Chapters Transcript Video Building the Best Model of Care for POTS Tae Chung, M.D., presents at the Johns Hopkins POTS Grand Rounds on September 2, 2021. Okay, all right, so the I think it's building the best model of care for parts, so I'm gonna go over the slides as best as I can and so we can um move over to to discuss it. So uh first of all um I think like to this audience, I don't even have to you know explain what are the challenges of for the parts care um you know um there are a lot of challenges for sure and I try to think about one of the main challenges um they are facing for part care. I mean first of all because we all know there are not many providers out there who's even interested in seeing parts of patients nor have any knowledge or expertise, very few people find and I think it's in part because of lack of knowledge there about parts, it sounds like something that we learned in the medical schools, the nursing school and also a lot of people are just not interested in seeing population and in part because there's a misunderstanding about the part patients, there's some stereotypes against part patients. Right? A lot of, I mean a lot of doctors don't even know parts even heard about parts, but even among those people who heard about parts, they, even though they've never seen participation, they somewhere someone heard about these patients being very needy, crazy psychological um um and I think basically just crazy people and they all try to run away from these patients which is completely opposite of my experience, I mean of course any disease that are disabling uh these patients are very desperate and a lot of people on disability. So of course um you know, providing care for these people are not easy, but it's, I don't think it's really specific for Parts patients. Maybe some of you may have different opinions about it, but I just really don't think these patients are any different from um any other patients who have chronic neurological conditions that are debilitating. I'm a prominent doctor, I've seen a lot of people with the spinal injuries through and I don't think these people are any different from, you know, part patients in terms of neediness because they're, they're all needy and desperate. But I just feel like this bus, you know, um like a lot of doctors um you know, kind of have some stereotypes against Parts patients. I mean, I think I told some of you guys before I went to a neurological conference in England and told one of these people that I studied parts cleaning program at johns Hopkins. One of the neurologists told me that um that I'm sorry to hear that you can see part of patients. So I, I think there's a kind of misconception but which is really not fair. Um and also another really big thing about parts care, especially in the United States, is that the finance aspects of it, as we all know in the United States, what makes money? It's a really simple procedure and high turnover kind of stuff. Um you know, a lot of cardiologists like dr boys here can make a lot more money by doing you know simple procedures and not I'm not gonna say simple but kind of procedures E. P. Procedures that are making a lot of money. These people are really making so much money but there's really no incentive on taking time and listening to patients and providing quality care something that's really needed for parts patients. And and you know a lot of big clinics or hospitals, they don't really want their doctors to spend too much time on parts patients. And that's actually another reason why there are not many you know providers out there in that optimal. I mean I'm one of the very few people who are bringing much to think that I can take pride in you know, you know providing good care while not making a lot of money and you can only find you know financially stupid people like me, there are very few people out there stupid enough to do this and they're not, there are a lot of participations out there so you cannot rely on this. Just very few people who are willing to do that. Um Also another thing is that you know part requires interdisciplinary approach, it's not just about one neurologist or cardiologist or criminal doctor but we need g i doctors to help these patients immunologists and you know so many different disciplines and and again in current medical environment especially in the United States, it's really not easy to talk to different people and collaborate. Um And at the end of the day there are not many options for treatment, not many drugs out there, there's actually no disease modifying treatment or drugs for pots. So with all these challenges how can you overcome challenges and provide better care? So this is um something that I try to do and then open for discussion matter. So so before that I'd like to kind of share how I see parts. Um and then you know based on that what kind of model of care that I'm trying to build. So I think a lot of people here will probably agree that part is a clinical syndrome is not just one thing. Um I mean I'm a neuromuscular doctor, I see things more neural muscular standpoint. So you know, my theories about at least some portion of parties, sympathetic ganglion 90s is overwhelming information to some gang um dangly ins in the Autumn universe system, but there are people who have hyperbole mia from a lot of different reasons where it could be mouse or some kind of pelvic congestion or you know, venus congestion syndrome that's causing of parts. Um it's probably heterogeneous ideology from my standpoint, still kind of focus a little more on autonomic neural function. And I think there's a pretty good evidence to suggest that this there's a lot of based on over dysfunction or dysfunction of this regulation of blood flow going on. I think fundamentally there's some kind of a um autonomic dysfunction and sympathetic nervous system. As you know, this is sympathetic, sympathetic based on model innovation. And I think there's some evidence suggests that there's a renovation of these blood vessels, either arteries or in pain and that's causing some kind of blood flow of this regulation. So from this uh from the symptoms standpoint that that based on moral failure or blood flow dis regulation because it's some kind of muscle cramps or to study intolerance, we're chronic fatigue. It kind of has a reduced cardiac or pulmonary pre load that causes a lot of the short and spreads just near the exercise intolerance and over fatigue and they have brain fog and reduce concentrations. So I called this pump failure symptoms. And I think it's primarily coming from some kind of based on this regulation or failure. At the same time, these people seem to have integer variable X. Function in the central nervous system so they can detect this, you know, based on motor failure and increase its sympathetic um um uh the neurotransmitters such as norepinephrine um to compensate for it. And and because it's quite a so it causes um some kind of sympathetic compensation, causing some kind of fight or flight symptoms including anxiety palpitation, tachycardia, sleep disturbance various G. I symptoms such as not they're vomiting indigestion or loss of appetite and excessive sweating at the same time. And and although this is kind of sympathetic as a compensatory mechanism, those symptoms can be equally disabling and very uncomfortable the patients. So this is a slide that I have to summarize uh parts symptoms. I don't, I mean I know some of you may or may not agree with this, but I feel like there are kind of two clusters of these parts symptoms. One is pump failure or vessel moral failure symptoms. Typically those are brain fog, Castillion tolerance, exercising intolerance and muscle soreness after exercise and which I think is coming primarily from, you know, blood flow dis regulation. Um At the same time the palpitation, anxiety, insomnia, G. I. Problems that are more kind of sympathetic overcompensation um or adrenaline. There one of the unique things about part is that uh you know, let's say even though it's coming from a single pathology which may be sympathetic renovation, the symptoms are very multidimensional. It's not like other type of neuropathy where for example if you have neuropathy in your distal legs, you have numbness, tingling and foot drop. Like in diabetic neuropathy. Um you don't have to your neurologist to know you have the neuropathy there. But for parts um it's autonomic nervous system, it's not something people can feel and because blood flow goes to different organs. The symptoms can be very multidimensional and that's actually one of the reasons it can be a little difficult to diagnose. Oftentimes you have to rule out other conditions that can cause, for example, brain fog, we're tachycardia for insomnia or G. I. Problems. So that's actually what complicates kind of this whole part diagnosis and recognizing parts um and also treat the part symptoms at the same time, which is actually it ends there. But there's even more complicated stuff to um as you well know, um Parts not only is a multidimensional in the symptoms, there's a lot of syndromes that are overlapping the parts too. All right, I'm talking about Alice Danlos syndrome, hyper mobile vascular E. Ds, massive activation syndrome, irritable bowel syndrome or other, you know, sybil or other G. I. Syndromes, thoracic outlet syndrome, fibromyalgia, chronic pain and various pelvic or venous congestion syndromes. And as you know, I've been trying to you know, find some lectures from other areas to learn more about it. Um But anyways, not only the symptoms are multidimensional, there's a lot of overlapping symptoms and you know, that's actually what complicates Parts care as well because it's not just one person can do everything right. I mean, there's no doctor whose place expertise in immunology, G. I. Neurology cardiology, there's just no such person exists. To my knowledge. So how do you treat the problem? I think theoretically there are two approaches. There. One we can fix the cause and um you know, be done with that or fix the symptoms. To just manage their symptoms. Let's talk about fixing the cost. Well, we're not there yet. We don't have any research software debt and we don't know. And like I said, it's also multi heterogeneous ideologist. Some people may have parts from, you know, you know, small fiber neuropathy or some people may have it from venous congestion. So, um, there's no one single approach there. And you know, and those are theoretical at this point, there's really not much proven for any ideologists. I personally feel like still at the same time, we can't just give it up though. You know, we still, we need to kind of do some investigation to try to find the kind of potential cause. I'm going to give you an example. Um, oh, that, um for example, I actually presented this case to this group I think a few months I think early this year and I don't know if anyone remembers the case. This is a guy In his mid 60's who developed, uh suddenly developed all this autonomic symptoms. Also said intolerance palpitations, anxiety and all that kind of stuff from somebody who is very healthy and it was very dramatic, sudden developer. But despite that, a lot of doctors dismisses autonomic symptoms for literally two years and he was in a Hopkins hospital seen by one of the, you know, very famous neurologist here. And you know, like literally said in the note, um, there's no absolutely no autonomic symptoms. He's fine. He's just getting older. That's what he said in the note. Um But he actually uh and I think he's a perfect blood, he's a male parent panel was negative, but luckily I got the CSF sample and then which was recent for male panel and came back as positive for anti Hugh antibody And actually found tables in the both century Kangaroo 90s and autonomic in the 90s. Um And also there was coming from lung cancer, so he was treated he was getting treated and he's actually still getting treated for lung cancer. It's kind of a remission now. But he he says actually most debilitating symptoms are, you know, the ordinary symptoms. And although he knows he has a kind of limited lifespan at this point, he was um he really wants to treat his autonomic symptoms and I've been giving I. V. I. G. For him which has some limited kind of benefit at this point, but it's helping him for sure. I kind of like to talk just a couple more things about I. B. I. G. Uh As some of, you know, I actually use I. B. I. G. Selectively on some parts patients and I get a lot of criticism for both sides. I think a lot of participations accused me for not trying ideas enough on the other side, especially from my neurology colleagues. Um Okay, so like using I. V. I. G. Um you know, uh without really clear evidence. Um I think um like I still try to do I. B. I. G. S as much as I can. Um And I clearly understand there's a lot of limitations about using I. B. I. G. Mostly from financial aspect of the insurance company is not going to prove it most likely in the United States at the same time. I like to kind of point out that I was I V. I. G. I think a lot of people are a little confused about evidence based practice versus clinical research. A lot of my neurology colleagues who criticized criticized me for using idea agent parts patients um you know, tells me that, you know, this is not an evidence based practices really. I mean, some people even just say it's male practice to even just give I DRG on those parts patients, which of course I disagree. I think, you know, like in my experience that works really well for part of patients, of course with the massive activation complication, a lot of people may develop some side effects from I. V. R. J. But that doesn't mean that it doesn't work for parts. A lot of people actually do benefit significantly from I. B. I. G. Um But was there is evidence there, We don't have enough evidence to do the I. D. I. G. On participation, but but I think a lot of people, you know, kind of get a little confused when you try to use something like ideology or immunological or any medications. It's not like when you're in a clinical practice we need we need to have all the clinical evidence or um you know um you know the I. C. T. Or randomized clinical trial level of evidence to use any medication or intervention for the for the patients. So uh you know of course the standard for clinical research and standard for clinical care, it's a little different and uh you know we didn't good if you have a good side. I mean that's actually my opinion. Like if you have a good scientific rationale, I think of course you can use it. I mean for example like I'm pretty comfortable using I. B. I. G. On this path patient and I use ideology and other neuromuscular conditions and and you know of course I wouldn't use something other things that I'm not comfortable with. But you know I know the risk and benefits potential side effects from the I. V. I. G. So if you know the pros and cons in advance and can share with the patients that's my opinion. I feel like I should be we should be able to use idea G if there is any good reason to suspect auto immunity in those patients of course we don't have any biomarkers that are proven from clinical research. So I think those are two different things. Of course I open for discussion about how people think about using ideas on participation or some other email, logical. So anyway so that's kind of one of the very few limited options to fix the underlying cars, but practically uh, um, most part communicator, primarily focused on fixing the symptoms at this point. And um, you know, like for for to try to fix the symptoms that there are probably two things because I told you there are two different clusters. That's how I see parts and for symptoms, I try to target symptoms that's coming from, you know, kind of pump failure or based on the dysfunction and that's volume expansion for me. And I'll try to tiger symptoms from sympathetic overload over conversation. So, uh, to start it. Um, ah, for volume expansion. So I see volume expansion, not just drinking water. I mean basically it's kind of like I just acknowledged to everybody, like it's just the water balloon, you're trying to fill the balloon with water to expand it so it doesn't collapse. And, and I mean I try to do very aggressive rehydration, like up to four liters for five g of sodium per day. Um, and some of, you know, like I try to die of dehydration, like on and off. Not, it's not a long term chronic solution that everybody has to get all the time, but when people are very struggling, especially in conjunction with physical therapy, they find idea idea hydration to be very helpful. Um, and then, you know, use some medications such as military influence. I also do physical compression and also physical exercise as well. Um as you know, physical exercise. I see exercise as a part of volume expansion because you know, through chronic cardiovascular exercise we can expandable volume significantly. Um And also the bottom expansion is actually not that easy. Uh It's like I try to take step wise approach for volume expansion. So the first thing that I start with is, you know, kind of hydration or dietary modification, increasing water and sodium and I feel like this is the basis of everything. I try not to jump into everything at the same time, I try to do one at a time, starting with some kind of aggressive hydration or hydration and dietary modification. I always always give my patients some kind of homework, try to track down the amount of water they take a trip down their blood pressure and heart rate so so they can monitor their volume expansion. And then um you know, uh you know, ideally theoretically after that. And then I tried to florida for vintage, ring on some of the medications to optimize the volume expansion or retain the fluid. Uh and then tell them about, you know, like avoiding caffeine or alcohol and other things. And eventually, as we all know, exercise takes time to kick in. So there's a lot of education should be. It's really needed to make patients understand that this is not exercise for a couple of weeks or a couple of days. It takes months or even years to build up their cardiovascular strength and you know, really do that, increase the volume. So this is kind of pyramid because I kind of start from the base and then go up. That's kind of my theoretical model for sure. And along the way, of course we uh these people patients have to kind of work with not only the main clinical providers in particle physics, such as Mds or mps, they also have to work with physical therapists, occupational therapists, ideally nutritionist, ideally psychologist. Not that I have those people uh in the team at this point, at the same time, if there's any other random symptoms arises, that's a severe migraine headache. I need to talk with neurologists. There's some limitation in managing migraine uh for me, uh some arthritis for rheumatologists and G. I. For G. I. Doctors. So this is kind of theoretical model for bottom expansion. And as you can imagine, building up this whole team to do this, bottom expansion can be also complicated um as well. Um And like I said, the therapist exercise, you know, I think the emphasis is that it takes a lot of time. And another thing kind of challenges for the bonding expansion is uh to set setting a goal like it's really difficult to set a goal uh You know, in terms of, you know, you know, how long they have to be on this kind of program. Um so, you know, again, this is not a cure, you're just addressing the symptoms. So a lot of people do respond to this whole combination of bottom expansion and exercise. But as you will know, it's not enough for a lot of people. And what is it actually clear goal when the patient, let's say their patients coming from out of state, they're coming to the program, um they're always asking, how long do they have to continue this kind of program? And nobody has a clear answer, like how to set a goal and this is the amount of time and money to spend to get to a certain point. I think we have a clear goal and some kind of objective measures for that. A lot of people will be more compliant, but we cannot just tell them to exercise for the rest of your life and do this kind of stuff. And that's that's one of the challenges that I have when I start this, you know, whole program for volume expansion or generally just particular and something we can talk about it. Um um this next symptom that I'm targeting is sympathetic symptoms. And I think this is relatively more straightforward. We can use a lot of medications such as beta blockers, Melatonin, some other medication I try to avoid uh you know, really, the carding medications for participation because it can cost them a lot of fatigue. And actually, a lot of politicians don't like to be on some kind of opioids or benzos in most cases because it makes them more fatigue on top of having fatigue. So I don't think I have a lot of problems. Unlike there's a stereotype against pot patients. I don't think I have a lot of problems with the kind of drug dependence or even you know addiction for party patients because these people really try to try to avoid those medications. Um You know and a lot of other psychological manipulation, meditation, mindfulness. I think those are really helpful but also require some kind of training and you know some psychologists on board who can help with this type of approach. Uh You know I generally advise patients to avoid physical or emotional stress. Uh That's almost impossible. I don't even know how to avoid my own stress. So that's really not easy. Um So again also don't forget E. D. S. M. KCbs, venous congestion. All the things we talked about, right? So bottom expansion and sympathetic. That's all good. But what about the other complications? These people have like severe ideas, having multiple, easy bruising multiple M. SK injuries. Um Some of them may have been as congestion. That can be actually solved with some kind of standing. So we still need to work with geneticists immunologists, surgeons, cardiologists and that's really not that easy. Um And especially somebody out of state patients or coming from distance. How do we arrange uh this, you know refers to different specialists really challenging for a party care. Um and also don't forget to rehabilitate interventions to like I try to include uh you know, address this rehabilitation aspects of it. Uh This is not about like treating symptoms or cause it's just about, you know, keeping them functional and a lot of as you know, like a lot of participations, they use cane walkers or wheelchairs to get around. Unfortunately, these affect very young people who are who are in school work, so they need to do something to, you know, keep them going and make money and try to work with the rehab therapists, especially more job with the occupational therapist. And sometimes it can be very helpful. Um and sometimes I just advise patients to sort of voice activation system at home like Alexa or google uh in case they, you know faint or collapse, they can get some help. It's kind of minor things can be helpful too. Um and ah so, so overall this is kind of, you know, structure that I'm thinking about. So I think still there's a really, you know, benefit of having main parts provider because it's a physicians, therapists and there's practitioners and who are probably the driving overall plans and studying with the diagnostic work up for sure, trying to cause it cause cause underlying cause um, you know, like I suggested before I start with the oral hydration, some kind of lifestyle changes. Um and then, you know, kind of start a little later on medication with some more aggressive bottom expansion the exercise program. I think it's just started early on. But in the, in the beginning they can go very easy on it. They can do very low level exercise but then gradually increase it. So ideally, theoretically, by the time they can do more exercise, they can graduate from some of the medications or if they're on I dehydration, they can come off of my dehydration. They continued rehydration for sure. But at the same time, if they have other overlapping syndromes, MK CS and Bs, but they have to imagine collaborating with other providers and cardiology, neurology and other things. So again, the model here is kind of complicated, but this is some kind of model that I'm trying to provide or establish your Hopkins. Um Well, luckily, I think a lot of doctors and from the different departments within our hospital giving very supportive. Of course. I met with a lot of skepticism too, but more people are very collaborative and very supportive and I still very appreciative but um trying to build up the whole clinic program, but still, again, a lot of challenges. We don't have enough people even reading john's Hopkins, They're like, we don't have enough therapists are willing to see populations and I'm really struggling at the same time, I try not to refer to rehab therapists or other therapists who really don't know much about parts, my minimum criteria for referring part therapist is somebody who joins our parts meaning thursday minutes, so we share knowledge or share cases. Uh And also this is another second thing is another thing that I'm always struggling with. I know my chairman has been very supportive and trying to you know, have our business uh business person to help me with building this party clinic, but we all know that this whole party clinic uh really doesn't bring a lot of revenues to the clinic or department. I feel like there must be a way to do that. We just haven't found one another really important thing is it was a good academic support. Um you know, I'm still kind of early to meet career um you know, clinician scientists and investigator, there's really no mentors to support it. Actually, a lot of mentors told me in the beginning and I tried to do particularly, they told me against it actually, they they say it's very dangerous for any early investigators to jump into parts or autonomic research because there's no infrastructure uh you know, and there's really, you know, not many mentors that can, you know, make me make my career successful and there's actually clear danger there there are not many good grand mechanisms to support pots, autonomic research at the same time. So I think especially in academics because we still have to rely on a lot of academic centers to provide this kind of care because of because of financial restrictions. I can't really imagine a lot of you know private practice doctors to provide care for parts. I mean it should be limited but they can but it's kind of limited. So it's still probably going to be better and more academic center. But if there's no good academic support or grand mechanism or mentors, not many people will be, you know, providing parts care. I think that's probably probably more practical limitation from my standpoint. So So this is all the slides that I have actually 12:35 So at this point I'd like to kind of open to discussion um about what you think about this whole bottle of care. Any suggestions? Um anything. So today I thought that was really that was really a good um kind of collection of sort of your thoughts and and your your approach. I wanted to ask so and when you talk about a pots program, does that mean that all the providers that you listed? I mean you you listed nine different types of providers next to your pyramid. Do all those providers see that patient? There you go. Um all the providers see those patients does one provider see those patients and then farmed them out, you know, how does that work? So um okay this is my plan and I really need some suggestions and modifications. So what my planning, my kind of ideal plan is there's a you know M. D. Or M. P. Level providers that are kind of driving this whole was the volume expansion of part care and these are the people who refer those patients to either neurologists or psychologists but sometimes the emergency room at the same time physical therapist or nutrition and so on. I think at the same time all these kind of nine providers and you know in the preferred I mean probably more than nine for sure. But these people whether it's cardiologists or physical therapist ideally I think these are the people, they're not the people who are seeing parts mainly but at the same time ideally they are the people who understand you know parts. So for example I I don't want when I send the patient to physical therapist I don't want our therapist to say oh this is a psychological, why why did you come to my pt economy? You should go to you know psychiatrists and and actually there are a lot of reasons for that. A lot of PTSD if they cannot. This is actually very complicated model for as far as understands especially for billing and reimbursement. Uh This is not really straightforward for their billing and reimbursement uh saying it takes a lot of time for them and you know they have to show clear improvement over there a couple of months of their P. T. Program and it's really tough to do that for their insurance reimbursement. So ideally somebody who understands the nature of parts. So sure. I mean the and the reason I ask is because a couple of years ago um after I left after I left chop I put together a document just for myself and then for whoever, you know whoever was interested in in potentially working with me on the on how to create a multidisciplinary pots program. And after having seen what the program was a chop and after sort of having thought about it, you know my my thought was a multidisciplinary clinic that included for all patients at least upfront cardiology, neurology, G. I. P. T. And psychology. Um And um and then with patients being sent out to other providers as needed. But at least at minimum you have a central core because those tend to be the biggest uh I guess sort of your biggest bang for your buck. Um From a financial standpoint you're right. I mean if all we're doing is talking about outpatient visits then yeah your your revenue generation is going to be poor but I think the way to think about it is you think about the downstream the downstream revenues. So number one G I does a lot of procedures on the patients typically um neurology does some um but then you also have emergency department visits, you have admissions, you have infusion center, you know ostensibly. I mean I think I think there are ways to um uh there there are ways to bring procedures in without being inappropriate um from pediatric cardiology standpoint, you know it's it's inappropriate to do echocardiography. Which is which is as we say in pediatric cardiology. Echo is the A. T. M. Of feeds cardiology. Um But it's appropriate to do an echo on every patient because by and large these patients are really clinically normal that there is concern amongst adults. And I know Lauren has has talked with some other folks about this that consideration of doing echocardiography in all adult patients with pop this is something that should be considered. So I mean I think there are ways to do it. Um uh the actually and I was just gonna make one more comment and let other people talk. Um You you talked about how other providers, you know have this impression about pots patients and and and being needing some of that. And the other thing too is because a lot of other providers were not taught about the autonomic nervous system um and didn't really have a lot of interest in it. You know, in the meantime you get, for example, electro physiologists who are who have these patients referred to them because of sync api palpitations, tachycardia, chest pain etcetera etcetera and electro physiologists um would much rather be doing E. P. Studies radio frequency ablation, putting in pacemakers, doing those things where you get immediate um immediate gratification. The concept of dealing with delayed gratification. And I think in in patients like this especially if you train for years and years to do completely different things is hard for for other providers to sort of wrap their head around. So now shut up. That's a great point. Yeah this is very challenging. Um Yeah I actually this is someone things that I'm trying to work on with our pieces person to like what are the procedures that can including their right? I do like proper procedure. I do E. M. G. Uh procedure and you know skin biopsy and other things try to kind of be reasonable. I don't want to overdo anything. These people are young people we do without insurance and they are spending a lot of money already in their care so I don't want to you know to have more stress from that. So I think that's a great point like I think we need to talk about. There may be some other procedures that make this whole financial you know I'll go the rest. I think Lauren mentioned something that I was gonna say yeah I d I just prescribed you know it doesn't have to be like a lot of old stable use for sure and yeah that's true like I think I mean generally medicine like I remember this kind of grandma on top of neurosurgery there's a neurosurgeon says you know if you have a brain injuries and bleeding in the brain they just you know open up their skull. It's not like there's R. C. T. On that kind of stuff. They just open it up using the reason. Right? So I see idea just kind of like that too. I think if you're careful in selecting the patients and first of all even if you weren't careful the insurance company is gonna make you careful because you can't get it approved. But you know I know you're careful but just generally speaking um selective and who get this. Um I think that if it's dosed in a cautious way and you know it's a a trial run to see how it works and if it doesn't work you don't continue it. You know I get so frustrated by doctors who kind of you know shame other doctors for doing this because pretty much a majority of the medications you're prescribing in your career or off label and and a majority of the I. V. I. D. Prescribed is off label. So I think it comes from the the shame is put on the doctor because there's some sort of anti pots patient mentality from a lot of clinicians you know just like why are you helping these people with a real drug? There is they're just crazy they don't need that real drug. Um So but you know obviously the real answer is we need good clinical trials we need real research. So um we are doing that at Ut southwestern with a small study. Uh The initial study was funded for 10 patients and we got griff als to give us extra free I. V. I. G. And albumin placebo. So it's going to be going to end up being about 40 patients. It's gonna have a path only cohort and a pots Children's cohort by the time it's done. So I have a lot of patients who want to get into that study recruiting patients or no, I think for Nino has more than he needs. So there's definitely more than 40 pots patients in Dallas who want I. V. I. G. So I understand. Well so that's actually the same question from Lawrence. Um what I liked was the selecting criteria. I really you know, I wish there's more biomarker like oriented but even circling receptor you know, especially the low tighter has been kind of criticized as being not specific enough for you know parts. So I mean to be honest personally I don't really look for that will be great. Um I do skin biopsy, I think skin box is just a little probably to convince a little more myself than anything else. Not really interesting because interest companies not convinced with anything but but when I'm not completely sure it's already noon or something like that. But I think what I'm looking for is more kind of like their clinical course that are more suggestive of order immunity something post infectious, just like post covid and acute onset of waxing and waning course. I think kind of good analogy is like the embrace syndrome? Like we don't really look for all the antibody when you do I d I join the Jamboree syndrome, it's enough to show imagination, cannot said CSF showing some high protein. None of these are really specific specific auto immunity, but we still use I. B. I. G. When they get hospitalized and then you know, um you know, just I. B. I. G. Is kind of one of these kind of standard stuff. So I don't know if you know, maybe Lawrence you may have different ideas and yeah, first of all I really liked your delineation of the challenges of starting a good pots clinic. I also want to remind you that you're surrounded by mentors tie. So impressed you put together this group and those of us who've been doing this for a while, I can I know I can speak for myself and probably others that we we support you and we all need to work together to support each other because you're right, this is kind of a lonely don Quixote esque type of campaign that we're on. We often are an island within our own academic centers and uh it's it's um often I find that my colleagues who have some familiarity with pots is only because they have a family member who has been affected and and you know, it's it's and so in that very often you just have to figure out what are people's story and how how did they get involved? How can you create alliances? Because you know, mike gastro neurologist who I coordinate with his daughter has pots and so that makes for an automatic link. So those things are helpful. The other thing I would add that I find very helpful is um is to measure and measurement is difficult and somewhat amorphous disorder. But one thing that is measurable is functional capacity. And so I use a functional capacity score that is used by the chronic fatigue syndrome group. It's a 1 to 10 score that basically looks at, you know, on a, you know, 10%. You're basically in bed and you need somebody to help you get to the bathroom and 100% is your, you know, your exercising every day, you're you're an overachiever. And then there are gradations in between and I try and figure out where are you on your best day? Where are you on your worst day in a typical two week period? And how many, you know, how many days of that two week period are you or what percentage of that time are you at your worst? What percent of your your best? And were you in the middle? And and what are the things that tend to favor one versus the other is a way to get it triggers And and then, you know, because patients will ask us, well, what can I expect in the future and you know, what is a reasonable expectation for my level of function. And you know, the answer to that is we want to bend that curve towards greater functionality and fewer days lying in bed and how do we do that? And so I learned a long time ago that To be a good husband. It's better if I under promise and over deliver and it works pretty well as a doctor too. So I'm shooting for 50% better. My goal is to get you 50% better. So if you are nonfunctional hanging out on a couch, my goal is to get you back to work. My goal is to get you out of the wheelchair. My my goal, you know from the wheelchair maybe to the to the walker. Uh huh and start increasing the amount of time of your where your function is a bit more optimal. You may have discovered as I did that many of my patients with pots have loved the pandemic because for two reasons. Number one, no one expects them to do much and number two, the rest of the world is living their lifestyle. And so they've kind of played catch up with everybody and this has taken enormous emotional and physical burden off many of my patients. And you may have discovered that as well. Um I think those are just a few reflections. I liked your list at the very beginning, those those are indeed the challenges. We could probably add a few more But I'll stop there. Yeah. I don't think it's about actually completely agree. I actually just forgot to mention that one of the criteria for me is actually the functional status. I mean I do selectively in parts somebody who are very debilitated for two reasons. I mean one of course they're debilitated and number two I work with the physical therapist and we do have a lot of kind of semi objective questionnaires or some measurements to show their disability. And that's, to me a biomarker. Um So when they get the idea that when they get better I know that they're getting better versus somebody who has more better functional status without any specific biomarkers. It's really difficult to show whether they're getting better from I. B. I. G. Or something else and and I think that's a great point. Um And that's kind of my selection criteria at this point. The idea when I if I go for idea idea on part patients, the thing with with what you're doing is get as much data as you can Whatever because in the end you'll be able to go back if you can get blood samples and freeze them at -70 Freeze Plasma -70 that you eventually will be able to go back and try to build those those biomarkers off. Um The biggest thing that I would tell you in terms of trying to survive in academics with something like this is figure out how to write a grant that tests the mechanism that's lateral to what you actually want to look at. Because if so if you want to look at how exercise helps pots, you may want to focus on how exercise increases blood volume in patients with X. Y. Z. And then it's a much more mechanistic grant application. They tend to do much better. The papers tend to do much better and at the same time because you're testing it in this particular population, you determine who doesn't doesn't do well with exercise and pots. And I would say probably half of the R. O. Ones I've gotten in my career have something which is right in the mainstream of testing a mechanism. Or we're looking for a marker that's critical or something like that, but also have some other critical piece that I'm trying to advance on the side. Oh and if you can a lot of times in essence you're flipping your hypothesis or you're flipping your aims. So the main part of the aim is the mechanistic piece in terms of how it's written and that's the piece that the rest of the world is looking at But buried on page six says, Oh and we will be looking at whether or not this interacts with hydrocortisone because we want to see if this interferes, we have reason to believe this interferes or we want to see whether exercise decreases inflammation and enables people to get off I. V I. G. And in the meantime you're seeing whether I V I. G helps people exercise. That's a great point. Thank you so much. So because I always thought that NIH reviewers will probably look at the pots and then okay, you're not getting the scores and stuff. It doesn't sound like it's necessarily the case, right. People. Typically what happens in an NIH study section, I sat in about 30 days is, and I am always ad hoc. I've never actually been a standing member in a study section. They called me into typically because they want my my expertise tends to be very broad and not as deep. Um, but but you'll see that you'll get three completely separate reviews. And it's not uncommon at all to get completely different comments. So the example I always give people and I'm I'm a biomarker person by, I mean that's what we've done historically has developed. We developed a lot of the early metabolism mix work. Um but I had a grant that had mass hardcore mass spectrometry and epidemiology and nutrition and I had a nutritionist and epidemiologist and a mass spectrometry ist and the mass spec person killed the epidemiology. And the epidemiologist killed the nutrition and the nutritionist killed the mass spectrometry. But so if you you have to deal with writing it in such a way that the pieces that are up front are ideally mechanistic and they're ideally very clear and very straightforward and we do some math now that everybody has spoken to at NIH says there's absolutely no way to get it through NIH because you'll never find anybody who's, you're not gonna find three reviewers who can understand what we're doing. And so the couple of times we've started to put it in, we show the method and we just say using an in house developed method. But the method is actually the huge peace. The other thing with biomarkers is developing really good usable biomarkers is not, it's doable, particularly for something that I think is a severe phenotype of some of the stuff you guys look at it in pots clinically. Um but nobody wants to do it because it costs. So for example I went I was talking with somebody who wanted to, wanted me to basically start a company and I think we could develop markers for em cast and markers for um small fiber neuropathy that are Very tractable, portable, quantitative, you could use them but it probably cost like 1-2, million dollars apiece. And they don't feel they can get enough money back. And if you're going to NIH with this, it's a it's not mechanistic, it's very um data dredging in a certain way. It's targeted data dredging. The example I always use is yes, I'm it's a fishing expedition but I am literally going to drain the entire lake grab every fish and then sort them out because they have to be in there. Um So it's doable but it's that kind of work is unbelievably difficult to get funded unless you can get good preliminary data. So if for example you take every patient you put on I. V. I. G. And you get a couple of blood samples before you do it and then you record carefully whether or not it works. You can take that and you put it through a proteomics panel and metabolize comics panel and the lipids panel and and you see what hits whatever hits you build the roos around. Great point. Thank you so much for somebody. I think I haven't I'm still in Calgary and I haven't done that one yet but I'm kind of planning on I'm almost at the end of case no drafting some hard ones for some other things in pots. The thing I would tell you is draft like 10 eights, Draft like 10 names and then go back a week later and you'll cut it to five and bring it back up to 10 and do that a couple of times. And I mean I'll be happy to look if you're if you're writing something in pots. I'm happy to look at it. I really appreciate. Yeah I'm actually working on that too quiet without the person. But if you can I'll email you about that. Just give me some notice because I typically get very busy around grant. Absolutely. Yeah. For sure. Thank you so much. Personal, critically important piece for popcorn. But that's because you need those papers academically. And I noticed I saw somebody um agreed it's it's something like M. S. Is A. Is a more defined field with more defined mechanistic questions. Thank you. I really appreciate it. Thank you so much for that. Um I know Nate. I don't know if it's still there conventional what the misogyny is about. You know? I think yeah I think that really plays a part. I'm not trying to be political about it but now female predominance I think they really play a role in stereotyping these people. And on top of that they look fine. Young female look healthy. So I think that really asked to have today. The we actually have some like data that really supports that. When we did the big pot survey, we looked at some length of diagnostic delay from the first time you saw a doctor for your pots like symptoms to the time you got diagnosed it was two years longer for women than it was for men, including pediatrics and adults. Um and so when you have a condition that's like 90% female and the women take two years longer to get diagnosed. That is a huge red flag that there is some serious um discrimination going on in health care Lauren was that corrected for severity. We didn't have um severity at time of onset data. So. No, but in general the women, we actually have other data showing that the women have more severe pots so that that wouldn't support that conclusion is not good to know. I know it's not good, but because that would be the big thing is and you know, I'm biased. I think there are really strong subgroups and pots. And if the boys have a different subgroup than the girls, you could easily imagine a scenario where that's why they get caught sooner interesting that you found that and I saw that presentation at the international conference as well about the difference or the difference in duration. Um for diagnosis because in our series there was no there was no statistical difference. So yours is just pediatrics. So there could be some differences there. We I think we looked at age. Nobody actually the presentation was pediatric for you guys to, was it Okay. Hey, we just had a paper accepted it. It'll be online in a few days with more data on the sex differences in the experience and the diagnostics and stuff. But in general, um our overall dataset shows that the female patients tend to have more more comorbidities. And I think you can see um the more comorbidities that are diagnosed the sicker the person is and it could just be that you got diagnosed with more stuff because you were sicker. You know, we don't really have, we don't have a cohort where somebody went through and screened accurately for all of these comorbidities. Right? So like this Venn diagram I don't think I. B. S. And mast cell are different. I think that they're actually overlapping too and that you know what I mean? Like they're not necessarily different things that we just give them different labels right now. Um You know pelvic congestion and E. Ds. Well maybe that goes together if there's some kind of vascular weakness there. And in there was an overlap between um favors disease which we know the mechanism of. Right. It's a genetic disorder that causes weird vascular stuff and autonomic neuropathy. These So they had um 70 like 70 or 80% of favors patients meet the EBS diagnostic criteria. So that kind of says to me maybe there's something about an autonomic neuropathy or a vascular thing that can look like E. Ds that can look like an autonomic disorder. Anyways I'm kind of babbling but I don't think this Venn diagram is right. I think that they're all grossly overlapping. Can I can I jump in at the end just to kind of bring back that immunotherapy point. It's very important. Uh So when Bruce said well you need to publish papers. So I want to ask all of you who are seeing great results on cases of very sick participations with immunotherapy. Please publish it because we think while it's one or two patients. Maybe it's six, maybe it's Seven but it adds up when we're trying to authorize these therapists for our patients. Uh And oftentimes I'm being asked to review these papers. So so that's good. We should help each other to populate literature with this case reports and case serious So far. We have like 38 patients from Jill Schofield. We have one or two from me. We're now working on another study of immunotherapy with subcutaneous and plasmapheresis to add that to the literature. But we should all help ourselves because there are a lot of naysayers. And when I presented that poster at the american Auto Economic Society meeting last year these naysayers told me oh that's great that it was subcutaneous and not I E A G because it could have been a placebo effect from the volume. That's how they think a lot of uh a lot of them are our colleagues who are you know reportedly on our sides yet they have these preconceived notions. Another big thing we need to think about. Like Lawrence said, well if they don't think parts is real why would I V. A. G be helpful. That's absolutely true. But the second one is cost. So there are forces there that want to prevent this very sick population from entering even for as candidates for I. V. I. G. And insurance right now companies are denying I. V. I. G. Left and right. So right now I am working with the department of labor to make sure that that very sick parts patients have equal access to I. V. I. G. Just like sick patients with you know autonomic neuropathy or um you know, see I ZP you know all of that. So it's very important. We help populate literature with case reports and case series and that we help each other because the forces are not on our side. Those forces work for insurance companies to tell them, oh no Pats, that's not a legit diagnosis to authorize coverage on patients that are greatly helped. You know the co author on my paper is now a full time resident. She was a medical student. She got sick with spots. She her case was quite severe. She became homebound, she could not start residency. So what helps her is a is a doctor who is open minded and the insurance loophole that allowed her to get subcutaneous immunoglobulin. She's not working full time as a resident. So it I think these therapists can be very helpful and can you know, they can be cost saving to. That's what I argue in my reports because a lifetime of disability and care is going to cost insurance and society way more than covering I. V. I. G. Or S. C. I. G. So that's my, let me interrupt you for one second because I had a long argument with somebody who sits on a board that oversees all insurance companies years ago about this point in terms of predictive markers for disease. Um, and what he told me is the insurance companies don't care about this, I said, but if you catch it early, you'll save money. And he said, they don't care because the turnover from insurance company, the insurance company in this country is so great that the patient that they're saving money on will not be their patient in two or three years. So those arguments, he said, absolutely stay away from, he said, because the insurance companies not only don't want to hear them, but they actually work against you because you are now paying money for some and for some benefit that they are not going to get. Yeah, that's why the department of labor cares because that's sort of in the discrimination way, like why the severe path. So that's why thankfully they hired me because if they hire somebody else, they would be saying, well, you know, parts isn't really autoimmune or not yet. You know, this is too soon. A lot of, you know, a lot of those arguments are circulating. Uh, and I think we're gonna stand there for decades if we keep, you know, listening to that kind of mentality. Well, it's not yet automation. Why would we use immunotherapy or you know, we can help them with exercise the levines, programs, salt and fluids and, you know, cBc. So I think the government cares on, on the note of, you know, equity and employee, right? Because if you get your health insurance from an employer and that health insurance denied that sort of, you know, like the discrimination situation. So I'm hoping that we can change that and allow severe refractory cases to enter the I. V. I. G. Coverage. If if you've got as somebody who's not a clinician who's looking at this from the outside, does it make sense to to take a meeting like this and say, okay, who's got? How many patients? What are the questions we need to standardize in this group and then create a common data set. Because if you've got the comedy, is that the thing that will help you the most is if you can get I. V. I. G. Into Aqua boys and then say, you know, Here's 50 patients, 25 of whom benefits. But we know that you just don't get this otherwise, then you need the real trial to see who benefits. And you may have enough data scattered around the people that use this to start to say, yeah, these are the people that didn't benefit. These are the people that did. Now you have blood samples from some of them go back and see what begins to differentiate them. It'll all be preliminary data. But that's the kind of preliminary data, you can build a real like larger NIH study around. See I just funded that study on ivy on autoimmune pots. So um stanford stream stream a pd and mitch Michalis from stanford applied in collaboration with french Goodman at Mayo Arizona and steve Bonino at Ut Southwestern for the first sort of multi center longitudinal autoimmune pots. They're going to sort of it's like partially retrospective looking back at everyone and anyone they gave immune therapies to or or who had who they thought had immune dysfunction with their pots whether it was an official autoimmune disease or not. And then there's a bit of a prospective piece kind of following these patients for some follow up and trying to um you know if you did skin biopsy on them before I. V. I. G. Can we do a repeat and see if the small fiber nerves grew back or if whatever antibody levels they had went down. So I'm kind of excited about it too. To see what comes out of that. Um veneno doesn't use I. V. I. G. Clinically a lot in pots but definitely had a lot of antibody tests in patients. Goodman is a little more, I don't know if the right word is liberal but a little bit more a little bit more liberal in giving out the I. V. I. G. And stanford has sort of somewhere in the middle there. So they're a good team. They're not all true believers in anything. They're just sort of gonna put their data together which I think is great. Mhm. But there are so many others in private practice. You know I have colleagues who do mass cell and we always talk about, oh that would improve the N. I. B. I. G. That would improve them subcutaneous. I think if you pull that that there is real strength to numbers. But you know when you're in private practice they don't ask you to the table all that much. So you kind of fade aside. And then the academic medicine there there like you said, they're not liberal at all actually, they're very conservative. They don't go with anything new. They try to you know protect like the establishment and and cut costs and all that. So yeah, it's it's a problem. But hopefully we can let you know at least some patients to you know to have I. V. I. G. Because in in new york state is extremely difficult to get it approved extreme. It's you'd say it's almost impossible, you know? So over on the West Coast, that's easier for Kaiser people because Kaiser only requires a physician order and justification. So they gather the proof. That's how julie Schofield has so many because a lot of her patients are part of Kaiser, Is she part of Kaiser? Yeah. Yeah. I asked her, I'm like, how do you get all of these patients approved? And that's what happens. And I think um Al Shimali has also like away with the special nurse together authorized because I'm always asking them, you guys are so lucky you can easily get the proof here. I'm writing petitions and reports and appeals. And then at the end the patient gets gets plasmapheresis because they were denied coverage for I. V. I. G. Mhm. Always there. Okay. That is thanks. Yeah. So the only patients that I got ideas approved for is the one who has secondary diagnosed as a hyper game or some previous history of cancer. Somehow they are very sympathetic I guess and there's a put the cancer and they just approved it. So I think we definitely need more research. Um And I was looking forward to during study of B. I. G. Um we'll see 10 minutes I'm actually interested in. I don't know John Maybe you don't have time. Like if you're 10 minutes test, I'd like to kind of learn more about the first protocol. I don't know what that is. Do you have time to do that or just introduced or? Yeah. Hi. So I'm the odd personality. I mean your your surgeon, I treat vertigo and it was just amazing too. Listen to tase very well organized presentation. Thank you. I feel like I'm finally up to speed on pots. Um Everything you said has an analogy in treating vertigo patients. Um I was dissuaded by colleagues by administrators. Um You know the physiology in some cases is very well understood and in other cases very poorly understood for common things like vestibular migraine and what we call triple pd persistent perceptual and postural dizziness and and there's an overlap with pots here. I I could expand your Venn diagram but I'm sure it's, I'm sorry about that. No problem. Um Yeah, so just some thoughts of things that we've done to handle the volume of patients. Um So we have a good group of vestibular physical therapists here and they have been eager to take more of a leadership role. And so mike schubert and Allison Nog have put together on the therapy side of what we call the vert program. And basically new patients calling our call center for vertigo will often not be scheduled with them as first visit. Many of these patients benefit from vascular physical therapy, no matter what the underlying causes. These people see dizzy patients all the time. They can they can pick somebody with Meniere's disease out of the bucket in a minute. Um and they'll get them to see me promptly. Um meanwhile the patients who have benign positional vertigo and need repositioning maneuvers, they'll treat them and get them in and out faster than if they went through the conventional Dr. 1st route. So that's something to consider. I don't know if there's a comparable kind of pathway that could be built for your patients with pots. Um Second is we've trained a couple of nurse practitioners actually several in the past as well. And one of my former ps went on. She now runs the dizzy clinic in one of the busy Seattle private hospitals. I think one of the one of the possibly low hanging fruits for conditions like this is for us to establish training programs for N. P. S. And P. A. S. The real world is hungry to have people who like seeing patients who aren't scared of managing basic medications and lifestyle changes um and who have backup from Mds for what they're doing. And I think it's maybe worth exploring a formal training program. I tell you when I when I go to national meetings now my colleagues always ask me do you have any fellows or trainees that you've trained to think about vertigo the way you guys do at Hopkins because we don't have anything like that. Um And I could never train enough mps to do that. So nurse practitioners, physical therapists, P. A. S. You know you have lots of potential allies. Yeah thank you so much for them. Um Yeah that's a great idea and I'll probably have to reach out to the email how you run. Well let's talk to me. Yeah I'll send you an email about that. I think that's a great idea and having cold center that can triage patients at the front line. And I should say, you know what Bruce said really resonated with me too. Um You know I will tell you in our studies, you know R. N. I. D. C. D. National Institute of deafness and Communication disorders. Those study sections are dominated by basic scientists who want to understand mechanisms. Um and and they're really more interested in normal physiology mechanisms than even disease mechanisms. And so every successful grant I've seen, you know, for any of these disease processes also starts with physiologic measures. And we're going to get to the mechanism that's going to have broad applicability to understanding this. Uh not only this disease but other diseases and normal physiology. And he hit it spot on. I think that that's the way you got to go. I see. I see. Well thank you so much for that. I really um you know, I think I'm glad that I you know, open this discussion and learn a lot about it. Um Yeah, I'll definitely send you an email to get some more kind of details of the protocol. It's great because it's Ian Hopkins and I think we should collaborate for. Sure. Yeah. Thank you so much for that. uh we're actually 15 minutes past any other comments or any questions or Created by Related Presenters Tae Chung, MD Assistant Professor of Physical Medicine and RehabilitationAssistant Professor of Neurology Dr. Tae Hwan Chung is an assistant professor of physical medicine and rehabilitation and neurology at the Johns Hopkins University School of Medicine. View full profile
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