Episode 76 – Pediatric POTS and Other Disorders with Dr. Boris https://tinyurl.com/potscast76

00:01 Announcer: Welcome to the Standing Up to POTS podcast, otherwise known as the POTScast. This podcast is dedicated to educating and empowering the community about postural orthostatic tachycardia syndrome, commonly referred to as POTS. This invisible illness impacts millions and we are committed to explaining the basics, raising awareness, exploring the research, and empowering patients to not only survive, but thrive. This is the Standing Up to POTS podcast.

00:29 Jill (Host): Hello, fellow POTS patients and most appreciated people who care about POTS patients. I'm Jill Brook, your hyperadrenergic host, and today we are lucky to be speaking with a physician and researcher who truly deeply understands POTS, as much as anyone can, anyways. And he is one of the very top specialists around. I think the technical term for what he is, is a Unicorn doctor. We all wish we had one, right?

So, our guest is Dr. Jeffrey Boris. He is a board-certified pediatric cardiologist and general pediatrician in private practice, caring for children and young adults with POTS, syncope, and other forms of autonomic dysfunction. He has been practicing since 1997 and caring for patients with POTS since 2002.

He has worked with patients who have multiple other diagnosis alongside their POTS, including joint hyper mobility, with or without Ehlers Danlos Syndrome, concussions, mast cell activation syndrome, Sjogren's syndrome, and, you know, all the fun that can go with POTS.

So, Dr. Boris advocates a creative multipronged and individualized approach to helping children get back to school, sports, and their lives. He has published research in acclaimed medical journals to help doctors better understand POTS so more physicians will be able to treat it and join research efforts to better understand this disorder, and he continues to perform and publish research today.

In fact, here at Standing Up to POTS, last year we awarded Dr. Boris a research grant to him and his colleague at Harvard, Dr. Bruce Crystal. So, we have been excited about that as well.

And in 2016, Dr. Boris was named physician of the year by Dysautonomia International. He was recognized for his warmth, compassion, and tireless commitment to his patients.

So, Dr. Boris thank you so much for speaking with us today.

02:35 Dr. Boris (Guest): Hi Jill, thank you for that very kind introduction and I'm glad to be here to talk with you.

02:41 Jill (Host): So, my first question is what led you to specialize in pediatric POTS and what keeps you doing it, because it sounds like such a niche-y little field, right? I'm guessing medical schools did not have any classes on pediatric POTS?

02:55 Dr. Boris (Guest): Any POTS, like not at all, right. Medical School was paid for by the United States Air Force. I was in a program that did that and they paid for my general pediatrics training and my pediatric cardiology training. And so, in exchange I was a pediatric cardiologist in the Air Force for seven years.

And in 2002 I had a young lady who came to see me who had lightheadedness and syncope and just a bunch of symptoms, and she wasn't behaving like sort of a typical patient with lightheadedness and syncope, despite my therapeutic attempts. And I had heard of POTS by then and I was wondering if this is this POTS thing. And so I looked in the medical literature and this name kept popping up of Blair Grubb, and so I cold called him and we talked on the phone for 2 1/2 hours, taught me a ton of stuff - we are still very good friends today - and that kind of started things off.

When I left the Air Force, I was in private practice for three years and took care of a few POTS patients and then left there and went to the Children's Hospital of Philadelphia in 2007. And at that time they had a lot of patients with POTS that were sort of diffusely seen through the system, or just not seen at all, and so I let people know that I could take care of these patients. They were very relieved, I guess to to have someone who had an interest.

I started seeing these patients and I recognized straight up these patients were way sicker than any patients that I had ever seen with POTS previously. Much more complicated, their symptoms were harder to get under control. So, I did a lot of talking to colleagues and a lot of reading and really worked to see what I could do to try to improve these patients' daily lives.

And, still loving the field of pediatric cardiology, the number of patients with POTS just kept going up and up and up and up. And so I finally said in 2013, like, OK, fine, I will be the POTS doc. And I went to my division chair and administrator and said, “Look, you know, I'll do this, but we need to do this on my terms. I need a nurse practitioner to work with me and to go out to our satellite clinics with me.”

So in January of 2014 we opened the POTS program at the Children Hospital of Philadelphia. And at that time, we had a lot of patients and my nurse practitioner, Tom, who many of your listeners may know, set-up kind of an informal spreadsheet sort of track our patients with..with permission. We turned that into probably the largest pediatric POTS database at the time in the country. We ended up getting almost 950 patients, and with that we were able to publish a fair number of papers for from a research standpoint.

So, to answer the second part of your question of what keeps me going, I guess the the two things that keep me going are: One, really being able to help the patients and their families to be able to understand this disorder and to be able to control it to get them back to school, back to sports, off to college, living their lives, doing their activities of daily living as best as possible. And the other half of it is the research part's fun, too, you know? I've always been kind of a fan of big data and so being able to utilize the large amount of statistics that we've been able to to capture through the electronic health record has made it really fun.

And I've been able to sort of highlight some aspects, I guess, of POTS that other folks haven't been able to look at.

06:44 Jill (Host): Yeah, and that's why we feel so lucky to talk to you today. So, maybe we can get into it because I have a feeling that when it comes to seeing the onset of POTS, you are seeing more of it and keeping track of it better than almost anybody.

And so, what can you tell us about that? Like, are there certain ages that, like, what's the youngest you see or what's the most common age you see? And what are the first symptoms that you tend to see?

07:15 Dr. Boris (Guest): Yeah, so we put out a paper looking at over 700 of our patients, looking at the demographics of those patients. And when we talk about sort of the typical age, we talk about either the...the average age or the mean age or the median age. In this case it was the median.

The median age of onset was about age 13. So yeah, a lot of the adult papers, you know, when they look at patients with POTS, they're already seeing these patients down the road, right? Because they haven't been seeing these patients at the time of onset. But we certainly were.

Lightheadedness, tachycardia, fatigue, headache are typically the big symptoms that occur early on. It's interesting to me - one of the things that I've been able to do recently is continue the research and we did - a long term outcomes study just last summer. We had over 225 respondents, which was fabulous. And what we see is, again, the tachycardia, the lightheadedness, the nausea, the headaches, the fatigue, those sort of things come on early.

But once you get down the road, the lightheadedness is often easier to control, but you still get a lot of the GI, and the neurologic stuff, right? So nausea we see a lot more, fatigue and brain fog we see a lot more, which is interesting how it sort of morphs or seems to morph over time.

As far as the earliest - so this is going to be a little controversial - as you and your listeners probably know, the official way of diagnosing POTS in pediatric patients goes down to age 12, right? Age 12 to 19 is sort of what the pediatric range has been defined as. That said, I have absolutely seen patients whom I believe to have POTS no differently from adolescents and/or adult patients, probably down to as young as age 6.

But, you know, not...not nearly as many as the older kids, but I do have a sense that these kids really do have it. It gets to be difficult from a diagnostic standpoint, because then you say, “Well, what are your diagnostic criteria,” right? And we're still - in 2022 - we're still sort of stuck with using either a standing test or a tilt table test and watching what the heart rate does.

And what has been found is that in children and adolescents, their heart rate goes up with upright position more easily, higher, than say...than, say, adult patients do, and so it it gets to be a little bit more problematic.

But you know, we still don't have a biomarker, right, a biological marker to say, you know, “Let's get this lab test. Oh, based on this lab test, you have POTS.” We’re just...we’re not there yet.

10:12 Jill (Host): Right, and it's silly to think that something magical happens at 12 years old where, OK, now you could have POTS, but you couldn't have it when you were 11 and a half.

10:17 Dr. Boris (Guest): Totally. In one of the other papers that we published, you know, using a 10 minute standing test, we showed that in patients who had a heart rate increase of 30 to 39 beats per minute versus those who had a 40 or more beat per minute increase on a 10 minute stand, there was no significant difference in the symptomatology and the symptom burden, it was all the same.

Unofficially, unpublished, I have looked at my data for heart rate of 20 to 29 and 25 to 29. And I do think, and again, this is...this is unofficial, unpublished, not reviewed, not even written up, but it does look like in those patients there may be a somewhat lower symptom burden.

But having said that, that's still, in my mind, that doesn't necessarily mean they don't have POTS. I think it just means that we still don't understand it particularly well.

11:14 Jill (Host): Well, yeah. So what happens to a patient who's having all the POTS symptoms but their heart rate only goes up 29 beats per minute, or I guess if it's a child 39 beats per minute? So they don't quite make the cutoff. So do they not get a diagnosis? Does it make a difference?

11:31 Dr. Boris (Guest): Sure. So I I think....it's kind of a long answer. Patients, parents - we all sort of hang on having a name for something, right? We need guidance, we need clarity, and it's hard if you say, “Well, you sort of make it, but you don't. And so, you know, what does that mean?”

Well, OK, the first thing that I would say actually is that we know that there's what's called diurnal variation in the heart rate response, which means that it depends on the time of day the standing test was done, right? So, if it was done in the morning, you're more likely to have a higher increase in your heart rate than if it was done in the afternoon. So that's one of the things to keep in mind.

Two, it's going to vary day-to-day. But three, it also in my mind, I think the therapy or the therapeutic approach for orthostatic intolerance is probably about the same. It is really about the same. You know, we're doing the fluid and solvent exercise and the non-pharmacologic therapies. And I don't have a lot of concern about utilizing medications that can help reduce the other symptoms, despite the fact that they might not necessarily meet the official criteria for POTS.

12:53 Jill (Host): So, do you notice anything special about the kids who get POTS, or do they just seem like normal kids to you?

13:01 Dr. Boris (Guest): So no, they they're really....this has actually been published at least once, but we notice it, too. A lot of these kids - not all of them - but a lot of these kids are very high achieving either academically or athletically or both, which makes it even more both urgent and rewarding to get them back to school, because that's what they want to do. That's where they get their...their best feeling is they're able to demonstrate what they really are able to do.

And so that's what makes POTS so much more devastating for these kids because they define themselves in these ways, right? They define themselves in part by being smart in the classroom or being athletic, and this is taking them away from everything that really that really helps them be them.

13:47 Jill (Host): So what on Earth is up with that? Why would something have this association with high achievement, 'cause you're not the first person to mention that, and I notice it. I enjoy it. I'm like, wow! I'm in this club where people just happen to be smart and nice, in addition to having a lot of similar life circumstances to me. But is it something about adrenaline or...?

14:11 Dr. Boris (Guest): I wish I knew. Could it be norepinephrine? If I was to make a completely uneducated guess, I would say that it might have to do with more with the receptors and where the receptors are than the actual chemicals. The way these folks' brains work, the way these folks' muscles work, but if it's so hard to say, you know, we and I think we're still having a lot of difficulty putting our finger on who gets, you know, who gets POTS.

I mean, in the United States, for example, we typically think of POTS occurring in females. We typically think of POTS occurring in Caucasian females, often with joint hyper mobility, yet how good is the access for folks who are African American or Asian or that kind of thing? And there's been fairly decent research coming out of China and Korea and Japan demonstrating that their Asian population gets POTS. And it's interesting - a lot of the stuff that they published tends to be closer to 50/50, male to female, that I recall.

Apparently, there's some new stuff coming out of India as well, that I've heard. It's still been hard to...to characterize what we've seen so far, at least in this somewhat narrower population of Caucasian females, is that they tend to be more high achieving in those domains.

15:33 Jill (Host): OK, so one more question about differences in kids: so, you know, there's some people who get POTS and they just have POTS and that's all that they ever have. And then the other side is people who start with POTS and then they discover they have the joint hyper mobility and the mast cell activation and maybe other things. Is there anything you notice that's different about the kids who snowball into more stuff versus only POTS?

16:08 Dr. Boris (Guest): Yeah, I think the first thing to keep in mind when we talk about POTS, and I think a lot of my colleagues in the field think this way as well, is that POTS may be kind of a common final pathway that may be associated with different conditions. In our long-term outcome study, it's kind of interesting, what we found is a little over half - 56% of patients - felt that their POTS started within three months of a specific event, right? So, like a trigger. That leaves a small number that also had sudden onset but didn't have it unrelated to any event and about...that's about a fifth of those patients.

The other 80% of the patients who didn't seem to have a trigger, the parents say, you know, there's...there's always been something, something, something going on, whether it's chronic headaches, whether it's chronic, GI symptoms, whether it's abnormal sleep. They...we've known they had joint hypermobility, but we didn't know it was this bad.

I think there is definitely something...we know that in the adolescents a growth spurt can trigger POTS. Because we've seen or many more females with it, we know that onset of menses can also be what we think is an associated trigger, right? So something hormonal. And I think that it's just so widespread in how we get there from here, you know, could it be that things like mast cell activation syndrome start with the hormonal changes also? Could it be that with more rapid growth in the setting of adolescence, joint hypermobility because the joint connections - tendons, ligaments, that kind of thing, you know, are they softer in the presence of the increased hormonal output? I don't know. I think it's hard to tell.

But you're right. I mean, we, we do see a lot of various comorbid disorders. I mean one thing, for example, you know median arcuate ligament syndrome or MALS - some folks have heard it. You know, why does that happen? And is that something that causes symptoms of POTS or is just associated with it? And, you know, and there are so many different things as you said - mast cell activation, joint hypermobility. Yeah, all of that.

18:24 Jill (Host): One of the comorbidities that you mentioned on your website is mitochondrial disorder.

18:30 Dr. Boris (Guest): Yeah.

18:31 Jill (Host): I think we have not spoken about that yet on this podcast, and do you mind just saying what that is and what it looks like?

18:40 Dr. Boris (Guest): Sure. So, to bring us back to high school biology, the mitochondria are the powerhouses within our cells, right? So they take the input of things like glucose and fatty acids and those kind of things and they are able to turn them into energy that our cells use to be able to do the things that we do on a daily basis.

So, in mitochondrial disorders there can be a genetic abnormality either in the mitochondria, and I think a lot of people don't necessarily know that mitochondria have their own DNA, interestingly enough, right? So they have their own DNA that can have abnormalities there, or there could be an abnormality in the cell nucleus that houses the mitochondria that puts out a protein that's supposed to help regulate the way the mitochondria works, and that mitochondrial protein is messed up, OK? So either way, we have disorders of the mitochondria.

And these patients also can be at risk for developing symptoms of autonomic dysfunction. With the group that I'm working with right now, we did a survey of patients, both adult and pediatric, with various mitochondrial disorders, and we did that a couple years ago. We still haven't published it yet, but we're putting those manuscripts together.

From the pediatric section, there's all kinds of different mitochondrial disorders. Very interesting to me that the patients who have mitochondrial disorders, as compared to those with POTS, their severity - although it can be significant and interfering with their activities of daily living - their overall severity is not necessarily as bad as those with POTS. Their overall symptom burden is not necessarily as bad as those with POTS. And the kind of symptoms we tend to see are a little bit different. So they tend to have somewhat less of the orthostatic type symptoms. They tend to have more of the GI dysmotility, so that feeling full early, constipation, nausea, abdominal pain, that kind of thing, and they also tend to have a lot more of the heat intolerance as well.

It's really interesting comparing these and the number of symptoms that these patients have, but it definitely seems to be a difference.

21:05 Jill (Host): So that's interesting to me, because when I think about mitochondrial disorders, I think, “Oh, here's a body having trouble making enough energy at a cellular level.” So then my mind starts going, OK, so why would those people have more GI problems? Do they just literally not have the energy to do it or I suppose nobody knows?

21:25 Dr. Boris (Guest): So, there have been a few papers published historically on associated autonomic symptoms in the setting of mitochondrial disease. Most of the time they look at the autonomic nervous system symptoms in the setting of one specific disease, or they look at one symptom over the setting of multiple diseases, right? It's either tall and narrow or short and wide, and what we tried to do was kind of increase it in both directions with that.

So, to answer your question, you know, why would they be more at risk for GI Dysmotility? Maybe, I think again, it's one of those things that we don't have great insight into. Does it make sense that they would have GI dysmotility? Sure. But then if that's the case, then why wouldn't they have other issues from a from a muscular or musculoskeletal standpoint, right? Their heart or their skeletal muscles, and I think it's not to say that they don't have tachycardia and lightheadedness and fatigue and brain fog. They certainly do, but it just seems to be less overall.

22:32 Jill (Host): OK. So I have a couple questions for you that came in from listeners and I think they're parents. So the first question is what are the chances that my kid might just grow out of POTS? Because I think that's a meme out there, the thinking that a lot of kids just do, so you don't have to really worry about it. How often do you see that?

22:55 Dr. Boris (Guest): I think it's more than a meme. I think in my putting together our long term outcome study, we recognized six papers that have been published on long term outcomes: three from the United States, one from Portugal, one from Korea, and one from China, OK? And it's interesting in reading these papers because they get really rosy, they say, “Oh, these patients do well and these patients, you know, are able to be functional and blah blah blah.” And not that I wanted to be a Negative Nelly, per se, but I wanted to have a clear-eyed view of how our patients are really doing.

And so we've looked at it in in multiple ways. We looked at it from an education standpoint, from an economic standpoint, from a social standpoint, and from the symptomatic standpoint. What we found in our long-term outcome study, and I want to warn our listeners that none of this in our long-term outcome studies - right now we have 4 manuscripts so far - they have not been submitted to a journal, they have not been published, they have not been reviewed. So this is just me kind of talking off of what we've found so far.

Specifically, when we looked at symptoms, we looked at did you have these symptoms? How bad were these symptoms when they were their best, their worst, and did you have these symptoms in the last month and how bad were they in the last month?

What we found out of 215 patients who responded to that question, 213 patients - so over 99% - had symptoms in the last month. Now it doesn't mean they weren't functional, right? It doesn't mean they weren't able to go to school or do their education with...with homebound or cyberschool, that kind of thing. It doesn't mean that they weren't able to go to colleges and they weren't able to work, although a number of patients do have that hanging over their heads as an impediment.

But I think what we need to recognize is, we again need to be clear eyed about this, that despite the fact that there are many things that we can do to help reduce the symptoms of POTS and to help allow these patients to be functional and to do their activities of daily living. In the large majority of patients, we have not been able to figure out how to make POTS go away, period. End of statement.

25:18 Jill (Host): I think probably most listeners suspected that.

25:22 Dr. Boris (Guest): That's awful to hear. As a parent myself, I mean, I, you know, I look at...I look at my kids and you're like, you put your heart and soul into raising these kids and you're...and you want them to have fabulous things for their future and to be able to change the world, right? Be... be the next awesome human in our society. And you look at a disorder like POTS that, at least at minimum up front, completely derails that and can cause people to lose hope.

But again, the good news is when people are able to get with a provider who knows what they're doing to try to help them reduce their symptoms, to try to help them find those other comorbid things so those can be addressed, too, it is possible to do good things. A fair number of my patients went to college, proportion of them went to grad school.

This was fun - when I was still at Children's Hospital of Philadelphia, which I left in 2019 and then it started my present private practice, when I was still at CHOP, I ran into easily three or four of my former patients, who became pediatric nurses at CHOP, right? I mean, I think that's just so cool where they were able to, you know, to make it work for them, and not only that, but...but go into the field of health care.

I'd be very curious to see what kind of fields people do go into. I don't think we really asked that a lot, but I do get a sense that a number of patients do go into some sort of health-related field. I had....I had a number of patients who went to medical school, nursing school, social work, physical therapy. I thought that was kind of neat.

27:02 Jill (Host): That's great, yeah, like they're finding meaning and purpose in the whole ordeal, at least.

27:07 Dr. Boris (Guest): Well and you know, I think we all want a job that gives us meaning and purpose but helps us relate to who we are and helps us use our experience to help other people to be able to move on, and specifically in the healthcare field, being able to bring that to bear to help guide people and and help them feel better is truly rewarding.

27:30 Jill (Host): So, you had mentioned that’s the experience you have when you work with probably a great doctor. Do you get patients who have been misdiagnosed in the past? We had one parent asking a question about what are the most common misdiagnoses that a child might get if they're showing POTS symptoms, but their doctor does not know about POTS?

27:54 Dr. Boris (Guest): Yep. One of the things that we see often in fact in our long-term outcome study, we saw that...so first of all, nearly 3/4 of patients before their diagnosis of POTS had a doctor who said, “You have a physical illness, but we don't know what to do about it.” But easily 2/3, 65% were told that they had a psychological problem, right? Depression, anxiety, conversion.

Over half - 55% - were told that their symptoms were all in their head and when only about 1/3 of them really were, in their own opinion, suffering from a psychological or psychiatric problem, right?

So, the misdiagnosis that we see or anxiety and panic, depression, conversion disorder. Sometimes we see fibromyalgia, amplified musculoskeletal pain syndrome, or reflex neurovascular dystrophy, chronic regional pain syndrome, post-concussion syndrome as well.

And then the patients who specifically had GI dysmotility and have a hard time eating, get full early, have abdominal pain, that kind of stuff are not infrequently diagnosed with eating disorders too, right? We see that all the time. So, it's hard.

Another study that was previously published demonstrated - this was the that from the big POTS survey - demonstrated that half of patients had at least 7 providers before they got a diagnosis of POTS, and over 1/4 of them had over 10 providers before getting a diagnosis of POTS. You know it's just difficult, it's just difficult.

And then you...and then you also get the providers who say, “I don't believe that POTS exists,” or they're like, “I've never heard of it,” or they're like, “OK well, I evaluated your heart and your heart's fine, so I don't know what to do with you,” or “I evaluated your GI tract, and I can't find anything wrong.”

There's a lot of blame the patient. There's a lot of “I can't find anything and I can't take the time to look any further, so it's your problem, and it's probably your fault too, so it's probably psychiatric. Have a nice day.”

And it's worse because so many patients are female, right? And through time, this is not the first time this has happened. You know, you go back in the annals of medicine, you look at diagnosis like hysteria. Women through time have been discounted in their diagnoses all the time - adults and kids. You know, it's even worse when they're kids, right, 'cause kids, you know, seen not heard. So it makes it more difficult.

On top of that, then you're dealing with parents and the parents at first blush, take what the providers say, but they're like, “You know my...my kid’s not well, they're not normal. I know this.” And so they have to become advocates for their kids.

And after a while, the doctors start pointing fingers at the parents. “What are you doing wrong?” Or “why are you so wrapped around the axle with this?” It makes it very difficult for them.

31:04 Jill (Host): I have wondered what's different - if there's doctors who tend to believe patients and then there's doctors who tend to not believe patients - and I've wondered if there's a lot of patients out there doing something to their doctors that makes them learn not to believe patients. Do you think there's a lot of patients who are, I don't know, just saying false things or trying to get out of work or trying to get out of jury duty...[Laughs]…or I don't know. Are there...are there things patients are doing that are making doctors predisposed to not believe them? Or what...what is?

31:40 Dr. Boris (Guest): So, don't get me wrong, I'm gonna have to be very careful when I say this. There is a very small proportion of patients, with any disorder, who either do have a psychiatric disorder that may be causing issues that they are hyper focused on, symptoms that they're trying to get some sort of aspect of secondary gain.

Do I think that's what's happening in patients with POTS? I will tell you I have seen, I mean, your readers may be familiar with the concept of something called Munchausen syndrome or Munchausen syndrome by proxy, where the patient or the patient's parents do something that makes it seem like there is a medical disorder going on. I've seen it in two patients. You know, does it...does it happen? Sure. But that is far and away the minority. I think these patients have better things to do. These patients would much rather be showing off their....their academic skills or their...or their athletic skills or just hanging out with their friends and being normal.

Do I think that there is some what we call ‘habituation’, you know, getting used to being sick? I mean, I guess, but that's...that's not what these patients want to do, and that's not what these parents want them to do.

And I do recommend to all my patients that having a chronic illness is tough and we don't have the tools in our toolbox necessarily to help manage chronic illness. So, do I think that counseling for managing long term illness - chronic illness - is important for everybody? Absolutely. That doesn't mean you're faking it. It doesn't mean you're making this up. It doesn't mean that you're making it worse. And, you know, the number of patients that I'm sure have felt like or don't even know that they've been gaslit is huge, unfortunately.

33:33 Jill (Host): Yeah. Probably most parents out there are not going to be able to take their kids to see you [Laughs] or a top pediatric POTS specialist, 'cause I don't even know if there's other like two of you around or not very many, but do you have any advice for parents of young POTS patients how to get the best treatment?

33:56 Dr. Boris (Guest): Oh, that's a hard one, right? Because I think there's amongst us providers, whether adult or pediatric, there is significant variation in the way doctors practice. I think there are some providers like myself who feel comfortable managing most, if not all...well, I have to be careful with the concept of “all” - I'll put that in quotes - of the symptoms and associated comorbidities with...with POTS. And that's something that I learned over time. I taught myself. I'm still learning. My patients still teach me stuff in 2022, and I'm...I'm better for it.

And then there are other doctors who were like, ‘Well, I'm just a cardiologist. I can only take care of the cardiac issues and I don't feel comfortable taking care of the neurologic issues, the GI issues, the pain issues, the orthopedic issues, that kind of thing.”

I do know that so many of these patients and families are well connected on social media, and so I hate necessarily that folks feel like they have to do “Doctor Google,” if you will, to talk with folks because everyone is different. I feel like people need to have a personalized approach to their care. And I think we have to be careful when someone says, “Well, this worked for me, so it should work for everyone.” It's not true, OK, because your kid is not necessarily the same as someone else's kid and having the same problems and that kind of deal.

There are lists of providers out there that exist on the Dysautonomia International website and, full disclosure, I'm on the medical Advisory Board for Dysautonomia International, and no, they're not paying me.

Beyond that, I think it's so much of it still is word of mouth. You know, who helped you? Who was good, who was not? And truth be told, I've been fired before, too. A lot of times. You know, people just didn't find it with me, and from my standpoint I don't care. I just assum the kid get the care that he or she needs to feel better. It doesn't have to be from me, right?

Beyond that, I wish there was a really good way to tell in the bigger picture of medicine. I think it's hard to tell who's a good surgeon, who's a good internist - think that's just hard to tell. Just because they've gone to a specific school and had this kind of training and been practicing for a number of years, that...that doesn't tell you squat.

36:34 Jill (Host): [Laughs] I learned that the hard way. I wish I heard you about 20 years ago. [Laughs]

36:39 Dr. Boris (Guest): Yeah, it's just rough. And again, part of the reason why people bounce around from doctors to doctors. And then these patients get accused of doctor shopping. I am licensed in Pennsylvania and in Colorado, and from a telemedicine standpoint, because my practice is only telemedicine, I can see patients in those two states. And then also the telemedicine laws are kind of interesting. There are three states - Minnesota, Michigan, and Ohio - that if you have a primary or specialty care provider in that state taking care of you, they can request a consultation of a out of state licensed provider who, as long as they don't quote "practice too frequently” in that state, they can do a telemedicine consultation.

37:22 Jill (Host): OK, great, well I could just talk to you all day and you are so full of knowledge and I appreciate it so much.

I have one more question for you: POTS has so many different symptoms, and I'm wondering if there are any that you particularly try to focus on with a child because it might affect their development, right? Like is there any symptom where, like, Oh yeah, I don't want that kid growing up with chronic pain or bad GI issues, or is it all just different? Or I'm just wondering for the parents out there, is there anything that can happen in POTS that you need to really get on top of as a priority because it could affect the kid's development?

38:09 Dr. Boris (Guest): That's a fabulous question. I haven't really thought of it that way, so I'm going to answer that in two ways.

The first is I think, you know, my approach is to be able to control the symptoms, both from a non-pharmacologic standpoint as well as pharmacologic if needed, so that the patients can then get to exercise because we know that for most but not all patients, exercise can help suppress and really reduce symptom burden so that they can be more functional, right? Again, that doesn't work for everybody and I and I recognize that.

So, I try to just get on top of things – everything - you know, when we can. What I'll do is I'll ask “What are your three worst symptoms? And you know, three or fewer worst symptoms?” That said, to your question specifically, and it's an interesting thought, we know from a brain development standpoint that chronic pain causes literally physical changes in the brain. And that's whether you're a kid or an adult. Now in a child, the brain is not finished developing until between ages 21 to 25.

And I don't know if we know enough to say, “Well, if we control the pain, will those changes revert?” I don't know that, but we do know that there are physical manifestations of chronic pain in the brain. So I think that's a thing.

I think the other thing, too, that I think about when I'm dealing with my patients is because I want them to be able to take in at minimum the fluid and the salt and that kind of deal to be able to have appropriate nutrition, getting GI symptoms under control to allow them to do that can be really important as well.

So, so the chronic pain thing, I mean, that can be the pain of GI, the pain of headache, the pain of small fiber neuropathy, the pain of joints in joint hypermobility, and so addressing those specific aspects I think is important.

You know, fatigue and brain fog are certainly come to mind because from, again from a development standpoint, those can be significantly interfering with...with the way these kids are able to function. I'm very gratified that when we published our data on the use of medications for the treatment of fatigue and brain fog specifically, that we had about a 69% effectiveness rate, which was much better than, say, therapies for lightheadedness, nausea, insomnia, pain, headache, GI dysmotility, which ranged from about 38 to 52%.

40:57 Jill (Host): Oh, that's interesting.

40:59 Dr. Boris (Guest): Right? So the good news is we do have tools that can make a difference for these kids. There needs to be, you know, more studies obviously, what we call ‘prospective studies’. In other words, not looking back, but looking forward and seeing what happens in the future.

41:13 Jill (Host): I think your website it talks about prioritizing just getting kids back to their life and back to their sports and back to their activities. And so maybe, it sounds like that's kind of the thing to make sure that they're not growing up without?

41:27 Dr. Boris (Guest): I think the thing to keep in mind there that, again, is that it's very personalized, right? So some patients when they say to me, “My three worse symptoms are headache, insomnia and nausea, those are the three things that are interfering with my activities of daily living the most, and that I would like to be able to get control of the approach there,” in some ways is going to be different from those that say, “Oh, it's the Lightheadedness, the fatigue and brain fog and, you know the pain of say, small fiber neuropathy or...or MCAS symptoms, or that kind of thing.”

42:01 Jill (Host): OK, sorry I lied. Can I ask you one more question before we go?

42:04 Dr. Boris (Guest): As many as you want.

42:06 Jill (Host): So, in the very beginning, you had mentioned that you started seeing more and more POTS patients, and I wasn't sure if you meant that you think there's a rise of POTS in the general population, or if they're just finding you more. Do you have any thoughts on that?

42:23 Dr. Boris (Guest): I was seeing more and more POTS patients because my colleagues in cardiology, in neurology and gastroenterology, in our diagnostic center were like, "You're familiar with taking care of them. You're comfortable taking care of them. All yours, have fun.” Right? And I'm like, “Bring it.” OK? So in part, that's why the volume went up.

Do I think that there is an increased prevalence of POTS in the general populace? It's a definite maybe, I think yes. If I was to really say that it's higher, I think it's being finally and due to the attempts of groups like Standing Up to POTS and like Dysautonomia International and a number of other groups who have really tried to advertise its existence who’ve really tried to work with providers and kind of get the word out there.

People are more aware of it. Having said that, I think it's also in some ways being overdiagnosed. I think there are a number of providers who understand just enough to be dangerous, and so they won't do a 10 minute standing test. They'll get a list of histories, they'll have them go from laying down to standing up for two minutes, “Oh, your heart rate went up too fast, you've got POTS. Go see someone else.” And in reality they might have something called initial orthostatic hypotension. They might have sort of typical mild orthostatic intolerance, which we often see in adolescence, but it's not POTS, right?

So I think it's difficult to say. But I think, and in talking with some of my colleagues, I think we kind of thumbnail it to say there probably is somewhat more POTS going on. I'm sure a bunch of your listeners are familiar with the fact that there is a thought that at least some or even most of POTS may be autoimmune, and I think there is also a recognition that there is an increased incidence of what we think are autoimmune disorders in general.....in the general population.

So, it's one of those things about, like all boats rising with the tide, right? So, there may truly be more patients with POTS as well.

44:34 Jill (Host): Yeah, interesting. Well, Dr. Boris thank you so much for taking the time to speak with us today. We really appreciate all the amazing work you do, both working with patients and in your research, and we can't wait to have all of these publications come out. And we didn't talk about the research that you're doing with Standing Up to POTS, but I think that's not going to be ready for a little while anyways, so maybe we can get you to come back sometime, but you're just a wealth of information, so huge thanks for all that you do.

45:05 Dr. Boris (Guest): Well, thank you very much for inviting me. Just a quickie on the Standing Up to POTS stuff - it's probably going to be another year. We're probably about...about halfway through with our data analysis and already some really interesting things are coming out, so can't wait to share it with everybody when...when we kind of get a better sense of what else is going on with our data.

45:24 Jill (Host): And to anybody listening who has donated to Standing Up to POTS, this is the kind of cool research that you're funding, so thank you.

45:32 Dr. Boris (Guest): Absolutely.

45:33 Jill (Host): Yeah, thanks a million. This has just been great.

And hey listeners, I hope you enjoyed today's conversation. We'll be back again next week. Until then, thank you for listening. Remember, you're not alone. And please join us again soon.

45:48 Announcer: As a reminder, anything you hear on this podcast is not medical advice. Consult your healthcare team about what's right for you.

This show is a production of Standing up to POTS which is a 501(c)(3) nonprofit organization. You can send us feedback or make a tax-deductible donation at www.standinguptopots.org. You can also engage with us on social media at the handle @standinguptopots. If you like what you heard today, please consider subscribing to our podcast and sharing it with your friends and family. You can find us wherever you get your podcasts or at www.thepotscast.com.

Thanks for listening.

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[Transcriber’s note: If you would like a copy of this transcript or the transcript for any other episode of the POTScast, please send an email to volunteer@standinguptopots.org]