vxmike

On 3/22/2018 at 1:08 PM, p8d said:

@Clb75 may I enquirer what other autoimmune disease you get IVIG for?  Feel free to pm me if that’s better.  I certainly will update everyone.  I am hoping we can use this as ammunition to get my insurance company to pay for IVIG and just figure out what is going on.  I was diagnosed with MCTD after POTS and am post-viral onset.  My mom is an autoimmune disaster, 4 different diseases and that side of the family is crazy with autoimmune diseases.  I am pretty positive my POTS is autoimmune too.  My rheumatologist isn’t keen on IVIG but neuro and cardiologist are.  Have you seen this https://vimeo.com/243160944?  It’s from a Dysautonomia International conference recently.  She talks about her protocol and doses for refractory patients.  

I plan to get my blood drawn and sent off Monday although my neurologist said there are issues with shipping but didn’t give me details.  I *think* I have that sorted with FedEx.  

 

 

If one truly has autoimmune POTS antibodies I would *assume* some kind of immunosuppression or plasmapheresis would be a treatment, but thus far I've not read any papers or recommendations of such. It's such a new avenue for POTS.

I believe frequent plasma donation will actually accomplish the same thing as plasmapheresis on a lighter scale assuming the body doesn't regenerate the antibodies quicker than one can dump their antibodies via the donated plasma. I'd try this but losing volume from donation isn't exactly a viable option for a POTS patient! Too bad the donation centers won't replenish you with a couple liters of saline afterwards.

Hello everyone,

Now that we are aware of several studies suggesting that autoimmunity alpha and beta antibodies are often found in POTS patients that don't fall under the Hyper or primary neurogenic categories, I am curious if anyone here has tried plasmapheresis as a treatment to remove antibodies?

I'm fairly certain my etiology is autoimmune. I was on prednisone for almost 15 years for skin conditions but was otherwise perfectly healthy. After receiving proper Derm care and stopping the Pred my health has fallen apart. My POTS came on 2 years after stopping Prednisone. I have multiple conditions suggesting autoimmune disease:

POTS not Hyper or neurogenic

Sicca/Sjogrens syndrome - dry eyes/mouth resulting in corneal damage, lacrimal gland atrophy seen on CT

Likely IgG4 related disease with all relevant positive serum markers plus positive PET CT scan

I likely need systemic immunosuppressant therapy especially Rituximab, but I'm curious to try Plasmapheresis to see if clearing my antibodies will temporarily ameliorate my POTS. 

All my issues were not present or kept under control while on Prednisone. I'd merely start the Pred again but once I do that further investigation is impossible and Pred is a poor lifetime medication. Ultimately I likely need Rituximab or other steroid-sparing immune suppression.

Plasmapheresis anyone? 

On 1/2/2018 at 5:19 AM, bombsh3ll said:

I really like that way of putting it!

Do you find the midodrine helpful at all or would it be worth considering stopping it? - you mention that alone it didn't help.

I tried midodrine briefly - my BP went high, pulse went low and I was still presyncopal & couldn't stand  I don't feel it is for me as I am already very vasoconstricted as a natural response to hypovolaemia. 

Alternatively you could maybe ask Dr Grubb about a short acting antihypertensive for overnight. 

I did not find Midodrine helpful solo (just no effect at all) but I think it helps in combination with Ivabradine for me. I have no theories why.

50 minutes ago, bombsh3ll said:

Thanks vxmike that is really encouraging that you've had a good response, and also good to know Dr Grubb's opinion on it. I haven't ruled it out entirely but do want to address volume issues first - I have a blood volume test scheduled in 2 weeks. I also do not feel I have pooling as such, just general hypovolaemia secondary to not producing renin or aldosterone. My resting HR is also not high which is a concern for me re this drug - I found some studies in which a resting HR of below 60 or even 70 precluded use of the drug (granted these were for other conditions not POTS) but I would be a bit worried about my resting HR dropping too low, particularly whilst asleep.

Has this been an issue for anyone or has your doctor said anything about this?

Dr. Grubb didn't mention resting HR at all as an issue. In the few days I've been able to do some testing my supine heart rate has been around 55-65 with Ivabradine on board. Without it my supine rate was usually 60-70, so it's a fairly minor difference that doesn't worry me at all. My only concern is that I want to decrease my supine BP at night. My daytime BP is abnormally high due to Mididrine plus my body's natural attempts at compensatory vasoconstriction, and that's not a great thing for my heart long-term especially with the Mitral Prolapse that was found incidentally in my workups. I had great BP usually 100/60 before all this mess started two years ago. I'm a little worried to try something like Clonidine before bed if I already have Ivabradine in my system too. I don't have regular access to a POTS physician willing to be creative  (Dr Grubb visits are few and far between as I am not local and he immensely in demand), so I'm in the experimental camp personally...thus far I haven't found a suitable vasodilator that doesn't also reduce heart rate. 

Even if you have hypovolemia instead of blood pooling POTS I suspect the effect would be similar. Neither of our hearts are completely filling with blood even if the underlying reason is different. If my blood is in my legs and yours doesn't exist it might as well be the same when we're standing!

1 hour ago, Jessica_ said:

I am working with an EP but he doesn’t have any answers and referred me to the neurologist *SIGH* any ideas what causes hyper-pots? I’ve googled and googled and I can’t find any answers that fit what my body is doing. All my tests for “underlying causes” such as RA and adrenal issues come back normal. Could this all be my heart? So many questions! 

I think the cause of hyper-POTS is even more elusive than the other variants, unfortunately. 

What were your upright catecholamine values? These for the most part can diagnose or rule out hyper POTS.

On 12/24/2017 at 12:33 PM, green said:

I posted about pheneylephrine a few months ago.

It definitely works for me - the blood pressure elevating effects are stimulating. And it is a cheap OTC drug, so I am pleased.

 

What dose are you using? I just purchased a bunch of pheneylephrine but I'm not certain what a good dose for POTS might be.

On 12/10/2017 at 8:34 AM, bombsh3ll said:

I was recently offered ivabradine but decided against it because I personally feel in my case the tachycardia is a compensatory physiological response to low blood volume, and that suppressing it would not be helpful. I felt worse on beta blockers for the same reason.  What dose of fludrocortisone are you on? 

I think you ought to reconsider Ivabradine if your physician is offering it.

I recently saw the renowned Dr Grubb and  he started me on Ivabradine. His explanation is that at a certain the increased heart rate is inefficient and offers no benefit. The baroreceptors sense they don't have enough blood, so the body naturally increases HR. In our case, however, due to blood pooling there is no blood to actually pump. The heart is working like crazy (using tons of energy/oxygen) to rapidly pump dry. If you can lower the HR without decreasing BP (which only Ivabradine can do) then the heart will rest longer between beats and fill with more blood resulting in a higher output per beat. 

Im close to labeling Ivabradine a miracle drug for me. I'm using it with Midodrine and my standing HR went from 140-155 down to the 90-115 range within 48 hours, and I've had massive symptom reduction. Midodrine was ineffective solo. I'm certainly not "cured" and definitely still impaired, however I've improved at least 50%. 

Beta blockers decrease BP which negates the benefit from reducing HR. It probably slows the heart but causes even worse blood pooling. Ivabradine is a genius solution for some POTS patients that respond well.

On ‎12‎/‎31‎/‎2017 at 12:09 AM, Jessica_ said:

So I am looking over the Neurologists chart notes and I just do not know what any of it means. Since she refused to see me since I “don’t meet criteria for PoTS”. Anybody know what any of this means? 

 

Other Scores:

COMPASS-31 Domain Scores:

Orthostatic Intolerance: 9

Vasomotor: 0

Secretomotor: 4

Gastrointestinal: 13

Bladder 0

Pupillomotor: 5

Total COMPASS-31 Score: 57.85

(expected score in normal subjects 9.6 +/- SD 8.1; neurogenic autonomic failure 39.2 +/- SD 17, autonomic neuropathy 20.9 +/- SD 13)

Composite Autonomic Severity Score (CASS): Sudomotor 1 Cardiovagal 0 Adrenergic 0 Total 1

 

Autonomic Reflex Study:

 

Comments:

1. QSWEAT responses were normal by volume, though a relative reduction in distal (foot) was noted.

2. Heart rate responses to deep breathing was and the Valsalva maneuver were normal.

3. The blood pressure responses to the Valsalva maneuver were normal.

4. The blood pressure response to 10-minute head-up tilt was normal; heart rate response was excessive during mid-portion of HUT, though improved by minute 8.

5. Pupillary responses were intact.

 

Autonomic Diagnosis Codes

101 Orthostatic intolerance (OI)

 

Conclusion:

 

Cardiovagal, cardiovascular adrenergic and post-ganglionic sympathetic sudomotor responses were intact. There is no evidence of neurogenic autonomic failure or autonomic neuropathy affecting sudomotor or cardiovascular adrenergic/vagal function.

 

There was transient, non-sustained orthostatic tachycardia on head-up tilt, though this did not meet criteria for Postural Tachycardia Syndrome (PoTS) today due to resolution during course of Head-up tilt. The robust increase in norepinephrine noted on orthostatic catecholamine testing 7/19/17 suggests a tendency towards a mild hyperadrenergic response to standing. 

******

 

Should be noted I am on a low dose beta blocker and i am still struggling DAILY. 

My biggest symptoms right now are nightly palpitations, even if my HR is 70 its POUNDING out of my chest and it feels awful. Anxiety is the next. HELP!

Sounds like you don't have primary neurogenic POTS or autonomic failure. That's good!

You're on a beta blocker, so maybe that's why your tachycardia isnt testing positive on the TTT? 

The last part suggests you might have hyper-POTS. Probably should seek out a cardiologist who specializes in this. 

7 hours ago, m@t said:

I was similar and able to do 5k runs, cross country cycling for a number of hours each week for a long time.

 

Sure my heart rate raised on standing by 70bpm and Id feel a bit dizzy from time to time but it didn't really worry me too much. My resting HR was down in the 40s so a rise of 70 only took me to 110 but I was used to sitting at an average 155bpm for 3 hours when riding so 110 felt like nothing.

 

Then I think my body ran out of things to throw at it over the course of a few months and it wasnt actually the heart rate rise symptoms that took me to the docs. It was the other symptoms of wacky stuff going on with my pupils and suddenly being totally exhausted from mowing the lawn.

Me too. I was in excellent shape too before the sickness, and I did a 120 mile trek at 15,000+ elevation in Peru three months before I started getting dizzy just sitting at work or in the car. Looking back I was abnormally exhausted and my performance wasn't quite what I expected so I think my blood pooling was starting and the symptoms didn't really manifest as noticeable for a while.

On 12/26/2017 at 2:23 PM, kaevne said:

I’m on Midodrine and salt. Hr was at +29 in two TTTs.  BP unchanged so it was not OI and I was given a POTS diagnosis, which seemed consistent with my symptoms.

I think this explains it right here. +29 is right on the edge of a POTS diagnosis. Even if you do have POTS your HR is far lower than most people here who experience 40-80+ beat increases. I went from 68 to 148 on my last poor man's TT.  I'm not discounting your symptoms or condition at all but your HR increase is at the very low end of the POTS spectrum. 

I do think there could be something to the male/female differences, however. Anecdotely it seems to me women report greater degrees of disability from POTS. I'm also the rare male with POTS and even with my 70-80 typical increase I can still work full time and even do moderate hikes outside. Men have a higher cardiac output to begin with, larger blood volume, and a higher hematocrit on average so we have more tools in the body's arsenal to fight POTS. There may be other physiological differences in the various blood pressure, RAAS, and vasoconstrictive responses in the body between the two sexes. 

POTS is like 90%+ female so it's interesting to hear from other guys.

On 12/28/2017 at 12:55 AM, KiminOrlando said:

The way it was explained to me by my cardio is that tachycardia is usually compensating for low volume / pressure which is good until a certain point. He said there is a point the heart beats too fast for it to properly fill with blood before it beats again. This partial fill means decreased pressure, signalling a need for faster beats to compensate for the sudden decrease in pressure, which then causes the heart to fill even less, triggering even faster beats, again less blood in the heart during the 'fills', etc, starting a downward spiral. The result is syncope.

He said even though it seems counterintuitive, breaking that cycle is imperative in controlling POTS and Neurocardiogenic Syncope. 

This approach made sense to me. The beta blocker didn't solve my problem, but it was a helpful tool for me. I'm not sure if we are talking about the same issues or not. Different types of POTS may work differently.

Brain profusion is a different issue and one I haven't figured out for myself yet. We also took a look at my red blood cells. Mine were at the very lowest number in the 'normal' range in the U.S. My cardio wanted to put me on a drug that increased RBC because as he explained it, 'they are what the O2 rides on. You barely have enough, then due to low pressure, they don't go very fast, so demand is greater than your body can deliver.'  Insurance denied the med and it was too expensive to pay out of pocket with everything else I take, so we never found a solution.

If anybody figures this out, please let me know.

Hi Kim,

Thats exactly how Dr Grubb explained it to me as well. The increased heart rate simply becomes less efficient as it pumps faster without sufficient blood to pump. It's literally pumping a fraction of the normal blood volume each beat. By itself the cardiac muscle uses a lot of energy and oxygen itself, so I'm sure it contributes to fatigue and shortness of breath. 

I recently started on Ivabradine, and it's been a wonder drug so far. It slows the HR without the accompanying reduction in blood pressure that a beta blocker delivers. Essentially my heart has more time to fill up with blood between beats and isn't wasting energy pumping inefficiently. My standing HR went from 140-155 down to the 90-115 range in addition to strong symptom reduction.

I too want to try EPO to deliver more oxygen per unit of blood delivered to tissue. My hematocrit is 40% and I feel like a boost to 50% would aid me greatly. Unfortunately there is no way my insurance will pay for it in this case, and it's indeed quite expensive