New Member - POTS & SFN

[hopeforthecure]

Hello,

I have posted once here before I was officially diagnosed. I did a TTT last week and my heart rate went from 84 laying down to 144 standing up almost immediately. My blood pressure stayed consistent throughout the test. My heart rate did end up going down to about 120 after about 20 minutes into the test so I didn't stay at 140 so I did feel a little better after standing for a while. The dr had already had me on toprol 12.5mg twice a day for the tachycardia before this. The only reason he did the TTT was because I have sarcoidosis and my pulmonologist felt that this autoimmune disorder was causing my tachycardia. He felt that the POTS was being caused by SFN. So the TTT test proved that I had POTS. Just wondering how many of you have SFN and what you do for it. My pulmonologist for sarcoidosis (he is the leading one in the US so very good) does not want to treat the sarcoid and wants to stick with my treatment that the electrophysiologist has given for the POTS which is the toprol, extra salt, lots of fluids, compression stockings and get up slowly, drink before getting out of bed, etc. I do have tingling in my hands and feet. I am already on topamax as a preventative for migraines. I increased my dose of this and it has seemed to help the tingling so obviously the SFN is affecting my hands. Anyways, just wondering who here has SFN, do you treat it, what type of dr do you see for it? Do you think watching and waiting and just treating the POTS and SFN symptoms is all I need to do? I just want to make sure I'm not harming myself. I have 3 children that I need to be here for. I am able to function. I know that I am better off than many as I am able to work, and function and I'm so grateful for that. I just want to make sure that I don't just sit back and do nothing when this could become very serious.

Thanks!!!

[Sylvie33]

Dear Hope,

I have relatively mild sensory and severe autonomic SFN. I had an acute onset just a year ago, and I'm still being tested to see if I have an underlying autoimmune disorder.  I'm eager to find this out, because there are real treatment options, like IVIG infusions, which can halt the progression or actually improve SNF symptoms...ONLY if I have some sort of autoimmune disorder...like yours!  

I encourage you to see a rheumatologist or other specialist well-versed in sarcoidosis, SNF, and infusion therapies.  

This might not be easy....so try to read up on this (lots on the web) so you know what questions to ask.  See if you can talk to the Dr. or someone medically knowledgeable in the office before you make your appointment.  It took a year, but I finally found a really good neuro at the Peripheral Neuropathy Center at Weill Cornell in NYC who can put all of this together. (Feel free to PM me if you'd like his name).

Not to be an alarmist, but the time factor for effective treatment is very important and a "watch and wait" stance can possibly lead to irreversible worsening of symptoms.  

You are clearly motivated....I wish you the best.

Sylvie

 

 

 

 

[hopeforthecure]

Thank you for the advice. If I were to use one of these treatments, do you know if it's something that you have to stay on forever or it will come back or do you just stay on it for a certain amount of time and you are done and the SFN/dysfunction is gone?

Thanks!!!

[Sylvie33]

Hi Hope,

Among the people I know who've had IVIG (three only!), two receive infusions regularly.  All have experienced real improvement.  

I hope someone who's undergone this treatment posts here...as noted, I'm still being evaluated, so I can't speak for myself.  

I've pm'ed some additional information for you.

---S

[Sylvie33]

Dear Hope,

I just would like to offer the caveat that I have only a year's experience with this.  It would be best to seek advice from someone who has received infusions.

S

[hopeforthecure]

Thank you so much for everything Sylvie33!!!

 

[Sylvie33]

Dear HFTC,

I wish I could be more helpful -- but you're so welcome!

--S

[Justin30]

Hi,

Dr Oaklander at Harvard and one of her colleagues has been doing a lot on SFN and its role in FM, Dyautonomia, GWI.  

She has a couole of videos online but it her colleague (cant remember the name) thats shows dymylination of the small fibers that constrict around the blood vessels to push blood along....

I am to wiped out to search for the names and that stuff ATM...searching Oaklander should bring up stuff.

Like others have mentioned they meaning Dr Oaklander is using IVIG with success in SFN.

I hope you get everything sorted out and get some treatment.

[jvherenow]

Here's a recent paper by Oaklander. It's high science but helpful. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4720682/

It looks like when we're talking about small fiber neuropathy with dysautonomia, we're actually talking about small fiber POLYneuropathy...this is when the nerves that innervate the organs (like heart, GI tract, urological system) get effected. I quote from the above paper:

"The cardiovascular symptoms include not only orthostatic hypotension and tachycardia, but also impaired cognitive function, headache, and exercise intolerance due to circulatory insufficiency"

The really good news is that there's a chance for the nerves to regenerate with treatment: 

"Among the 11 early-onset patients treated at least 3 times with IVIg, 2 g/kg/month, 38 % did not respond and discontinued treatment, and 62 % had significant improvement and continued treatment [74]. Adverse events were as expected and largely manageable. Repeat skin biopsies and AFT provided objective confirmation of axonal regeneration."

[toomanyproblems]

I have SNF and POTS. Also a lot of other autoimmune conditions. I have CRPS that I take gabapentin for and that helps the SNF symptoms. I just had my first round of five IVIg infusions last week. I'm having some known adverse effects but can see improvement in some areas. Not my severe dysautonomia yet unfortunately but I'm hopeful with further treatments.

 

[Clb75]

This is very interesting, thanks for posting the link. I have CIDP, which causes large and small fiber damage. I noticed blood pooling in my feet after I first became sick, and my doctors kept telling me it's not related to neuropathy. I became sick with POTS a few years later, and was told by my neuro that autonomic issues are not common with CIDP, though my cardio said there is a subset of POTS  called neuropathic pots. Small fiber damage to the nerves that are connected to the blood vessels makes sense. 

I've been on ivig for almost 5 years. I've always maintained that it did nothing to prevent the onset of pots nor has it done anything to help improve my symptoms now that I have it. However, I am on only 1 kg/g per body weight. The people above were treated with 2kg. I wonder if that would make a difference in pots symptoms or at least the blood pooling? Very interesting to think about!

[toomanyproblems]

I'm on 2g/kg per month. Too early to tell but I guess I'll see. I don't have a lot of options left.

[Clb75]

Will you continue with 2kg for each infusion? They had me on 2kg for my first infusion where you get it  over 5 days. The next one and every one since I'm on 1kg for only one day a month. It works to keep the CIDP from progressing, but the numbness has never gone away totally. I notice some improvements after my infusion then I can feel it start to wear off and the numbness increases a bit. 

Are you tolerating it ok? Something that was helpful for me was to drink about 6 glasses of water a day 2 days before the infusion, as much as you can during the infusion and into the next day. You may already be doing a lot of fluids if you have pots. I found this helps a lot with the headaches. Also, keeping the infusion rate slow can help too. I'm at 120, it takes all day but if I go over it causes problems! Hope it helps you.