Episode 64 - POTS in Kids with Dr. Maxwell

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 beautiful people who care about POTS patients. I'm Jill Brook, and I'm so excited for today's episode of the POTS Practitioner because our guest is someone whose work I have admired immensely. Today we are speaking with Doctor Andrew Maxwell who is a pediatric cardiologist in the San Francisco Bay area. He is double board certified as a pediatric cardiologist and a pediatrician. He received his medical degree from the one and only Johns Hopkins Medical School and completed a residency in pediatrics at the University of California at San Francisco. He followed with training in pediatric cardiology at Stanford Hospital. He pursued additional training in thoracic organ transplantation, which sounds pretty impressive to me, at Children's Hospital of Philadelphia. Finally, he returned to Stanford University for additional training in intensive care cardiology, non-invasive and interventional cardiology, and he has won awards for his prolific research. He also kindly volunteers on our Medical Advisory Board here at Standing Up to POTS. He is a top top expert and thought leader on POTS and you should really check out his presentations on YouTube, some of which are truly brilliant for how they synthesize so much information relating to POTS and related comorbidities. Dr. Maxwell, thank you so much for taking the time to be here today.

02:05 Dr. Maxwell (Guest): Well, it's a pleasure, Jill. Thank you for having me on.

02:08 Jill (Host): So, I know your expertise goes way beyond just cardiology, but I was wondering if we could start with a few questions about POTS and the heart which our listeners have sent in. Would that be OK?

02:21 Dr. Maxwell (Guest): Sure, yes.

02:23 Jill (Host): OK. So the first question is just in POTS - is there anything wrong with the heart itself or is the heart just reacting to other stuff going on?

02:34 Dr. Maxwell (Guest): Yeah, no, there's absolutely nothing wrong with the heart itself, and the best way to think about POTS is that this is a condition of pre-cardiac failure. So, some people think, “well, maybe you know there is heart failure with POTS.” And no, the heart is... the heart muscle is working perfectly fine. The ability to generate a good cardiac output is there - it's potentially perfectly fine. But this is pre cardiac failure. In other words, it's the problem of the return of the blood just before it enters the heart to actually make it into the heart.

03:13 Jill (Host): So it's kind of like the blood pooling problem that we talk about.

03:16 Dr. Maxwell (Guest): That's exactly right.

03:18 Jill (Host): Pooling instead of going back to the heart.

03:20 Dr. Maxwell (Guest): That's exactly right. I mean, the blood should return to what's called a central venous pool - that's just before it enters into the heart. And there are various different problems that can prevent it from being there to be ready to enter in the heart. Some of those are an autonomic nervous system failure, and some were actually structural issues with the connective tissue. And so, it allows blood volume to hang lower in the body than it should, particularly when gravity is involved in an upright person. It will sag down and hang lower, and it's not ready to fill the heart.

03:58 Jill (Host): OK, so maybe that's related to the next question, which is how does deconditioning change the heart? And how does exercise change the heart?

04:10 Dr. Maxwell (Guest): Deconditioning and exercise training or two sides of the same coin, and there's many many different effects on the cardiovascular system that goes along with exercise conditioning or aerobic conditioning. But if we limit the discussion to what's going on with the heart alone, then the probably the most important change when it comes to the heart is the ability of the stroke volume of the heart to increase with increasing exercise. You can imagine that the concept of cardiac output - how much blood flow is delivered to the body per minute - that is basically the equation: stroke volume x heart rate. So you have to have a good stroke volume, otherwise you're going to have to compensate with a higher heart rate. So, stroke volume is where the problem lies. And as you're conditioning and becoming more and more physically fit, you're training the heart to increase its stroke volume. And that's... a lot of that is through what's called “the compliance of the heart” when it's relaxing and opening up and allowing more blood to come in. So, you're training your heart to have more compliance. And then of course, having a good strong stroke to deliver that larger stroke volume. And so the flip side, of course, is deconditioning where you lose that ability. Now, there's a difference between the deconditioning of a healthy person and that of someone who has POTS. So, both of them have trouble getting the blood into the heart to have that stroke volume. But an otherwise healthy deconditioned person loses that compliance through deconditioning, so that even if there's plenty of blood to enter the heart, the compliance isn't there to relax and allow that volume to fill to give a good stroke with each heartbeat. That's as opposed to someone who has POTS, who they could have a very good compliance, their heart can be ready and waiting to open up and allow lots of blood to fill it, but the blood is just not there ready to fill it because of that sagging pool. Now, over time, when a patient with POTS and doesn't have that blood volume to fill that well-conditioned heart, you're going to also lose that and a patient with POTS is going to have the same kind of deconditioning of an otherwise healthy deconditioned person. They're going to lose that compliance as well. So, they’re going to have a loss of stroke volume for two reasons. So that's why it's so important that a patient with POTS does the exercise prescription, usually the typical like Levine Protocol so that they continue to at least have the conditioning of the heart that a healthy person should have. [Transcriber’s note: the Levine Protocol is an exercise protocol developed by POTS specialist, Dr. Ben Levine.] They should continue to to strive for that good compliance and stroke volume. And if you think about how that's best achieved, you’d understand why the Levine Protocol is something that's useful, that if you lie in a recumbent position, such as in a swimming position, then that volume that's trying to enter the heart is ready to enter the heart and it will fill the heart and allow a good volume, and then your regular conditioning of regular exercise conditioning can occur.

07:46 Jill (Host): That makes sense. OK. So does that have anything to do with this hubbub that came up a few years ago about the Grinch syndrome? And I think you probably are familiar with how I think on the TV show Jeopardy!, they brought up the question.

08:05 Dr. Maxwell (Guest): The Grinch heart.

08:06 Jill (Host): This is known as the “Grinch syndrome.” They were referring to POTS, and I think they were referring to a study that came out a few years ago. Do you mind talking about that, and is that real?

08:18 Dr. Maxwell (Guest): Right, so the question would be, is the heart of a POTS patient smaller than a typical otherwise healthy person? And of course, that the answer is no. And I think that was a misunderstanding that arose from the way this was described during a study. The bottom line is no, the heart of a patient with POTS is the same size as anyone else’s heart. And the issue, again, was that the stroke volume is less, so anyone would say, “Well, if the stroke volume of the left ventricle is less than the left ventricle, must be smaller.” No. It's again how much blood is coming in and allowed to fill that ventricle to have that stroke volume. If the blood is there to fill the ventricle, it's going to be the same size as anyone else's heart.

09:08 Jill (Host): Ah, OK. That answers the question. OK, so the next question we had was what do we know, if anything, about chest pain in POTS? Do we know what causes that or how many people get that?

09:23 Dr. Maxwell (Guest): Right. Well, I mean we should probably back up and say whenever anyone has chest pain and say they have POTS or not, but those who have POTS as well, they probably deserve a full evaluation to try to understand the nature of the pain. And all the normal common things should be thought of and considered first and ruled out or ruled in. For young people, we think less of coronary artery disease causing angina and chest pain. Certainly, the most common cause of chest pain in a young person. During exertion, the ones we worry about most would be from the lungs, something like reactive airways. So, we want to make sure that the lungs are perfectly fine and so I always exercise test all my patients - all my POTS patients - and not making sure that their lung function is good and usually it is, usually that's not a problem. But sometimes it needs to be addressed. So, once we ruled out that then we start to focus a little bit more closer to the heart. And again, there's some things that are less likely, but need to be considered. Most of your audience was probably aware that POTS is often associated with mast cell activation syndrome and being that, I always consider a phenomenon called Kounis Syndrome – K-O-U-N-I-S Syndrome, and that is basically an allergic spasm of the coronary arteries. And I've seen that plenty of times where patients actually get relief of their chest pain with anti-mast cell meds, even in the face of their lungs working well. So the mast cell meds aren't working on bronchi or lung function. It's working on something and I think it's the coronary vessels are relaxing and opening up. Now, with respect to POTS itself, there was a study done in 2016 at the Brigham and Women's that showed that the exertional dyspnea and chest pain was due to this phenomenon of the poor ventricular filling. If you could not have good ventricular filling as I've been describing earlier, then the very first symptoms of that is an exertional dyspnea and chest pain along with that. So, that would be the number one cause that's directly related to POTS. Fill that ventricle and, you know, all the different strategies we have for that, and usually the exertional dyspnea improves and the chest pain improves.

11:50 Jill (Host): OK, that's so good to know. And you know when it comes to chest pain, I always remember - so I used to work at Caltech and we would get a once a year CPR certification and the first time I did it with a group of Caltech people, the CPR instructor said it's very important that anytime you have chest pain you get it checked out by a physician. And everybody in the room started cracking up because I think it was a group of people under a lot of stress, working very hard, and they felt so much chest pain so often that the idea that they would get it checked out every time they had chest pain was funny to them. Can I ask for your reaction as a cardiologist to that?

12:34 Dr. Maxwell (Guest): Yeah, well I would hesitate to think that anyone would have chest pain from just stress alone unless there was an underlying issue. So, I would say at least get it checked out once. If the doc found nothing wrong with you after looking pretty good from your chest pain that you think is stress related, then maybe you can relax a little bit at that point. But I wouldn't just brush off chest pain with stress.

13:00 Jill (Host): OK, that's good to know 'cause I think sometimes we tell ourselves to suck it up and be tough and so, OK listeners, you heard it. Get it checked out if you have chest pain. [Laughs] OK, this next question is also a great one. Does orthostatic tachycardia - for example, if you just have to stand still in a line for a while - can that count as a POTSie’s cardiovascular exercise for the day? Like, is that actually a productive good workout for the heart?

13:32 Dr. Maxwell (Guest): No, of course not. You know, the tachycardia is coming as a compensatory mechanism to the lack of the stroke volume. And yeah, it needs to be there to help with cardiac output. As I said, stroke volume times heart rate is cardiac output. Stroke volume is not there, then the body tries to compensate by increasing heart rate, and that's through sympathetic overdrive, increased adrenaline that's going to make your heart go faster. In reality, it doesn't matter how fast your heart rate goes, if there's no volume to fill the heart every time it beats. And so the tachycardia, either succeeds in keeping you conscious or it doesn't succeed and you end up fainting anyway. But, the fact that your heart is racing is not going to be much help with exercise conditioning. Where your conditioning really needs to take place as the opposite. We need to work on the stroke volume and so with all the different strategies we have. In addition, you know, you're spending a lot of time forcing your body to be in sympathetic overdrive, and there's a lot of other consequences besides fast heart rate – insomnia, anxiety, and then what I see in my patients, and I think will become more of a known phenomenon, is subtle hyperventilation. So everybody sits there quietly breathing faster than they should because of this phenomenon of sympathetic overdrive. And if you were to measure their entitled carbon dioxide at any given time while they're awake, it will be driven down to the low 30s, and that's not good. And it’s not good for a lot of reasons. First of all, you’re on the edge of a panic attack because you can feel your fingers and toes tingling and having some loss of sensation, and that's because you're hyperventilating. But on top of that, the blood vessels to the brain – the most sensitive determinant of blood flow to the brain - is your blood carbon dioxide level. And so, you’re blowing off CO2 and constricting down blood vessels that feed your brain. So not only is your cardiac output not delivering blood enough to the highest part in your body – your head - but your blood vessels are working against you, by clamping down. So you've got two reasons among another four or five reasons for brain fog. So not a good way to try to get your exercise.

16:03 Jill (Host): OK, good to know, because I actually know some people who kind of figured that a trip to the DMV where they knew they were going to have to stand there for 20 minutes was going to count as some sort of good exercise. But that is interesting about the carbon dioxide, and that would be fun to dig into more at some point. You know, heart health, of course, gets a lot of attention as people age, and so we're wondering, do you think that POTS patients have any special considerations about heart health as they age? Or should they just do what everybody else does around heart health?

16:37 Dr. Maxwell (Guest): Yeah, I mean, I've been thinking about this question and I I really can't think of anything that a POTS patient has to do differently as they age that anyone else. And of course, maybe that's because I haven't spent a whole lot of time at that end of the spectrum of age, I've spent most of my time watching patients go from, you know, early pediatric to into adulthood and all the phenomenon that go with that, but you know, maybe I need to consult with my adult cardiology POTS people to see what they see with that.

17:15 Jill (Host): Well, I'd love to ask you more about what you just said that you see the beginning side of POTS. So.... so maybe you could tell us like what age kids you tend to see? And.... and I imagine that you see POTS onset more close up than doctors who work with adults. So what does that look like to you?

17:35 Dr. Maxwell (Guest): If you've seen some of the videos out there that I've done, I consider myself the embryologist or the pediatric cardiologist and maybe pediatric neurologist who consider themselves the embryologist of dysautonomia, mast cell, because we're there pretty much from the very beginning, watching it evolve through time. But I see.... I've come to the conclusion that, you know, there's many, many different ways that dysautonomia starts. Sometimes it starts completely de novo, where it's nothing but pure health prior and then suddenly the onset of dysautonomia and POTS. But a lot of times there's an underlay of mast cell activation syndrome that predates that, and then of course you know there's an association with Ehlers Danlos. And so, the number of patients that you realize, “Oh, you know what? There was a a predating of Ehlers Danlos issues prior to the dysautonomia and POTS is now becoming evident.” Other parts of the history are being pulled out that you didn't know before, and now I can't really say this was a pure onset of dysautonomia POTS. I see that again and again. I used to think that the concussion - onset of POTS following a concussion - was kind of the perfect example of the patient to follow and watch for that... what we see with the evolution of pots de novo, because, OK, you have a perfectly healthy... say, athletic person that it took a hit to the head. Now the next day they suddenly have onset of POTS and everything that follows, and so this was an ideal way to study POTS by itself in isolation, and I learned a lot from that. You know, I would say that those patients kind of have a course that they all take and tend to resolve over time, but at some point I realized that a lot of these concussions were only occurring because of their underlying Ehlers Danlos. These patients actually had a connective tissue issue that probably allowed for much more jarring concussion than they otherwise would have had, had they had not had the connective tissue disorder. So, then it complicated things as far as saying, OK, concussion was the perfect way to look at POTS de novo in isolation. So, that always has to be considered – is a concussion only a big deal because they have underlying Ehlers Danlos? I think there's a lot of other, you know, a lot of other learning experiences that can arise from kind of being embryologists as well, and I think I've talked about these in other... other places as well. And I'm constantly learning. I think my other pediatric colleagues are also learning from this as well.

20:34 Jill (Host): And your ‘Embryology of POTS’ is the video that I would recommend that all our listeners go Google it and watch it, because there's so much information in there that we could never even start to get into it right now. But, yeah, I really appreciate how you are putting together the various issues like Ehlers Danlos Syndrome and mast cell activation and autoimmunity and other things, and how these things might fit together.

21:01 Dr. Maxwell (Guest): I wasn't the one who came up with the idea of connecting the dots between them all, but I certainly have started to focus a lot on how the dots are connected. And, boy, after time you realize there's an infinite number of ways they can all be connected and feedback on each other and reconnect. So, there’s definitely not one way that a patient becomes a POTS patient or a mast cell patient, or Ehlers Danlos patient. It's very, very interesting in that respect.

21:32 Jill (Host): As a teaser to our listeners, hey listeners, after watching this video I eat a lot of cabbage and there's some good reason why you might consider doing it too. [Laughs]

21:43 Dr. Maxwell (Guest): All right, yeah, good point. They’re going to be asking why that is next.

21:47 Jill (Host): [Laughs] Watch the video! So, does any of this help you figure out, like, which kids just grow out of it, and which don't? And do kids just grow out of it? Like, that was a meme that we heard a lot a few years back? In fact, there's kind of this idea that most kids just grow out of it, and what's your experience? And have you noticed any trends in who grows out of it or who doesn't?

22:12 Dr. Maxwell (Guest): Well, it's still kind of an open... open question to me. You know, I've over the years taking care of a lot of patients that seem to have gotten better, and I've had patients that it was not that difficult taking care of their dysautonomia. They come in saying they're struggling at school. They had to drop out of sports. But then I put them on a little midodrine and teach them about all the lifestyle changes and suddenly they’re back running cross country and very successful. And, you know, the plan is, OK, we probably we probably just need to ween you off of this as you developmentally grow out of it. Certainly there are those cases. But some of these patients at the end of high school. They're coming back and still saying, “I still need my midodrine. Why is that?” And so, that's kind of made my thinking evolve on well, not all of these patients I thought were going to be so easy and just recover are. And in my practice, what I believe is a underlying environmental exposure issue that a lot of these patients have. And so, sometimes them moving on to a new environment, they suddenly get better, and that still remains to be seen whether that was temporary or whether that, you know, they just got lucky and found a very unique environment where they're better, and yet they're going to struggle in 90% of the places they might be. So, a lot of that is still an open question to me. Things that were when I thought a patient looked like they were going to be entirely better, yeah, maybe there's still a little bit of underlying thing that might pop out again in the future.

23:52 Jill (Host): OK. So, you had mentioned lifestyle things. I think your website mentions that your offices offer a dysautonomia management program. Can I ask what that's about?

24:04 Dr. Maxwell (Guest): Well, I'm a single provider on my own, so I can't... we can't do a whole lot in this clinic, but most of it is through very established networking connections with physical therapists and the physical therapists come in a couple different forms that... there's the general physical therapists that are taught the Levine Protocol and put my patients through the Levine Protocol, then there’s the cranial cervical instability physical therapists - the CCI physical therapists – that really work on that particular area. And then there's another type called the Myofunctional therapists, and I worked with a couple of those that do a fantastic job with my patients. And then a couple of nutritionists, and you got those that understand low histamine diet and the FODMAP diet, and so we work with them pretty closely. And then, you know, pretty close working with the radiologists, neuroradiologists at Stanford and UCSF. So it's really the connections and the networking that we have that make it a full program.

25:13 Jill (Host): In my mind, any child that finds you and gets their POTS diagnosed while there's still a child is super lucky. So, under these conditions, what kind of an improvement is realistic in your in your patients?

25:29 Dr. Maxwell (Guest): Everybody is different. It's as different as each individual. And, you know, some patients get remarkably better when you do a few little strategies and they come back and say, “Oh, My GI tract - all those symptoms improved, and once that those improved, everything else just fell into place and I feel a lot better.” But there's those who it’s an ongoing struggle, and you're constantly putting out fires and that's, I guess, the part that I'm there for long term is, you know, helping understand these fires and put them out long term, but they don't necessarily, you know, see the improvements that the other patients see.

26:14 Jill (Host): So we keep hearing reports about POTS and mast cell activation syndrome as part of long-COVID in adults. Are you seeing that in children?

26:24 Dr. Maxwell (Guest): Oh yeah. Mmm hmm.

26:26 Jill (Host): Do you think it's teaching us anything about POTS that they get this during COVID?

26:31 Dr. Maxwell (Guest): I think it's more teaching the world about.... less like COVID teaching us about POTS than COVID teaching us that POTS has existed and mast cell has existed a long time, and awakening the world to this phenomenon that's been going a long time. I mean, I don't think of long-COVID any different than I think of long Epstein Barr virus or a lot of other things that we've been seeing all along. So I think that's the biggest learning experience as the whole world has been woken up to what all of us has been seeing all along.

27:07 Jill (Host): OK. So this next question is something that we get a lot and I'd really be curious as your perspective as a pediatrician - do you think that kids have more health problems in general these days compared to, like, 50 years ago? And if so, do you have any ideas why?

27:27 Dr. Maxwell (Guest): Yeah, I think that's a very, very tough question to answer. I think that how much of what was experienced in the past was swept under the rug, was never understood, was pushed aside. Some people that just had to suffer in silence. So, I don't think that there's any evidence that we're sicker now than we were before. I think that there is a case to be made that our dirty environment is making us sick, but, you know, there were dirty environments before too, and I don't necessarily think that there's more problems now than there were before. I just I... I just don't think we have the evidence to say that.

28:23 Jill (Host): When you talk about environment, have I heard you mentioned before the idea of an environmental sabbatical, and if so, do you mind saying what that is?

28:32 Dr. Maxwell (Guest): Well, I mean, right now it's become one of my most successful management strategies as a location sabbatical. And basically what my feeling is, and if you look at maps of my where my patients live, there's definitely geographical distributions to my patients. And it's really raised the question is what is going on in these areas that are really high density of patients, not only in the numbers but how sick they are. You know, I've come up with this strategy that what's called the DQ score - this is something that Jill Schofield and I developed together to quantify a patient’s illness, and zero to 100 essentially for the DQ score. And if you map out the patients’ addresses where they became sick, and then color coded according to their DQ score, you know you get a pretty good sense of that there's a geographical distribution to these patients, and then you ask the question, “why?” And we've looked at lots and lots of things from: could this be some odd presentation of valley fever? And, you know, if we looked for coccidiomycosis in these patients - and that was the very first thing I looked at many, many years ago - and maybe it's not the coccidia, but maybe the breakdown product, the cell walls of these coccidia that are aerosolized and they're breathing these in. [Transcriber’s note: coccidia are a type of parasites that can produce gastrointestinal infections.] And that was looked at many years ago with nothing found. And then well, could it be industrial toxins? You know, in a lot of these areas that these patients are, border on farmland, and as this farm runoff? And also some other industrial things. So, we looked at all the Great Plains testing, 172, toxic exposure, labs and everyone always came back with something, but there. was never any unifying toxin that looked suspicious. And then of course the question of mold and mold toxins has been forefront, and then the question of, well, if it's that, which we haven't dismissed, why would it be in, you know, such high density in certain areas and not others? Was there some issue with what the developers were doing when they were building houses? When they were all half built at the same time was there some environmental catastrophe that caused all these houses become moldy? And then they continued to build them and people moved in and got sick? Yeah, maybe, but the other question is, is something wafting in from the greater environment and they're breathing this in? And so that is a question. And then the question is, could it be brought in through the drinking water? And so, that's where this idea... could this be blue green algae blooms? And as we know, blue green algae blooms are becoming longer and stronger throughout the world, but one of the hottest spots in the US is now becoming some parts of California and some of that is because of what's called eutrophication - that basically farm runoff causing increased nitrogen and phosphorus in the water, which are perfect nutrients for algae, and then of course the cyanobacteria live on the But the other is California wildfires - wildfires produce a tremendous amount of phosphorus and nitrogen and then that all is drained into the San Joaquin River basin and in the delta, and so that is potentially a reason why we're seeing longer and stronger algae blooms in this area. Under certain conditions, toxins that have never been described before now being produced by these cyanobacteria. Basically, dormant genes are turned on when a certain phosphorus level in the water is hit, and so toxins that have never been described before being produced. So, are these making their way past the filtration system? Are they making their way into our drinking water, our showering water? And are they causing some of the problems? That's one of the things that my practice is looking at. We have some blue green algae bloom specialists from across the country - in the University of North Carolina, University of Georgia, Berkeley, and UC Santa Cruz all have groups that are looking at this question now, and trying to see, could this be the source of human illness in general, and in particular what I'm looking at.

33:17 Jill (Host): Wow, that's so fascinating. And I don't want to make this about me, but I grew up on a lake in Wisconsin that obviously had a lot of farm runoff going into it, and we'd get a yearly algae bloom. And I remember I was an avid water skier and I remember water skiing, having the, you know, you throw up a wall of water and when you're slalom skiing, you know, that's half the point is to make a beautiful wall of water. And I remember it being a pure wall of blue green and it never occurred to me. You know, we just swam in it and we just skied in it and so that's fascinating. And I'm so glad that you are looking at all of these things that seemingly nobody else is looking at. And I can only imagine, I think you mentioned that there's 172 toxins that can be tested for, but I think that there's probably...

34:03 Dr. Maxwell (Guest): Thousands.

34:03 Jill (Host): ...millions or trillions.

34:04 Dr. Maxwell (Guest): Yeah, I mean, yeah. There's the long and short coming of the of that test. I mean, they're supposed to be classes of 172 different classes. So, you know, if it's a similar compound to something else that fits in 172, it’d be picked up. So that's the that's the wrong part of it is that, yeah, it picks up more than just 172, about the other part of it is, well, you know, is it something that's necessarily going to be excreted in someone’s urine, and can we detect it? And if it's high in their urine, does that mean that they have a lot of it in them, or that they're actually doing a very good job of excreting it and getting rid of it? So, there's a lot of questions that come up with that method of looking for toxins.

34:51 Jill (Host): Yeah, that's interesting. So, in... in my world of nutrition, there's a lot of thinking about how increases in obesity might be related to a very healthy protective effect of your body storing and sequestering away toxins in fat. And that's how it is protecting you and keeping it out of your bloodstream and keeping it away from your organs. And so, I can see where if your body is sequestering it, then you're not excreting it and it becomes pretty tough to test for it.

35:20 Dr. Maxwell (Guest): That's right, yeah.

35:22 Jill (Host): OK. So back to this idea of a location sabbatical - is that something that you recommend people do? Or what... what is that exactly?

35:31 Dr. Maxwell (Guest): Yeah. Well, I mean the first thing I would say is, you know, I'm not talking about you packing up and moving. Lasting thing we want is a family saying, “Well, Dr. Maxwell's suggesting that where we live is making us sick. We need to pack up and move.” Couple things wrong with that: #1 is in any given patient, that's not necessarily true. They could have it, you know, from a different cause, and I've got plenty of patients where it's probably not going to be a location effect. But secondly, everybody is different and what's making them sensitive. And so some people, it's a simple matter of moving to anywhere else, and they're going to get better. With other people that could be only, you know, 10% of the surface area of the earth where they're going to be able to live successfully. So they need to go and test themselves in in different places to find out where they feel good. And as a general rule, I would say people need to try a hot arid environment. Now you say, “Well, POTS patients do terrible in hot environments,” and that's... that's true. And I would never have said this except for the number of patients that move to – POTS patients of mine - that move to warm arid environments that put up with feeling worse and then suddenly you know was like, “Hey all of its gone. I feel better.” And it made me realize hey wait a minute, what if, for example, they are, you know, mold patients that have some mold that’s colonizing their sinuses, and this warmer and drier environment is drying out these sinuses and maybe not killing the mold, but certainly putting it into dormancy where they are. And they're suddenly feeling a lot better by doing so. So, I started actively asking patients, “Hey, what would it be if you spent maybe this summer in Death Valley or Palm Springs or the other places that have been successful have been Santa Fe, New Mexico. Not just anywhere. And you gotta be outdoors, you can't go rent a motel that's moldy and just sit around in some place and expect to get better. And it can't be so hot that you end up spending more time indoors with HVAC than would've otherwise. It's got to be something where you feel comfortable being outdoors, breathing warm fresh air, and being active because you want to be breathing it deeply enough that it's making a difference. And so, I've done this with many, many patients now and like for instance, this summer I had 13 different patients try this, and 11 of the 13 said yes. As soon as.... some, it was three days into it, others took all two or three months to feel the difference. And some they didn't even recognize the difference until they returned home and went downhill and said, “Oh, OK. I didn't realize how good I was actually feeling.” And a lot of it was because, “Well, I still felt fatigued everyday.” “Well, what did you do with your day?” “Well, I went hiking. I played three hours of tennis.” “Yeah, and what were you doing before you left?” “Well, I was laying in bed all day.” So, they didn't even recognize themselves that their fatigue was actually from a lot of physical activity that they were happily doing. You know, sometimes you gotta wait until they actually come home to see them go downhill again. So, you know, it really shows I think that - and again, I don't know whether this is a phenomenon in my area and this is just unique to my practice, but there seems to be an environmental phenomenon with a lot of my patients.

39:13 Jill (Host): That's so interesting, and that tracks with me because I was a California person who didn't do well in the heat. So I moved to Alaska and it was very nice for avoiding heat, but in the end, I ended up moving back for exactly the reasons you said. Something, despite the heat, the dry, the dry outdoor stuff was better. So, I wish I had talked to you before I made that expensive mistake. [Laughs]

39:39 Dr. Maxwell (Guest): I try to make the patients that are finishing up high school and going on to college, you know, a lot of the times we're now making college decisions based on that. And again, I say it can't be so hot that you end up spending all your time indoors. And so, you know, that that fits the warm, dry, arid, but also outdoors and not in such a hot place that you're spending all your time in HVAC.

40:06 Jill (Host): Wow. Well, I know you need to get back to your clinic and we could talk to you for hours and hours. But this is such great information. Is there anything that you wish more people knew about POTS, especially doctors?

40:22 Dr. Maxwell (Guest): I'm really, really happy that there's been a lot lately in the press about POTS. So, just the awareness has grown, and I think that less and less doctors can legitimately try to claim that it just doesn't exist or it's all in your head. I think they're becoming more and more in the minority. I think the next big hurdle is the connection with mast cell activation and Ehlers Danlos, that they become aware that there is a pretty strong association with those. And then getting toward the mast cell side that mast cell activation syndrome is not what is described by what we now call the Consensus 1 Criteria, where it's, you know, if you don't have an elevated tryptase then you don't have mast cell activation, and most of us who are in the field know that's just absolutely not true. That's the last thing you need to do determine whether you have mast cell activation is what your tryptase is doing.

41:23 Jill (Host): Smart. So, we'll put in the show notes a link to the Consensus 2 paper that says what the other consensus of experts thinks about diagnosing mast cell activation syndrome. That'll be really helpful. Thank you for saying that.

41:37 Dr. Maxwell (Guest): Perfect.

41:38 Jill (Host): Perfect. Well, Dr. Maxwell, thank you for all the fantastic information and for your tireless, ongoing work to help patients and your research to help uncover the bigger picture. You are truly just such a shining light in the POTS world and we're so appreciative of your taking the time to speak with us today and also the time you take to volunteer to be on the Medical Advisory Board of Standing Up to POTS. We really appreciate you.

42:06 Dr. Maxwell (Guest): Jill, thank you. It's been a pleasure. Thank you for inviting me.

42:09 Jill (Host): Well, hey listeners, as always this is not medical or any kind of advice, but please consider following us because it helps us get found by more cool people like you. Thank you for listening. Remember, you're not alone and please join us again soon.

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