So yes, the headline is true. This was a case of POTS that somebody had for eight years. They were in a wheelchair when they came to see us, and within just a few weeks was able to leave the wheelchair behind, get back to exercising, get back to living and doing some great things. And I know that seems big and hyperbolic, but that's why we're going to talk about it — because this case was really useful in my evolution of understanding POTS as a syndrome, and hopefully it'll give you a chance to look at it through a little bit different lens.
Her Story Before Coming to Us
She had suffered with POTS for about eight years prior to me getting a chance to meet her, and it was one of those ones that just kind of came out of nowhere. There was a viral infection in the year preceding her diagnosis, but nothing that was quick and obvious.
She was able to get good care early on and started a POTS rehab and a cardiac rehab program. And actually in that first little stage of time, she was able to build up some tolerance and get back to exercise and actually being able to run a little bit. And then she had an episode or a setback where she experienced some severe bradycardia, wound up in the ER, and then after that was not able to exercise in a meaningful way — a classic case of exercise intolerance in POTS. Even in recumbency on a recumbent bike would feel super exhausted, would be knocked down for two to three days afterwards.
So then went to using a beta blocker, using midodrine sometime thereafter, which caused some big blood pressure fluctuations. By the time I had seen her, she had seen some other physicians along the way and they were suspecting that she had some Ehlers-Danlos syndrome, Mast Cell Activation Syndrome, and a neuropathic form of POTS — which for most of you, that sounds pretty familiar.
What Our Exam Showed
So when we started our exam, we were looking at it through that lens. We had also found that at some point she had a neck injury and had an injury in the middle part of her cervical spine as well that she was trying to deal with.
She had suspected neuropathic POTS — suspected damage to the nerves that innervate blood vessels to push blood back to the heart so that you can start it recirculating again. And when I looked at that exam, what I noticed was that she did have some kind of faulty signs. So we measured vibration and pinwheel and joint position sense — some of these things that help us get a picture of small diameter fibers versus large diameter fibers and how those communicate back up to the brain.
One of the things we noticed was the ones that were affected for her were all on the left side of her body — in her legs on the left side, in her trunk, in her arms, and her face. The whole left side of her body, but not so much on the right side. Which is very interesting if you're thinking about a form of nerve injury — usually when we think about neuropathies, they're bilateral and they usually start distally, like down toward the feet, and they migrate their way back up the legs and into the fingers. So it was a unique presentation. It made me think about looking at some different things.
“If you don't feel things well, it's harder to move them and it's harder to be coordinated — and that's what we saw.”
So then we looked at coordination. Doing finger tapping on the left side — it was slow and it had hesitations. Using pronation-supination, so rapid alternating movement on the leg, we saw that the left side was a lot slower than the right. And then when we looked at sliding the heel down the shin with the left side, the movement wasn't smooth. Usually we think about the heel sliding smoothly down the shin to the toe, and in the case of her left foot it would slide and kind of fall off of her other foot.
Balance and Eye Testing
We compared that to a Romberg's — that's where we have somebody stand on a pad, a foam pad so it kind of has some squish, so it makes you work for it a little bit. And you stand and close your eyes. When you close your eyes, all of a sudden now you can't rely on your vision as the main source of input to help you keep your balance — you have to rely on information from your body and information from your inner ear to kind of keep you positioned in space.
What we found was that when she would close her eyes, she would tip over toward the left side. When she turned her head to the left, she would tip. When she pitched her head down, she would tip. And then when she put her head back, she would tip. The only place that she didn't fall was with her head turned to the right — which is really interesting and kind of correlates with some of those other findings.
We looked at just the station of her eyes. When her eyes are relaxed, do they aim straight forward? Is that the relaxed neutral position? Or when we cover one and have them relax, do they drift to a different location? And that's what we saw — she developed this exophoria, or her eyes drifted out, which tells us that to maintain that nice stereo vision where she's got two eyes looking at the same place, that's taking effort, which is not the way it's designed to run.
There were errors in just simple eye movements of pursuing targets, holding your eyes to the side, and being able to detect kind of motion in her vision. So all of these things lined up with what we're seeing on that left side and point to the fact that maybe this isn't just a simple neuropathic problem. This is a problem that actually looks like it's a little bit higher up in the brain in coordinating these efforts on that side of the body. And what we would call that is something of a functional hemi-neglect — and that's actually what she was working with.
The Tilt Test and Valsalva
So then apply that to kind of the autonomic syndrome, right? She's got POTS, which means she's got this tachycardia developing when she's coming up. That's why she's using a wheelchair — she gets very dizzy, very nauseous, very short of breath anytime she's trying to be up and at it in the world, even just sitting up and being around.
Doing a tilt test, she was only able to last about five minutes before requesting to come down. We kind of encourage people to stay in that tilted position for as long as we can so we can collect data, but her heart rate just kept scooting up and she felt more dizzy, like she was going to pass out. So we brought her down after only about five minutes. It was a pretty quick reaction and she was not able to recover that very well.
And then on a Valsalva test — we have people blow hard into a tube, and what that does is create a pressure in your thorax, in your abdomen. That pressure forces your cardiovascular and respiratory system to have to modulate to that pressure quickly so that you don't pass out. What we noticed was her response was very slow. Normally we get a change in blood pressure and it drops down and then it starts to kind of come back up to keep blood in your head — and that's what keeps you from kind of passing out. And hers didn't really respond. So we had what's called a late phase two failure in the Valsalva test.
“All those things kind of go together to give this diagnosis of she has POTS, yes, but it doesn't look neuropathic — it actually looks more like a functional hemi-neglect.”
How We Treated It — Step by Step
Here's the caveat: the things we did for her don't apply to every neglect, don't apply to every case with POTS. But I'm just going to explain kind of the things that we did along the way that helped her out.
So the first place we started was understanding that anything that we did in recumbency or standing or sitting, she was still short of breath, she still had a high resting heart rate. We used a red saturation test where we look at the saturation of the color red in her vision to help get a gauge of how much blood flow she's got coming through that ophthalmic artery — just as a quick bedside test to see where she's at metabolically, like how far we can push her in that moment.
What we did was we brought her down until she was able to hit a stable heart rate, stable blood pressure, stable breathing, and we practiced just these really simple one-sided leftward head motions — kind of like we do with gaze stabilization in vestibular rehab, but instead we're going just the one direction because we've got that one-sided neglect. And we're doing it with just a small amplitude while she's laying down so that we can make sure she's got plenty of blood flow to the head. So we're exercising this system in the best context to deliver fuel to the brain.
Once she was able to hold her eyes steady with that gaze, then we started also adding in using a peripheral nerve stimulator on the left side. We would stimulate her peroneal nerve in her leg, tibial nerve in her leg, the anterior portion of the quad in her leg, median nerve in her arm, and then some points along her face as well. We would practice going from using the head movement to stimulating her body so that she could start to link those two sensations together.
Once she started to be able to feel those vibration and pinprick tests equally on the left and right side, we started to actually bring her up — so rather than doing it just laying down, we'd do it at five degrees and then 10 degrees and then 15 degrees. Once we hit 15 degrees, we started to add in doing more motion with the limbs — electrical stimulation and then a passive movement of the limb to continue to kind of scale that therapy up.
Then we would start to just oscillate, gently bringing her down to zero and then bringing the tilt up and then bringing it down to zero and bringing the tilt up afterwards. This is taking place over the course of several days, but I'm just giving you the progression of how we're adding to that.
From Laying Down to Standing Up
We did that until we were able to get to where she could go from laying down to being up to 30 degrees and she could oscillate between those without having a change in her heart rate, without having shortness of breath, without feeling dizzy, without feeling lightheaded or syncopal. And once you can start to do that, then we would add doing active things for her — so this is where we transition into exertion.
She wasn't doing exertion seated or standing — she was doing it where she could tolerate it. But here's where we have her start doing some marching on the bench, start doing some active limb movements while she's on the tilt table. And at this time also we started adding in some cervical spine mobilization while she was on the table, with isometric exercises of her neck.
So now we went from kind of having her body just focusing on — can we get these sensations in and make them accurate — to now we're actually having her start to use them. So she can take the sensory information in and process it into a motor output where she's creating motor activity. Which is huge. Super fun.
Once we hit 45 degrees where we go 0 to 45 with no meaningful change, then we start moving into recumbent exertion exercises, shifting everything to seated exercises with the head and neck movement, with the peripheral stimulator. We're able to start adding in recumbent bike and then we use a recumbent stepper, which is pretty cool — it's like the stair stepper machine that you have in the gym where you kind of walk up the stairs and it's super tall so you can look down on everybody else and feel awesome, but this one is you're laying back at 45 and it has a stepper that moves so it lets us engage a little bit more with the leg musculature without having to be in a seated position all the time.
“Within a couple weeks we had it so that she could come in without the wheelchair — which is pretty cool — without getting short of breath, without getting dizzy.”
Getting Back to Life
Then we started to have some fun with it and looked at using standing exercises. The way we do that in the beginning isn't just jumping into standing — we start off with just 30-second little walking activities and then we do more complex activity. But we like to make it fun so you're not just focused on how you're feeling when you do it. So we use things like Blaze pods, playing sports, playing catch, playing hockey — different ways that we can make it exciting or kind of activate some different brain areas that allow us to be able to dual task a little bit.
We moved into that and then eventually into doing it for 30 seconds to a minute to two-minute intervals, and that was the progression. And within a couple weeks we had it so that she could come in without the wheelchair, which is pretty cool — without getting short of breath, without getting dizzy.
In the transition to home, we worked on going a little bit longer, a little bit longer. We added one interval at a time until she was able to start walking down the end of the road, around the block, then adding in some biking and doing things like that.
Why This Case Matters
So that's kind of the full progression of where she came in using the wheelchair as a daily tool to be able to get around — most of her time is sedentary, not doing much of anything — dealing with the kind of deconditioning that makes POTS worse — to being able to get some active lifestyle again and start making plans.
To me this was a really great case where we're thinking about how we can look at that exam just a little bit differently, how we can apply treatment in a way that allows her to be able to get to the goal without over-exerting the system. I'm hoping that's useful for you to think about — maybe even just give you some inspiration to look at things in a new way.
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