POTS: Symptoms, understanding, and management

Tae Chung (0:06): I always tell my patients that diagnosing POTS can be either super easy or super complicated. Almost 80 90% of POTS patients are disabled to a certain extent. There are people who just cannot work or go to school. A lot of doctors who don't see a lot of POTS patients, they usually say, Okay, well, exercise helps. Just go to gym and try that's just not gonna work. A lot of POTS patients have exercise intolerance. If you do too much cardiovascular training, more than they can handle, they always pay the price.

Emily Kate Stephens (0:42): Welcome back. Welcome Gez. How are you doing?

Gez Medinger (1:02): Today, you know what? I'm suffering less than usual, so I want to have like a little party streamer... So, obviously, less than it has been for the last week or two. So that's good, isn't it?

Emily Kate Stephens (1:12): And I think that what we have to say on that day is you need to still just take it as easy as you do on other days, because there's such a tendency to go, Yes, I feel good. Let's get it all done.

Gez Medinger (1:24): Yeah, that was a lesson I learned probably in year one, quite hard, and I still continue to learn it now in year six, funnily enough.

Emily Kate Stephens (1:32): Yeah, wow. Well, at the top of the show today, I would like to once again ask our audience to comment like share this podcast, and in today's show notes, I am going to add a link in which you can feed back to us directly via a form. And we would love to hear some of the stories of our listeners. And particularly, I'd be really interested to talk to anyone about the financial impact that having a long term sickness has had on you and your family. And I'd also be particularly interested to hear from people who might like to talk to us in the capacity as carers or supporting those people with these conditions. So we would like to try and get some of our audience involved in some of our upcoming episodes. And if you feel like that is something that you could do and you might have the energy to do, please get in touch with us. Gez, today's episode, we are going to be talking about the often misdiagnosed, much misunderstood condition of POTS. Am I right in thinking that POTS is something that has affected you?

Gez Medinger (2:44): I have sort of pseudo POTS, which means that I get a bit funny if I'm standing up for too long. But I don't think it fully qualifies POTS. Now what I really ought to do, and what I've been encouraged to do, and for some reason, I have put off for many years is a NASA lean test, and we can talk about that at the appropriate time.

Emily Kate Stephens (3:03): I think we should call it an active stand test, because some people find the NASA lean test title quite incorrect, because apparently it has nothing to do with NASA.

Gez Medinger (3:12): Well, my research tells me it does, because one of the problems that astronauts have when they come back from space is, funnily enough, orthostatic intolerance, because they've not been subjected to gravity. So this is one of the things that people with chronic, complex illness have in common with astronauts, is that basically, it's like we've been in space for a few weeks, and our bodies are struggling to deal with the onset of gravity. See, it's fun to compare us to astronauts, but I think it is

Emily Kate Stephens (3:41): Before we go into looking at things like that, the NASA lean test, active stand test, and ways that we might be able to help diagnose and potentially alleviate symptoms from POTS. Let's have a listen to Dr Tae Chung of Johns Hopkins University, he set up the POTS clinic there in 2017 and still leads the team. Let's hear from him what POTS is, and some of the strategies that people like him, who are at the top of the field might have when dealing with POTS patients and afterwards, perhaps you and I can dig into some of the strategies that people can take away to use for potential diagnosis and self management. First of all, we should probably explain what POTS is in terms of its full title and the symptom set that you see there.

Tae Chung (4:53): Okay. Well, POTS stands for Postural Orthostatic Tachycardia Syndrome and is considered to be largely autonomic nerve disorder, and they have hard time handling their blood volume, and as a result, they have a chronic fatigue and dizziness and lightheadedness on standing, especially but they do also have non orthostatic symptoms, meaning they can have a lot of symptoms without the position changes that can include brain fogginess, migraine and a lot of GI symptoms as well. And in combination, those symptoms can be very debilitating.

Emily Kate Stephens (5:31): Unusual to come from your background in physical medicine and rehabilitation and with your neurology strands as well to looking at POTS, because I think a lot of POTS specialists come from a cardiology background, but you say that you're seeing these people with the brain fog, with the it's a different symptom set. So tell us about how that translates into you understanding that as a POTS diagnosis and that chronic fatigue, the way that plays into POTS.

Tae Chung (6:00): First off, I can say it's unusual for even any cardiologist or neurologist to POTS patients. So I really appreciate any doctor of any background willing to see POTS patients. I really appreciate that. I really want anybody or doctors to see POTS patients. Just first of all, prior to me seeing POTS patients, most POTS doctors who see POTS patients were either cardiologists or neuromuscular neurologists, right? So like I said, I did my neuromuscular training, so it was kind of little natural transition for me to start seeing POTS patients, and this is considered to be largely autonomic nerve problem, so kind of fits into my expertise. But in addition to that, there's a statistics actually shows that almost 80 90% of POTS patients are disabled to a certain extent. There are people who just cannot work or go to school. There are some people who can actually go working full time, but mildly limited in their activities. But about 80 90% of patients are limited in their daily function. And as a physical medicine rehabilitation doctor, that is actually my patient target population.

Emily Kate Stephens (7:08): What's the target, in terms of an improvement of quality of life for your patients, or to get them back to fully operational?

Tae Chung (7:17): Actually, pretty much everything I know a lot of people don't understand much about probably didn't hear much about PM&R as a specialty. Simple way of explaining PM&R (Physical Medicine & Rehabilitation) specialty is that we are population based specialty PM&R doctors. We see patients who have physical disability, and to take care of these patients, we have a special set of training to address their needs, recognize their needs, and how to help those people, okay? So by nature of PM&R, we are very multidisciplinary. There's a lot of overlap there, right? For example, a lot of typical PM&R patients are patients with a lot of orthopedic problems, right? Maybe, or amputees. So of course, we work a lot with orthopedic surgeons, or a lot of our patients are stroke patients who are neurologically impaired, and we work a lot with neurologists, given the nature of POTS, which is also multi systemic, that's why I think a PM&R doctor with training in neuromuscular was the best situation to see POTS.

Emily Kate Stephens (8:20): And tell me about how you give that diagnosis of POTS? Now I spoke to Dr Peter Rowe, who is a specialist in orthostatic intolerance. Now this is Postural Orthostatic Tachycardia Syndrome, so there's another element. There's a difference between this and orthostatic intolerance. Can you explain the difference with POTS and another term that's often applied is dysautonomia. Can you explain if those are the same things or how the symptoms vary?

Tae Chung (8:53): I first have to actually say that, I guess there are some different opinions about how they interpret these different terminologies, even in the field, even among some doctors. But the way most people, I guess, including myself, interpreted as, first of all, dysautonomia, is a very umbrella term, any dysfunctional autonomic nervous system. We can call it as dysautonomia. I think probably better analogy would be psychological disorder. You don't have to specify schizophrenia or there are a lot of,

Emily Kate Stephens (9:24): I thought you were saying that dysautonomia is a psychological disorder, no, okay?

Tae Chung (9:28): It's an umbrella term. It's an analogy. Don't get me wrong, that there's nothing to do with psychological disorder, which is to call anything psychological disorder, doesn't mean that it can be depression or schizophrenia, this very umbrella term, so to say something is a dysautonomia is just like that very umbrella term, anything that can be coming from autonomic nerve dysfunction. You can call this dysautonomia, before we get to orthostatic intolerance terminology when it comes to POTS I also want to emphasize that POTS ends with S, and S stands for syndrome, as I just mentioned, syndrome, as opposed to a specific disease, by nature, is a heterogeneous condition. When any doctors call syndrome, that means we don't know much about it, but it's a thing, so we need to label it, so that's when we call syndrome. We don't know much what's causing exactly POTS maybe all POTS patients, they may have POTS in a different path, they may not exactly or same, or even are all POTS patients having dysautonomia is all coming from autonomic dysfunction, maybe, or maybe not. Maybe there's some people may have POTS from some other cause other than autonomic nerve dysfunction. At this point, we don't know. So I'm not even going to say entire POTS is under this dysautonomia umbrella. There's a probably 90% maybe big overlap there. Again, I still also leave a room for some other non-autonomic causes of POTS as well. So that's how I view the whole syndrome, you know, Venn diagram. I think orthostatic intolerance, or chronic orthostatic intolerance, is a more of a term. As far as I know, there's not many great consensus in terms of different societies having some clear diagnostic criteria. This is a little more symptom description, and of course, chronic orthostatic intolerance usually overlaps a lot with the POTS and chronic fatigue syndrome and connective tissue disorders in a Venn diagram as well.

Emily Kate Stephens (11:41): So okay, so a patient comes to you with a chronic fatigue condition, debilitation, muscular debilitation, what enables you to give that POTS diagnosis?

Tae Chung (11:54): So I always tell my patients the diagnosing POTS can be either super easy or super complicated. Tell you why the way it's easy, because the syndrome is defined very broadly. So anything that can cause orthostatic symptoms, chronic orthostatic symptoms, like I said, dizziness or sometimes they faint or headache, and all the other orthostatic and non orthostatic symptoms, that's going on for three to six months, and then they have this orthostatic tachycardia. And orthostatic tachycardia is defined by more than 30 points increasing their heart rate from a supine to standing position. It can be from the tilt table or just active stand. And of course, there are some other additional things, such as, if you're a pediatric 40 points rather than 30 points. And there's another technical definition, but that's largely that definition of POTS.

Emily Kate Stephens (12:50): But it's that increase in heart rate, right? That's specific that the part that enables that diagnosis.

Tae Chung (12:56): But the more important thing about the diagnostic criteria is that, like, if you really look at the criteria, there's a little corner that says, oh, but that, by the way, those phenomena should not be explained by structural heart disease. So that makes it actually sometimes either diagnosing rather very easy or very difficult, because in many cases, actually, as you know, POTS patients, they're relatively younger patients, in their 20s or 40s, and more female than male. So let's say patient comes in who are previously very healthy and athletic, and suddenly they're having POTS symptoms. Let's say after Covid infection or maybe Lyme infection, those are relatively easier cases, because, well, first of all, I don't have any reason to suspect they have a structural heart disease. Somebody was like, maybe 19 years old, who's a running marathon, are suddenly having these POTS symptoms. It's very unlikely that they have a heart structure heart disease. Those people, as long as I can rule out any structural heart disease, such as cardiac arrhythmia or heart failure, and then just simply showing orthostatic tachycardia is relatively easy to diagnose POTS. Now, having said that they have a POTS, does that mean that they have autonomic nerve dysfunction, or is there another cause of POTS? I don't know, but we can still label them as POTS at that point. It gets way more complicated when patients had some pre condition or comorbidities, let's say they have some mild degree of some heart condition before the POTS, then the question becomes, is this some manifestations of heart condition, or they have a pre existing heart condition, and they have a POTS on top of that? Teasing that out can be a little difficult, and I'm being very cautious when I diagnose POTS in those cases.

Emily Kate Stephens (14:47): And is POTS normally something that comes on following virus or some kind of insult on the body like that? Or do you see patients who have just gradually, over time, develops this syndrome,

Tae Chung (15:03): Both actually. So I think there's a there's really not many great epidemiological researches on POTS, but there's few pretty good survey from 1000s of patients, and from those surveys, just about half of these people have some identifiable infection prior to onset of their symptoms, such as Covid infection or Lyme or EBV infections, Epstein-Barr virus infection. So it is often attributed to a virus just about half or 40 to 50% case, I'll be kind of attributed to those infections. Others can be variable. It can be sometimes somewhere very early in their childhood, maybe some potentially genetic basis to it where the whole family member has POTS so it can be almost everything else. Some other small number of patients also reported some kind of concussion, head injury that's prior to onset of their POTS symptoms too. So it can be very variable, like I said, it's heterogeneous. Syndromic diagnosis, the

Emily Kate Stephens (16:06): Go to treatment of POTS is rehabilitation via exercise with a physiotherapist. Is that? Is that fair to say

Tae Chung (16:18): Or yes?

Emily Kate Stephens (16:21): You tell me about this sort of, not necessarily what you do, but the normal way that POTS is viewed and treated. There are multiple strands to it, but a lot of it involves that physical rehabilitation piece, which involves exercise correct

Tae Chung (16:42): So I'm gonna go in a little breakdown of the different aspects of treatment. But before that, I like to actually give a little bit of a kind of proposed pathophysiology about what causes POTS symptoms before even talk about treatment. Because if you don't have a target, you don't know what to treat. So basically, the core of the pathophysiology of POTS is reduced blood volume, return to the heart and lung. So that's actually why, when they stand up, the blood is not being returned back to their heart. You know, as you know, heart has to circulate blood. They pump out the blood and receive the blood so that they can circulate. But POTS there's a lot of evidence suggesting that there's reduced blood volume return to the heart, and that's causing fatigue, because they're circulating low blood volume, and exercising tolerance, because they cannot increase their blood volume to your muscle, and brain fogginess, because they cannot increase their blood flow to the brain and so on. So as a heterogeneous clinical syndrome, it can be coming from a lot of different reasons why they have a reduced return there. It can be autonomic nerve dysfunction, where the blood vessel is not squeezing blood back enough to the heart, or there may be some blockages somewhere down there, pelvic congestion syndrome, so called. They can be another proposed etiology for POTS as well, but whatever the reason is, as a final common pathway to reduce blood volume back to the heart and lung that's causing, you know, all the POTS symptoms. So the main target of the treatment for POTS is to increase blood volume that's returning to the heart. And to do that, we call this volume expansion therapy. The goal is to increase blood volume. And there are certain ways to increase blood volume, and you know, as you probably know, like drink a lot of water, salty diet, and there are some medications that can retain fluid, so they can increase blood volume back to the heart and lung too. Physical exercise can be also viewed as a part of volume expansion therapy as well. Okay, so normal people, when we do cardiovascular training or exercise, there are two kinds of things that can happen acutely in the exercise. What happens is, especially if you use a lot of lower extremity muscles. Our lower extremity muscles squeeze the blood back to the heart and then momentarily increase blood return back to the heart

Emily Kate Stephens (19:07): Just in that moment, though. Or is there the suggestion that by doing exercise, it creates a sustained effect?

Tae Chung (19:15): It's momentarily, actually, their cardiac output increases, like dramatically, but when you stop the training, then it goes back right away. However, anybody actually normal, healthy people, meaning normal, healthy body function, I guess if you do a chronic cardiovascular training over time, over weeks, our body starts making a lot of red blood cells because we have to deliver more oxygen, and that increases our blood volume substantially So, and it applies to POTS patients too. So if you do cardiovascular training over a long period of time, our blood volume increases substantially, and that's actually why POTS patients benefit from long term cardiovascular training. However, there's a big caveat there. Like I said, there are a lot of POTS patients have exercise intolerance. If you do too much cardiovascular training, more than they can handle, they always pay the price, and they crash. So it's actually really not as easy as people think. A lot of doctors who don't see a lot of POTS patients, they usually say, Okay, well, exercise helps. Just go to gym and try. That's just not gonna work. I usually strongly recommend that POTS patient exercise should be closely supervised by somebody who only understand this physiology.

Emily Kate Stephens (20:29): So is that easy for you to identify from the outset whether the patient will respond favorably to exercise or not?

Tae Chung (20:37): To be honest, I wouldn't say easy. More and more I see patients, this is actually one of the probably more difficult aspects managing POTS. Actually it's really sometimes we see these patients who we think they may respond well, but they don't. And then vice versa too. And also I'm realizing, increasingly realizing, that when you implement exercise therapy, it needs to be in close conjunction with medical therapy as well. So oftentimes we have a whole team. We have actually designated POTS physical therapists in our team, and oftentimes their physical therapist will just email saying, Okay, this patient needs midodrine or IV fluid or some other medications as they progress their physical therapy program. So it is not really separate from medical pharmacological treatment and physical treatment. It has to be very, very related. What is midodrine? Midodrine is one of the medications that are called vasoconstrictors. It pushes the blood back to the heart and lung, and is one of the common medications that we use.

Emily Kate Stephens (21:37): And there are a few others as well that you regularly use. Am I right in saying you use things like ivabradine?

Tae Chung (21:39): Yes, ivabradine is a little different class of medications that lowers the heart rate. It doesn't have any effect on blood volume increase, but ivabradine is also very common medications tachycardia, to a certain extent, can be a little helpful for POTS patients. It's considered to be somewhat compensatory mechanism. For example, if you have a low blood volume. Basically, body is trying to be heart fast so they can compensate for low blood volume. That's why POTS patients, their heart rate goes up. But if it becomes excessive, it can be counter productive as well. And in such cases, any medications they can slow down their heart rate can be helpful, and those medications include the class called beta blockers, or ivabradine, which is a very new medication that slows heart rate without causing a lot of side effects.

Emily Kate Stephens (22:33): And what are the others more in the category of increasing the blood volume that you would go to? Well, there

Tae Chung (22:39): Are not many, to be honest, but the common use medications include, like I said, midodrine and fludrocortisone that retains fluid in their body at the kidney level, and another drug called pyridostigmine may act as a little bit of vasoconstriction and also helps with the GI symptoms as well. Those are very common these medications. There are actually quite a few or several medications that probably not commonly used, but frequently used by some other different doctors in different situations. Okay? And on top of that, non pharmacological care, if you actually look at the kind of literature, there are some small studies about exercise and drinking water hydrations, but there has not been really good randomized clinical trials about it. So we actually wanted to really answer all the questions whether this non pharmacological care works. We know what non pharmacological kind of care suggests. I mean, this is like hydration, diets and exercise and so on. We realized that a lot of practices, like I suggested. Well, we can go to textbook website, or if you have POTS - exercise and diet, there's a one page, 1, 2, 3, 4, 5, things to do. Just give it to the patients. Versus our clinic, or some other clinics in the country, we actually follow them up, making sure they are actually doing the exercise and how we follow very closely, and how much they drink, how they do actual care, like really coordinated care, with the multidisciplinary team.

Emily Kate Stephens (24:07): Obviously you had a big uptick in POTS, then Covid hit. So you started getting more, not post viral necessarily, but infection associated, viral associated, POTS patients, yes, so how much of your clinic now is taken up with that viral associated

Tae Chung (24:31): Just about half of our patient population. They can pinpoint some specific infections prior to the onset of first symptoms among them, how many patients have Covid specifically? I'll probably say 20, 30% but that's just rough estimation. I don't have a specific number that's updated since 2022, so

Emily Kate Stephens (24:52): And is there any difference in that Covid related POTS versus ME/CFS or Lyme related POTS, those infection associated POTS.

Tae Chung (25:02): Well, clinically speaking, I cannot tell much difference at all. To be honest, I've been asking about my colleagues about the same question, if anybody walks in with all the symptoms that I mentioned, and meeting the POTS criteria, can you tell there's a difference if they have Covid infection versus Lyme or something else? Yes, nobody can actually discern. I still believe anybody gets POTS from Covid versus Lyme or Epstein Barr Virus, at least at the molecular level, it may be a little different. And maybe if there's a better way to recognize their symptoms, or analyze their physical symptoms, you may find some difference, but at this point, it's hard to differentiate. So in

Emily Kate Stephens (25:48): Terms of presentation, it's the same. So does that mean that a trial looking at treatments for POTS related to Covid would also have relevance in POTS related to other viral conditions. So for those ME/CFS patients who have POTS, or for those other patients that have POTS, we will be able to learn from this Long Covid trial, because obviously RECOVER has this huge focus on Covid, but there is pushback within the community, within the ME/CFS and fibromyalgia and all of these patients who say, but you haven't done anything for us for all this time. Okay, obviously there are people like you who have been treating people, but in terms of the world funding research and trials into those other things, we're quite far behind, correct. So do you think that kind of clinical trial looking specifically at Covid treatment should be able to inform us in other viral insults as well.

Tae Chung (26:47): Absolutely, yes, there's actually one of the big reasons that I'm working on this post-Covid POTS. It's not that I'm only interested in post-Covid POTS and nothing else. Yeah, but reason why we selected post-Covid POTS population. There are a few reasons for that, but one of the things, main important things, we don't have a good biomarker that can show that our if you have this, then you have POTS. And this is autonomic nerve dysfunction, and more importantly, this is autoimmune condition. That's why you're having this problem. We don't have any biomarker for that this point at all. Now, even for post-Covid POTS, post-Covid onset POTS, for example, that we call the Autonomic trial for IVIG. We wanted to really emphasize that anybody who came to trial are the ones who did not have any POTS, ME/CFS symptoms prior to Covid infection, and somebody has a very new onset POTS after Covid infection, there are not really many other explanations, other than autoimmune condition that can cause these POTS and also at the same time, unlike Lyme, EBV, some other infections, everybody tests for Covid. That's actually why we selected as a more scientific reason, it's more, potentially, relatively more homogeneous population that are more likely to respond to certain treatments, such as IVIG, which is why we select this population. But clinical trial is not about just testing the drug. You're collecting a lot of bio samples, and then this huge amount of resources for future discoveries. And from there, maybe you can find biomarker and then share it with EBV, post-EBV onset POTS and other things too. To me, this is a, really, the starting point of all these POTS research. You're not just limiting into post Covid.

Emily Kate Stephens (28:42): It's interesting, isn't it? I've spoken to other doctors who say the same, and actually what that post Covid enables us to do is have a specific infection that we're relating it to and looking at, whereas so many people with prior infections, or people who have ME/CFS prior to Covid. Don't necessarily know what the driver was, so it's almost that we're using that Covid as a way to control these trials, because we know that it was specifically that I think you were part of the study that did a large amount of proteomic analysis, yes, and looking at POTS in post-Covid again, correct? You established that there were within those proteomics, potential biomarkers, yes, for that post-Covid POTS specifically, not necessarily diagnosing post Covid. Can you tell me about those potential biomarkers?

Tae Chung (29:38): Yes. I mean, that study is just the beginning of the beginning. I mean, there's so many more things to do, and that just opens up really possibilities. And my goal, I do have a probably more different interest in what to do with the POTS, I mean, the trials and finding good treatment, but also, like I said, finding really reliable biomarker, I think, is really critical, and there's a very direct implication on patient care. Having specific biomarker finding that can open up opportunities for why they have POTS. Maybe the biomarker is related to certain molecules that are involved in, let's say, auto immunity, or something else, they can really crack the code into why they have POTS and maybe you can even develop treatments that can even cure POTS too. So a finding biomarker has a huge implication, and not only diagnosis or treatment in POTS as well. And as you know, there's no really reliable biomarker for POTS. The idea is that we can get blood samples from POTS patients and normal patients, and we can, well, it's more agnostic kind of approach. We don't know what the protein markers are going to be. We just measure 1000s of proteins at the same time and see what separates them. And in the first paper, we saw some bunch of proteins that can separate POTS and normal people. That's potentially a really good starting point. Maybe among those proteins can be a good biomarker. Now, to find what exactly that can be a good biomarker requires years of actually follow up studies. I'm planning to actually really longitudinally, follow up the patients, analyzing their test and other things and matching their blood markers over time. I think it's going to take some time before we actually come up to very reliable biomarker, but we are very remain very optimistic that we will be able to find something

Emily Kate Stephens (31:35): Down the road. So then finally, I was talking with Todd Davenport the other day, and he mentioned that you because I noticed that you've done the systematic review together, and he said to say hi to you, oh. And then he said that you're working on another study together. Is it the second part of the same review? That first review

Tae Chung (31:55): Is more meta analysis the existing literatures, and the second review that we are kind of working on together is more perspective reviews on how we approach exercise in POTS patients, because there are not many really good studies to be really meta analyze on. But you know, the basic group of people who see POTS patients like Dr Davenport and chronic fatigue syndrome, as you know, Dr Davenport is a physical therapist, and we approach them very similarly, whether it's POTS or chronic fatigue syndrome, sort of different, though, for sure, it's not exactly same, but one of the big themes, as you know, post exertional malaise or post exercise or flare of the symptoms, and like I said, that's one of the big challenges in treating patients with exercise, therapeutic exercise, and I think we're realizing that, first of all, nobody has one answer, and it's very difficult. And also, as you know, POTS patients, they have a lot of overlapping syndrome, such as Ehlers-Danlos Syndrome - EDS, MCAS, Mast Cell Activation syndrome, all kinds of different comorbidities. They can also affect our exercise intervention as well. I mean, the most common thing is Ehlers-Danlos Syndrome. We see a lot of EDS patients and whenever they do exercise, there's a lot of subluxation, the hip, musculoskeletal pain. So it's not really that easy. Like I said, more and more we get into this therapeutic exercise, there's a whole lot of complexity there. Well, fortunately, we have a good group of collaborators across the country, like Dr Davenport and some other people in the Utah and our Hopkins group, very experienced therapist, and we are getting together try to really brainstorm what's going to the best way to POTS patients.

Emily Kate Stephens (33:45): That's amazing. I love the amount of collaboration that is going on. I know that historically in ME/CFS, there have been these working groups across the country, but I think the amount of collaboration that there is now in this post Covid society is fairly remarkable. So thank you very much for getting together with them, and I'm working with Visible now. Do you use... I know a lot of your patients do, do they?

Tae Chung (34:06): Yeah, yeah.

Emily Kate Stephens (34:11): But it's not something that you necessarily advocate.

Tae Chung (34:15): I tell my patients to use wearable device. I mean, that's really, really informative, but I at this point, I just say, well, you can do Apple Watch or Galaxy or Fitbit or whatever is available. There under a lot of financial stress, right? They stop working as well. So I don't necessarily want to make anyone spend too much money on things like that, so, but it's very, very, very, very helpful. I mean, just the wearable device just makes so much difference. What I can

Emily Kate Stephens (34:44): Help it in terms of patient pacing?

Tae Chung (34:47): Oh, absolutely pacing. Or not only that, especially, like I checked their heart rate with our trend over the day, yeah, and that really determines whether I should use beta blocker or ivabradine, or when I should use it, and so on. So the more information I get was so incredibly helpful, really.

Emily Kate Stephens (35:04): So it's not just about their understanding, their energy usage and their what they're doing for themselves. It's actually useful for you in terms of your treatment

Tae Chung (35:13): Strategy, right? I know the physical therapist is very helpful for patient pacing, but for me, it's more for okay, what should I use, Corlanor, or when and what and how, and it's incredibly helpful.

Emily Kate Stephens (35:26): I hadn't thought about that in terms of actually, the way in which patients can then take it to their doctors. And it's not just about, oh, look, I've got really low energy, or this is my symptom exacerbation, specifically for that heart rate change. Thank you very much for your work, and I'm looking forward to

Tae Chung (35:45): The biomarker. Oh, thank you. I'm looking forward to you too very much.

Emily Kate Stephens (35:56): Let's first of all talk about this idea of diagnosis, because I think that is probably the starting point for many of our listeners. There is this huge crossover between multiple of these conditions and POTS. As this syndrome actually falls under many of the conditions suffered by our listeners, I think of it more as a set of symptoms I also think that working out the cause of why we have this is so fundamental to how we might be able to treat it, but that is not really prevalent in our medical systems. I believe my daughter has POTS because I repeatedly have tested her with the active stand test that I would love you to explain for our audience, but one of the key things that you and I always say is these conditions are relapsing and remitting. So tests done by the hospital very badly, in my opinion. For my daughter, did not alert the doctor to the fact that there was any problem. When I observe her day to day, I see that she struggles with her heart rate every time she changes position. Tell me about these tests that we can use and medical professionals should be using to help people understand whether or not they have POTS?

Gez Medinger (37:16): Well, first of all, I just want to pick up on one of the words that Dr Chung mentioned, which was syndrome, and syndrome being a word that we use when we don't really understand what's going on. And I think this is at the heart of the problem. We still don't really know what's going on. We have an idea that there is this basic problem of, we're not getting enough blood volume return to the heart and lungs, right? But why? Why and how, and we don't understand that. And it's very difficult, as he said, to treat it until we can work out why and how this is going on. And I think a little bit like ME/CFS, this sort of lack of understanding of the condition leads to it's not really appearing in textbooks, and doctors don't read about it in their medical training, and so it's not the sort of thing where it appears on their radar, like these are the signs for appendicitis, and this is what you do. It's not there like that is and so it gets missed. But at the same time, it's not necessarily a difficult thing in principle to diagnose. And when I say diagnose, orthostatic intolerance versus POTS, are they the same thing, or there's overlapping. But certainly it should be relatively straightforward to determine whether you have a problem when standing up. Right? First of all, do you just get an increase in symptoms and you stand up and there's a set of symptoms that are on the list? There things like dizziness, brain fog, headaches, but then also, does your heart rate jump? And you can see this relatively straightforwardly, if you've got a wearable or some description, and if you want to do an active stand test personally, I'm going to call it the NASA lean test, because I look, we can't compare ourselves to 100 meter Olympians or anything like that, right? But if we're like astronauts, that's the one thing, right? That's the one kind of together with so, so I'm going to call it the NASA lean test. So what's it looking for? The answer is it's looking for a heart rate increase of over 30 beats per minute within 10 minutes of standing

Emily Kate Stephens (39:07): Up, and 40 beats per

Gez Medinger (39:08): Minute in children, correct? And we're also checking blood pressure as well during this now you can do a sort of a reduced version, if you don't have a blood pressure cuff, better to have one, but if you can just measure the heart rate too. Ideally, you do also need an assistant with you, because that assistant needs to check that you're not about to collapse and fall on the floor, also to take the readings and write them down. What do you do? The answer is, so before you start, don't do any strenuous exercise of any kind. Before you start, don't have just eaten, don't have just taken a load of caffeine. Be well hydrated, but not overly so don't do it in a hot room. Do it in a room where the temperature is comfortable. And before you start, lie down for 10 minutes. Whilst you're lying down, take your heart rate, and if possible, your blood pressure. Take two or three readings over the 10 minutes and average them, and then that's your baseline. Next thing, get up. You find a wall to lean against. You put your feet about 15 to 20 centimeters away from the wall, and you lean back against it. What you're doing there is you're stopping the muscles from holding you up, which basically makes it a little bit more challenging for your autonomic system to regulate the blood flow. It's why Tilt Table tests have you lent back? It's about 60 degrees because that means that your autonomic system has to work harder to return the blood stand up against the wall and start taking heart and blood pressure readings at one minute, three minutes, five minutes, seven minutes and 10 minutes, and also record any symptoms. So if those intervals, if you are feeling brain fog, dizziness, breathlessness, chest discomfort or nausea, mention it to your assistants, and they will write it down. Stop the test early. If you feel like you're going to faint, your blood pressure drops significantly or the symptoms become severe. So once you've had a look at those results, you want to be able to see if your heart rate has jumped by over 30 beats per minute at any of those points up until 10 minutes. And if they have then, essentially you have a positive test results for POTS. So once you get to the 10 minute mark, you can lie down again immediately. And if you're having any symptoms at that point, notice how quickly they resolve. So if they rapidly resolve upon lying down, that's a positive indicator in itself for orthostatic intolerance. So looking at the results, a few possible options here. If your heart rate goes up by over 30 beats per minute at any point in the 10 minutes or more than 40 if you're under 30 years old, that suggests POTS if you have a systolic blood pressure drop of over 20, that is an indicator for orthostatic hypotension. If your symptoms are relieved by lying down, that's an indicator for orthostatic intolerance. But if you have normal heart rate, or heart rate that jumps by less than 20 beats per minute, and your blood pressure stays fairly constant and those symptoms, this means you probably don't have POTS. Now I haven't done this test properly, but from the sort of the wearable tests I've done, when I've been standing up for a while, I feel like I need to lean or I need to perch. And if I do, check my wearable, if I haven't, my heart rate's probably running at about 20 to 25 beats above normal, which suggests I'm somewhere between the normal increase, which is between 10 and 15 BPM when standing above line, and the 30 that indicates POTS. So I am autonomically challenged there.

Emily Kate Stephens (42:26): Yeah, it's suggesting dysautonomia, isn't it? In that general umbrella,

Gez Medinger (42:30): Correct, but not full blown POTS. I had a tilt table test three years ago and failed dramatically, and when I say fail, I essentially passed out at about the six or seven minute mark so that, again, is also indicative of orthostatic intolerance, if not necessarily a full diagnosis of POTS.

Emily Kate Stephens (42:48): And we reiterate we are not medical professionals, but what we're trying to give you here is tools for you to then take to a medical professional and say, I would like to be assessed for this. And a big problem that you might come up against is, as Gez mentioned, there seem to be few people who really understand this condition in the primary care line of medicine. And there is, as Dr Chung mentioned, this sort of discrepancy between the people that treat POTS, because a lot of the time it's cardiologists, but it seems that POTS is not a heart problem, it is the autonomic nervous system dysfunction, as Dr Chung said in 90% of cases.

Gez Medinger (43:35): So let's say you've done your active stand test, or your astronaut test, as I will continue to call it, let's say that you've got some results there that suggest orthostatic intolerance, orthostatic hypotension, or POTS. Then take that piece of paper to your GP, and you can actually present them with something that they ought to understand at that point. It's a very useful thing to have done, and that will help you get better care going forward. So ultimately, the results in themselves might help you understand what you need to do practically in your daily life, to help manage your symptoms. But if you want to actually get help for POTS, and you can get help for POTS, whether it's lifestyle changes or medication, then yes, take those results to your GP.

Emily Kate Stephens (44:16): And as Dr Chung said at the end of the interview, there a wearable. And Gez has mentioned, a wearable is an incredibly helpful device for you to be able to see on a regular basis what is happening to your heart rate when you change position. Just enables you to check in and see what is going on. Dr Chung briefly touched on it, and he talked about medication, but you mentioned there Gez some lifestyle changes. Now I am going to link quite a few different resources in our show notes, things like POTS UK, Standing Up to POTS. There's a great society called the Australian POTS Foundation that has some really, really good resources. I'm going to link all of those in the show notes for people, but let's talk about some of the lifestyle changes that seem to come highly recommended, although, as Dr Chung said, they have not necessarily rigorously checked through any kind of clinical trials, these are things that anecdotally, seem to support the primary thing that people with POTS are advised is to increase fluid and increase salt intake. It's very difficult to give figures, but the general advice across the resources seems to be two three liters of water a day and increasing your salt intake by six to 10 grams. I just wanted to give one word of warning on electrolytes, because people might immediately think, yes, electrolytes are a really good way to increase salt content, because they are. A lot of them made up predominantly of sodium chloride. I want to flag that a lot of them also contain a lot of citric acid, and that can really exacerbate mast cell activation. So where we're dealing with conditions that have these multiple layers, you are going to have to be mindful of adding something that might have potentially other side effects to it. I think that a lot of the time, it tends to be better to increase your salt content by increasing your salty foods. It's a minefield Gez because a lot of the sites they have salted nuts, that is really also not great for mast cell activation. So then, in terms of that intake, a lot of the time, it's suggested eat more smaller meals, because of the way that your heart rate changes when you're digesting a heavy meal. So instead of two to three meals a day, it suggests six to eight small meals a day and a decrease in carbohydrate and an increase in protein. I think the other big thing to do is to check for deficiencies, because iron, folate and B12 can definitely drive these POTS symptoms. What would you add to my list

Gez Medinger (47:05): So you don't feel cool putting them on, but just tell yourself you're an astronaut, and it will be fine. Compression wear. So these are basically super tight leggings. You call them tights, and you also get ones that go around your torso as well. And again, this helps stop blood pooling and helps that return of blood volume to the heart and lungs. So compression stockings are up there on the list of things to try. The other thing to be aware of is, if you've got POTS, you probably also struggle with temperature regulation. And along with eating big meals, the one thing that will suddenly make your POTS a lot worse is probably heat. So wherever possible, try to again, it's difficult, depends where you live, but you probably find heat difficult, and you may find cold difficult. And again, this is just part of the autonomic regulation of the system being out of whack, and it's something else to manage. So you might find that you get hot flushes that come across and again, your body is simply struggling to manage heat at any one given time. And if you're in the middle of that, the POTS is going to get much worse too. So I think layers, along with the compression stockings, layers that can be removed or added according to needs are a helpful piece of advice.

Emily Kate Stephens (48:16): The compression thing is really interesting, because for someone like me, a lot of the time I want to get rid of any tight clothing, because in terms of sensory for me, it's too much. So there's that comes into play as well. But I do know that there are a lot of compression garments, and a lot of the doctors who use that as one of the first line treatments. There are things like showering that can be really challenging in these conditions. And actually there you might a shower stool that so many people have suggested that with many, many of these conditions, that there are simple additions that you can make to your life that just might ease things.

Gez Medinger (48:56): So we always talk about it pacing again, POTS and your symptoms are directly proportional to the amount of time you spend on your feet. So simply breaking up your tasks so that you spend as little time continuously on your feet as possible. Take frequent breaks if you get particularly symptomatic after seven minutes of standing up then every five minutes, sit down for five or lie down for five. Have to manage the way that we attempt to get through our daily life, and that can make a huge difference, actually, to your experience of that day by simply breaking things up.

Emily Kate Stephens (49:29): There are things like that can have positive impact on POTS, whether they're all related, then also has positive impact on other of your symptoms, and whether or not you could then crash in some of these conditions. One of the things that Dr Chung said there is work with a specialized physiotherapist or exercise physiologist, and if you are lucky enough to be in a specialist POTS team, you might have access to those. I would like to give a warning here to people who are referred to non specialist, Exercise Physiologist. I was sent to see someone at an NHS hospital, and he told me to start doing weights, because this age old thing, he thought that I was deconditioned. It is there in the 2021 updated, NICE guidelines of treating ME/CFS that pushing exercise on patients is not appropriate, as Dr Chung said, there are some people who might respond favorably to exercise, but we need to be so, so careful. And I would like to give warning to everyone to be mindful when you are advised by people who are not specialists to simply go out and start pushing yourself for exercise please be mindful that they are not in your body. They don't know how you feel, and you know in your body how things are affecting you. And I encourage people to trust listening to your own body, please. It can be really, really detrimental. I completely agree,

Gez Medinger (51:00): If you fit the criteria for ME/CFS, or you have exercise intolerance, don't do exercise even if the doctor tells you to. Can I throw something at you, Emily, please? On the frontier of treatment: we have the established treatments for POTS, which include the lifestyle changes we've mentioned, the medications like beta blockers, midodrine, pyridostigmine.

Emily Kate Stephens (51:20): Something that both you and I have tried in various forms I believe, for improving autonomic function is tVNS, transcutaneous vagal nerve stimulation. There is evidence to suggest that can regulate your heart rate variability and improve vagal nerve tone?

Gez Medinger (51:41): Yeah. I feel like the solutions we're going to find for this part of the autonomic nervous system regulation, are going to be slightly out of the box. I don't think it's going to be take a pill and suddenly the autonomic nervous system just behaves. I think we're going to have to think electrically, magnetically, electrically, something like that as ways to approach this and also holistically, too, as with all these things.

Emily Kate Stephens (52:05): You know me, I'm a massive advocate of breath work, and that form of nervous system regulation. And there is the suggestion with some of those things in the same way as electronically stimulating your vagal nerve, that because the vagus nerve is essentially an electrical pathway. We are all made up of electrons that stimulation, by whatever means can have an impact on that. So I think this crossover with that electronic the magnetic resonance, is hugely fascinating, and an area that we should definitely delve into the more evidence that comes out, and the more research that is done into it.

Gez Medinger (52:46): So just in this whole world of electromagnetic stimulation, there is a boundary between the stuff that has evidence and the stuff that has very weak evidence. You have to be a little bit careful. I think it's really important the next place for us to be looking for complex chronic illness is for treatments that have been shown to be effective with some form of condition that may be adjacent to complex chronic illness, because we know that there is a physiological effect. Because the world of snake oil out there is huge, and I think we have to be careful of it, because a lot of that snake oil is very, very expensive, and it wants your money, and it will tell you very nice things.

Emily Kate Stephens (53:21): So much of this is so expensive, there is a really good evidence base with some of the tVNS, but not all of it. And this goes to that point that everything has to be well researched. I do find it exciting that we're potentially at the fore of actually going way deeper into understanding so many of these conditions that have this huge overlap, that are treated very poorly and a lot of the time primarily suppressing the symptoms chemically. And I guess my hope with all of this research that's going on is perhaps we can actually have a far greater understanding of the human body and it's malfunctions at large, rather than this only being appropriate to one condition or a couple of conditions.

Gez Medinger (54:09): I think one of the interesting things that complex chronic illness has, in its own way, shone a light on the established, you know, Western medicine mindset, is that the specialties which we've traditionally had in western medicine. Are very good at treating things like a broken leg and diabetes and heart pressure issues, cardiology, orthopedics, Gynecology, all of these specialties are important, clearly, but we don't really seem to have very big chapters in the medical textbooks on the autonomic nervous system, on the really fundamental nature of metabolic and mitochondrial function. These are two subjects which are so deeply woven into the fabric of complex chronic illness, and we've sort of ignored them in our quest for gathering evidence based, my hope would be that as time goes on, we do start to get fuller chapters in those textbooks on the autonomic nervous system, the function of the nervous system. We don't fully understand it. We don't fully understand what's going on on a mitochondrial metabolic level yet. And this is really where the next leap in medicine is going to come from, I think, and I hope over the next generation or so, absolutely, and

Emily Kate Stephens (55:22): I do feel like that autonomic nervous system and metabolic and mitochondrial function piece will actually influence so heavily in those other conditions, hypertension, diabetes, these things that are at the moment treated, balanced, but not cured this. And I know you hate using that word, but I'd love a cure. Perhaps the leaps in medicine have the potential to actually help us to reverse in understanding of the human body.

Gez Medinger (55:52): Well, I think this is fundamentally it, isn't it? We're coming at it from the outside in, not the inside out. So you treat the surface symptoms, as opposed to going deeper inside and solving the root cause that's actually generating the disease in the first place. So we're treating the symptoms of type two diabetes. We're not removing type two diabetes?

Emily Kate Stephens (56:10): Yeah, absolutely. And it's the same with all of these conditions, symptomatic treatment, not going upstream. What was the cause of any of this, and perhaps it's all autonomic dysfunction, autonomic dysregulation,

Gez Medinger (56:27): Yeah, connected to immune dysfunction, connected to metabolic dysfunction, connected to inflammatory dysfunction, you name it, right.

Emily Kate Stephens (56:32): What with the whole body being connected one system? Wow. Gez, it has been such a pleasure talking to you today. Thank you so much for joining us and to all our listeners. We look forward to speaking to you again soon. Thank you for listening to Make Visible. Please do like follow or subscribe to listen to our next episode where we'll be uncovering more insights into complex chronic illness. This was brought to you by the team at Visible, a group of scientists and engineers whose lives have been affected by energy limiting health conditions. We're building wearable technology that's helping 100,000 people measure and manage their complex chronic illness to find out more about what we're working on and how Visible could help you visit our website, makevisible.com

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