E186:Behind the Scenes of The Triad Film on POTS/MCAS/hypermobility: A Crossover Episode

Episode 186 January 11, 2024 00:53:05
E186:Behind the Scenes of The Triad Film on POTS/MCAS/hypermobility:  A Crossover Episode
The POTScast
E186:Behind the Scenes of The Triad Film on POTS/MCAS/hypermobility: A Crossover Episode

Jan 11 2024 | 00:53:05


Hosted By

Cathy Pederson Jill Brook

Show Notes

We have the entire medical/production team of 5 physician/researchers to give us an update and behind-the-scenes look at the documentary they are making about the trifecta of dysautonomia/POTS, MCAS and hypermobility spectrum disorders.  We hear from doctors Tania Dempsey, Larry Afrin, Linda Bluestein, Lawrence Kinsella and Leonard Weinstock, along with patient Jill Brook, to hear how filming is going so far and how we can all help support the film.  This is a crossover episode with Bendy Bodies with Hypermobility MD, Dr. Linda Bluestein.  You can learn more about the documentary at MCASfund.org.


00:00 Introduction

00:37 Creating Awareness and Increasing Treatment Options

03:09 The Birth of the Documentary Project

05:20 Filming in New York

07:19 Filming in St. Louis

10:32 The Motivation to Work with Complex Patients

15:49 The Journey of Recognizing MCAS

17:34 The Impact of MCAS Treatment

21:00 Personal Experiences and Incorporating MCAS Treatment

22:42 The Need for an Educational Library

25:29 The Challenge of Condensing Information

26:21 Describing MCAS in Sound Bites

31:10 The Importance of Raising Awareness

34:42 Growth and Development Abnormalities in MCAS

40:03 Main Points about Hypermobility Syndromes and Dysautonomia

44:48 Partnership with LDN Research Trust

51:47 Final Words and Call for Support

54:26 Recognition and Treatment of Unrecognized Patients

55:44 Importance of Learning and Trying

56:13 Gratitude for Dedicated Doctors

56:47 Closing Remarks and Resources


You can learn more about the physicians in this episode at:

https://aimcenterpm.com/ for Dr. Tania Dempsey and Dr. Lawrence Afrin

https://www.gidoctor.net/leonard-weinstock-md for Dr. Leonard Weinstock

https://www.ssmhealth.com/find-a-doctor/doctor-details/laurence-j-kinsella-md for Dr. Laurence Kinsella

https://www.hypermobilitymd.com/ for Dr. Linda Bluestein

You can read the transcript for this episode here: http://tinyurl.com/potscast186

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Episode Transcript

Jill Brook: [00:00:00] Hello, fellow Triad patients and everyone who cares about Triad patients. Dr. Linda Bluestein: We have a triple crossover episode for you today. Joining forces between my podcast, Bendy Bodies with a Hypermobility MD, the POTScast with Jill Brook, and Mast Cell Matters, Deep Dives on Mast Cell Activation Syndrome, or MCAS, with Dr. Tania Dempsey. Dr. Tania Dempsey: Today's episode is bringing you an update and behind the scenes look at the nonprofit documentary and online educational library that we are creating to help raise awareness about the triad of syndromes, MCAS, Dysautonomia, and Hypermobility Spectrum Disorders. Unfortunately, many patients suffer with all three conditions, and we are working to increase awareness and the number of doctors who can effectively treat triad patients. Jill Brook: Hooray! Our guests today are the amazing physician researchers who are making this [00:01:00] documentary possible. They include Dr. Leonard Weinstock, GI specialist, research publishing machine, and world leader in finding answers and treatments for complex syndromes. Dr. Larry Afrin, widely considered the father of MCAS, with tons of publications and presentations, plus he literally wrote the book about MCAS called Never Bet Against Occam, and in addition to all that, he spends countless hours every week mentoring other healthcare providers about MCAS. Dr. Tania Dempsey is not only one of our hosts today, but also part of the documentary film team. She is a Johns Hopkins trained physician and researcher. She's a thought leader in the space of MCAS and related conditions. And just like her partner, Dr. Afrin, she volunteers a huge amount of time educating other providers about these complex disorders. We've got Dr. Laurence Kinsella, neurologist extraordinaire, who among other things, [00:02:00] specializes in helping complex patients at the intersection of Dysautonomia, Hypermobility or Connective Tissue Disorders, MCAS, CSF leaks, chiari malformation, neuropathy, and all manner of very complex neurological issues. And Dr. Linda Bluestein hasn't yet been filmed for the documentary, but she will be soon. And Dr. Bluestein is a Mayo Clinic trained anesthesiologist and world leading pain expert, especially when it comes to the complex and many types of pain involved with hypermobility, MCAS, and dysautonomia. And finally, I'm a triad patient, and my 17 year diagnostic delay almost did me in until Dr. Weinstock saved me. So now I volunteer in several capacities to help make things easier for patients in the future. So that's the roster, and thank you everybody for everything you're doing and for being here today. Dr. Linda Bluestein: All right, and the first question goes to Dr. Leonard Weinstock and Dr. Tania Dempsey because this entire project, I [00:03:00] believe, started as your brainchild. Can you get everyone up to speed on what this project is and why you have been pursuing it for years now? And let's start with Dr. Weinstock. Dr. Leonard Weinstock: Well, we had a Mast Cell Activation Syndrome meeting in Colorado 2019, and it was something that, you know, got me thinking. We had all these difficult patients and so few doctors out there. How were we going to get it brought to the attention of the medical community, and part of it came as an idea of what about a movie, what about a documentary, and because it's so hard to change the academic curriculum, and so, but the patients were knocking down on our doors, how were we going to get it to other doctors, and possibly just to more people so they recognize their own condition. So, I came up, and probably simultaneously with Dr. Dempsey, the idea [00:04:00] of something out there like a documentary to bring it into the public domain. Dr. Tania Dempsey: Yeah, exactly. I couldn't, I, well, well said, Dr. Weinstock, but also I'll just add that, you know, this has been a few years in the making because when we started initially thinking about it, we had reached out to some and talked to some filmmakers and it was at that time prohibitively expensive to even consider. And, you know, we're fortunate now to have support in a way that we can actually get this done, but we're, we do need more support, and we'll get to that towards the end of this podcast, but, you know, it's, it's coming to fruition but it's been a long process, and again, unfortunately everything you do like this does require funds. So that's, that was our big obstacle for a while. Dr. Leonard Weinstock: COVID was the other obstacle. Correct. Moving ahead during the season of COVID, not good. Absolutely. Dr. Linda Bluestein: Right. Um, and Dr. Dempsey and Dr. Afrin, you just [00:05:00] recently completed the first phase of filming in New York, and we would love to hear what that was like and if you could set the scene for us. And let's start with Dr. Afrin. Dr. Lawrence Afrin: Pretty straightforward for me Dr. Bluestein. I sat down where they told me to sit, and I faced the camera and answered the questions the producer asked. It was a very straightforward interview. He appeared well informed and asked, Some insightful questions and thought we had a good conversation. Dr. Tania Dempsey: It was a, it was a great experience. I, I, I had to do a lot of the behind the scenes stuff. So, Dr. Afrin was, was lucky because he was able to just walk in and he was set up. But I was working with the filmmaker to try to find the right places in the office that would work. And, and I've posted on my Facebook and my Instagram behind the scenes pictures of, you know, where we were being interviewed and and how it went down but it was, it was a lot of fun a lot of [00:06:00] work, but but I think that the information that Dr. Afrin provided and that I provided, I think will just really make this movie I think reachable for both lay people and for the medical professionals. I mean, that was my sort of approach and I think Dr. Afrin the same. You know, we, we want to make this a film that anybody can watch and, and get, get the points and, and hopefully again, reach and reach more and more people. So it was great. So now I'd love to actually find out what, how it went in St. Louis. So I'd love to hear from Dr. Weinstock and Dr. Kinsella what your experience was like. Dr. Lawrence Afrin: Laurence, go ahead. Dr. Laurence Kinsella: Yeah, the first day we'd had some time to set up, and we recorded a patient first, having a tilt table test to demonstrate what that technology is like for patients to go through. We had a very in depth interview. We [00:07:00] had a number of questions that we went over. We had a chance to repeat what was needed and focus on certain areas of interest. Dr. Leonard Weinstock: In my day with Rob was spent setting up the room and interesting camera work, you know, actually had this very low light situation. And but, you know, he knew what he was doing and had very sophisticated equipment and spent a long time getting sound bites from me. Hopefully it won't be overfill, but he, He did tell me about good experience he had interviewing my patients. I had four patients that were interviewed. A good friend of mine gave up his home for the day, very beautiful home, and that gave time to set up different areas. And Different look to them. And you know, they said, the camera man said that one of the individuals, it was just a lot of emotion was going on and [00:08:00] telling her story. So, I am looking forward to seeing that. I, I know the individual and she's just a. wonderful person who I always say to her, or frequently will say, you know, you're my greatest patient but my worst patient and I, you know, she has intravenous Benadryl going continuously and if she waits too long to change the canister she'll start to have anaphylaxis and on one trip to the emergency room where things weren't going well, she had epinephrine five times in the ambulance. So, I mean, it's just amazing how this syndrome affects patients and their life. And so, we're going to have some of that in the movie. We want people to understand how tough it can be and I mean, yeah, you can treat some patients so easily and it's wonderful when it's like that and they've gone to [00:09:00] a bunch of doctors who don't know how to add up the symptoms and look at the review systems and suspect the condition. But even when you've done so in some patients, it can be really tough. Jill Brook: Well, that brings up the next question that I wanted to direct to anyone, but I'd love to start with Dr. Afrin, which is, what brings you to this corner of medicine working with such complex patients and not just one complex syndrome, but three or more that seem to be intertwined? Because obviously there's a lot of people suffering with a lot of different medical conditions out there. What is it about the triad that makes you donate so much of your time on so many projects, including this one? Dr. Lawrence Afrin: Well, certainly wasn't planned. I was 13 years past 11 years of training 13 years into practice after 11 years of training, before I you know, came to figure out after [00:10:00] nearly a year's effort and my first patient I came to recognize as having what we now call MCAS but it was saying, I mean, her life had been destroyed by the disease. And just one month into treatment. I, well, we got lucky. I mean, serendipity is so much of many significant advances in medicine in, in all fields, and we got lucky. And the very first treatment I selected. She came back in a month and I did a double take on walking into the exam room. It was incredible how much better she was doing and that improvement has held up for more than a dozen years now. And then the next patient and I, you know, it doesn't take too long [00:11:00] before if you're. Paying even halfway decent attention to what's going on in your patients, you start to realize, even though the, the superficial details are quite different from one patient to the next, there's an overarching pattern to this, and you begin to realize, it's incredibly prevalent, and in truth, every doctor has been seeing this left and right, all day long, every day of their whole careers. They just couldn't previously recognize it for what it is, because number one, they had never been taught such a thing exists, and number two, it is so variable in the details, and honestly, most doctors only have, you know, what, five or ten minutes in a visit? You don't have time in the typical doctor patient encounter to dig into the details. You can, you know, [00:12:00] address briefly one superficial issue or another, and that's it. You've got to move on. So, I understand the challenges, the reasons why, in spite of the prevalence of the disease, not a new disease, newly recognized disease, I understand why Despite all the smart doctors in the history of medicine, we haven't, we didn't recognize this until, didn't start to recognize it until just about 16 years ago, but nevertheless, if you're paying attention, you begin to realize this is incredible. These patients have been seeing uncountable numbers of doctors consuming enormous amounts of resources and generally not getting very far with any of it, either diagnostically or therapeutically. And you see the opportunity in the first few patients, even as different in their superficialities as they were. You see just this [00:13:00] extraordinary opportunity to turn their lives around and especially considering that most of them, even if they don't get diagnosed and treated, they're probably going to live a relatively normal lifespan, so this becomes all about a much better quality of life for for, for most of them for decades to come. And for some of them it's just a quality, it's productivity too. They've been disabled you know, sick to the point of disability. And when you can take somebody who's totally disabled and get them back to work for decades to come, that's significant. So, I started. You know, figuring this out on more patients, and I began publishing, and I think that's an important part of it. And these patients who you know, thanks to the internet, they've got access to way more medical information than ever in the past. They, they come to figure this out [00:14:00] much sooner than their doctors do. And so they started coming to me from all over creation. It really was remarkable. And that's a long story, but here we are today. Jill Brook: Have others seen a similar story? Dr. Leonard Weinstock: I have. So, you referred to your own case history and you and I and your other doctor, Dr. Goodman published a case a case report in 2018 and we worked on it since basically your treatment over, since 2016. Anyway, bottom line, we, published this new treatment for you and and then if you Googled POTS and MCAS for four years, it came up as, you know, one, two, or three on Googling, and that led to many emails and people asking if they could be my patient or where could they find this treatment and [00:15:00] so forth and you know, the internet is powerful. I had a patient not too long ago who basically wrote down all of her symptoms, typed in all of her symptoms, and it came up with MCAS and my name. Because obviously she was from Missouri, but you know, that, that just shows you where people are going. Patients are ahead of the game so my, my day is, you know, 12 colonoscopies, as a gastroenterologist, come back to the office and see 7, 8 patients who either have MCAS or think they have it and often do have it. So my life has turned around dramatically since 2018 when we published that case. Dr. Laurence Kinsella: For me, it was a patient who came to see me, had a very ruddy complexion, flushed and had POTS and she said, [00:16:00] doctor, I think I have Mast Cell Activation Syndrome. Well, what is that? I've never heard of this. This has, this is, well, pre pandemic, I'd say around 20 14, 20 15, and I'd never heard of it, and my initial response was resistance. And, over the course of time looking into this more, measuring her own serum triptase, which turned out to be elevated, very lucky, because very often it's not elevated. but I had some, I had some argument for this diagnosis, and so I started to become interested in it. She mentioned that she had been in communication with Dr. Afrin, I said, who is he, and started looking him up. And, over the course of time, you know, my resistance slowly melded into if not acceptance, at least a [00:17:00] begrudging willingness to consider it but I wasn't sure how it really related to neurologic disorders, specifically small fiber neuropathy and POTS. But over time, as I got to know Dr. Afrin and became a member of our Masterminds listserv which is enormously helpful. I've, I've eventually come to the point of acceptance and, and even adoption. I, it turns out that I probably have some mast cell symptoms myself. When I sneeze, I only, I never sneeze once or twice, it's 10 times. My father had terrible rhinitis medicamentosa because of a lifelong sinusitis and basically, got himself addicted to you know, the Sudafed in old time inhalers. And so it's probably something that does run in the family. And you know, again, begrudgingly looking at potential allergens [00:18:00] like mold, I've been very resistant to that, but I'm coming around. So, I am, I am a slow adopter, but I am very impressed with that in order to convince people, you have to convince them that they might have it, or maybe their daughter or son or spouses have it. And that is a very good way, and it turns out my daughter has Mast Cell Activation Syndrome and has done well on the very easy, over the counter cocktail that Dr. Afrin and others have described. So, you know, the barrier to entry to treating this disorder, I think, has a huge advantage for us in getting people to adopt it because It's something that their doctors don't have to prescribe some controlled substances to make this work, so the hurdle is low. Dr. Linda Bluestein: Like a lot of other people, this was not a planned thing for me. I [00:19:00] grew up with severe asthma. Terrible allergies, went through all the immunotherapy, eczema, you know, irritable bowel, migraine, a whole bunch of things that we now know are associated with mast cell, and practiced for over 20 years in the operating room. And then when I opened my clinic in 2017, again, not a planned thing, but to do pain management for people with EDS. My second the patient had been diagnosed with Psychogenic Non Epileptic Seizure Disorder and was, had stopped driving and she, she was young and the mom had asked me, you know, during one of my talks, do you think you could help my daughter? And I was like, I have no idea, but I'll, I'll try. And so I prescribed and did some of the things that I had learned from Dr. Afrin. And she came back after 30 days, and I was shocked. She had had no events, and I almost fell out of my chair. I was like, it actually worked? Because, you know, you really don't know until you actually try. So, it definitely has been more of an evolution for me [00:20:00] that I've been incorporating more and more into my practice and doing more screening and, and things like that. And so, I've, I am so grateful also for the listerv and for the incredible wealth of information that you know, everyone in this space shares, which is just really fantastic because we learn so much more than if we just, you know, go off of a you know, publications, obviously, there's a lot of great publications as well, but so yeah. So for me, it definitely, as I started to incorporate more and more mast cell directed therapies in my practice, my outcomes got better. Dr. Tania Dempsey: And I think my experience is very, very similar. And I remember, I think one of, one of the ways that Dr. Afrin and I like kind of connected was I, I had done a podcast. I had already had my first patient identified. I was already getting interested. I had connected with Dr. Afrin to, to consult on MCAS. And then I started, like, once you see it, you can't unsee it. So, I just kept seeing more and more [00:21:00] patients. And, and my, my lens just shifted so dramatically that patients that I didn't have a lot of answers for or solutions for, I finally started having that, you know, like Dr. Bluestein, you know, you start being able to help more people, and I was doing a podcast and I had sort of determined I did a very, very quick chart review before the podcast, and I just wanted to see, you know, what, what percentage of my patients at that time I think it was around 2017, had presumptive Mast Cell Activation Syndrome you know, many of them proven, but you know, just broadly based on my clinical impression. And it was, you know, almost like 90%. And I think I said that on, on, on the air. And at some point Dr. Afrin and I connected, and I think he was sort of impressed that I had, had started to identify that many patients. You know, the truth is that not, obviously, not everyone has MCAS, right? I'm not trying to say that 90 percent of people have MCAS, right? But 90 percent of patients who were coming to me [00:22:00] who were sick, who didn't understand, didn't have an answer, who had been to so many specialists, who were trying to figure out, you know, a path forward. Many of them had Mast Cell Activation Syndrome, or have it and so again, you know, that, that's why I'm so passionate about doing this, this documentary, working together on educational work, because it's out there. You know, you know, based on some literature, right, we think maybe, maybe 17 percent of the population maybe, you know, has some form of Mast Cell Activation Syndrome. That's a lot of people and there are a lot of people out there who are not understood. There are a lot of medical professionals who want to learn, I, you know, I want to give them the benefit of the doubt. I think they want to learn, they just don't, they just don't know what they don't know. And and their families that have family members who are ill and they don't understand, right? So I think that if we can, because of our experiences, right, if we can put this [00:23:00] information in this film as we're doing then we're gonna, you know, hopefully change a lot of lives. That's my little plug. You know, I'd love to talk a little bit, you know, from my own personal experience with the filming. It was, I thought, difficult to really put the information into the sound bites that we needed. You know, the film will be about an hour. Obviously, you know, even with one patient, right? We spend hours with each patient, right? So it's such a complex topic. So I found it difficult putting together the ideas so that it was relayed properly in the, in the film. And I was wondering what Dr. Afrin, Dr. Weinstock, Dr. Kinsella thought about that, that piece of the filming. You know, how do we, how do we give, get that information into those, those short, snappy sound bites. Dr. Lawrence Afrin: I'll go first. I mean, I was asked that question, you know, how do you summatively describe [00:24:00] such an extraordinarily heterogeneous disease? And I said, I've been struggling for 15 years to find. A soundbite for this, because I realize there are all sorts of reasons why attention spans for everybody, including professionals, is pretty short. And I said you know, the best way I've figured it out so far is chronic it's a chronic multi system illness with general themes of inflammation. That's the universal constant that's present in every mast cell patient. Plus minus allergic type disorders, plus minus abnormalities in growth and development and potentially any tissues in the body. So that's the soundbite, but each of those words are, each of those words is important [00:25:00] and you can quickly start to see the, the potential for the variability, the heterogeneity in that admittedly very nebulous description. You can see the heterogeneity from one patient to the next, you know, inflammation, that's a very general concept and the symptoms you get from inflammation in one system or organ or tissue in the body can be very different from the symptoms you get or the consequences you get from inflammation in other tissues in the body. But again, if you back up, and I mean these patients come to us with, you know, 73 different problems already long established on their problem list, but so many of those problems end in ITIS. [00:26:00] ITIS, ITIS, ITIS, ITIS, ITIS, ITIS, ITIS, ITIS. It's all inflammation, inflammation, inflammation, inflammation. And so, you know, many of you have heard me say this, I'll say it again, what's more likely? That this poor patient is so uniquely unlucky as to have coincidentally acquired so many different problems, all of them developing independently of one another, or is it more likely they've got one thing going on that really is biologically capable of causing, either directly or indirectly, most or all, probably all, of what's long been going on in them. And yes, it's more likely there's one thing going on. I mean, this is the principle, you know, Occam's Razor. This is why I titled that book the way I did. Never Bet Against Occam. It's [00:27:00] just unfortunate that that one disease that really is capable of causing so many different problems and different patients, it happens to be, and again, not too surprising given its extreme heterogeneity, but it just so happens to be an extremely biologically complex disease. So, this creates that variability. So, when all you have is five minutes in the exam room, and all you can do is focus on one thing, you can't pay attention to the rest of it. But, if somehow you can find the time to look at this from the 100, 000 foot level and realize They just can't be that unlucky. There's got to be one thing going on, and yep, it's regrettable. I wasn't taught any such one thing that could do all of what's happened in this patient, [00:28:00] but that doesn't change the fact that it's still much more likely that there's one thing going on. So now you got to go trying to figure out what that one thing is. Unfortunately, we now have figured out what that one thing is. Again, as Dr. Dempsey said, we're not saying that MCAS is the diagnosis in every chronically, multi systemically, mysteriously, ill patient, but nevertheless, it's turning out to be the diagnosis in a lot of them. So this is all I've been trying to say for 15 years now. I'm not saying every mysteriously ill patient has MCAS. What I'm saying is, if you have a patient who is chronically, multi systemically ill with general themes of inflammation, plus minus allergic issues, plus minus dystrophisms, then it becomes reasonable for you to to consider the possibility that maybe it's [00:29:00] MCAS that's what's at the root of the patient's problem. It becomes reasonable to include this previously unrecognized disease in the differential diagnosis. That, that's, if you don't even think about it in the first place, if you don't consider it, you'll never get to the diagnosis and then you won't have any way of actually helping the patient get better. Treatment depends entirely on diagnosis. So, this is one of the most important messages, just asking doctors who have never heard about this before for understandable reasons, and the reasons why they might initially reflexively reject the notion and I, I, I get it. You know, you were, out of 10 years of medical training, you got one minute of teaching that there's, you know, there's allergy, well, [00:30:00] okay, that's a prevalent issue, but pretty straightforward to take care of in most patients, and there's only one other known mast cell disease, the incredibly rare disease, the cancer of the mast cell called mastocytosis that most doctors will never see a case of in a decade of training and three or four decades of practice. So if it's that rare, of course, you're only going to get one minute of teaching about it. But now we're beginning to realize, oh no, there's this other mast cell disorder that's kind of the polar opposite to mastocytosis in its prevalence. And therefore, now, there's every reason in the world to go for all medical training programs. I don't care if it's, you know, doctors or nurses, PAs, NPs, NDs, whatever, all medical, all health care professional training programs need to [00:31:00] be incorporating teaching about MCAS because every health professional is already, they've been seeing these patients. They just couldn't recognize it. Once you recognize it, you make the diagnosis. It's like any other disease in that respect. You give the right treatment for the right diagnosis. Look what you can accomplish. Dr. Leonard Weinstock: Very, very, very good. So many of my patients come in and talk about inflammation. That's something I had not heard patients come in and complain about more than five years ago. So, something's happening there and on the internet. Number two, I do want Larry to explain what you mean by dystrophism to the audience today because I think it's your theory on one of the aspects is really interesting in terms of the triad. Could you talk to that? Dr. Lawrence Afrin: Sure. The word dystrophism, the medical gobbledygook [00:32:00] dystrophism is just a you know, $400 word for encapsulating the notion of abnormalities or aberrancies in growth and development in potentially any tissue in the body. I mean, we know at this point that the the mast cell puts out more than a thousand mediators and many of them absolutely do drive effects that you would kind of superficially classify as inflammatory and there are many other mediators that would drive effects you would classify as allergic, but it turns out there also are quite a number of mediators that are integrally involved in guiding growth and development in every tissue in the body. So, if you get a bunch of dysfunctional mast cells, whether diffusely or even in one part of the body, that are just chronically [00:33:00] inappropriately producing and releasing various and sundry of their growth guiding mediators, then why would you even expect to get normal tissue growth and development in that area? You would expect the opposite. You'd expect to get all sorts of abnormalities. And in fact, that's what we see. Now, fortunately, they're quite often quite modest. They're almost always benign, so they usually don't have all that much clinical effect, but nevertheless, they are abnormalities, and there's got to be a reason why they're developing, and it's just another clue as to what the underlying issue is, because again, they're not so unlucky as to have all these different issues going on independently. There's one thing going on that's driving all of this. So, these patients [00:34:00] frequently I mean, certainly some growth and development anomalies with this disease are more common than others. So we very commonly see poor healing, poor wound healing. For example, we very often see development of excessive scarring or fibrosis and that kind of ties into the inflammation piece of this because fibrosis kind of is the end stage of inflammation. We very commonly see development of cysts. breast cysts, ovarian cysts, kidney, pancreas, liver, lung, thyroid, brain, spinal cord cysts. Again, usually not clinically significant, but nevertheless, they're there. There's got to be a reason for them. And many times, too, we find normal growth and development in vascular formations, [00:35:00] aneurysms, hemorrhoids tiny little red spots on the skin we medically call telangiectasias or hemangiomas. Again, not, not of any great clinical significance, but there's a reason why it's there and it's providing another clue as to what the root issue is. Dr. Leonard Weinstock: So I would just also go at your own literature that you've written about Ehlers Danlos Syndrome, hypermobile joints from growth of ligaments and tendons in childhood, and that and also other literature about endometriosis, and then going back to literature that you and Dr. Molderings published on cancer, increased risk for cancer. So, You know, I think that growth is a real big issue as part of those three conditions that you spoke to before and the [00:36:00] triad. Dr. Lawrence Afrin: It's just that the growth issues, for very understandable reasons, are kind of the hardest of the clinical issues with this disease to even recognize because the growth and development issues, the dystrophisms develop the slowest of all of the features of this disease. Inflammatory symptoms, oh, you know about that pretty quickly. The allergic issues, you know about that pretty quickly. Growth and development is usually a relatively slow process and it's very easy for patients to come back, visit after visit after visit for even many years with a given doctor and things are changing in the patient growth wise but the doctor's not recognizing it, and sometimes even the patient isn't recognizing it. But it's all [00:37:00] part and parcel of, of the disease. Jill Brook: Well, I think this is getting to why we are going to need the educational library, because there's so much to know, and this sounds like it can account for so much, but that Educational Library, just to let people know, is planned to contain resources and lectures and presentations from top experts on all of these subtopics so that if somebody wants to go deep into one of these areas, they can find all that great information, but so we're really seeing how difficult it is to pack so much information into a one hour to 90 minute documentary film, but I would love to ask Dr. Bluestein and Dr. Kinsella, what they think you you think are the main points to include about hypermobility syndromes and about dysautonomia? Because each of these things is only going to get a few minutes in the main [00:38:00] documentary before we hope to get people to go to the online library. So in those few minutes, do you have ideas about what you think the main points are that you hope people take away about these areas? Dr. Linda Bluestein: I would like people to know that joint hypermobility, or increased range of motion of joints, is actually very common and is not always problematic. But if it's symptomatic, so if you have symptoms that are likely related to that, then you have a higher chance of having dysautonomia and or having Mast Cell Activation Syndrome. So, if a person has symptoms, they're likely attributable to having joints with greater than expected range of motion, then I think it's really important for them to be aware of these conditions and so that they can take as many possible steps as possible to improve their quality of life because I think if you just address one aspect without addressing the others, you won't get as far. Jill Brook: Dr. Kinsella, in your few minutes in the documentary, what do you [00:39:00] hope are the main points people take away about dysautonomia? Dr. Laurence Kinsella: Well, the you know, as a neurologist, I got interested in this because um, uh, there was no autonomic specialist in St. Louis, and I was seeing a number of these patients. They would walk through what I would call the dizzy door, and the eventually I converted my EMG lab into an autonomic lab. I borrowed an old tilt table test. From the physical therapist, they abandoned that equipment because, guess what, it makes people faint. So they stopped using it. I picked it up and then I was able to do heart rate, deep breathing, and valsalva maneuver on my EMG machine. So, I, you know, just to add water, I had an autonomic laboratory, and I started doing this about 2005, and I've been doing it ever since. And once you hang a shingle as a dysautonomia specialist, very quickly your people beat a path to your [00:40:00] door. So, you know, as we have the hypermobility spectrum disorder, I think we also have a dysautonomia spectrum disorder. That this covers not only POTS, but also Inappropriate Sinus Tachycardia, IST. And then there are a number of patients who come to me with a, with a diagnosis of POTS, but they actually, you know, you can't demonstrate it at the bedside, but it doesn't change the fact that they get severe postural dizziness, palpitations, they have all the same symptoms. You know, you can't always demonstrate it. It can be difficult. So I think that's where the tilt table is really helpful as a closer to help people garner that diagnosis. So that plus the other autonomic studies that we do. So I think the take home point is that this dysutonomia is a spectrum, it is multi system, just like mast cell, just like hypermobility. We're all dealing in these multi [00:41:00] system disorders and they can be overwhelming for patients and their families and their doctors. Dr. Linda Bluestein: Before we wrap up, does anyone want to quickly talk about our partners in the UK? Dr. Leonard Weinstock: Well, we all, as mast cell and POTS and EDS doctors, needed to get the word out. And we were lucky enough to partner with LDN Research Trust. Linda Ellsgood and her film maker, who's done her other documentaries, Rob Jones. And they've done a good job and we hope with our input it will even be better. And throughout you know, our resources make it even more interesting. And we do need support. We've raised a fair amount of money, about half of what we need, but we do need more. And so if you go to MCASfund. org, M C A S F U N D dot org, [00:42:00] you'll see a nice preview of the movie and places where you can donate. And no matter how little or much. We just need your help and support. Dr. Tania Dempsey: And I'll add to that, that you know, these types of projects require enough funds to actually bring it to, you know, actually make it go live, right? So, there are a number of ways that this documentary could be promoted. It could be on the LDN Research Trust website and, you know, that would be amazing just to have this information out there. Our hope is that we can make it a little more widespread, a little bit more public, and so we need the funds to to do that, and you know, I'm a little bit naive when it comes to these sorts of things, and I'll be honest, I didn't really realize what goes into, where the money goes, right? And so it's maybe not a bad idea to just even just share with our [00:43:00] audience, you know, the filmmaker has to get, has to get paid. He puts in hundreds of hours probably at the end of the day in editing, obviously filming and editing, and then obviously housing this, this this film somewhere is going to require, require funds. All of us are doing this as a volunteer project. None of us are, are gaining any, any funds from this, right? So, it's really just about, like, making this film. And getting it out there. And so, we do need, we do need people to contribute. And that's, I, you know, I hate to be in this position to ask but again, we're so passionate about it. We think it's going to be an amazing film. I think it's going to help a lot of people but to get it out there. Again, as Dr. Weinstock said, we're about halfway there. We're getting close, but, you know, it's possible that the project won't make it all the way, and that's, that would be such a shame. We've all put so much time and effort and so that's what, that's the one [00:44:00] point that I think we should know, is that there's that possibility that we don't have the money to finish this project, which would, you know, be a real disgrace. So, please help. Dr. Leonard Weinstock: And on the other hand if we can make it as good as a Netflix film, then it could have widespread impact for those who suffer or are partners in the ailment, whether it be the spouse, child, or parent of the individual affected by MCAS, POTS, and EDS. Dr. Laurence Kinsella: There are over 10, 000 diseases and most doctors only know a fraction of those. And your contribution goes to helping raise awareness, to make your doctors, your clinicians, your families aware of this diagnosis and to help us do the research, but at least at this stage, the education to raise awareness to impact so many lives.[00:45:00] So, please join us in getting this made. Jill Brook: And I would say selfishly as a patient, sharing our website, that's MCASfund. org, has benefits in not only raising the funds to make this possible, but what you'll see at that website is that it actually has a quick explainer of each of the three conditions and how they fit together. And so you can send this to your family, to your friends, to your colleagues, not only to help raise awareness and see if somebody might help give us financial support, but just to have them get that quick, easy explanation that helps them process, oh yes, these are real and these are the underlying mechanisms, I'm starting to put it together. And so that's what I'm also excited about in sharing this. And finally, I just wanted to also let people know that there is going to be a virtual brick wall made with [00:46:00] names. So if you are interested in just donating $10 you can become part of that brick wall that will have your name. So it'll be known that you were part of this movement. And for people who do have the means to donate more, there are different levels of sponsorship that also come with a few perks. So you could check that out at MCAS fund. org if you're interested. Dr. Linda Bluestein: And I think that's such important information, Jill, that you, that you were just sharing that even if you can't donate financially, if you can share the website, if you can talk to people about the film, because the goal really is to raise awareness and provide education. There's a lot of, we know there's a lot more documentaries being made nowadays, but this is really, I think, such an important one because it will be providing real information that people can use and especially with the educational library as well. Before we wrap up, does anyone have any final words to share? Dr. Lawrence Afrin: I'd just like to recognize all of my [00:47:00] colleagues for taking the the brave step of stepping out of the comfort zones of their own specialties, subspecialties to basically, do what's right for the patient. Um, uh, I mean, what Dr. Weinstock, Dr. Kinsella, you, Dr. Bluestein, you, Dr. Dempsey, and hundreds of other physicians around the globe have done beyond their training to learn and to at least try to help these initially quite mystifying patients, that that's what makes all the difference. I, I understand the, the challenges in modern practice, why people, why doctors often don't or can't continue with learning or trying, [00:48:00] but to best serve the patients, and that's what we're supposed to be that's supposed to be our principal objective here to best serve the patients. I really do encourage as many doctors as possible to keep learning even if initially it might not seem like, these topics could have any intersection with your domain, but I I promise you, mast cell disease absolutely intersects with every domain in medicine. So If you can just see your way to continue learning and, and to at least try. Most of the drugs and other treatments that make sense to make biological sense to try for this disease they're, they're, they're safe. They're easy for any physician to prescribe and to manage. They're [00:49:00] just treatments that most doctors have never heard of before because they've never had to take care of these disorders before. But if you're willing to learn, willing to try, my experience and what I've seen the experiences of many hundreds of other doctors who are coming into this area, that my experience says you'll, you'll wind up helping a whole lot more patients than you might have ever suspected could be possible. I've had many, I've been very privileged over, you know, 15 years now to have been able to assist a growing number of doctors come to understand this, come to recognize it in their first few patients, and many of them have circled back to me a year or two later and said, oh yes, it's definitely in a lot of my patients, and many doctors tell me how much enjoyment it's actually bringing back to their medical practices because these [00:50:00] previously very mystifying, very frustrating patients, they could not figure out what was wrong with them, they thought they were psychosomatic, they could not effectively treat them. Now, they're getting better and I've come to describe it as sort of chicken soup for the doctor's soul and many other physicians have told me they agree with that. So, that's my closing message. Learn, be willing to learn, be willing to try. That's what your patients, your patients aren't expecting a guarantee that you can get them better. They're asking you to try. Dr. Tania Dempsey: Well said. And I'll just take a moment to thank you all for your dedication and support for this project. This has been an amazing group to work with. I'm so honored to be a part of it with all of you. And you know, we have a ways to go, but we'll keep you all listening. We'll keep you up to date and, and provide, you know, progress as we go. And thank you all for, for [00:51:00] being here and listening. Jill Brook: And I just want to say thank goodness for doctors and humans like you guys. I just love you so much and I know all the patients do too and I hope you know what a gift you give to all of us. You're just incredible. And so I guess that's all for for me for now. Thank you for listening. Patients, remember you're not alone, and please join us again soon. Dr. Linda Bluestein: And remember to check out the MCASFund. org website for more information. And just want to remind everyone that you've been listening to the Bendy Bodies with a Hypermobility MD podcast and POTScast and Mast Cell Matters. And this has been such a great conversation. And thank you so much to everyone for your incredible work and for coming on the podcast today, and this is just such a great conversation.

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