reported that a much larger antibody study (75 patients) found that no less that 92% of POTS patients had autoantibodies which were attacking that receptor, and other autoantibodies were elevated as well...

... Grubb said he’d suspected autoimmunity in POTS all along but the testing used to be too expensive. It’s still expensive, but a lot less than it used to be. The autoimmune finding looks like it’s going to redefine at least a very large subset of POTS patients and possibly the disease itself. Not only that, but it’s affecting other fields as well. Taking a clue from the POTS findings, Dr. Scheibenbogen found evidence of autoimmunity in about 40% of ME/CFS patients. Reports from the ME/CFS Montreal Conference suggest that a follow-up study from another lab is getting similar results.

The big question is treatment. The next generation of drugs will probably be biologics that affect the autoimmune processes in POTS... Mestinon has been a favorite drug of Dr. Systrom’s in both POTS and ME/CFS for quite some time... While Grubb hasn’t been particularly happy about the treatments available, he’s very high on Ivabradine... Grubb said Ivabradine has a 75% success rate: that’s unheard of in this complex condition..

Dr. David Kaufman was one of two ME/CFS practitioners that I saw at the conference... Dr. Kaufman said that antibody results and the multisystem effects in ME/CFS were causing him to lean towards an autoimmune cause. He was the first to approach Dr. Kem after Kem’s autoantibody talk on POTS. Kaufman said he was having good results with Rituximab in patients targeted using Dr. Scheibenbogen’s CellTrend antibody screen (which is different from the Mayo screen some doctors are using). His major new interest of the last couple of years, though, is mast cell activation syndrome (MCAS) – which, in his experience, is common in ME/CFS. Dr. Kaufman is also finding a very high incidence of small fiber neuropathy (@80%) in his ME/CFS patients.

Mass cell activation syndrome (MCAS) cropped up frequently in the conference presentations. It’s becoming a big deal in dysautonomia/POTS, etc. The big room was packed and afterwards Dr. Maitland was swarmed by patients asking for more... It’ll be interesting to see over the next five to ten years how all these disorders – MCAS, ME/CFS, EDS, IBS, POTS, FM – come together. Will they merge or split apart?

There is much more detail in the blogs including an additional section on Vagus Nerve Stimulation and CSF leaks. It is definitely worth reading!

 

[Gingergrrl]

I totally forgot I was going to tag a few people who might be interested in reading the blog: @Peyt @Forçe e Honra @Lalia @Malea @Learner1 @crypt0cu1t @MartinK

 

[Lalia]

Thank you for tagging @Gingergrrl, I can’t wait to read the full articles tonight. I wouldn’t be surprised at all if a big subset of CFS ends up being several yet-to-be classified autoimmune diseases.

Just got my recent blood results which show positive smooth muscle abs, so looking like autoimmune hepatitis. It doesn’t explain the severity or breadth of my symptoms tho, waiting to get results for autoimmune gastrointestinal dysmotility - really interested to see if those abs come back positive too.

 

[crypt0cu1t]

Thanks for tagging me @Gingergrrl I'm not sure if I have POTS, but I do believe a percentage of us have undiagnosed autoimmune issues.

I am in the process of getting high dose IVIG approved for my autoantibodies and Im awaiting another test for antiganglioside antibodies. so hopefully my insurance doesn't give me any issues with it..

 

[StarChild56]

@Gingergrrl - Thank you so much for tagging me! It will take me some time to read everything but wow.

Reading the part about SNF which I didn't really know was a big deal was - surprising. Especially since I am getting biopsied for it (soon, I hope). Woa.

The big news in POTS has been the discovery of autoantibodies that are whacking a receptor (adrenergic-1 receptor) in the legs that causes the veins in our legs to narrow or vasconstrict as we stand.
This is the only one I tested positive for on Cell Trend (the only other was "at risk" for M4). Wow

While Grubb hasn’t been particularly happy about the treatments available, he’s very high on Ivabradine... Grubb said Ivabradine has a 75% success rate: that’s unheard of in this complex condition..
I'm still waiting for insurance approval for this. I really, really hope it is approved soon.

 

[Gingergrrl]

I wouldn’t be surprised at all if a big subset of CFS ends up being several yet-to-be classified autoimmune diseases.

I still have no idea what ME/CFS is going to turn out to be (at the root cause, I mean) but I suspect it is going to involve many different sub-groups and at least one is going to be auto-immune (just my personal opinion).

Just got my recent blood results which show positive smooth muscle abs, so looking like autoimmune hepatitis.

Wow, @Lalia, what does that mean? What is the treatment for autoimmune hepatitis?

I am in the process of getting high dose IVIG approved for my autoantibodies and Im awaiting another test for antiganglioside antibodies. so hopefully my insurance doesn't give me any issues with it..

I would almost just be prepared that your insurance is going to give you a hard time and then if it doesn't, it will be a nice surprise!

Reading the part about SNF which I didn't really know was a big deal was - surprising. Especially since I am getting biopsied for it (soon, I hope). Woa.

I thought it was interesting that the blog said that Dr. K was finding SFN in about 80% of his patients. I was never tested for it but I don't really match with the symptoms and once we found that I had 11 autoantibodies, I think he felt it wouldn't add anything to keep testing b/c it would not change my treatment plan.

I did have a QSART test (as part of a bunch of autonomic testing) which showed neuropathy to the long branch nerves of the feet, and abnormal lack of sweating, but then absolutely nothing was proposed or recommended for it (this was from a Neuro and not Dr. K). I think the blog (or something else I read recently?) said that it didn't even recommend QSART testing vs. the biopsy that you are going to have. I'll have to find that quote again.

This is the only one I tested positive for on Cell Trend (the only other was "at risk" for M4). Wow

That is interesting. I tested positive for 7/9 Cell Trend (in 2016) and I will be re-doing the tests in Sept, but all eleven this time since there are now two new tests since I did them in 2016.

I'm still waiting for insurance approval for this. I really, really hope it is approved soon.

I hope you are approved soon (for Ivabradine) and am curious to hear how it goes.

 

[StarChild56]

I thought it was interesting that the blog said that Dr. K was finding SFN in about 80% of his patients. I was never tested for it but I don't really match with the symptoms and once we found that I had 11 autoantibodies, I think he felt it wouldn't add anything to keep testing b/c it would not change my treatment plan.

I did have a QSART test (as part of a bunch of autonomic testing) which showed neuropathy to the long branch nerves of the feet, and abnormal lack of sweating, but then absolutely nothing was proposed or recommended for it (this was from a Neuro and not Dr. K). I think the blog (or something else I read recently?) said that it didn't even recommend QSART testing vs. the biopsy that you are going to have. I'll have to find that quote again.

I ended up reading both links and found it very interesting, as well. Yes, it was recommended not to do the QSART.

That is interesting. I tested positive for 7/9 Cell Trend (in 2016) and I will be re-doing the tests in Sept, but all eleven this time since there are now two new tests since I did them in 2016.

It will be very interesting to see what the difference it now!

I hope you are approved soon (for Ivabradine) and am curious to hear how it goes.

Thank you so much. POTS sx are one of the most debilitating - as soon as I sit up, I feel it then standing up I am almost out of breath immediately, along with all the other yucky things (faint, dizzy, tingly feet, lead feet, shaky, etc.). It makes my life pretty impossible. I really, really hope it is approved and is not too expensive and it works. It seems effective in such a large percentage of patients I am hopeful.