Episode Transcript
Jill Brook: Hello, fellow mast cell patients and marvelous people who care about mast cell patients. I'm Jill Brook, your hyperadrenergic host, and today we have another amazing, not to be missed episode of Mast Cell Matters, Deep Dives in Mast Cell Activation Syndrome, or MCAS, with our incredible, most appreciated, phenomenal guest host, Dr. Tanya Dempsey. We are so lucky, Dr. Dempsey, thank you for hosting, and which of your illustrious colleagues did you bring with you today?
Dr. Tanya Dempsey: I'm so excited to have Dr. Jeff Boris here today. And I'll go ahead and introduce him and then we'll dive right into the questions. Dr. Boris is a graduate of Washington University School of Medicine in St. Louis, Missouri. He completed his residency in general pediatrics at Keesler Medical Center, then completed his fellowship in pediatric cardiology at the Children's Hospital in Denver.
He is board certified in general pediatrics and in pediatric [00:01:00] cardiology by the American Board of Pediatrics. He practiced as a pediatric cardiologist in the US Air Force in Germany and at Keesler Medical Center, and then was in private practice in Asheville, North Carolina. He was at the Children's Hospital of Philadelphia from 2007 to 2019.
He is an outpatient pediatric cardiologist, but he has been caring for patients with POTS since 2002. He opened the POTS program at the Children's Hospital of Philadelphia in January of 2014, and created one of the largest pediatric POTS databases in the country. with which he has published several research articles.
He received the Dysautonomia International Physician of the Year Award in 2016, recognized for his warmth, compassion, and tireless commitment to his pediatric and young adult patients. His academic interests include clinical database research in pediatric [00:02:00] cardiology and in POTS. Welcome, Dr. Boris.
Dr. Jeff Boris: Thank you so much, Tanya. It's good to be here and good to chat with you about this.
Dr. Tanya Dempsey: I'm, I'm really excited to, to hear your perspective on, on a lot of different things. We're gonna we're gonna cover, of course this is an MCAS podcast, right? So we're gonna talk about MCAS, we're gonna, we're gonna talk about POTS, of course, that's your area of interest. And so what I'm really curious about is, as a pediatric cardiologist, how much MCAS do you think you're seeing in your patient population?
Dr. Jeff Boris: Yeah, so, you know, it's, I have a very interesting relationship with MCAS, to be honest with you. So, as you said, you know, I've been taking care of POTS patients now for almost 22 years, and certainly when I was in the Air Force and in private practice, you know, I saw a few patients, but when I got to Children's Hospital of Philadelphia, I saw a lot more patients, and a lot of them were much more severely [00:03:00] debilitated, severely ill than I had previously seen.
And around 2014, 2015, I had this one patient who, no matter what I did from a POTS standpoint she just didn't get better. And she went and saw a renowned MCAS specialist and I'm not sure how she ended up getting to see her, which saw a renowned MCAS specialist in New York, diagnosed her with MCAS, treated her with MCAS, and she was so much better.
And I was like, what is that? Right? So what is MCAS? And as, as time has gone on, you know, I sort of was slowly recognizing MCAS. Kind of more and more in my, in my clinic and when I left CHOP and, and I have, I'm now in a telemedicine only consultative private practice that's been open for three years now.
And so I've been really paying attention to [00:04:00] this you know, in the literature I have seen 10 percent of POTS patients, having MCAS as quoted. We did a long term outcome survey that we just submitted for publication. The survey was done in summer of 2021. Had 227 patients, so pretty sizable.
Average age of the patients was like 21 1/2 and average duration of symptoms from onset to the time of the survey was 9 1/2 years. So pretty long. In that survey, which we asked a ton of questions 20 percent of patients reported that they had Mast Cell Activation Syndrome. And I'm like, oh, interesting.
So that's higher than what's been reported in the literature. I, I have been wanting to do this and, and when, when I was asked to do this podcast, it sort of kind of pushed me to do the, to do the rest of this. And so I went through and [00:05:00] looked at the data through my clinic that in my, my present clinic now. And what I think you're gonna hear is amazingly surprising, right?
So what I did was we looked, I looked at several things. I looked at connective tissue disorders, and I also looked at MCAS. So in my present clinic, which has now been open for three years. I have 147 patients, the mean age is 18 and a half, 127 of them or 86 percent, are female, right, so the rest are male. I'll skip over the connective tissue disorder stuff, and we can talk about that later if you like. But, here we go. So, for patients with POTS, so POTS, just POTS alone with MCAS, I'm seeing 103 out of those or 70%, 70. 1%. Huh. Right.
Jill Brook: I knew it!
Dr. Jeff Boris: And, I, now, [00:06:00] to be fair, I think there are some very strong limitations that we need to apply to these data.
Number one again, I have a consultative only service, I don't accept insurance, and so that limits the kind of patients who come to see me, right? So, so there, so that's that. Number two, from an MCAS standpoint, and we can, we can discuss this further, what I am using as my criteria, diagnostic criteria for MCAS are the following.
Because there, because there are a number of overlapping symptoms, within MCAS and POTS. I put a lot more of my emphasis on the cutaneous manifestations. So, pruritus or itching, urticaria or hives, flushing, the hot flashes, dermatographia, or the you know, the ability to if you, if you scratch[00:07:00] your skin you get either an elevated an elevated rash, or you get worsening itching or burning.
And you know, plus minus a combination of oral allergies, oral, oral food, oral responses to, to foods and that kind of thing. Because my patients are from all over the country, I have, I have licenses in multiple states across the country, I can't necessarily depend upon using laboratory studies. So I, what I do also is I do depend on the use or the response to medication therapy so antihistamine therapy, etc. for their mast cell activation syndrome. And so, and I think to be fair, you know, there, there, as I'm sure you know, and as I'm sure our listeners know, there's a fair bit of disagreement amongst various [00:08:00] factions on the diagnostic criteria for MCAS out there, but in, you know, and I think you could, I think we could also potentially say that um, you know, could these be just straight up allergies and not actual MCAS. And the answer is sure, right? I mean, these cutaneous findings, you know, could be sure. But what I see is, and I don't have specific data for this, but what I see is in part, you know, I'd probably say about 30 to 40 percent of the patients I'll also see improvement in some of the GI symptoms, so nausea, bloating, diarrhea. Okay, and in about 10 to 15 percent of the patients, I would say that I would see improvements in the cardiovascular symptoms, the orthostatic intolerance. So, lightheadedness, tachycardia, even the brain fog, right? [00:09:00] Because I think we do have good data to demonstrate that brain fog is directly associated with decreased cerebral blood flow.
So if you improve the cerebral blood flow, you can have improvement in in in fatigue and brain fog. So, so that's kind of what I'm seeing. So even if, let's say for the sake of discussion, even that 70. 1 percent is an overestimate of MCAS in the association with POTS. It's still, when, when, when push comes to shove, I would still say it's still going to be more than 20%. So, short question, long answer.
Dr. Tanya Dempsey: No, that was perfect. That's perfect. Because I think that's in line with what many of us are seeing. But because of your interest in POTS, to see that strong correlation, or association, I think is important. You know, what I'm curious about, because you mentioned also that you did [00:10:00] look at hypermobility syndrome as well. Yeah, I'd love to hear your numbers on that because we're gonna segue into, you know, what we'll call the triad after that. So please talk a little bit about what you've, what you were seeing.
Dr. Jeff Boris: Absolutely. So with so, for the POTS and, and by the way, with, for the MCAS, there was no the difference between males and females, i, I always split it out by, by sex as well, there was no statistically significant difference in MCAS between males and females. Same. for the Connective Tissue Disorders, interestingly enough.
So, in the, for my POTS patients, 73. 5 percent had any kind of a Connective Tissue Disorder, okay? Within the Connective Tissue Disorder, 69% had hypermobility spectrum disorder and 19% had hypermobile Ehlers Danlos syndrome, and then another 10 ish percent had what, what would be referred to as [00:11:00] localized joint hypermobility if we're going by the 2017 papers, that, that came out. And then what I did after that was I said, okay, so now if we combine POTS and then any connective tissue disorder and then, you know, my, my sort of soft definition of Mast Cell Activation Syndrome, 53. 7 or 54 percent of patients had POTS and a connective tissue disorder and MCAS. And I also broke that out by, you know, 35 percent had the, 35 percent of patients had Hypermobility Spectrum Disorder, 6 percent had Localized Joint Hypermobility, and 14 percent had Hypermobile Ehlers Danlos Syndrome.
We published a couple years ago on just the prevalence of joint hypermobility syndromes in POTS didn't pay attention to MCAS, and overall that was 61 [00:12:00] percent of patients had some sort of joint hypermobility. So, and that's, that's kind of, so these data are sort of in line with that, I would say, maybe a little bit higher.
I've seen Amanda Miller has published stuff on this, and, and I've seen other folks have published on this as well, and their numbers are, are a little bit less than ours. Some, some some of the research has only published on hypermobile Ehlers Danlos Syndrome and don't talk about patients who don't have EDS but still have joint hypermobility.
And, you know, in the big picture you know, it's all a connective tissue disorder, right? And, and so, um, it's, it is interesting to me and we can talk about this more, it's interesting to me that in the bigger picture, I'm not sure that there's in the end going to be a whole huge difference between hypermobile EDS and hypermobility spectrum disorder.
Dr. Tanya Dempsey: Well, that, that's interesting. And [00:13:00] then, you know, if we sort of segue then from talking about the triad, let's say, and we know that some of these patients with these three disorders also have other comorbidities. What I'm curious about is in your experience, and maybe you didn't look at the numbers, but just sort of anecdotally, do you have a sense of the order in which many patients develop these various conditions?
Do you have a sense of, is it MCAS first that then evolves into POTS? Is it POTS first that, you know, do you have a sense of that order? I have a sense in my patients, but I'm curious what you see.
Dr. Jeff Boris: I'll be very curious to hear what you have to say on this. The short answer is I don't. What I do have data for is in our long term outcome survey, 55 percent of patients were completely normal. And then within three months, or three months or fewer, three months or less prior to their POTS, [00:14:00] they had some sort of a trigger infection, concussion surgery, non concussion trauma, like a fracture, growth spurt, menarchy.
Okay. And then, and then of course the controversial one, vaccination. Right. Um. And then 33 percent there was, you know, they said there was always something going on. Abnormal GI, headaches, abnormal sleep, since they were little. Some of them even little, little, like infancy or toddlerhood kind of thing.
But most of the time it was during some, you know, some of these symptoms were noticed during elementary school age, that kind of deal. And, and things just progressed from there. Do I have a sense as to which, do I have a sense as to which started first? I don't. Um, I, I will say that most, [00:15:00] but not all, of my patients with hypermobility, knew they had hypermobility from when they were younger, and, and that, that just sort of was a thing.
Having said that, it always makes me laugh when I, when I demonstrate hypermobility to a patient who didn't know they had it, right? And I'll do a Beighton score, and they'll be like, I didn't even know I could do that Right.
And you know, I, I've heard, I've heard some folks suggest that mast cell activation syndrome induces joint hypermobility.
I, I am, I am skeptical, you know, pretend I'm from Missouri and show me. I, I, you know, I'm kind of, I, I, I question that. But, having said that, I have had a couple of patients where the parents were stone cold sure that their kid did not have joint hypermobility, period, end of statement, have a nice day, [00:16:00] and they all of a sudden did.
So, you know, I think that's probably going to be a small percentage, though, a significant minority, or a small minority, I should say. I think most of the patients who had hypermobility kind of came to the table with it.
Dr. Tanya Dempsey: Yeah, I think that's, I think that's my experience as well. I think, I do definitely like you have maybe a small subset of patients that developed more hypermobility, let's say the worse their MCAS was. And I think that, you know, from my experience, there was this, a little bit of that, I'll call it a vulnerability or a, a touch of what could be MCAS. Already, at that young age, where there was hypermobility as well, like, they're starting to see that, and there's just some hint of something that could be in line with MCAS. It could be [00:17:00] headaches, it could be GI issues, it could be the skin issues, or allergies, or, you know, just these subtle, very, very subtle, along with that hypermobility.
And I agree with you, those triggers then seem to just bring out.
Dr. Jeff Boris: Right. And so there are a couple of things that come to mind. One is we also published a a study on family history, right? And so in pediatric POTS patients, we found that 13 percent, sorry, 10 to 13 percent, had a family member with POTS. 31 percent had a family member with orthostatic intolerance that includes POTS or just lightheadedness, syncope, that kind of deal.
20 percent had a family member with a connective tissue disorder of some sort, joint abnormality, that kind of deal. But if the patient had a connective tissue [00:18:00] disorder, had joint hypermobility, that bumped up to 26%, having a family member with that too. And then, and then just for kicks and grins, 45 percent had a family member with autoimmune disorder. Okay? So the second thing is that I, I'm very much on the, in the, in the group that believes that, that POTS, at minimum POTS, is probably, falls into probably this concept of the two hit hypothesis, where patients have some sort of a predisposition in one way, shape, or form, and then they are exposed to a second hit.
Right? We got a grant we got two grants actually from two families that we are doing genomics research and have identified a couple of different putative gene [00:19:00] markers for for, in association with POTS. Hopefully we'll be able to publish that next year. So, we'll see.
Dr. Tanya Dempsey: Very exciting. So is this something that you think eventually, once you publish, this could be something that we could use as a screening tool for, for patients? How do we, you know, the question is how do we intervene before patients get really sick, right? Is that something that you think could be useful eventually?
Dr. Jeff Boris: I mean, in one way, shape, or form, I think do I think it'll, do I think it is going to be able to be useful from a a preventative standpoint? I don't. Because like with everything else you know, there's probably going to be a lot of people who carry this gene marker. That doesn't necessarily mean that they're going to develop this disorder or, or these sets of disorders. I think the second thing that goes along with that is, like everything else in the body, everything [00:20:00] is interconnected and so many of the tools that we use as therapeutic agents are still quite blunt instruments. And the unintended consequences, the downstream effects, the things that we don't expect to happen would, what I think, be unpredictable.
You know, and so I think, I think we're just not there. Now, having said that, you know, once we identify these, you know, gene markers and we can, and we can try to look down the road at some sort of prevalence of this, would those be useful for coming up with some attempt at a therapeutic as opposed to a preventive measure? Maybe. Maybe.
Dr. Tanya Dempsey: So speaking of therapeutics, I would, I'm really curious about your approach to a patient with POTS. So they present to you, you have a consultative practice. What is your, sort [00:21:00] of, approach to that patient who presents to you with POTS? Where, where do you go with that?
Dr. Jeff Boris: As you can imagine, I not only ask about symptoms of POTS, and I have almost 30 symptoms that I go through that, that I you know, review with them. But then I also ask about symptoms like I talked about before, actually about symptoms of MCAS and I ask about also dry eyes and dry mouth looking for Sjogren's Syndrome. And you know, and we, and well, and, and so I try to consider as I'm talking with a patient, what other things, because there are a lot of other comorbidities that can come along with POTS between cranioscervical instability, Median Arcuate Ligament Syndrome, Small Fiber Neuropathy, you know, all kinds of not so fun entities.
So I try to get a sense of what's going on from the, from that standpoint as well. And [00:22:00] then once it comes down to therapeutic approach first off, I say, you know, and I tell all my patients this, in 2023, we're still quite clueless as to what exactly is happening in POTS, right? So we do not understand why what's happening is happening.
We do think that a large proportion, if not all have an autoimmune or an autoinflammatory etiology for it, but we haven't been able to prove that yet, and I'm hoping the next 5, 10, 20 years we'll have better research that will point us in that direction. But in the meantime, we are absolutely able to control symptoms and make these patients more functional.
So my approach is, in the bigger picture, my approach is to suppress these symptoms for two reasons. One, to be able to allow these patients to be able to do their activities of daily living, go to school, hang out with their friends, you know, be as normal as they can be. And I, and I sort of joke with them, I'm like that all, and I, I joke with them, and I say that also means cleaning your room and taking out the [00:23:00] trash, because these are adolescents, right? And then, and, and the other goal is to be able to allow these patients to exercise because we know that exercise that is done consistently and progressively helps to suppress the symptoms of POTS. And with the caveat that what works for one patient doesn't work for everybody, and I've absolutely had patients do all the right things and still feel like garbage. And so, the first thing that then comes to my mind is, what else am I missing? Okay, right? So you have to go back to the drawing board and say, you know, obviously you don't blame the patient for this, you say, you know, did I miss something? And but from a therapeutic standpoint, I use both non pharmacologic and pharmacologic therapies. I, I enforce with, reinforce with my patients that the non [00:24:00] pharmacologic interventions are foundational. And we do, you know, the significant fluid and salt loading and then talk about such things as elevating the head of the bed specifically on risers or a cinder block, you know, and I give specific details on that, and if you want, we can discuss those specifics. When you wake up, just chug 16 ounces of cold water straight away when you get up. Use of an abdominal binder. The data show that abdominal binders work better than compression stockings. Use of a cooling vest. Good sleep hygiene. Um, you know, those kind of things.
And then, and then we'll talk about the exercise protocol that, that I recommend. From a pharmacologic standpoint my approach is very personalized for the patient. What I like to do is, I, I, I find that a lot of, I find that a lot of places do one of several things. One is, if they're a cardiologist, that's all [00:25:00] they take care of is the cardiac stuff.
So if they have, you know, brain stuff, if they have GI stuff, they don't want to hear from it. They have to send you to someone else. I don't do that, right? I mean, I'm double boarded. I've been doing this long enough. I sort of feel like, how hard can this be? You know, it's, it's, it's, just learn about these medications, use them, talk to friends first, you know, talk to your colleagues first, learn about them, and use them. It's okay. And then the second thing is I find that there's It's often a one size fits all approach by a lot of providers therapeutically, and they don't always use medications for the right reasons. So, and I'll get to that in a second. So, what I do is I I look into, you know, I ask, what are your three or fewer worst symptoms that are interfering with your activities of daily living?
Because then I go after those. And so it varies amongst the patients. Sometimes it's the orthostatic [00:26:00] intolerance, the lightheadedness, the tachycardia, the palpitations, that kind of thing. Sometimes it's the fatigue and the brain fog. Sometimes it's their headaches. Sometimes it's their GI dysmotility. Sometimes it's their chronic pain.
Right? So, so it, it doesn't make sense, it doesn't make sense to go after orthostatic intolerance if their GI dysmotility is, is causing a problem, and especially if they're having bad GI dysmotility, they're not going to be able to take in, a lot of times, they're not going to be able to take in adequate fluid and salt intake, so, I mean, you could knock out orthostatic intolerance, or at least reduce it anyway, if you had adequate fluid and salt intake, but if they're having early satiety, feeling full early, severe nausea, severe constipation, bloating, that kind of thing, you know, you're not going to get anywhere.
The other thing is how I utilize the medication. So, for example, there, as, as, you know, as you and probably [00:27:00] a number of your listeners are aware, one of the potential therapeutic approaches to treating POTS is the use of medications to slow the heart rate down. So either low dose Propranolol, which is a beta blocker, or Evabredine, which is a special channel blocker that works at the sinus node, the natural pacemaker of the heart.
And the way it, and, and what it does, the use of the, the use of those beta blockers, what they do is they slow the heart rate down to prolong diastole, to prolong the relaxation phase of, of the cardiac cycle. By prolonging that, that increases the amount of blood that fills the heart. What that does then, that increases your cardiac output and that causes improved blood flow to the brain.
Cool, right? I shoot for a resting heart rate on those meds under 80. If we [00:28:00] say that the normal resting heart rate for someone aged 16 and over into adulthood is between 50 and 100 I sort of use 80. I kind of just arbitrarily use 80 as a cutoff. And the deal is, is that if I have a patient who's come to me and their resting heart rate is in the 60s or 70s, then what's the point of putting them on a beta blocker?
But a lot of providers don't think that way. They're just like, I'll just put you on this and slow your heart rate down even further. And that makes no sense to me. You know, I think, I think you have, there has to be a rational, personalized approach, to managing these patients. And so that's, that's how I think of it. You know, another one I'll use Ciproheptadine or Periactin. Antihistamine. It's great old antihistamine, it's been around for decades, and it's one of the few antihistamines that's actually prescription still. But it has a lot of wonderful attributes.
It does [00:29:00] four things in my estimation. One, as an antihistamine, obviously it can help with MCAS. So there you go. Number two, it makes you sleepy. So if you have insomnia, difficulty getting to sleep and or staying asleep, you take it at night, that can help. Number three, it is also beneficial in the prevention of migraine.
And number four, it increases gastric accommodation, so, I've had a lot of patients have improved nausea, have improved appetite with with the use of Periactin to the tune that some of them have actually gained unwanted weight, like, I mean, they've really put on weight, extra weight that they, you know, it's almost too much.
So I, I think, I think again, you know, the use of rational therapeutic approach in, in caring for these patients makes a better difference for them in being able to get back to what they want to do. And it, and honestly, it allows the patients to feel like they're being heard, you know, as opposed to just, [00:30:00] here's the therapy, I'll see you in a month.
Dr. Tanya Dempsey: We sort of touched on this earlier, and I want to get back to the exercise part that you, that you mentioned, but you mentioned earlier about, you know, the percentage of patients that have MCAS and you know, certainly I'm assuming that if they have those symptoms that, that are suggestive of MCAS, you are, are attempting to treat them for MCAS as well.
Is that, is that correct?
Dr. Jeff Boris: You bring up an interesting point. One of the things that I'll do if I have a patient, for example, who has, you know, maybe the skin manifestations, the cutaneous manifestations of MCAS, but also has nausea, diarrhea. So not constipation, but nausea, bloating, diarrhea. Then, I will say, you know, instead of, from a GI standpoint, let's say not, you know, let's say GI was one of their biggest complaints, instead of going after GI with, say, Ondansetron or [00:31:00] Zofran, which is an anti nausea medication, I'll actually try to go after their MCAS first with antihistamine therapy, with H1 and H2 blockade, and if I have a patient who, who has MCAS or who I whom I think has MCAS and their lightheadedness, tachycardia, that kind of thing, that orthostatic intolerance is a concern, I will again not infrequently start them on antihistamine therapy with the understanding that, again, specifically in, in, in, again, it's my experience with over almost 150 patients, that in those patients who have MCAS, which is what did I say, about 103, which was 103 of them. In those patients who have MCAS, I've only seen 10-15 percent of them have improvement in their orthostatic intolerance with the addition of antihistamine therapy. So not a lot, you know, I mean, you would, and for our listeners out there, [00:32:00] the reason why we think that patients have lightheadedness palpitations and all those things in the setting of MCAS is because the inflammatory mediators that are put out by the mast cells also cause the blood vessels to be leaky.
The goal of a mast cell is to get the immune system to respond to what they think is a foreign protein, right? An invading protein, bacteria, virus, that kind of thing. And so to allow the white blood cells to get out into the tissues and to fight off this foreign invader, you have to make the, you have to make the the blood cells leaky.
So if you are now having continual exposure to these inflammatory mediators and you're causing these blood vessels to be leaky, that can cause the, the leaking into your tissues, right? And so you get you can get, have some edema, you can have decreased blood pressure, [00:33:00] decreased cerebral blood flow etc.
I've been kind of unimpressed. I've had a few, like I said, I've had a few patients do better, but most of them, yeah, not so much from, from that standpoint, which is still, it doesn't mean that their cutaneous manifestations don't get better, right? The itching and the hives and all that other stuff, and they, they appreciate that improvement, but I still then say, and usually in that, in that same clinic note when I'm suggesting antihistamine therapy for the lightheadedness, I'll say I would have a low threshold to start either Propranolol or Fludrocortisone or Midodrine or, or, you know, something like that to help support their blood pressure.
Dr. Tanya Dempsey: How often do you use other MCAS targeted therapy beyond the H1 and H2s, like low dose naltrexone or Cromolyn? Because I'm wondering if those other agents might have a different effect.
Dr. Jeff Boris: [00:34:00] Yeah, so, and, and I think to your point, you know, there, there are so many different inflammatory mediators that, that are put out by the mast cells that we, we don't know which ones are necessarily going to be which therapies are necessarily going to be effective. Right? So, yeah, I mean, I I have had patients try Quercetin.
I've had patients try Montelukast or Singulaire. I've had patients do low dose Naltrexone, especially in the case of chronic, those with chronic pain. I've had patients add oral Cromolyn or Gastrocrom especially if they have GI symptoms. I actually had one patient, I, I, I I took this from the group that you and I are a part of that discussed this.
I had a patient add Cromolyn to some topical cream and it really helped to reduce her cutaneous rashes. I think that [00:35:00] it is interesting, you know, specifically with Cromolyn, with Gastrocrom, we talk about how Cromolyn is not systemically absorbed, right? So, if you inhale it, it stays in the lungs.
If you put it on the skin, it stays on the skin. If you put it in the GI tract, that's where it works. But, I think there are some, I think there are some patients that if you specifically give them Gastrocrom and you are able to stabilize their GI mast cells, the inflammatory mediators that they would put out would still go systemically.
And so if you reduce that, that can still, that can still to a point help to reduce some of their systemic symptoms. So it's interesting. I mean, like I said, I continue to learn all the time. In some ways, I mean, I, you know, in seeing some of the things that you have said and some of, and your colleagues have said in this group that, that we, that we kind of discuss patients and [00:36:00] patient care on I, I, I feel like I'm, I'm learning a lot and I feel like I'm quite behind in some ways.
Dr. Tanya Dempsey: No, no, not at all. We're learning from you and, and listen, we're all learning every day because, you know, there are new presentations. Every patient is different and putting those pieces together for the individual patient, right, is, is the challenge. I want to, I want to go back to the topic of exercise, because you mentioned you have a protocol that you use with your patients.
And it's, to me, exercise is a little controversial. You know, I treat patients with ME/CFS, and the ME/CFS world and Chronic Fatigue Syndrome, it's exercise could make it worse, them worse. We have to be really careful about our recommendations for that and how we approach that. You know, I would say that for many MCAS patients, it's the same.
Having said that, though, I know that ultimately, if I'm getting the patient better and they start to [00:37:00] exercise, exercise is only going to help them actually get even better, right? So, but it's sort of like, well, how do you start that and how do you approach it without making them worse?
Dr. Jeff Boris: So, and, and it is, it is really a hot button, not actually not a hot button topic, it is a third rail for, you know, to, to talk about the concept of exercise in the setting specifically of ME/CFS. And and so we do see post exert, you know, essentially we see this concept of post exertional malaise in those patients.
In the POTS patients, a few things. Number one, classic story, POTS patient has a good day, exercises like normal, they're dead for the next three days. Okay? And so we, we, we understand this. We, we know that this happens. But having said that, I think we need to keep in mind that upright exercise specifically worsens the [00:38:00] symptoms of a POTS patient who hasn't been exercising routinely.
Number two, a lot of patients with POTS, because they feel so poorly, either go to bed or avoid exercise to avoid feeling worse, and that leads to deconditioning. There are some folks out there that believe that deconditioning causes POTS. No way, Ray. It's the other way around. POTS causes deconditioning.
Okay. And and so, the deconditioning absolutely makes these patients have no exercise tolerance whatsoever. So, some years back and, and I'm probably a number of your listeners have heard of this Dr. Benjamin Levine at the University of Texas Southwestern, which is in Dallas, put together an exercise protocol.
One of my patients was actually in his original exercise assessment. And she did [00:39:00] significantly better using the exercise protocol. He was kind enough to give me a copy of the exercise protocol back, back when, some years ago. And what I did with, when I was at Children's Hospital of Philadelphia, what I did with one of our physical therapists was we sat down, we rewrote the protocol, made it a little bit easier to use made it more adolescent friendly, made the heart rate charts more appropriate for adolescents, because that's what I do you know, that's the patient population that I'm seeing.
And and I think what, what I, as I say to my patients about this, I, I refer to this as the Dallas Protocol, and, and you can, you know, our, our readers can find, or our listeners can find this, if they look online, they can do a Google search for Dallas CHOP, CHOP, POTS, right? The the Dallas protocol that I rewrote or revised is hosted by Dysautonomia International on their website, and it's an 8 month protocol. It comes with 8 months of calendars, so it tells you what to do every day for the next 8 [00:40:00] months of your life. It does leg and core strengthening, and it does cardio. And as I say to my patients, it starts out low and slow.
So the low is you don't exercise upright, you exercise recumbent. So recumbent bike, or rowing machine, or swimming laps. Somewhere around month four you transition to upright. The slow is day one is like ten to twelve minutes. But it builds up over time. So by the time you get to the end of month two, you're doing about an hour a day, five to six days a week.
And what my patients tell me is they get to about month four, five, six, and they're like, you know, I really do feel like my symptoms are better controlled. Now, it's interesting. There's one to two days, non consecutive built in each week that you can skip a day. And the patient's like, yeah, I can skip a day and be alright.
But if I skip two days in a row, two consecutive days in a row, by the end of day two, they feel it. They notice it. They're like should have exercised.
Jill Brook: I was just going to ask about that. You [00:41:00] hear that all the time. What's going on?
Dr. Jeff Boris: And I think what this means is just like everything else, whether it's exercise, whether it's the non pharmacologic approach therapies, whether it's the medication therapies, we're just masking. We're just masking the symptoms. And I think that there is for whatever reason, if you, you know, if we stop exercise and stop doing whatever we're doing from either an autonomic standpoint or stop what we're doing from a muscular venous pump, a systemic muscular venous pump standpoint it's causing the hypothalamus to go, sorry guys, we're, we're back into it again.
Have I had patients who don't even tolerate that first day? Yes. So what I do is I say, that's fine. Start with 1-2 minutes, 2-3 minutes. Start just even less. And build up over time. And and it is what it is. You [00:42:00] know, you just go from there. It's hard. What I've, what I've found is a couple of things. Number one, I find that patients, that the kids who were athletes previously, do better than those patients who are not athletes.
This is anecdotal. I don't have data for this. This is just, you know, and, and I think part of that is because, number one, they're really driven to work because they want to get back to their sport. And number two, they're used to setting the time aside each day to exercise and to work out. They like the way it makes them feel.
They like the way, you know, that they do what they're able to do. And then, I think the other thing too is, you're dealing, at least I am dealing with adolescents, and adolescence are learning to adult. And so, forget having a chronic illness, much less a chronic crappy illness like POTS. [00:43:00] Just forget having a chronic illness.
Just the transition of having to take responsibility from being a child to being an adult. Just the transition of having better, better interoception. Recognizing what aspects of your body, what things that you do to your body, or don't do, make you feel better or worse? You know, these are all things that kind of feed in to I think how, how patients end up managing their disease and managing exercise and being able to get through it.
And I, the other thing is I find that the patients who do the best are the ones that say, I got this, I'm doing this, I'm taking this on myself, as opposed to the ones where they have their parents going, don't forget to do this, make sure you do that, did you do this, blah, blah, blah, blah, blah.
And it's sort of, it's, you know, more getting nagged than anything else.
Dr. Tanya Dempsey: Agreed.
Dr. Jeff Boris: Is, you know, this is them taking it on.
Dr. Tanya Dempsey: Yeah. [00:44:00] It's a tough situation, actually, sometimes, right, when the parents are, are trying to get the kids to do things that they don't want to do, right? And they're, they're trying to assert themselves as adults or trying to become adults, right? They don't want to be told all the time to do it.
Dr. Jeff Boris: I say to them, I hate like heck making you grow up quicker, more quickly than you were expecting to do so. I mean, we all have to, you know, have to grow up to a point over time. And certainly people do it at different rates and at different times. But this is one of those ones where you sort of just, there's some folks who just sort of have to kind of wait it out and wait until they're ready.
Dr. Tanya Dempsey: Right.
Dr. Jeff Boris: Wait till they're ready to take it on.
Dr. Tanya Dempsey: Exactly.
Jill Brook: Having you two together is amazing, and I'm wondering, are there any other places where you see the intersection of the heart or the cardiovascular system intersect with mast cell issues? And I know from the patient side, sometimes you'll hear patients like they blame their high [00:45:00] cholesterol on MCAS, or I don't know, they talk about being concerned about other things that might have to do with exercise.
Like, is there anything else that you come across in a day to day basis or when it's the heart and MCAS, is it always POTS?
Dr. Jeff Boris: So the short answer is no. The cholesterol MCAS thing is intriguing. Is that a real thing, or is that just someone? Is it really?
Dr. Tanya Dempsey: It is a real thing. Yeah. Now I, the way I see it is that or one of the theories, let's say, is that cholesterol is really a result of stress in the liver. Well, certainly there's hereditary cholesterol issues, right? So, so apart from those, right, where there's very clear genetic component if there's a stressor on the liver the liver then, you know, produces more cholesterol.
This is sort of how my simple understanding of this. And in some patients the liver is affected by MCAS. It, you know, it could be it could be toxin exposure, it could be [00:46:00] infection. I see very often, because of my interest in infectious diseases and vector borne infections in particular, I see this very often where the liver actually is involved.
We'll sometimes see liver enzymes elevate. The liver's actually fine. You know, we can scan them, you know, structurally looks fine, but there's some stressor on the liver. The liver enzymes are high and the cholesterol goes up. And and then when we treat that underlying, it could be we're treating the underlying trigger or sometimes we're just treating the, the symptoms or let's say the mast cell component of the issue.
Sometimes you'll see their cholesterol will come down. It may not come down to normal, it may not, it's not as profound as let's say putting them on a statin, but but yeah, it's, it's really interesting. So I do think there is a, there's a correlation for sure.
Dr. Jeff Boris: That's interesting. It would be interesting to to do a comparative trial, right, of patients who have Mast Cell Activation Syndrome versus those without, who have hyperlipidemia, who don't have [00:47:00] familial, right, so don't have familial hyperlipidemia. They sort of have just kind of the typical, you know, dietary induced, if you will.
Dr. Tanya Dempsey: Or they say, very often these patients will say, you know, no one in the family really has high cholesterol, I don't know why. And very often they'll say, you know, the diet is, is really is perfect, you know, or as good as it can be. And some of those patients also complain of abnormal weight gain. That's also a kind of a common complaint.
For no reason they, they gain weight, whether some of it is, it may be edema, you know, it may be leaking of the blood vessels, but I think some of it is metabolic. And I certainly see this connection between the metabolic processes, insulin resistance, and Mast Cell Activation Syndrome. And so I think that there's a, that's a path also.
But, but again, maybe, maybe you don't see that as much in the adolescent population as you do maybe in the adult population that I see.
Dr. Jeff Boris: Right. And, [00:48:00] and again, you know, this, this goes back to a tenet that, that I and I think all of us really in the field pay attention to more and more, and that is, if you don't ask, you won't know. Right. And so for the longest time I didn't ask about, say, for example, the cutaneous manifestations, the skin manifestations of MCAS.
And now it's part of my template, you know, for, for asking. So, you know, I routinely recommend all my patients have a thyroid panel, a vitamin D level, a ferritin level, a morning cortisol. Okay. Looking, you know, looking from that standpoint. Do I get, do I get cholesterol or a lipid panel? I mean, I've had a number of patients come already who've had lipid panels just because their pediatrician or adolescent medicine provider has, has has already screened that. That's kind of interesting to me, that that that would do that. From a weight [00:49:00] gain standpoint, I think one of the other things that we'll, that we'll sometimes see, especially from an adolescent standpoint, is Polycystic Ovary Syndrome. Where you know, we'll see with the hormonal imbalance stuff, that they'll get PCOS and that'll cause significant abnormal weight gain.
Dr. Tanya Dempsey: Absolutely, and then also there is that connection that, that I've actually presented on that connection between Polycystic Ovarian Syndrome and Mast Cell Activation Syndrome. There is an immune component of PCOS that is manifesting, and whether it's the MCAS driving the PCOS or the PCOS driving the MCAS, I haven't figured out yet, but that is a sort of bi directional relationship that I'm seeing.
Dr. Jeff Boris: Well, and I think this all plays back to, you know, since we're just sort of riffing on this, I think this all plays back into the hormonal influence. The fact that there is a significant female [00:50:00] predominance in, POTS, at least. I don't, I can't speak for MCAS, and I'll be curious to see, you know, if you, if you find the same thing, but there's certainly a female predominance from a POTS standpoint, and we published a case series of three patients who were transgender males, in other words they were transitioning from female to male, and, you know, they were doing typically fine, they were doing alright with usual non pharmacologic and pharmacologic interventions and exercise, but as soon as they started taking exogenous testosterone, their POTS symptoms disappeared. Which is not to say that I'm recommending that all our POTS patients go out and start taking testosterone, okay? Don't do it. Thank you for asking. But, but what I, but what I am saying is that it, it, and also, interestingly, in our long term outcome survey, [00:51:00] female patients had more symptoms, more symptom severity, and longer duration of symptoms as compared to male patients.
So there's, there's really something to be said for the hormonal influence for that. So now, let's throw it back to you, what are you seeing? Are you seeing a sex difference in MCAS?
Dr. Tanya Dempsey: Yeah, I think similarly, we're seeing a predominance in females. We certainly have male patients, as you do as well, with POTS, but there does seem to be a skew towards female.
Dr. Jeff Boris: The you know, in, we, in, in our, our demographics paper from a few years back had a three and a half to one female to male ratio. What I've seen as much as 5 to 1 in the literature this, the data that I just gave you early on in this podcast with our 147 patients, the ratio is 6. 5 to 1 female to [00:52:00] male.
But again, you know, it's, it's, it's not, it's hard to say that this is a generalizable cohort, right?
Dr. Tanya Dempsey: So I'm going to look at my data. You've encouraged me to go back and do a chart review to see what my numbers are. Next time we speak, we'll go over our numbers, because I'm curious now, yeah?
Dr. Jeff Boris: Yeah, you know, it's, before, before you came on, Jill and I were talking about kind of my foray into, into research, and really, I think, I think you, you don't have to be uh, you know, phenomenally you know, doing phenomenal research where you're doing double blind, randomized, placebo controlled prospective trials.
I mean, just reporting on, on the data that you have, being able to collect those data comprehensively and evaluate them, I think is so important because how else are we going to move this field forward?
Dr. Tanya Dempsey: I couldn't agree more. Wow, this was, this was an amazing [00:53:00] podcast. I was so excited to have, have you on. Jill, do you have any other questions?
Jill Brook: Well, I know there's a lot of listeners wondering which states they could work with you in or where they can find you online.
Dr. Jeff Boris: Yeah, so my website is pretty simple. It's www. jeffreyborismd. com. I have, so here's the way telemedicine works. You have to be licensed in the state where the patient is physically located. So what that means is the patient can either be in that state, you know, they can be from that state, or if they want to travel to one of those states. And just be across the border. It sounds sort of bizarre and arcane, but that's just kind of how it works. So, I have licenses in Washington, Arizona, Colorado, Texas, Alabama, Illinois, Wisconsin, [00:54:00] Minnesota, Michigan, Ohio, Florida, Delaware, Pennsylvania, New Hampshire, West Virginia. I may be getting one in New Jersey really soon. My website lists them all though. It lists the sites. And, and again it's, you know, um, I think it's like from, specifically, it's Michigan, Ohio, and Maryland, those three states. I don't have licenses in, but if you have a physician in that state that requests a consultation, then I can see patients from those states, too.
So that's how that works. It's sort of a different, it's sort of a different construct. So I see patients up through age 23 years. So, you know, every now and then I get, I get older patients saying, Hey, you know, can I get your help? I'm like, sorry. But but yeah, so age 23 and under.
Dr. Tanya Dempsey: Great, [00:55:00] great.
Jill Brook: Well we are so grateful for everything that both of you do. It has been so wonderful having you here together. We just cannot thank you enough for your expertise and your time and your research and everything. So thanks a million. This has been such a treat. And hey listeners, that's all for today. We'll have a normal POTScast episode next week and another Mast Cell Matters episode with Dr. Dempsey in about a month or less. But thank you for joining us. May your health be good to you and please join us again soon.
