Tilt Table And More This Afternoon, We'll See What The Doc Says

[peregrine]

Had my first TTT, QSART, and other things (breathing out hard through a spirometer-type thing, rhythmic breathing with 5sec in/5sec out) test today at the University of Washington. All told it was a 2.5-hour process but I had the nicest technician in the world. Other than the TTT everything wasn't too surprising, but the TTT - ah, goodness, I am so glad I have read what folks here have had to say about them. Here's my symptomology during:

0 minutes: tilted, started to feel like I would fall forward. Got a bit spacey.

2 minutes: lower legs started having an electric shock feeling (I associate this with blood pooling)

4 minutes: lower legs feeling really heavy, definitely pooling (she said they looked dark pink/purplish)

5 minutes: begin breathing rapidly due to shortness of breath

6 minutes: tech asks if I can hold on for a few more minutes to get data. She seems concerned. I nod!

7 minutes: begin sweating heavily all over (one of my two main "tells" for presyncope; the other one, nausea didn't show, I'm guessing because they had me not eat for 2 hours prior and by the time we did the TTT my stomach was empty and hungry). still SOB and almost hyperventilating despite attempted slowdowns.

8 minutes: tilted back to lying flat.

12 minutes: stop breathing shuddering gasps, begin breathing normally

15 minutes: heart rate back to normal, still have overall shivers/tremor

She said throughout the entire process my HR and BP were all over the map; I knew that about the HR since I could feel it in the finger BP cuff and (hilariously) see the reflection of her EKG monitor in the paper towel dispenser. At one point the BP apparently got down to something around 75/68, which is super-weird (gotta love narrowing). I ran into my ANS doctor on my way out - she was in scrubs! I knew she was involved with this testing center but not that she was running around it in scrubs. She said hi, "see you tomorrow," and asked how things went; I explained the 8-minute failure (or success, hard to say when you expect/hope to fail since failure = data = help with diagnosis and treatment) and she was a bit dismayed ("but I've seen shorter, yours is just shorter than I expected"). We'll see what she says tomorrow.

I felt well enough after (despite some serious fatigue and tremor) to go back to work and do some light data analysis (I'm in grad school, so work is super-flexible and the hospital is right across the street from my office), but not well enough to do anything heavy, and I just crashed energy-wise an hour ago.

Also, QSART was painful-ish for me (similar sensation to a physical therapy electrostim but more painful and sharper), but - oh man - they use acetylcholine! So neat! One of my advisors and I squeed about it for a while, since he's a neurobiologist. We'll see what my ANS doc thinks tomorrow afternoon. Oy. But hey, at least we know I am pooling!

[Chaos]

So exciting to fail a test isn't it? LOL Hopefully the ANS doc will be able to give you some answers tomorrow.

Keep us posted!!

[peregrine]

And here's the update. We had a long chat. Here's what she wrote in the test results (I got a copy of all of the graphs!):

"Abnormal autonomic testing. POTS with venous pooling. Normal heart rate variability at rest, with deep breathing [the metronomic test] and Valsalva. Mildly abnormal sympathetic vasomotor dysfunction evidenced by excessive BP oscillations with head-up tilt. Borderline normal bilateral induced sweat responses. This study provides evidence for significant POTS with witnessed venous pooling as well as increased BP oscillations. The study demonstrates intact baroreflex mediated HR variability suggesting normal cholinergic (parasympathetic) function. There is evidence for mildly decreased adrenergic (sympathetic) vasomotor function with head-up tilt. Bilateral postganglionic sudomotor function [QSART] was borderline normal in a patchy pattern. Partial Ross syndrome would be in the differential."

To interpret that with what she said... basically, I have a weird type of POTS - basically hyperadrenergic and pooling together. We're going to do some labwork - urinalysis (24-hour urine sodium, urine protein via various methods) as well as bloodwork (anti-SSA/Ro to rule out Sjogren's, HB A1C to rule out diabetes, HIV just in case since it can cause some of these symptoms, protein electrophoresis and immunofixation to look at serum proteins, and vitamin B12 to rule out deficiency). There are more tests we could do to look at more things, but they're part of the Mayo panels and thus insanely expensive, so we're saving those for in case these all come back normal. She says there's likely some small fiber neuropathy going on (which I guess would explain the pooling, which was very noticeable on the tilt test). Regarding the GI tract symptoms I explained, she thinks gastroparesis is not unlikely (and what I describe makes her think small fiber neuropathy even more) but that there's not much point in testing for it unless it gets severe. She says I also have NCS based on my history of passing out with needles plus the behavior on my TTT.

We're going to stick with the compression stockings and up the salt to 10g a day (since I tolerate 5g just fine with no nausea/vomiting). Also going to drop the atenolol (while I've been off of it pre-testing I've noticed much less fatigue) and add propanolol (she says it's better because it decreases sympathetic nervous system drive in the hypothalamus, whereas atenolol doesn't really hit the brain). Also more exercise (starting swimming next quarter), trying to prop up the head of my bed, avoiding the things we usually avoid, etc. Plus a referral to medical genetics to talk about the hypermobility.

And, guess what, guys?! She referred me to DINET and dynakids! I am so happy. You guys all rock and I'm glad she thinks so too!

[peregrine]

Ooo, wanted to add more now that I've stared at the 12-page test printouts some more! (Okay, I am a dorky biologist, I am not supposed to be excited about bad tests results, but we knew something was wrong, so sue me)

So apparently my pulse pressure was reduced during the Valsalva, which can be caused by one of deconditioning, dehydration, or sympathetic nervous system failure. But we know it's not the third due to the normal response to metronomic breathing.

Regarding the TTT specifically, "Excessive oscillations in mean BP > 20mmHg for more than 50% of head-up tilt duration. Symptomatic abnormal heart rate response with increase > 30bpm [whoo, POTS] and >>120bpm within 10 minutes after head-up tilt with increased oscillations, consistent with POTS, reaching a maximum of 160bpm. Remark: patient started hyperventilating during the last 30 seconds of this test. She had visible venous pooling." For the TTT specifically, those last 30 seconds or so my heart rate stopped fluctuating and went to 160bpm steadily; my doctor said that if they hadn't tilted me back it would have rapidly dropped to zero (umm), my system was in overdrive, and I basically would have undergone a brainstem reset similar to how opossums play dead when they're being chased by a predator (which is NCS in her words). So we are opossums being chased by an invisible predator called POTS! *grin* The blood pressure chart looks very weird (it's not supposed to fluctuate that rapidly) - at the worst my systolic blood pressure was 62.5 and my diastolic blood pressure looks (based on the graph) like it was about 50.

She also gave me Dr. Grubb's paper "Postural Tachycardia Syndrome: Perspectives for Patients."

[HopeSprings]

Sounds like you got a good autonomic work-up. It is very validating when they find dysfunction (sad as that is) - it's like see, I told you something was wrong! Curious about the small fiber neuropathy. Did the neuro say how this fits in? Did she say anything about looking for underlying causes of SFN? Let us know what happens with the additional testing. Curious about the 24 hour sodium in particular. Hope the adjusted treatment plan helps!

[juliegee]

Good to have THAT behind you. So confirming to have those abnormal tests! You doctor sounds wonderful- hope it all leads to a better treatment plan!!!

[peregrine]

Naomi - I'm not sure how the SFN fits in, although from what I understand she's implying that it might be responsible for my blood vessels failing to constrict appropriately, thus causing pooling. Plus she says it may be causing the gastric symptoms I describe, and she says she think it's more likely SFN based on my bringing that up. From what I understand the QSART and similar tests are about it for SFN diagnostic techniques, with the exception of biopsies which I understand are pretty rare. She is testing for diabetes specifically (the HB A1C) because that can cause SFN and thus dysautonomia. I will let you know about how the tests go - I'll get the labs drawn on Monday and the 24-hour urine sodium started Monday morning and returned Tuesday.

Mack's Mom - thanks! I count myself as super-lucky to be (unintentionally) living in a place with two dysautonomia specialists, a gastroparesis person, a medical genetics team that likes hypermobility and knows about dysautonomia, and an actual autonomic testing setup. I honestly hope this becomes a lot more common so folks don't have to travel and people know about these illnesses. And rarely gets above 80F in the summer. (I guess that's my plug for Seattle?)

[peregrine]

Welp, more updates. The HB A1C, HIV, anti-SSA/Ro, vitamin B12, protein screens, etc all were normal, and my 24-hour urine sodium shows I'm not dehydrated, so the narrowed pulse pressure due to the Valsalva has to be something else. Time to pull out the more expensive tests for paraneoplastic syndrome and amyloidosis (TTR specifically, so looking for familial amyloid neuropathy, which affects the ANS). Ah well. At least I'm drinking enough fluid (passed 2.65 liters during the 24-hour urine sodium test) and am not dehydrated!

Doctor writes:

"All your lab results have returned very normal. So no common cause for autonomic small fiber neuropathy. I am sending you a lab slip by snail-mail for the two special tests (paraneoplastic panel and TTR), which you can get done at your convenience. Your urinary sodium is nice and high, so you are drinking enough and using enough fluids to get rid of it."

[Chaos]

Fantastic that you got such a good and thorough doctor!! Are they considering doing the skin biopsies for the SFN? I know at both CC and Mayo they said that was the "gold standard" for diagnosing SFN.

That's impressive that you got a 12 page print out from your TTT etc. That should have given you a lot of bedtime reading. LOL VERY COOL that she referred you to DINET and gave you Grubb's paper. Glad you are getting SOME validation and answers at least.

[ramakentesh]

Who did the test? very comprehensive! they didnt happen to measure your MSNA by any chance? nerve firing rate?

[Chaos]

Has anybody in the states (US that is) have MSNA testing done? Where were you able to do that?

[ramakentesh]

In australia. I know some do it and it used to be consider an indicator of hyper v non hyper. But maybe less so now. its a needle they stick into your perosomething nerve.

[Chaos]

Perineal (nerve)? Outer shin area?

[ramakentesh]

thats the one! Mine was 4 1/2 times normal and made the two docs say 'woow'. Still NE was in normal ranges!

[peregrine]

Hunh - I didn't realize there was such a test! They haven't talked about the biopsies; I don't know what she thinks on that but I will ask her next time. Right now we're waiting for me to get back in town on 3/17 and then get those other tests drawn on 3/18 (so will probably see her ~3/23 or 3/30 as she's only in on Fridays).

[HopeSprings]

She sounds smart. My neuro also did the paraneoplastic and TTR genetic testing, plus a lot more. There are 50 million things that can cause SFN and I basically asked to be tested for all of them. Keep pushing.