POTS: Diagnosis & Management

Podcast Transcript

Introduction: Neuro Pathways, a Cleveland Clinic podcast exploring the latest research discoveries and clinical advances in the fields of neurology, neurosurgery, neuro rehab, and psychiatry.

Glen Stevens, DO, PhD:

In the United States, it is estimated that one to three million people suffer from postural orthostatic tachycardia syndrome or POTS. And there is mounting evidence that the number of individuals afflicted with this disease has grown, and continues to grow with the arrival of the SARS-CoV-2. In today's episode of Neuro Pathways, we're discussing the often challenging diagnosis and management of POTS. I'm your host, Glen Stevens, neurologist/neuro-oncologist in Cleveland Clinic's Neurological Institute. And joining me for today's conversation is Dr. Robert Wilson. Dr. Wilson is a neurologist in Cleveland Clinic's Neuromuscular Center. Rob, welcome to Neuro Pathways.

Robert Wilson, DO:

Thanks for having me.

Glen Stevens, DO, PhD:

So Rob, during my PhD almost 40 years ago, I hate to admit that, my PhD was on orthostatic intolerance. I'm not sure if you know that or not, but I studied orthostatic intolerance, and we were mostly interested in orthostasis related to zero-G gravity. So, we worked with the space program, and our interest was when people go to space, they get very compliant, they don't have gravity, so when they come back to Earth, when they stand up, the blood pools in the legs, they're very compliant, it doesn't get pushed back to the heart, and they pass out. And we were looking at mechanisms whereby we could get the blood to go back to the heart and maintain orthostasis in these patients. And the other PhD candidate with me ended up becoming an astronaut and went up in the shuttle, and did some of our experiments in the shuttle, so it was quite exciting. But now I don't do any of that, of course. And I'm glad that we have you to do this.

Glen Stevens, DO, PhD:

But take us through the different types of POTS or orthostatic intolerance. What is it that you do, and what's the disorder that you see?

Robert Wilson, DO:

It's interesting. We'll get to that, but I always reference the astronauts when I talk to the patients to get them to understand orthostatic disorders because of how gravity is relevant for them not to have a concept for the disorder, and why it's important, and the early astronauts, and how exercise matters, and these concepts. And I'm actually in neuro-oncologist fellowship training. I did neuro-oncology for years, and I learned about orthostatic disorders from really mainly working with people who have sort of chronic disease aftermath and oncology treatments, late effects, when I was at Penn and CHOP. So, that's how I learned about it. So you and I very maybe mirrored more than we realize. So, I was glad to be here today. So, your question is about this sort of the types of POTS, right?

Glen Stevens, DO, PhD:

Yes.

Robert Wilson, DO:

And it's interesting, it's probably more than we realize. I mean, generally there's sort of big three groups. As a neurologist, there's the neuropathic POTS which we always talk about, which is that more of that neuropathy group. They have often associated small fiber neuropathy, they're the low blood pressure types that we see. Often, we'll find them in a clinical exam. When they come in, they have low blood pressure, you might actually see if you do bedside orthostatics, they drop their blood pressure. They're the ones who find them more conventional in our learning, in our knowledge of practice that you'll see they have the blood pressure changes. Many of the POTS patients will not drop their blood pressure. You might find things in clinical exam that they have more conventional small fiber neuropathy findings on the [inaudible 00:04:09] neurologic exam. Their testing might show in Skinner biopsy. You have the opportunity to get a QSAR in your community, you might find abnormalities.

Robert Wilson, DO:

Those are those patients that we probably, as neurologists or whoever, might listen to this, we might understand as the more conventional orthostatic patient. They also might have those skin color changes that we all sort of note, associate with, with like the blue legs or red legs. They're fatigued or light headed, they may faint. That's the neuropathic POTS, which are often associated with sometimes autoimmune, Sjogren's, celiac, chronic disease aftermath.

And then we also have that sort of what you call hyperadrenergic POTS, which are the ones I think that probably we see a lot at the clinic, the patients who maybe come to us who may get diagnosed, or have those sort of mish-mash of symptoms, go more of that baroreceptor dysregulation. They're much more jittery, probably ones who are more challenging for people to handle because, their symptoms can be misunderstood as anxiety. They're jittery, they're nervous, they have digestion, they have brain fog, they hard of focus, their heart rates are dysregulated, they might not have as much fainting. They can actually faint from high heart rate, low cardiac output. They might not drop their blood pressure, they may have moments of hypertension. We might find elevated catecholamine levels in their blood tests.

And then there's this other entity, which I find very fascinating and mysterious, the hypovolemic, which may have a different mechanism. There's sort of three groups, but there's probably more than we realize. And a lot of the patients might have some overlap, I personally believe. And there's probably much more to it. And when people ask that question, I always think it's more of future research opportunities, and these patients probably are not as so siloed into these three boxes or three circles. They're much probably Venn diagram. And they probably might evolve into other things over time.

Glen Stevens, DO, PhD:

So, I was very excited to hear you say the word baroreceptor, because all my work was on baroreceptors. I haven't heard baroreceptors for some period of time, but very excited to hear that.

Robert Wilson, DO:

Well, that's an important concept. I think the way to think about these patients with orthostatic disorders, and that'll be the one thing for any clinician, is when you think about them, think about the baroreceptor. That's when problems with physiologic things. It gets dysregulated, it becomes propagated, it becomes automatic. That's how people with head injury can get baroreceptors dysregulation. It gets very sort of like a circuit. And it's a very important mindful space for the clinician to think about these patients. It explains why their heart rates get sustained, why these drugs make sense that we use for these patients, a lot of the mindfulness work it does. And it's a very important concept to think about these patients, and why even these patients in a dysregulated state can have moments of hypertension. Personal orthostatic tendencies can still do the baroreceptor dysregulation, can moments of hypertension.

Some of the colleagues we work with at clinic are actually even sometimes are kidney specialists, and are cardiologists or hypertensive experts. So, that's an important thing to think about this, and the people I work really closely with, we think about baroreceptor dysregulation often.

Glen Stevens, DO, PhD:

Yeah, I think the most challenging patients sometimes I see in the hospital on consults are those that are having trouble maintaining upright posture because their blood pressure drops, and then when they lie down, they have supine hypertension.

Robert Wilson, DO:

Oh, yeah. And there's actually patients who might have these really unique disorders of orthostatic hypertension we'll see, and very other things, and they're all over the board, and the ones that I see in my practice, I don't see people who just dropped their blood pressure 30 points when they stand. They're easy to manage. They might respond to medications. But I see them more the baroreceptor dysregulation, which they are high blood pressure, and then they drop, and then they're up and down, their heart rates are all dysregulated. Those are much more challenging ones. And clinically, their symptoms are very all over the board, and they can be, I always say, mish-mash symptoms, and can be very hard for people to manage because their symptoms are very big and expansive, and one moment they can be one way, the next one will be another way. But if you think about the baroreceptor and how it works and how it can do, you can get an easier way to approach these patients, and a friendlier way to get a feel for them.

Glen Stevens, DO, PhD:

So if I'm an internist, a family physician listening to the podcast, and I think, "Boy, I've got somebody that has these types of problems," how do I decide? Do I send it to the cardiologist, I think there might be a rhythm problem, or do I send it to the neurologist, or should I send it to both? Or what do we have for these people?

Robert Wilson, DO:

I think the thing is that's a really great question. I think these patients can be first thing, I think the important thing is at least think about this first. I feel for the primary doctors, the internists, the pediatricians. As neurologists, we have the luxury of more time. These patients are coming in, they're dizzy, they're jittery, they're nervous, they feel anxious, they have digestive issues. Only about 10 percent or so might faint. They're dizzy, their lives are often derailed. So, the people on primary care are frontline, and they don't have the time.

So, I think sometimes is they might need to look at first, what are their best resources locally? We're in a very luxurious place at the clinic. We have a lot of people you can pull to. So, sometimes it depends on who they have locally. Are the cardiologists probably more adept for syncope, or sort of these more diverse symptoms? Is it a neurologist? Who are their best resources for these type of big symptoms, these diverse symptoms?

If you do have a concern of a rhythm, a cardiac rhythm, you do want to have someone at least seen by a cardiologist at times. I do think people need to follow their clinical judgment. If you do think there is a lot of neurologic symptoms, you're not certain, you do want to have someone seen by a neurologist.

And sometimes, it is both of those specialists. Because you are hitting a point, it might sometimes seem like a cardiac issue. Ultimately, it is behaving like a neurologic process. The baroreceptor, and how the brain gets involved, the neurocircuitry, I do think it's a neuro process, ultimately.

Glen Stevens, DO, PhD:

But I guess you can see that if the patient says, "It feels like my heart's beating really fast," somebody would interpret that as they're having some type of conduction problem and sending them to a cardiologist.

Robert Wilson, DO:

Yes, yes. A lot of our patients have seen numerous cardiologists, and you have to be very sympathetic for what the people locally in our local communities go through and what resource they have. And I had a patient in the Upper Peninsula of Michigan where the local cardiologist has done a spectacular job and had a very neurologic, sophisticated approach.

Glen Stevens, DO, PhD:

So, you mentioned some of the different types of POTS that we can see, which then make some of the things more obvious, but who's at risk for POTS?

Robert Wilson, DO:

I love that question. That's personally my obsession with the times that we live in recently, because I almost became an OB guy, and I changed to neurology last minute, and most of my patients are women. I actually like women's health. So, most of my patients are women. And it does seem like even what we're going through with COVID, we'll get into that a little, but most of the patients we see with post-COVID POTS are women. It does seem to be a more female demographic, young, which seems might raises lots of questions about could there be something about hormonal inflammatory states? Women have more pro-inflammatory states. There's some data about even head injury with women doing worse in some literature. Could be more autoimmune aspects. So, there's a lot of things we see about young women and that aspect.

We do see a lot of times people then with postviral syndromes. We knew when COVID happened, we were going to see some stuff. I've been doing some grand rounds with post-COVID POTS, and I called it same script, different cast. Because we knew like mono was associated with this, and we see with mycoplasm and some other infections, postviral syndromes. We see other [inaudible 00:12:47] is associated with it, which is probably more common than we probably realize. Once you start looking, it's there. And then other autoimmune things, maybe the older demographic, Sjogren's. We've done some research and publications on Sjogren's. That's more than just dry eyes and dry mouth and dry skin. Causes neuropathy, it can lead to POTS. Celiac we mentioned. A lot of chronic aftermath disease with chronic health issues.

And then sometimes we'll see people post pregnancy, we'll see people have a lot of life events, subgroup, a lot of life events we're looking into. So, adverse life events. And there might be something we call susceptibility, why some people versus others. We'll look at some people, person A, person B, why person A? What is it about that person? We'll see sometimes clustering in families. And that's probably a great research question. Why that person?

Glen Stevens, DO, PhD:

I'm sure you have some strong opinions, but what about some of the biases and misconceptions of POTS patients?

Robert Wilson, DO:

You know, it's interesting. I think my medical students, I help run the neurology clerkship, and we have a lot of medical students on the floor. I think they say it best. They come up here and they mean well, and they go, "Yeah, I hear it a lot about these POTS patients." And they come up here and I go, "They're actually really nice, and very engaged, and very involved in their care, and very motivated." We have a whole sort of care package with education, appointments, and get them exercising. And they really are involved. They're very involved in being educated. And they like information, our demographic. They love information.

I think it's hard when the autonomic nervous system is everywhere in the body. It does eye secretion, pupils, mouth, digestion, bladder, blood pressure, skin. I mean, it's everywhere. So, these symptoms could be everywhere for the people, everywhere. And they feel fatigued. They don't feel well. So, it can be an overwhelming experience. People come to a clinician, and we are limited with time. And so, these patients can be overwhelming, I think partially for a healthcare system that often doesn't have much time, and we're limited. I think also, there's an issue sometimes, not enough neurology education in the healthcare system. Not everyone has enough neurology practice and their training to understand how to approach these patients. I think we're often still thinking that shortness of breath, chest pain, racing heart rate, is always cardiac, and not thinking it could be a neurologic process.

And I think sometimes there is still, if someone's coming in saying, "I'm dizzy, I'm lightheaded, I'm chest pain, and I'm fatigued and I can't function," we might still think it's anxiety, and maybe if they're feeling bad and they're anxious and they're feeling anxious cause they just don't feel well, there's a little bias always going to the anxiety card too quickly.

Glen Stevens, DO, PhD:

Do we have any idea what the lag time is between patient symptom onset and diagnosis of POTS?

Robert Wilson, DO:

So, that's a great question. I mean, there's a lot of sort of patient experiences, and some data out there, three to five years. Many for physicians, six physicians. There's a lot of this data out there, which is, you as a neurologist know that with other neurologic conditions, like migraine, that later people are intervened and treated, it can impact how people do. And that's why sometimes I think it's fascinating, these COVID POTS patients where we're seeing them, I was a normal person, functioning. Had COVID, got POTS< and we're seeing them four months into their symptoms. It's fascinating to see how these people sort of are putting this fire out. Helping them earlier is a fascinating concept.

Glen Stevens, DO, PhD:

Well, I thank every day for you that you have a real interest with these patients. It really requires somebody to have an interest and want to listen to patients. So, what's your initial approach with these patients, besides just actually taking a really good history and exam?

Robert Wilson, DO:

Well, you're very kind. I'm not much different than you are. I think any neurologist, we love a good story, and that by doing an exam, the exam comments a story. So, I think neurology honors what being a doctor's about. So, I just think it's just our work is just incredible every day. It's just a pretty neat journey, a great way to spend our days.

So, I think getting these people answers, and there's such a neat thing about neurology about pattern recognition. When you start really following these patients, all the stories often seem very similar. Most of them have had something, a tipping point. We've been doing these group appointments on Zoom with these patients, and we call it timeline, where they notice this day when everything's changed. And we do this teaching point where everything's evolved and changed, and then this cascade of symptoms.

So, I call working with these patients, I call it the E's with them, the E's, like the letter E. Educating them. Once they're diagnosed, we believe in educating these patients, really getting them educated. Because a lot of people nowadays are on social media, they come with a lot of sometimes, by time they come to us, they've been to many medical providers. They're often confused. We do a lot of work educating through these Zoom appointments, educational process. We have a patient liaison that works with us. We really educate our patients, really try to get as much tools. We empower them. We try to use a medication if we need to, like a beta blocker to control these adrenergic symptoms. Ivabradine, if you have low blood pressure, we use midodrine. We try to find what works for them.

So, we use education, empowerment. Empowerment in a sense to get them feeling confident for the education to start doing things, all the typical orthostatic self-care of hydration, salt if needed, compression stockings, mindfulness work, learning how to sort of hand hold their bodies and brain to feel less adrenaline, sympathetic tone, and then really exercise their mind, body, and spirit. We get our patients exercising. We really try to get our patients exercising, but we know that exercise helps. Going back to where you started again, the astronauts who had orthostatic intolerance disorders, the treatment is exercising. Even now, exercising the astronauts is a big part of the deal in space. We really get our patients moving.

Glen Stevens, DO, PhD:

Do all these patients need tilt table testing?

Robert Wilson, DO:

I'm very big about people having tilt table testing. That's a great question. So, you can imagine these are often young people, these are young women, many of them. There's a lot of times they have diffuse symptoms. They have a predilection of bias against them. Often, they have high care needs. They might need some medications that require insurance hurdles. Having this sort of testing to have this objective data that shows this for them now long term is helpful for confirming diagnosis, make sure I'm not missing anything else, and really care planning long term. It really does help.

Glen Stevens, DO, PhD:

And what about tests for small fiber neuropathies? Sweat tests, biopsies, those types of things?

Robert Wilson, DO:

I think it's being in an academic center like the Clinic, we have all those things really at our fingertips and just down the hallway where I am. We might order it more. I think use your clinical judgment. We actually have a project we're doing, and our people are coming to our department, 20 some percent of patients might have small fiber neuropathy. What do you believe? The exam, which has relevance? The Skinner biopsy and QSAR are important data that could be confirmatory. But we actually have patients,

Glen, who might come to us who have POTS, and then they might then also have gastric motility disorders. And they might fail Schirmer's test, but then their QSAR and Skinner biopsies are normal, because that testing might be too distal, the QSAR and Skinner biopsy, to miss it.

And what do you believe? You believe those testing, there are more core organ involving. So, nothing's been standardized yet for the absolute for small fiber neuropathy. Maybe there needs to be a scorecard for some of these autonomic disorders, symptoms, neuro exam, low blood pressure, skin color changes, composite QSAR, Skinner biopsy. Where we were looking at the corneal confocal microscopy as a preliminary study, it's the densest nerve plane of the body, as another additional test. Really, these patients might need a composite of testing to really show an autonomic disorder, to show small fiber neuropathy.

Glen Stevens, DO, PhD:

And in terms of your exercise, aerobic, anaerobic, resistance, combinations, doesn't matter?

Robert Wilson, DO:

I love your last sentence. These people feel so poor, they feel so flu-like, so run down. I think some of the best examples, some of these COVID POTS patients in the 2020 phase, they got COVID, and then a few weeks later they got POTS. They kept going to the ER thinking they had COVID again. That's how flu-like these people can feel. We start people wherever they are, just getting their bodies moving. In a chair, laying down. We get them moving their body. We'll do chair yoga. We'll do Tai Chi. We'll give them Qigong. We can start at that level. So really, just simple body range of motion. Then we'll get people to a more aerobic exercise, recumbent bike. And then from there, if they can, treadmill. Then from there, weights. Really getting people going. We'll have people starting maybe five minutes a day. Some people are so debilitated, we'll have them just even just look at a video, just really getting people starting that slow. It can take people up to a year, some of our patients, to get up to 15, 20 minutes on a recumbent bike.

Glen Stevens, DO, PhD:

And what about the COVID POTS patients? Different phenotype?

Robert Wilson, DO:

It's interesting. We knew we were going to see something, and I thought we're going to see, like because of viruses, can be viral toxin. I thought we were going to see a lot more neuropathy, positive Skinner biopsies, QSARs. We have some research we're doing, something at the AAN. Not as much nerve destruction, but much more this more hyperadrenergic POTS state we've been seeing. It's almost like these people who are having it, it's like their bodies are in a sort of very stressed physiologic state. And it was fascinating how they all started coming in in the fall of 2020. And it was much more high heart rates, and sometimes their blood pressure would shoot up in a very hyperadrenergic state.

Glen Stevens, DO, PhD:

And is it self-resolving, or what's it doing?

Robert Wilson, DO:

Well, you know, I love your questions. One of the challenges are we're going to do a research project, one of the medical students are, we have a registry, and these people have been through a lot. They've

had social determinants on them, health insurance issues. They've lost their jobs. Some of these patients had virtual appointments, they got their testing. So, they've been all over the board with us, and we're going to sort of thumbprint them, and then track them where they are in terms of return to function, their quality of life scores, their autonomic scorecards, and see where are they?

I know where a lot of them are, the ones I follow more closely. A lot of them have done well with beta blockers, mindfulness work. We're going to show you how they control your adrenaline, hand hold your body and your brain, show it how it can be not in a stress state forever. Show it mindfulness, wellness. Get them exercise. I know we've done with our patients, but it's going to be interesting, because these patients who went through this phase, like all of us culturally, had this major upheaval, and a lot of them have just grabbed the healthcare when they could get it.

Glen Stevens, DO, PhD:

Well, Rob, as I mentioned at the start, I love the fact that you're so passionate about this field, because my experience with these patients is that that's what they need, right? It's a puzzle, like a lot of neurologic disorders. But it's easy for me to look at a scan and, when you used to do neuro-oncology, there's a tumor in the right frontal lobe, it's pretty obvious. You have to look a lot deeper. So, we appreciate every day what you're doing. But I think it's just good to just remind everybody that we all just go back to the basics, take a good history, do a good exam. And that probably really tells us the majority of times what these patients really have. We do the confirmatory test, but we probably know.

Robert Wilson, DO:

Exactly. Well said.

Glen Stevens, DO, PhD:

So, appreciate your time today, and look forward to interacting with you again in the future. Thank you very much.

Robert Wilson, DO:

I appreciate you, Glen. You're a good man.

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