Episode Transcript
Jeff Boris/Jeff Moak
Jill Brook: [00:00:00] Hello, fellow POTS patients and dearest people who care about POTS patients. I'm Jill Brook, and today we have an episode of the POTS Practitioners where we are going to speak with two superstar pediatric POTS experts who have published a really exhaustive and impressive review of pediatric POTS. They dug up research findings that I had never heard of before and I thought I followed this stuff pretty well.
Our first guest is Dr. Jeff Boris, whom you've heard on our podcast before. He is a pediatric cardiologist. He is famous for being a past dysautonomia physician of the year, a recipient of one of our a hundred thousand dollars Standing Up to POTS research grants, and also for being almost a practical human encyclopedia of POTS science and research finding.
Our second guest is Dr. Jeffrey Moak, who is the director of the Electrophysiology and Pacing Program in the Children's National Hospital Division [00:01:00] of Cardiology, where he has held the position for more than 20 years. He is a professor at George Washington School of Medicine and Health Sciences. He treats children, adolescents, and young adults with cardiac arrhythmias and autonomic nervous system dysfunction.
Dr. Moak, along with Dr. Boris has been a co-author on pretty much all of the POTS expert consensus statements, and they are here to share some of their latest work. Dr. Boris and Moak, thank you so much for being here today.
Jeff Boris: Thank you for inviting us.
Jill Brook: So you two published a recent paper in the journal Pediatrics called Pediatric Postural Orthostatic Tachycardia Syndrome, Where We Stand.
And when I read it, well first I had to give you credit for the nice pun, but then I also really thought it was the best summary of POTS I had ever seen. It was so thorough, so exhaustive, so obviously labor intensive. [00:02:00] It covered research that I had never heard of, including POTS research from China.
I was just so impressed by how much deeper your article went than the normal POTS reviews that we always get. So I'm really excited to go over it with you today, especially the famous table two. I have been thinking about your table two for weeks because it is so packed of amazing thought provoking stuff.
But can I start by asking what made you decide to write this and to make it so
Jeff Boris: exhaustive? So I'll take this one. This is Jeff Boris. So this actually goes back about five years. In 2017, Lauren Stiles, the president of Dysautonomia International, asked me and also Glen Cook an adult neurologist to do a congressional briefing on POTS because there wasn't enough, or there wasn't research funding. And so we were able to do that. And when Congress decides to do [00:03:00] something they can't say to the National Institutes of Health, you must spend X amount of dollars on research. But what they can do is they can say, you need to look at this. So that's what they did. In 2019, NIH had a one day workshop where they looked at the science and the practice of POTS.
So Dr. Moak and I were the two pediatric presenters. And we, as part of the all day thing, we gave a 30 minute talk on the aspects of pediatric POTS. And then about a year, year and a half later, We were asked to write it up and we were gonna go ahead and have it, have everything published as part of the larger compendium.
So we put together a 1500 word section on pediatric POTS. We're like, Nope, too big. And so we cut it down to 750 words. Nope, too big. And it ended up getting relegated to, honest to God, two small paragraphs. And so Jeff and I were like, What? And, and around that time, another paper had come out in the pediatrics world, saying that POTS [00:04:00] was a quote, functional disorder.
And so between the fact that we had done all this research and the fact that there was this annoying paper out there that was actually written by one of our colleagues, to be honest with you we felt like we wanted to really put out there everything that we could on pediatric POTS and bring things as much up to date as we could.
Understanding that so much of the research has been done in adults and there's not nearly as much in kids.
Jill Brook: Great. Anything to add?
Jeff Moak: Yeah, there's a huge divide in the POTS world between the psychological realm and the medical realm. And is, Jeff alluded to the paper from the Mayo Clinic was kind of emphasizing more the psychological world.
And we had hoped to emphasize here in this paper, in the medical findings. We're not saying that there isn't psychological overlays to having POTS but there's obviously a very strong medical [00:05:00] component highlight that is more here to the story.
Jill Brook: Yeah. I really appreciate that you did that but let's dive into the article. Your introduction sets out the goals for the article. What are those?
Jeff Moak: So in the paper itself, our goal was to really write a comprehensive description of physiological abnormalities in this population of patients.
Not all of it is understood. But the goal was to really compile an extensive medical review of known physiological consequences of POTS and to show that there really is more to the story than has really been told.
Jill Brook: Great. Okay. So your paper starts out by talking about the diagnostic criteria. Do you wanna kind of summarize the important parts that you've got about that?
Jeff Boris: The diagnostic criteria have remained fairly stable since I think at least 2011. There have been several consensus conferences [00:06:00] 2011, 2015. There was another one subsequent to that as well. And in kids, we say that the latest criteria, and this was in part what came out from the NIH workshop, was chronic orthostatic intolerance. So that means that you have difficulty with being upright and having symptoms associated with that for at least three months.
As well as an increase in the heart rate of 40 points over supine position in the first 10 minutes of being upright. And that should be a consistent increase, not sort of an up and down kind of thing. And then no decrease in blood pressure more than 20 millimeters of mercury, over 10 millimeters of mercury systolic diastolic.
So in other words, not orthostatic hypotension. And then also elimination of other potential medical problems that could cause this thyroid disease. Low vitamin D, low iron, that kind of stuff. [00:07:00] And when we look at this, one of the things that I have really kind of fussed with for a long time is that I have argued that for children the heart rate criteria should really be 30 beats per minute as well.
The reason for that is a couple fold. One is because of a paper that I published previously where I showed that symptom wise, there is no difference between patients who have a 30 to 39 beat per minute increase in heart rate versus a 40 per minute heart rate increase or more. Second, because the original data was performed with tilt table testing.
And if you look, there's actually a couple of papers in the medical literature that demonstrate that a tilt table increases the heart rate more so than a 10 minute standing test. There was a good paper out of Vanderbilt. First author is Flash that really kind of pays attention to that concept. But there was a [00:08:00] surprising article out of the Cleveland Clinic that I had never even seen before that also mentioned that, and they sort of breezed right by it. But they found that tilt table increased the heart rate more. And so the original suggestion that heart rate threshold should be higher, came out of a paper from the Mayo Clinic in which they went to a bunch of normal kids and did a 10 minute stand on them.
And they found that asymptomatically, their heart rates went up to 40, 42, 45, whatever. And my response to that is, that's fine. Sure they don't have POTS. But that doesn't mean that we should just ignore those patients who are symptomatic and who have the exact same degree of symptoms, the same degree of debilitation who don't have that heart rate increase.
I actually just for fun looked at my data in patients who had a 20 to 29 beat per minute increase and also a 25 to 29 beat per minute increase, and this is not published, but [00:09:00] found that those patients actually did have fewer symptoms overall. Interesting. Right.
Jill Brook: Wow. So does that mean that you treat patients in that 30 to 39 category as if they have POTS?
And do they tend to
Jeff Boris: respond? Yes, they respond appropriately. Yes.
Jill Brook: So if there's people listening out there who have had trouble getting treatment because they were at only 38 beats per minute, maybe they should bring your paper into their doctor's office.
Jeff Boris: Maybe. I think, the problem is in some ways it's a matter of what's in a name.
And I think in some ways it's like, okay, fine. So if you're not gonna call it POTS, then what are you gonna call it? Are you gonna call it orthostatic intolerance but not POTS? And does that mean in the end that there is actually a difference from a therapeutic standpoint? And I would say that there isn't.
Because these patients still have, not just the tachycardia, not just the [00:10:00] lightheadedness. They have the fatigue and the brain fog and the insomnia and the GI dismotility and the chronic pain and all those other things that we know that patients with POTS have. So we're gonna treat 'em anyway.
Jeff Moak: I agree I remember at this meeting we had with NIH mentioning this issue, the pediatric cardiology world the diagnosis of POTS is a lot different than sometimes in the adult neurology world. In adult neurology world really depends on the tilt table test, and they're very fixated on this issue.
Most pediatric cardiologists will not do a tilt test. So mostly do a standing test. And in fact, a lot even gone away from even doing a standing test. So just kind of diagnosed POTS based upon the patient's clinical symptoms and gestalt about their issue.
So the pediatric world's a lot different than the adult, and as Jeff alluded the number that you [00:11:00] get varies from different parts of the day. I make this little joke in the clinic, like the patient stopped off at 7-11, 30 minutes before the clinic visit and had a 64 ounces slurpy. Their test is gonna be negative.
Jeff Boris: There's that, and there's also, as Jeff was saying, the time of day, right? So we know there's what's called diurnal variation. So in patients who have tachycardic response in the morning, I've seen them have an absolutely stone cold, normal, or at least under 30 beats per minute, increase in heart rate on a 10 minute standing test in the afternoon.
Jill Brook: Wow. Okay. So it sounds like we have some more work to do on finding really great markers for POTS. Everything right now is just kind of best guesses in what we can measure.
Jeff Boris: And I think that's a holdover from when people like Philip Lowe and Blair Grubb and some of these other folks got together way back when and said, Well, we know the heart rate goes up, so why don't we use that as a marker. But we really need a biological marker.[00:12:00]
Jeff Moak: Well, the heart rate issue assumes that the primary problem with the patient is really the heart. And it is true that the heart is involved in POTS, but there's other organ systems that are sometimes involved even more.
And so, in my mind it's simplistic to depend on the heart rate because many times what I find is the major morbidity of the patient, even the cardiac symptoms, there are other symptoms like the GI symptoms. Neurological symptoms. There isn't always one to one correlation, of the cardiac symptoms with some of these other symptoms.
The heart rate changes are minor, but yet the patient has, major morbidity consistent with what we consider POTS. But maybe 10 years from now we consider POTS will be more divided up into other entities?
Jill Brook: Right. Okay. So your next section talks about risk factors and outcomes, do you wanna start by kind of giving us an overview of what are the important risk factors?
Jeff Boris: I think [00:13:00] that there are a lot of things that people are already familiar with. We know that infection, concussion, non concussion trauma, growth spurt, onset of menses, all those things have been associated as triggers.
Can we say that they're triggers? We haven't proved anything yet because we don't have a biological marker, so that makes it really difficult. But at this point, we think that from a time relations standpoint, those things really are triggers for POTS.
It has been recorded in the literature that patients really have been high achieving from an academic standpoint or from an athletic standpoint, which I think is really fascinating and I'm not sure why.
Jill Brook: That was gonna be my question. Any ideas? Is anybody floating hypotheses out there or they're just
Jeff Boris: noticing this?
Just noticing it. I'm not sure why. One of the things from, actually, that we've also seen is from a risk factor standpoint, patients who have joint hyper mobility
We did report that [00:14:00] an overwhelming predominance of Caucasian or white patients, and that is what's reported in the literature for the United States. But if you look in places like Africa, there's a huge African or black population of patients with POTS. If you look in Latin America there's a huge Hispanic population.
If you look in Asia, there's a huge Chinese, Korean, Japanese population of patients with POTS. And so I think this is definitely pointing to some of the disparities in access to care, some of the inherent, unfortunate, prolonged biases that have remained within medicine and some of the cultural issues as well that just make it difficult for some of these patients or a lot of these patients to be diagnosed.
So those are sort of some of the things that we have definitely seen, for the hypermobility aspect of it. I mean, one of the papers that I published as much as 61% of patients had either hyper mobile [00:15:00] Ehlers-Danlos syndrome, or hypermobility spectrum disorder, it's a lot, right?
That's a huge proportion of patients which also means almost 40% of patients aren't hyper mobile, but still that, that's a lot from that standpoint.
Jill Brook: Absolutely. Okay, so let's move on to outcomes. We used to hear that POTS patients would just grow out of it if they were pretty young, and did that pan out or what's the state of the art in thinking about outcomes?
Jeff Boris: So, it's interesting. I actually just completed a long term outcome survey. It hasn't been published yet. But hopefully we'll be able to submit those manuscripts for publication within the next few months. It's probably the largest study, over 220 patients. It's probably the longest study. The average duration from onset of symptoms to the time of filling out the survey was nine and a half years.
Okay. Wow. And there have [00:16:00] been six other studies in the literature, three from Mayo Clinic, one from Portugal, one from Korea, and one from China. And they've all been really rosy. They've all been saying, Oh, these kids get over it. But there have been a lot, or these adults, but there have been a lot of problems with those studies.
If you read between the lines or you read deeper into them. A lot of these patients can't work. A lot of these patients are still on medications. A lot of these patients still need extra assistance in one way, shape, or form. One of the studies didn't look at a full complement of associated symptoms or what we would expect to be associated symptoms.
So it was just very problematic. Probably one of the better studies was one of the studies from the Mayo Clinic where they suggested that as many as 19% of patients had spontaneous resolution. But even if you read about those at least a third of those patients had worsening symptoms at various times.
[00:17:00] So with that as a backdrop, when I did my long term outcome survey, one of the things that I found was that honestly, 99% of patients had symptoms as late as one month prior to filling out the survey. Oh.
Jill Brook: So when they said they felt better, they meant they had felt better for a few weeks.
Jeff Boris: So it's interesting, what we did was in that survey, we looked ranking the severity of each symptom. So at its worst, at its best, and in the last month. Okay. And so yes. Did patients improve? Yes. Were there ways to get patients to improve? And, we looked at the various aspects of how we could get patients to improve from a non-pharmacologic standpoint, from a pharmacologic standpoint, et cetera.
And so, in the grand scheme of things, a large majority of the patients did have clinical improvement [00:18:00] in their symptoms, but they didn't disappear. And so in 2022, we're still pretty clueless as to what's going on and why it's happening. So there are things that we can do to mask it and to make people better. In that survey, 97% of patients either graduated from high school or got a GED and 80% went off to college and it was amazing. Right. But that doesn't mean that they're not asymptomatic.
Jill Brook: Dr. Moak did you have anything you wanted to add?
Jeff Moak: Well, in my own experience, I see a lot of parent child combinations cuz we know there's a very high incidence of familial through to POTS. And many of the parents tell me when they're 30, 40 something that they tend to be better than they were as teenagers.
But some of them will still be symptomatic. If I try and drill down a little bit more carefully, is it due to true biological improvement or learn adaptation? And so it's a very [00:19:00] complicated issue, to kind of look, is their biology better or they just have learned to live with the symptoms and so they feel better because they're not bothered on a daily basis as much, but they still have the symptoms.
And my own personal experience, the parents is that there is maybe some true biological improvement, but a lot of it is learned adaptations and adapt to the symptoms.
Jeff Boris: Knowing what things set you off, knowing what things make you worse, how to avoid them.
And I think part of that is learning to adult, right? As a child you're not particularly familiar with your body. You don't think about things about your body. And in the absence of POTS, when you transition through adolescence, learning to be an adult, those are things that you learn about anyway.
And so this is just more of that on a much much more urgent scale. I think one of the other things too, so you get out of adolescence and the hormones calm. And so does that mean [00:20:00] that with the hormonal changes that occur in adolescence once things sort of settle out a little bit, does that make a difference? One of the other things for my long term outcome study that we found was male patients definitely did better than female patients. They got better sooner.
They needed less intervention. They had less severity of symptoms. It they had less pain overall as well. It was very interesting. So, I hope to have that published within the next year.
Jill Brook: Yeah. That's fascinating. So in this section you mentioned that the presence of parental care makes a big difference. Can you talk a little bit more about that, Dr. Moak?
Jeff Moak: Hopefully every child has a parent and the hopefully the parent is loving and wants the best for their child, which I believe is true for most parents who are of sound, psychological health and relatively financial health.
And that is a rare, very, very rare situation where a [00:21:00] parent wants to harm their child or somehow take advantage of their child. It could be that the child, again, if the parent has POTS, somehow there's a projection from the parent onto the kid and wanting to help the kid in certain kind of ways that they wish that maybe their parent had helped them.
And that so there's over indulgence sometimes in these symptoms. I think sometimes the parents in these situations, not only with POTS but other any kind of chronic illness. We do see it where the parents will be overly protective of the kid and maybe not let the kid play sports. Maybe not let the kid do certain socialization activities or participate in camp.
So I think it's true to any chronic illness that there's a certain percentage of parents that are overprotective. And sometimes the overprotectiveness inhibits the kid's growth in terms of their [00:22:00] biological growth and that the kid is restricted in their development of alternative coping strategies.
Jeff Boris: Yeah, I think people have at times misinterpreted what was said in the article and it has probably taken outta context. We both understand that the vast majority of parents want their kids to get better and want to help their kids to get better. These parents have had to fight and advocate for their child for a long time.
We know from other studies that at least half of patients have seen at least seven providers before getting a diagnosis and, and a quarter of 'em. I've seen over 10 providers before getting a diagnosis of POTS. So we know that the medical community has been harsh toward these parents and toward these patients.
And so once they get the diagnosis, it in some ways it can be difficult to then back down. And forgetting POTS, just transitioning your child from being a [00:23:00] child through adolescence, to being an adult, learning to fail, learning to take on responsibility. It is difficult , and there's a wide range of the ways that people do that, some of them not particularly beneficial for the child.
And then put on that as, as Jeff was talking about, a chronic illness and you feel bad for your kid, they feel awful. They're debilitated, they have pain, they have fatigue, they have all kinds of symptoms that make them feel awful, and you just as a parent want to take it away. And that can sometimes be a problem in inhibiting the child to being able to then take responsibility for themselves.
But truth be told, I've actually seen the opposite. I have several cases in which parents have actively caused problems. In other words, one of 'em was smearing feces on an IV catheter port and was caught on camera doing so to make their kids' symptoms persist.
[00:24:00] Another one literally faked their two kids POTS because the mom told the kids that they were gonna get to go to Disney World. it's just all kinds of stuff that has happened. And so that's the great minority and I think that needs to be kept in context, right? So the vast majority of patients and parents are here for all the right reasons.
Jill Brook: I think it's important for the patient community to remember sometimes that even though it is sometimes very frustrating not to be instantly believed with everything you say, that oftentimes the medical professionals are having to see both ends of this spectrum and figure out who's telling the truth, who's doing some of these amazingly horrendous things. And those cannot be easy calls to
Jeff Boris: make. And again, in the absence of a biological marker, we are pretty stuck. And it's not like you can just draw some blood and say, Oh yeah, it's POTS right. Yeah.
Jill Brook: Wow. That sounds [00:25:00] amazingly tough.
Jeff Moak: On the other hand, also, the issue is that sometimes it goes the other way. The highly performing academic achievement and highly academic school, and the kid can't do it yet the parent is really pushing, to the detriment of the kid. The kids can't live up to the parents' expectations, and so there's a failure of the parent to learn to modulate their expectations for their child.
Jill Brook: Yeah. I am not a parent, but I cannot imagine how tough it is that this first of all happens in the teens pretty often, which is tough enough. And then to add this layer on top. Yeah. So let's move on to what I think is the most fascinating part of your paper, which is all of the abnormalities found in POTS.
You have a section called neuro hormonal and hemodynamic abnormalities. What's the best way to just pick out some fascinating parts? Do you wanna take turns? [00:26:00] Maybe just mentioning studies that are in there and just giving us a little snippet about what was found.
Jeff Boris: So there have been some really interesting studies that have come out of China that find that certain hormones including resistant co pepin, antidiuretic hormone, things like that are abnormally elevated. And, when we put this review article together, one of the things that we wanted to do was make sure that the papers that we were quoting were decent research.
We wanted to see that they had a controlled population that they were comparing against. And some of those things that I just mentioned were seen as well. So which came first, right? Is that chicken, is that egg?
Is that because of POTS or is it causative of POTS? And the answer is, we don't know. There needs to be much more research done from that standpoint to look at some of those things.
Jeff, you wanna take some? Sure.
Jeff Moak: So, even in our table here, there obviously is a ying and yang. Up to [00:27:00] 48% of POTS patients were symptom free at one year follow up, and 85% were symptom free at six years. So again this stuff is a little bit controversial, mainly because there isn't really high level research. It's mostly like observational data. And the whole field needs to be brought up into more controlled trial type data or registry data. Rather than these little small anecdotal studies.
His figure shows a very high incidence of GI symptoms, like 80, 85% of patients have GI symptoms. And we see that and we see some patients who even have what sometimes the gastroenterologists like intestinal failure, like they can't eat they're on NG and or NJ fluid supplementation.
That's still not enough. And then they're getting TPN like try to get a sense of what is really going on with these patients. I have lots of colleagues who think these patients are just lingering yet so we tried to look at manometry studies and we did see [00:28:00] that some of them do have significant issues like gastric emptying problems condition called neurogenic intestinal dysmotility, where their stomach will temporarily stop functioning and they'll have regurgitation of the fluid food from the small intestine into the stomach.
So, again, is this the final understanding of these problems? No. But it's beginning to show definite physiological abnormalities happening in the patients. And most of these findings I would consider preliminary and needing further confirmation and a better synthesis of how they interrelate. But showing for sure that it's not all in their head.
Jeff Boris: Yeah, the bottom line is you can't fake this stuff. You can't fake that your GI motility is abnormal. Right. Just looking back at table two, there are abnormalities in nitric oxide and hydrogen sulfide and some other markers.
But one of the things that stands out to me is the fact that there is increased pooling of blood [00:29:00] in what's called the splanchnic circulation. So the splanchnic blood vessels are the blood vessels that feed the GI tract, right? The blood vessels in the gut. And so beyond that, there was a fabulous study that came outta the University of Calgary last year where they compared no compression garments, using compression garments on the legs, using an abdominal binder, and then using a combination of an abdominal binder and compression garments on the legs. And what they found was the combination reduced symptoms the best, but right behind it was just the use of an abdominal binder over the leg compression garments. Which has really changed my practice because I used to always refer patients to get the waist to toe compression stockings, 20 to 30 millimeters of mercury where it while awake, that kind of thing. And some patients really liked it, but it makes you kind of warm. It's this really tight sensation for patients who have some GI problems.
It can cause worsening [00:30:00] abdominal pain. It's a 15 minute wrestling match to put it on. And when I saw that, about the abdominal binder. I've really changed, I've actually been having patients wear abdominal binders more so than using compression stockings and have found that that again, I mean with everything with POTS what works for one patient doesn't work for, for another one.
But for those patients for whom it works, it's really been life changing and it doesn't use a medication.
Jill Brook: Yeah. That's a great one. Did you have any more that you wanted to pull out, Dr. Moak? Yeah, I
Jeff Moak: think this divide between beta blockers and ivabradine for idiopathic sinus tachycardia and POTS, I think is the development of this knowledge base and experience of using ivabradine has come a long ways.
I mean, beta blockers cause some patients get better and some patients actually make them worse because the beta blockers can cause fatigue and lower blood pressure. But the use of ivabradine it has more selective effects and [00:31:00] slowing the sinus rate. So I think that the it was a recent study in adult patients showing how ivabradine was markedly effective.
Jeff Boris: Just to sort of talk about that a little bit further. I think people don't quite necessarily understand what beta blockers and what a ivabradine does from a cardiac standpoint.
So the original papers came out of Vanderbilt where they took adults with POTS and they gave them a single dose of propranolol, 20 milligrams, and then they gave them a dose of 80 milligrams. And what they found was the adult patients who had 20 milligrams of propranolol had reduction of their symptoms.
And so, a number of years ago, I had patients who were on metoprolol or atenolol, which was sort of longer acting beta blockers. And I'm like, Okay, I'll do 20 milligrams of propranolol. And I converted a bunch of patients to 20 milligrams twice a day, and they all passed out. So I didn't realize that at the time it took me a number of years cause I just thought, Oh [00:32:00] maybe propanolol is just bad for pediatric patients.
But what I realized was the dose was probably too high. So I actually start with 10 milligrams, then maybe bumped to 15. But the bottom line is what both beta blockers and Ivabradine do is they slow the heart rate down enough that you get proportional, increased slowing, I guess is the best way to say it.
And increased time for the heart to fill with blood. And so if you have longer time for the heart to fill with blood, that means you have more blood in the heart to eject. And since you have more blood in the heart to eject, you have more blood to send to the brain and to the rest of the body. And that's what we think is the mechanism of decreased symptoms in the setting of beta blockers.
Now, I ivabradine has been out on the market probably for about five, six years or so. And so it's still on patent. It's very expensive. A lot of insurance companies give pushback. I think the [00:33:00] article out of California that Dr. Moak was referring to was probably the first real double blind, randomized placebo control study of patients with POTS.
And that was using a ivabradine in the show that a ivabradine was really fabulous. So we hope that the use of that article will help both providers be more comfortable with using it as well as insurance providers paying for it. But a lot of times the insurance providers want you to try the propranolol and if you're not able to tolerate it because of either low blood pressure or depression or worsening asthma or that kind of thing, diabetes, an absolute contraindication to the use of beta blockers, then you can go to a ivabradine.
Jill Brook: Okay. So you have to jump through hoops maybe to get there a little bit. Yeah. Are there any other bits of table two that you wanna pull out and discuss?
Jeff Boris: Do you wanna talk about the normal saline. Yeah, I know a lot of patients are very interested in getting routine saline infusions. They're like, [00:34:00] Oh, this fixes me. Especially like if they go to the emergency department and because they've been feeling really bad and they get a liter of saline, like, Oh my God, I feel great for a week now.
I want this regularly.
Jill Brook: And for listeners, you're making a face.
Jeff Boris: Well, yeah. So the research came out of Jeff's group and I think it's important to discuss for several reasons, but I'll let Jeff go ahead and talk about it.
Jeff Moak: Yeah, I sometimes make this statement to my colleagues in the clinic and sometimes the families, if you had one therapy, you can offer a POTS patient. I personally believe that fluid expansion in some form like this, where normal saline really improves a very high percentage of patients.
And many years ago, I was involved in the paces group that put together a consensus of use of different therapies for POTS and idiopathic sinus tachycardia. And I mentioned this, and some of the thought leaders in the group, they were like shocked and stunned but on the side, some of them did do it, but they, as a group I think a [00:35:00] class three recommendation means not to do kind of thing.
But the reality is that most of the patients actually do better. And we looked at this more medication refractory patients had poor quality of life and most of them had pretty significant improvement. The problem came up with two aspects of it is blood clots from the lines and infection.
And having worked with one of the hematologists at Children's Hospital, we have nearly cured the issue of the blood clots. But the issue is the infection. We haven't really found a very good way to prevent infection, but we do find that you can achieve about 80, 90% of the effect of the saline with entro fluids, entero fluids being like NG fluids or in some of the patients, they're willing to have like a G button put in a gastric button, peg button.
Jeff Boris: So one of the other things that Jeff was talking about with the risk of infection in the setting of central lines [00:36:00] is that I have had patients who've had central lines and the parents have been amazingly fastidious in keeping the lines clean and keeping the lines sterile and that kind of thing.
And they go into the emergency department or they go into the hospital, they go into a clinic, whatever, and some other healthcare provider accesses those lines and does a poor job. And it's not to sit here and beat up my fellow healthcare providers, right? Because there's plenty of healthcare providers who do an awesome job.
But I can't tell you the number of times that I've had parents come back to me and say they used really poor, sterile technique. I had to call 'em out. And I was like, Don't you put that needle in my kid's port until you do a better job. And so I wonder from an infection risk standpoint, if some of that at least plays into the increased incidence of of infections that that Dr. Moak's research was finding.
Jeff Moak: And so, we do see that the effect of the saline does help. [00:37:00] And it makes sense physiologically because the excessive increase in the heart rate is really due to the pooling of blood in the GI tract splanchnic bed, as well as the lower extremities.
And so that in itself drives up the heart rate. We showed that saline expansion really prevent a lot of these kind of changes. The Cleveland Clinic, they described many years ago that the patients had hypovolemia.
It's not too surprising that fluid supplementation the IV or entro really helps the patient. The problem is one is its delivery method and its complications. And the other relates to how one could do it in a kind of safe way.
And we're looking at ways to prevent infection and we alluded to that there are patients who have almost GI failure and are on tpn. And so unfortunately they're stuck with ports and picc lines and things like that.
But those who have better functioning GI tracts, we. finesse giving them enough fluid to make them better. [00:38:00] Now, I'm not saying this is prime time for everybody, but you probably have experience with patients who despite treatment with the usual non-pharmacologic intervention, pharmacologic just doesn't make them better and their quality of life is poor? So, if you have a heart for them, you wanna make them more functional. You have to kind of think outside the box.
Jill Brook: Well that's really good news about maybe solving the blood clot problem, because I do hear a lot of patients worry about that. Do you just use blood thinners...
Jeff Moak: Yeah, we used one of two drugs. They they called direct oral anticoagulant drugs, either a drug called Eliquis or a drug called Rivaroxaban or Alta. And we see very little problems if the patient's compliant with it. And it started just around the time, or sometimes even the day before they get the line placed, that the incidence of infections are extremely, extremely low.
So initially we found that there was about a 10 to 15% incidence of blood clots[00:39:00] (DVTs) with these lines. But now we, we hardly see any. So, that's
Jill Brook: huge. That's progress. Wow.
Jeff Boris: That really is important. And you can either have a central line, either a picc line or a port, or you can have an IV placed every time you have a normal saline infusion. I've had patients on both sides of that fence. And as he was saying, you sort of feel bad for them. I had a patient who, no matter what I did for her vision was always grayed out unless she was standing in a swimming pool because she was having the external compression of that water pressure.
Right? And then we started getting her IV saline made a difference. Now, I've had pushback on this because if you give a liter of normal saline. You're basically giving salt and water, right? The water is gonna be collected by your kidneys and peed out within six to 12 hours, right?
The salt [00:40:00] is gonna be collected by your kidneys and excreted in 12 to 36 hours. So why do these patients feel better for three to seven days? Right? Yeah. And so, the short answer is we don't know the long answer. My hypothesis is that by volume expanding these patients, what you're doing is you're cutting off that bad feedback information to the central aspect of the autonomic nervous system and saying, Everything's fine now.
And so the autonomic nervous system just kind of chills out. And then over time hopefully the patient is able to maintain oral or enter al hydration and kind of help keep things up. But as time goes on, things sort of start drifting down and drifting down. And then symptoms start popping up again as the autonomic nervous system says, Well we're not in a good position right now.
Jill Brook: That's so interesting. And I'm so excited that we have smart people like you thinking about [00:41:00] this. Because that seems like an important clue. So for anybody who is interested, I just want to point out that that table two has 30 or 40 different studies that you refer to, and we only touched on a few of them, but I do encourage anybody who's really interested to go check out the rest of that table because I plan to go look up every one of those things and geek out a little. Cuz you did an amazing job pulling that together.
Jeff Boris: I do want to put in a plug. This article has been made open access which means it was paid for. Such that anybody can access this article online, which is really good.
Jill Brook: Hurray wonderful. That's huge. Okay, so moving on. There's just a couple more sections. The next one is clinical assessment. What are your main points that you want people to take from that?
Jeff Boris: I think that the clinical assessment in these patients includes getting a very thorough history, and as time has gone on, at least in my practice, I [00:42:00] have continued to increase the questions that I ask. This is one of those things where if you don't ask, you won't find it.
And so, it's not just about the symptoms of POTS, it's about the symptoms of joint hypermobility. It's about the symptoms of mast cell activation syndrome. It's about the symptoms of cranio cervical instability and those kind of things, right? And so it's on physical examination, it's looking for the joint hypermobility, it's looking for the venous congestion, venous pooling the legs. It's listening for the sound of what's called a bruit, which is a sound of turbulent blood flow in the upper part of the abdomen cuz that's associated with median arcuate ligament syndrome, right? So I mean, just things like that. One of the things that I have been increasingly also finding is that patients who have had a history of concussion, even if they get over the concussion, they can still have persistent symptoms [00:43:00] of vestibular dysfunction.
In other words, vertigo. And they can also have symptoms of what's called convergence insufficiency, which means that they have difficulty making their eyes work together, especially in close up reading and close up vision and people just kind of blow by those things. And so I again, it's one of those things that if you don't ask, you won't find it.
So it's making sure that we, as physicians and nurse practitioners and physician assistants do our job to elicit all the history and all the physical examination aspects of what these patients are bringing to us.
Jeff Moak: So I'd like to interject, Ask Jeff a question. As you alluded to, sometimes when you take the history of the patient, their symptoms of dizziness, lightheadedness seems consistent with orthostatic changes, but sometimes their history is consistent with vertigo.
Jeff Boris: Yes, and actually what I say to my patients now is I [00:44:00] say the term dizziness is an umbrella term. It can include lightheadedness, which is sort of that swimmy feeling like you're gonna pass out. But dizziness also includes vertigo, which is that feeling where you are sitting still and the room is spinning around you.
Right. And we need to make sure that we differentiate between those two aspects of things because the treatment for those are different. I recommend that for patients who have persistent vertigo, who have persistent convergence insufficiency be evaluated by either an E N T or a physical therapist who's familiar with post-concussion syndrome, because the therapy for those are exercises that can make that go away in two to three months.
Really? Yeah. Yeah. And really, I found historically that if you have post concussion syndrome type of symptoms, it's very hard to treat the other POTS symptoms. So if you can make the post concussion stuff go away, then you [00:45:00] have a better chance of helping to at least suppress. I can't make it go away, but you can at least help make the rest of the POTS symptoms suppress better.
Well,
Jill Brook: that's really important. I just wanna make sure that I got that right. So you're saying that if someone has some of these post concussion syndromes, such as the vertigo. Yeah. And what
Jeff Boris: were the other ones? So, so vertigo is a symptom of what's called vestibular dysfunction. Right. So that's the inner ear.
And the other one is problems of convergence. So the typical one is convergence insufficiency. You can also have convergence excess. But convergence insufficiency is the big one that gets seen. And also just being able to track and follow abnormalities and those are all things that can really be fixed.
Jill Brook: I think that we've had a chiropractic neurologist on talking about some of those things. And so what you're saying is that you see it too, it can be fixed with some exercises, and that by addressing those things first, now you make more progress with the POTS and everything else.
Jeff Boris: I find it easier [00:46:00] to be able to help suppress the POTS symptoms. Yeah. Once the symptoms of Postconcussion syndrome are managed.
Jeff Moak: I'd like to ask you, do you see outside of the post-concussion patient. I mean, some of the patients who seem to have at times symptoms of vertigo and other times symptoms of orthostatic dizziness.
And yet there's no history of again, head trauma or anything like that. So how there's this overlap. And then I think in the past, pediatric cardiologists and providers would just blow off the diagnosis of POTS when the history was consistent more with a vertigo. And cuz I've seen many of those patients, they've been previously valued by E N T doctors and the ENT doctor's done vestibular testing and hearing testing and all that stuff is normal.
They've seen neurologists and neurologist says, no there's nothing neurologically wrong yet. Just because you have a history of what seems to [00:47:00] be dizziness, lightheadedness, more consistent with vertigo doesn't necessarily mean that you don't have POTS.
Jeff Boris: No question. So the patients whom I see who have POTS, the concept of orthostatic intolerance goes along with lightheadedness, right? You're not getting enough blood pressure to your brain. You feel lightheaded. The vestibular and the convergence issues can also occur separately from that.
And I mean, to your point I'll ask about a history of head trauma. And sometimes people sort of remember, oh yeah, there was some of this like years ago, like 4, 5, 6, 7 years ago that they had head trauma and they had a headache for a few days and they felt kind of crummy and then they felt better.
But they still have sort of this stuff now. I think the other thing too though, is that you can have a postviral. vestibulitis right? So you can have this post viral inflammation, infection of the inner ear, and that can cause vertigo as well. You can also have for whatever reason displacement of the [00:48:00] crystals in the inner ear.
So for folks that are not familiar with this in the inner ear there are these hair cells that are bathed in this fluid and the hair cells are in three different planes. So they can tell us which direction we are, if we're up, if we're down for right, left, front, back.
Okay? And as the fluid moves, as our head moves, it moves these hair cells. And on the ends of some of these hair cells are these crystals. And sometimes what can happen is these crystals can be displaced by trauma or I guess whatever else. And then the crystals sort of fall through the fluid and land on some of these hairs.
And so the crystal is now pushing on the hairs and stimulating abnormally these hair cells. So it explains what's called the Epley maneuver, which you may have heard of. The Epley Maneuver is something where a knowledgeable provider, not me, can take the head of a [00:49:00] person and sort of rotate it and kind of twist and have them lean back in a certain way.
And it can reset where those crystals sit and that can actually get rid of vertigo in and of itself, if that is the cause of it. That doesn't often happen. But that is one of the ways of resolving it. Usually it's doing a bunch of exercises that retrain the nervous system to better assess its position and space.
I think the thing that we need to keep in mind is we have three different systems in our body that help us maintain our balance. We have what's called the Golgi tendon organs, which are in our tendons. It's in our joints and stuff like that. And they tell us what position we are in space.
We have our eyes, right? We use our eyes to see where we are in space, and we also have our vestibular system. And so when you have a disagreement between at least one of those systems with the other ones, that's when your brain goes, I quit. And it makes you feel bad because, there's not a concordance between those two systems.
Jill Brook: I [00:50:00] could listen to you talk all day. Nobody else is thinking about this and putting it all together like this. Now, like I said, we had heard from some chiropractic neurologists who talk about this, but you're putting it in the bigger context of kind of where it might fit in through somebody's POTS issues.
And I find that really helpful. That's amazing. Wow. And here I thought I was just gonna hear the normal stuff about clinical assessment . Okay. So I gotta ask about treatment. What are the important points there?
Jeff Boris: From a treatment standpoint? I think that the non-pharmacologic interventions are truly foundational, right?
The fluid, the salt, 80 to 120 ounces of fluid. Although, truth be told, I had patients in my long term outcome study that are. Honest to God, drinking two gallons of fluid a day. It's whatever you need. Right? From a salt standpoint we, we have historically said eight to 10 grams of salt a day.
There was a study that came out of Calgary last year that used [00:51:00] 17.3 grams of salt. It's a huge amount, right? But that showed that it suppressed the patient's symptoms. Elevating the head of the bed can be very beneficial to patients. And it's not extra pillows, it's not a wedge mattress, it's the entire bed frame just six to eight inches. And that can make a really good difference for them. Good sleep hygiene, making sure that the patients are getting rid of the electronics, getting the lights down before they go to bed. Making sure the room is dark.
Using a cooling vest. Cooling vests are great. They allow my patients who have heat intolerance to be able to go out into the world and to be able to tolerate these increasingly hot summers that we're gonna be experiencing. The use of compression garments such as an abdominal binder. And then the reason for using all these therapies is to suppress the symptoms enough so that the patients are then able to exercise and using an exercise protocol like Ben Levine's protocol or like the one that I modified, which is the CHOP Dallas Protocol which I modified for [00:52:00] adolescence specifically.
Helps to further suppress the symptoms, helps to give these patients reserve, helps to allow these patients to be able to tolerate the stresses of life. That sounds kind of cheesy, but typical POTS patient has a little viral upper respiratory tract infection or cold or whatever.
And for most folks, they wouldn't even notice it, or, it would knock 'em down a day or two. For POTS patients, it kicks their butt for a week and a half, two weeks. Yep, yep. So what we see with persistently and progressively using something in exercise protocol like the Dallas protocol, we see that when they get to a month, 4, 5, 6, that they have that reserve, that they are able to be able to deal with the stressors, that they don't have that response from a viral standpoint. From a medication standpoint, there's definitely different ways to approach medication I use florinef I use Propranolol, I use midodrine. That's what I use.
There are some folks that [00:53:00] because they're cardiologists, they're like, I will only take care of the cardiac aspects of things. And so the headaches and the GI motility. You're on your own. Find some other doctor. And then other providers, and I fall into this, a approach is I treat everything. I like to hit the three or fewer worst symptoms so we can get those down. And again, so we can allow the patients to be able to do their activities of daily living and to be able to move on to exercise.
And one of the things that I've also seen anecdotally with patients who get down the road with a Dallas protocol is they can come off of some of their meds. And that's really the goal. The goal is hopefully not to have these patients on medications for the rest of their lives, or at least as many medications for the rest of their lives.
It's one of those things where everybody's different.
Jill Brook: Great. Anything to add, Dr. Moak?
Jeff Moak: I agree with all that Jeff said. The one thing obviously is our goal is to try and make the patient as functional as possible, right? The end game is to try and [00:54:00] restore functionality both intellectually as teenagers to be able to go to school.
And the other obviously is those who wanna participate in sports and physical activity to try and get them to achieve those. And so we try and push as Jeff said, as much in the non pharmacologic as well as pharmacological realms to try and establish a more normal, healthy patient.
Jeff Boris: I think we both had success in getting kids back to school, getting kids back to sports, getting kids off to college . That's the goal. Right.
Jill Brook: Excellent. So I think we kind of covered the clinical management section. Was there anything extra from that that you wanted to add?
Jeff Moak: Yeah, we're talking about more medical things, but also the other element of it is an equal balance is also improving sometimes the patient's psychological state.
When we [00:55:00] started out in the beginning of the session talking about the long term natural history, that many times a lot of the gains in long term function is learned adaptation. And so, that is important element in helping the patient learn adaptation, not only physiological adaptation, but sometimes psychological and emotional adaptation through cognitive behavioral therapy is the buzz term. Maybe psychotherapy if the patient has underlying psychological problems or even genetic, psychological problems.
And then sometimes proper use of antidepressant medication like the serotonin uptake blockers or drugs and so forth. so it isn't all just about water and salt. I think it's also learning to deal with the psychological overlays and sometimes the parent are sometimes very depressed about the whole situation.
And like mother, like father, like child, ? And so sometimes it's the parent who sets the [00:56:00] tone. And so there's a family unit that need to have a reset in an optimistic kind of way
To
Jeff Boris: that point I quote Blair Grubb often on this point. I heard him give a talk and he said, We are not born with the tools in our toolbox for dealing with chronic illness.
And I think that really says a lot because if you have a problem with your heart. You go see a heart doctor. If you have a problem with your bones, you go see a bone doctor. If you have a problem with your brain, you go see a brain doctor. But the problem is that there's such a stigma.
And really what I want to impart to the patients and families is if you go to see a counselor for, at minimum, counseling with the assistance of managing chronic illness, you're not a failure. You're not a bad person. You're going to someone to get some advice, to get some tools, to get some tips for helping to manage this stuff. Because POTS sucks. We know this and it sticks around for [00:57:00] a long time. And so helping to cope with that makes a huge difference. I think the other thing too, that Jeff was talking about with regard to the more severe psychological illnesses is anxiety and depression don't cause POTS.
Okay. But do I think POTS can cause anxiety and depression? Yep. it takes these kids, it takes them away from what they wanna do. It takes them away from their friends, it takes them away from normality. And can that be depressing? Yep. Can you have symptoms that come on at any time that you're not prepared for and that come on at the worst possible time? Yep. And that causes anxiety too, right? And then can there be a genetic component to it? Absolutely. And so those are things that do need to be addressed as appropriate. Not everyone has it. But for those that do, it does help to be able to to put those in perspective, to get those managed appropriately.
Jill Brook: Those are such wise words, and I cannot tell you how many times I've had patients say to me how much it meant to them [00:58:00] just to hear a physician say, I get it. POTS sucks. So thank you for saying that I can just tell right now there's people listening who are who are grateful for that. So yeah, what I'm hearing is that you take the gains where you can get them and you apply that energy, that mojo to get your next gains and you bootstrap as well as you can.
Jeff Boris: It's definitely the marathon, not a sprint approach. Right.
Jill Brook: Okay. Well, talk to me about future steps. What are the main points you wanted people to take from that?
Jeff Moak: I think that the future is bright that there's more and more research being done in into POTS. The Covid pandemic is causing a lot of children as well as adults, mostly adults have dysautonomia, post COVID.
And so there's gonna be tremendous research done in the dysautonomia world that will have paid big dividends, I think not immediately, but in the the medium to long [00:59:00] term to help.
One of my own personal pet peeves is the issue is I'm a cardiologist and we deal with the dizziness and the faint thing and the tachycardia. but that's not the whole sum game for the patient.
A lot of the issues are fatigue, mental fog, mood issues, sleeping issues, those things. I think you can give all the midodrine in the world, You can give all the IV fluids in the world and you're not gonna cure that kind of stuff. And that's where this the future research will come particularly in the post covid population.
Really understanding the primary causes for fatigue and mental fog, which is again, I think my big pet peeve is really that adolescence is a period of growth, not only a maturation of your body, but also of your intellect. And if you have mental fog you're screwed.
I mean, in sense of your ability to learn and grow is inhibited. And so one of the things we didn't really talk about what the long term outcome, what's the employability [01:00:00] of POTS patients? What kind of jobs could they have had if they didn't have POTS?
And I think as a parent, that's one of the things I think that otherwise the angst that they have, like I had plans for my kid to be a lawyer, or I had plans for my kid to be a doctor or an engineer or whatever. And you see that, that isn't gonna probably happen in this tremendous disappointment in that.
I have many, very successful POTS patient in college, a number of physicians who have POTS and stuff. But for the majority of patients it does pose a very high mountain to kind of climb when you have these issues.
And the hope is that research funding and now the this long haul COVID situation will provide insight and answers and potential treatments for these issues of the fatigue and the mental fog and that interfere with their life.
Jeff Boris: Yeah, it's interesting. I think that because we've recognized, and I saw this a couple years [01:01:00] ago when we started noticing that long Covid was creating a POTS or POTS like syndrome. I'm like, this is interesting because what's gonna happen is there's gonna be more people interested in treating it, more people interested in researching it, and when we get more data, all boats will rise, right?
And so we'll be able to not only take care of the long COVID, but POTS folks as well. I find it fascinating the number of my patients who've gone into healthcare fields medical, Nursing school, social work, physical therapy.
I'm just amazed so many people. I wish I had good data good numbers for that to actually be able to report on that, but I think it's just so cool that so many people are interested in doing that. I do have data with regards to employability and employment. That's from my long term outcome study as well. So hopefully in the next year or so we'll have those data published. Also. Yeah, that'd be great.
Jeff Moak: See? Yeah.
Jill Brook: Yeah. We cannot wait to see [01:02:00] that. Well, thank you so much. This article is brilliant. ,
Dr. Boris and Dr. Moak, thank you so much for speaking with us today. Your article is brilliant. I hope it's setting a standard for future articles about POTS. You do such wonderful, painstaking compassionate work to educate the medical community about POTS and to help your patients, and I really do believe that with articles like this one, I think more doctors are gonna be aware of POTS and truly, truly interested in it. So we are really grateful for all of your ongoing work. Thank you for being here today.
Jeff Boris: Thank you so much for inviting us. I'm hoping the the adults will come around and do a review article similar that kind of compiles everything that can really put it all together too. That'd be cool.
Jill Brook: Excellent. And hey listeners, we hope you enjoyed today's conversation. We'll be back next week, but until then, thank you for listening. Remember, you're not alone, [01:03:00] and please join us again soon.
