80. A Multidimensional Approach to EDS and HSD Care with Dacre Knight, MD

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Linda Bluestein, MD (00:13.418)

Welcome back every bendy body. This is the bendy bodies podcast and I'm your host and founder, Dr. Linda Blustein, the hypermobility MD. This is going to be a great episode. So be sure to stick around until the very end. So you don't miss any of our special hypermobility hacks. As always, this information is for educational purposes only, and is not a substitute for personalized medical advice.

Today, I am so excited to have Dr. Dacre Knight here with me. Dr. Knight established a specialty clinic for treating patients with hypermobility syndromes, including hypermobile Ehlers-Danlos syndrome, otherwise known as "HEADS", and hypermobility spectrum disorder, HSD, at Mayo Clinic Jacksonville, Florida in 2019. Prior to joining Mayo Clinic, Dr. Knight was an assistant professor of medicine at St. Louis University. He also served four years as an Air Force physician.

He also served four years as an Air Force physician at Scott Air Force Base and was the director of a Phase II Clinical Laboratory Education Program and chairman of the Life Support Function Committee. Dr. Knight, hello and welcome to Bendy Bodies.

Dacre Knight (01:20.047)

Yes, hello and thank you so much for allowing me to be here with you today. I'm really excited to be here.

Linda Bluestein, MD (01:25.854)

I'm super excited to chat with you. We've chatted a couple of times, and I'm so excited for the audience to get to hear directly from you and hear about your incredible experiences. And I wanna start out by asking you, how did you get interested in EDS in the first place?

Dacre Knight (01:40.587)

Yeah, I know it's a funny thing because some people, they come on to it and they probably didn't want to be here and then some that it came on to it because they were interested in it. And I would say it's probably more the latter because when I had completed my service in the Air Force and joined Mayo Clinic, there's a world of opportunities of research and all sorts of clinical conditions and rare diseases and things like that you can get involved with.

One of them came up as running the Ehlers-Danlos Syndrome Clinic because they had some shift in staff and things like that. And really what it came to is I was volunteering to do it because I found interest in it and I thought it would be a good area to build up some research and kind of move forward in our understanding of the condition because it was very intriguing to me.

And I had encountered patients in the past, not in a formal setting of an EDS clinic, who had these conditions. And so knowing that there was an opportunity to really develop it further struck my interest. And it's been uphill and hit the ground running ever since then. So very exciting.

Linda Bluestein, MD (02:55.379)

I'm sure it must be quite an interesting experience and I just can't wait to hear all your insights and everything from all these patients that you've seen and research that you've done. In terms of the specialists and the multidisciplinary type clinic that you have, are there other physician specialists there or is it, I know you also refer out to other specialties, how does that work?

Dacre Knight (03:19.355)

Yeah, so in the typical Mayo Clinic model is that we have as many specialists as we can all in one setting, all under one roof so that we can work together. And it's a very unique setting. It's not unique as nobody else does it anywhere in the world, but it's unique for the purpose of giving patients the benefit of having.

the specialty evaluations without bouncing around and trying to reduce some of that silo effect, which I know is out there in the community a lot and can be a hindrance. But for example, there was some of this to some degree when I was in the Air Force, where we had a clinical setting with radiologists just down the hall from me and things like that. So it's not unique, but it is very beneficial to patients. So yeah, so within our EDS clinic specifically, it's two providers, it's working here with

Shilpa Ghazrawala, who is a PA and has recently joined us and has been a great help to allow improve access for our patients to come in and decrease the wait times and things like that. But then also we work very closely with our physical therapy and occupational therapy team, our pain psychology team and so forth.

Linda Bluestein, MD (04:38.386)

And I trained at Mayo, so I definitely am very aware of that. I've also been back as a patient to Rochester. So I definitely have been the recipient of that benefit of, you know, oh, something else comes up, let's see if we can get you in. And in some instances, they got me in that same day. And then it did seem like there was much better communication between the different departments.

Dacre Knight (05:00.439)

Yeah, yeah, it's not perfect, but you know, anything we can do to improve that communication, that flow, and that was something that was really interesting to me when I was offered the job at Mayo Clinic is because I was talking to a lot of the colleagues who had been here for a long time and they say how they still just, you know, pick up the phone and call the oncologist that they got a question about it, you know, blood counts and things like that on some test results.

We can get formal consultations if we need, but if you need to just curbside someone, hey, I've got this patient, what's going on? What do you think? What should I look at? It's so helpful and it's daunting when you're coming out of medical school or residency that you're just, you wanna go out there all on your own. But to know that there is support in this network to other like-minded individuals that wanna help patients and understand the conditions and things like that, it's really a nice safety backup and follow back.

Linda Bluestein, MD (05:53.398)

Yeah, that's really fabulous. One of the things that I thought was interesting is looks like you do an echocardiogram on all the patients that come to the EDS clinic. Are you still doing that? And then what laboratory testing is typically performed? And of course, we'll talk about genetic testing later on. So I'm thinking about what other lab testing is usually done. Yeah, yeah. That's the first one.

Dacre Knight (06:11.175)

Yeah, yeah, I'm happy to go through all those things because a lot of these things evolve, as you know, and as our understanding evolves scientifically from whatever the condition is. And in EDS and HSD, there's so much that we're learning. It's still so much we still have yet to learn. But one of the research studies we're just wrapping up, actually, we're just submitting it for publication now, is data that we've collected on echocardiograms for our patients. Because you're right, and looking at

Linda Bluestein, MD (06:16.206)

Mm-hmm.

Linda Bluestein, MD (06:37.654)

Mm.

Dacre Knight (06:40.959)

our papers that we've done previously on kind of the flow and the process of our clinic, we did typically get echocardiograms on mostly all of our patients. Because, you know, several things, one is that there's part of the diagnostic criteria that looks at echocardiogram findings, so mitral valve prolapse and aortic aneurysms and things like that. And there have been some other similar studies on this same topic in the past. But...

What we wanted to look at with this recent research study was just to see what are kind of the findings that we see at a larger scale. So with a larger sample of patients, there was some evidence that we're seeing that the echocardiogram findings are actually not much different, really not different at all compared to the general population. And so that, as previous studies had also established, warrants to, of course we do,

cardiac exams on all of our patients. So we do the auscultation, we listen to hear what we can find at just using our stethoscope and doing the things that we do on physical exam. So that's still absolutely warranted and indicated. And if there are findings from that, then we would have an echocardiogram ordered. But just off the bat, just because there is presence of hypermobility and knowing through these large samples of patients that there is not.

any increased prevalence compared to the general population, then there's no indication for doing echocardiogram for every single patient, unless there are those cardiac findings on exam. So to answer your question, no, we don't do an echocardiogram on every single patient, unless we find some abnormalities or some information from the history or family history, things like that, that might serve us to investigate a little bit further.

Linda Bluestein, MD (08:31.378)

Okay, great. And then what about other like blood work that you might do?

Dacre Knight (08:35.907)

Yeah, so that has changed a little bit in some ways. Actually, I would say we probably broadened that, you know, whereas like the echo cardigan things like we kind of narrowed it down, zeroed in our focus, try to conserve resources and, you know, insurance billing and things like that, if it's not necessary, if it's not changing management. But some things for the lab work have been pretty consistent in some regards. So we do.

And also we have to consider what maybe are the labs that patients have recently received. You know, if they just saw their primary care doctor and they got certain labs done, you know, of course we don't need to repeat them. If it's just a matter of days or weeks ago, then things look normal and no major changes or medication changes since then. But still we do want to get things, you know, like a complete blood count, metabolic panel, things like that. Just so we get some basic assessments on.

other contributors that might be symptoms of another condition separate from HSD or hEDS. So things like, you know, anemia can be contributing to fatigue. So do we need to look at the blood counts, hematocytes, hemoglobin, and things like that, or other chronic infections? Maybe we look and see if there's any leukocytosis elevated white blood cell counts. And so we do that, just a complete blood count, general chemistry, same thing. We want to make sure our organ function.

as far as signs we can take from blood work. So kidney function, liver function, electrolytes, sodium, potassium is all where it should be. And then some other ones in vitamin D level, thyroid of course, as we know, thyroid function can have implications on fatigue, energy levels, temperature, tolerance as well. So we do those pretty standard. And then the ones that, so we've added on

probably a little bit more of an investigation out of some other autoimmune markers too. But we don't really dig too deep, but just kind of things on the surface. So maybe some inflammatory markers, CRP, ANA, and then some mast cell screening as well. So tryptase and some urine mediators too. Because as we're learning more that there may be some involvement of those conditions too with our patients with HSD. And this is just something that we gain from what is out there in the literature.

Dacre Knight (10:56.255)

research that's going on.

Linda Bluestein, MD (10:58.674)

So that's really interesting. So you're doing some MCAS testing through tryptase and maybe urinary leukotrienes and things like that on most of your patients or at least a substantial portion. Oh, great.

Dacre Knight (11:09.275)

Yeah, yeah, because like I said, most of our patients have some degree of fatigue, some degree of whether it's an allergy condition that may not be just typical allergies, but maybe there is some gut involvement, some gastrointestinal disturbance or some neurologic effects, even that's kind of in the range of headaches. So much overlap from mast cell and all those symptoms too of HSD and hEDS So

We want to make sure we don't miss anything there.

Linda Bluestein, MD (11:40.426)

Yeah, definitely. And you've already mentioned some ways in which the clinic has evolved since its inception. Are there other significant changes that you would like to share with us?

Dacre Knight (11:52.291)

Yeah, so I feel like it's always something new every week. Because, you know, there's the number of patients we have seen has increased and that's great. I mean, as you know, and others who are listening in, I'm sure know that there's a lot of long wait lists at a lot of places, there's some difficulty with access in some locations, providers who know. So we wanted to improve on that.

Linda Bluestein, MD (11:56.357)

Yeah, I'm sure.

Dacre Knight (12:20.511)

And we have. So like I said, we added a Shil pa to our team. But other ways that we've changed so that the testing, you know, made some minor changes there, lab work echoes and things like that. Our involvement with physical therapy and occupational therapy has been pretty consistent. I mean, that's one thing that is pretty continuous. It's a constant since our inception.

We've added a treatment program too, although that's not that new anymore now. That's our day long virtual group treatment program. And there's some, of course, some adjustments that we make there on length and topics that are covered. So it's always there's always something new.

Linda Bluestein, MD (13:05.414)

I'm sure. And I love how the clinic was developed and immediately integrated with research. And you've already published some great papers, including your 2022 paper establishing an Ehlers-Danlos Syndrome Clinic Lessons Learned. And then you talk about one of the tables has like the referrals. And of course, it's not surprising to see, you know, PT and OT near the top and also pain clinic and GI were also near the top.

Rheumatology was a little lower down on the list, and I think it was like 33% of patients are referred for rheumatologic evaluation. Are a lot of patients coming to you already having had a rheumatologic evaluation, and what are your thoughts about, you know, kind of ruling out rheumatologic conditions that might explain a person's symptoms?

Dacre Knight (13:52.251)

Yeah, that's a great question. And so yeah, the first part, we do have a fair number who have already had rheumatologic evaluations prior to being seen. And again, back to the access issue, I know many patients kind of look far and wide to see who knows anything about these conditions. Sometimes it's rheumatologist, or maybe sometimes rheumatologists in the community have been put to task and just try to evaluate some of the symptoms like joint pain or widespread pain.

Linda Bluestein, MD (13:59.606)

Sure.

Dacre Knight (14:20.979)

They ruled out the autoimmune condition part, and then they suggest maybe it's hypermobility or something. It's kind of out of my scope, and then patients kind of just by course of elimination go through the next steps to see who else might be able to evaluate their symptoms. So yes, the short answer on that is we do have quite a number of patients who have had rheumatologic evaluations, and sometimes even internally too. So we do take referrals from our rheumatology colleagues here at Mayo Clinic.

if it's kind of the same course, they've done evaluation for joint pain and, and then they ruled out autoimmune conditions and suggest, or see signs based on Beighton score and things like that, that it might be some features for hypermobility. So then as far as your question about what do we do about the autoimmune investigation. So just as I tell a lot of our patients too, and we get this asked a lot, you know, because I have, you know, potentially some sort of inherited disorder of the connective tissue.

which as we define it is separate from acquired disorders of the connective tissue, which is where we're talking about autoimmune and things where your immune system is attacking the connective tissue or causing symptoms that way. Two totally separate pathways. We get asked, am I more prone to having rheumatoid arthritis or another autoimmune condition? The answer off the bat is no, not necessarily because these are two separate pathways. One, we're talking about how the tissue.

develops and how it's formed in the body versus one how it is Interrelated with the immune system and that may be overactive in some ways So separate pathways now, however, what we do see very commonly is that overlap of the symptoms So joint pain can certainly be involved with both So it does warrant I believe an investigation of both with your when you have a presentation of What could be either inherited or acquired disorder the connective tissue?

So hence the reason why rheumatologists are sometimes seeing patients with what may be a genetic condition, and sometimes geneticists see patients who may have a rheumatologic condition in the end. So from our end, when we see patients who may have rheumatologic concerns, so we do a joint inflammation, kind of cursory to what a rheumatologist might do, but at least to just get an initial assessment to see if there is there significant swelling, or redness, or warmth in the joints.

Dacre Knight (16:45.659)

Is it affecting multiple joints or is it just one-sided? And then some of those, like I mentioned, there's laboratory markers, which we've expanded on a little bit just to make sure we're covering all aspects of screening that we can. So inflammatory markers, ANA, rheumatoid factors and things like that, at least help us get an idea where to go from there. So we do ultimately do some sort of an autoimmune evaluation as well.

Linda Bluestein, MD (17:12.786)

That's great and it's really appreciated that you're explaining the difference between an inherited connective tissue disorder and an acquired one, because I think that is something that is often really confusing to people.

Dacre Knight (17:24.971)

That's right.

Linda Bluestein, MD (17:26.526)

Okay, so POTS clinic was the third most common referral after PT and OT. And I do orthostatic vital signs on a lot of my patients, you know, like 10 minutes. It takes up a lot of time out of the visit, but I do that quite frequently because of course a lot of our patients have orthostatic intolerance at the very least, and then we wanna evaluate them if they might possibly have POTS. So what are your criteria for sending someone to the POTS clinic, and do you know what percentage of those referrals are ultimately diagnosed with POTS,

OI or another form of dysautonomia.

Dacre Knight (18:00.083)

Yeah, another great question. And this is again, kind of on that theme of where we have so many overlapping conditions, right? And so many overlapping features. And then our job as a clinician is to try to parse it out and see what's what and kind of help patients understand what's causing what. Although we may not always know. So we do want to screen for whatever we can. So the POTS and the dysautonomia orthostatic intolerances

Linda Bluestein, MD (18:06.962)

Mm-hmm, right.

Dacre Knight (18:27.971)

is a big one as at least part of our screening goes. Now what the findings may be, it can certainly vary from patient to patient. So at least we start with asking, have you even been evaluated for POTS? Do you even know what POTS is? Has this ever come up in your history? More often than not, I should say, because our patient population, as you also know, is very well educated. And at that point, they've come to see us. They've already done a lot of reading. And they've got themselves up to speed on a lot of these comorbidities. So most often, I can simply just ask a patient, have you ever had an evaluation for POTS? Has that ever come up? And either, yes, I have, I've been diagnosed. Okay, well, what's kind of the treatment that you're under right now? How's that going? Or I had been evaluated and it wasn't POTS. Or, you know, in a lower percentage, I would say is, I have no idea what is that. And then so we'll

kind of explain, well, these are some of the symptoms you might notice. So, you know, racing heart, positional tachycardia, upright specifically, so sitting to standing, the orthostatic intolerance might be some of the conditions of the dizziness, lightheadedness that are positional as well. So we just kind of go through that pathway to see what they might report. And if it...

does look like it's indicated. So then we do testing for pots. So this, we're fortunate that we have one of the 22 sites I think I just learned because I was actually with our autonomic technologist yesterday kind of shouting her going through the process of their flow, just understand that better. But 22 sites I think in the country that have this specific form of autonomic testing that we do that is quite comprehensive. So long story short, that we do the screening, ask the questions, if it's indicated, put in the order for that autonomic reflex screening. This is a test where they check their heart rate and blood pressure in different positions. It's not a full tilt because some of those symptoms can be quite profound if someone does have postural tachycardia syndrome. So what they will ultimately do and they'll do some of the sweat testing was called QSART So we'll do it with acetylcholine on the skin and try to assess sweat gland activity to get another type of autonomic function assessed.

Linda Bluestein, MD (20:23.885)

Mm-hmm.

Dacre Knight (20:52.543)

But then looking at the heart rate and see the variable changes with symptoms, which would if present be diagnosed as POTS. And then if that is the case, then that's kind of as you're asking, how do we route determine the route patients to our POTS clinic? So that's the answer of its diagnosis session. Now, if it's not, and but we come back and we've reviewed the test results for the patients, and we still determine they've quite significant symptoms, even if they

that specific heart rate change, then this could certainly be the orthostatic intolerance that so many more patients are dealing with. And a lot of the same management practices go into that. So then we'll have some counseling on how do we manage orthostatic intolerance in many ways similar. So adequate oral hydration, lower extremity, maybe compression stockings, resistance exercises and things like that. But to answer your question about numbers. So I think...

Linda Bluestein, MD (21:28.977)

Mm-hmm.

Dacre Knight (21:49.503)

It's pretty close to what we have seen in other studies about prevalence of POTS and orthostatic intolerance in the community. So where we are, I would say that about 20% of our patients with HSD or hEDS have some clinically defined POTS and then a much higher percentage of those with some degree of orthostatic intolerance and I'm talking about three quarters of our patients with that.

Linda Bluestein, MD (22:19.458)

Mm-hmm.

Dacre Knight (22:20.207)

So we still have a real importance on getting them that information and counseling on treatment.

Linda Bluestein, MD (22:29.91)

So there's only 22 centers in the in the US I take it this is that actually do the full autonomic reflex screening Wow

Dacre Knight (22:39.195)

Yeah, yeah. So there's various ways of doing it. I mean, some ways you can, you know, kind of do the poor man's tilt, right? You just, you sit down, you stand up, you check your heart rate and things like that. But, and that works if that's all you got, it's fine. It's something to go off of. But if we want to get all the details of what is the variability and how much of, you know, implication might there be as far as other

Linda Bluestein, MD (22:45.578)

Right?

Dacre Knight (23:05.767)

defects in the autonomic nervous system because there's a lot to it right it's not only heart rate control so If there's anything else that could be Warranting therapy. Yeah, it's good to have that information

Linda Bluestein, MD (23:10.134)

Right.

Linda Bluestein, MD (23:18.294)

That's great information to have, and I get frustrated when oftentimes patients will have seen a cardiologist and the cardiologist does a tilt table test. And I feel like so often, at least in private practice, they will view it as a cardiac condition and really just look at the cardiac effects and not consider the gastrointestinal and the temperature regulation and all those other things that you mentioned.

Dacre Knight (23:40.551)

Right, exactly, yeah. A lot of problems arise very quickly, as you can imagine with that, yeah.

Linda Bluestein, MD (23:45.11)

Yeah.

Definitely. In the same 2022 paper, you talked about that on the first 483 patients that you did genetic testing on, you found no pathogenic connective tissue variants in 70 percent. You found VUS's inconclusive results in 28 percent, and pathogenic variants are variants of unknown significance, likely pathogenic in 2 percent. Do you think those findings are pretty consistent with what you've been seeing more recently?

part, how do you determine which patients to test? Because I think this is something that a lot of people find frustrating and confusing.

Dacre Knight (24:24.811)

Sure, and I've been asked that quite often actually, yeah, about the genetic testing. But to start with, so yeah, the numbers on genetic tests, and I should say this is something that has evolved in our practice too. So we initially were doing a lot more testing. And I think that, you know, it's a good thing to point out here, the...

a big change that we've seen in our practice is been the type of patient we're seeing. So what, and this is just from my own observation. So from starting the clinic from day one, or even before we hit the green light that we're gonna open and operate, we had a waiting list of a hundred patients already, even before the door opened, just because there's a lot of communication in the EDS community, which is...

Linda Bluestein, MD (24:59.14)

Mm-hmm.

Dacre Knight (25:20.287)

great, you know, shared information and knowledge and stuff. But one of those is like this, the clinic starting up here. So we had a waitlist for the very beginning. And I think and this was probably continuous for the first few months, maybe in the first year or so that a lot of those patients who we had seen initially were those who are already pretty well established in the community and were in touch with a lot of the goings on and research and things like that. I mean, certainly we still see a lot of

Linda Bluestein, MD (25:31.804)

I'm sorry.

Dacre Knight (25:48.243)

very well connected and educated patients now, but those at the beginning were the ones that had, they're kind of like the OGs, the old timers who were very knowledgeable and were seeking, kind of more guidance and resources and things like that. So to that effect, a lot of, and some of those patients we'd seen where the worst pathogenic testing is another example of that. So we had actually acquired patients who had already had a lot of those things, some of them seem to be able to work faster. And this is another example. So we said, as far as the R&D, many of our things have R&D problems. But we don't have that. That's it.

Dacre Knight (26:17.747)

diagnosis, many of our patients had already had diagnosis from the very beginning, and they just were kind of seeking more treatment guidance, or maybe they wanted to be involved in our research studies. So I think that has shifted with time, you know, now we get more of the kind of the internal referrals from other departments where like they don't know about this, or patients don't know about this, or just came upon it, someone told them about it, this might be something you're interested in looking into. So that shift has happened. And then

So, yeah, so there's been some variations there. And I suspect, and we haven't put together the data from those genetic test reports, which is what you're asking about, would it stay the same? I suspected that would change in a sense that we would probably have less of those pathogenic mutations being found, even though it's a very small number. But more of our patients now, like I said, are just those that, you know, kind of just learn about it.

or just have a new referral. And so, and most often than not, so as we know, hEDS and HSD, the genetic testing would show no pathogenic results. And so I suspect that could change in some way there. I think you had another part of that question too, if you remind me.

Linda Bluestein, MD (27:34.793)

Okay. In terms of, yeah, in terms of how you decide which patients to test, because I think within the community, there's so much confusion, because they feel like, wait, I think I'm pretty similar to this other person. They were offered genetic testing, I wasn't. This is a question that comes up a lot, yeah.

Dacre Knight (27:38.552)

Ah, yeah.

Dacre Knight (27:50.069)

Yeah, yeah, absolutely. So.

For the diagnosis of HSD and hEDS, it's pretty well understood. And even from that data, we're showing hundreds and hundreds of patients being tested and negative result. And that's as we know, there's no genetic test that's gonna be positive when we know the diagnosis is hEDS or HSD. So there are still some patients who have a lot of, you know, consternation about, you know, having this anxiety related to vascular EDS or classic EDS, or, you know, a grandparent who had a...

abdominal aortic aneurysm that ruptured and things like that. And so what it comes down to is the individual setting. And so we do need to ask every patient, what is your family history? What are some features that might be alarming to us that could warrant genetic testing or could give us concern that there's something more going on about your family history that shows signs of one of these more rare types of EDS?

So some of those, just like I mentioned, so aneurysms are one, and closer the relationship, the more importance. So if it's like your mother or father had a brain injury or a rupture and they died at the age of 30, that's a huge red flag. And it's more distance the relatives, kind of the less that alarm is, and then the other ones, or maybe there's even just sudden death, even if you don't know what was the cause of death, but sudden death could be a ruptured aneurysm.

potentially. And then some others, other red flags that are kind of signs of symptoms of the rare types of EDS would be colon rupture as well. So, and we're not talking about, you know, colon cancer or surgery, but spontaneous rupture of the intestines, maybe multiple hernias that's kind of go along the same lines there. Cleft palate, retinal detachment, those can be features of other rare types. And then

Dacre Knight (29:50.939)

Non age-related hearing loss. So those are just some very cursory red flag questions we ask and I have to give credit to dr Frank Lamont out for her long experience these disorders because that was actually one of the first questions I had for her when I came on seeing these patients because it's important to know and because You know, there is a downside to just doing a genetic test on every single patient It doesn't come like no strings attached.

Dacre Knight (30:18.287)

There is, you know, cost and time. There's the anxiety of waiting. There's the anxiety of results. And if those results just end up being an uncertain variant, which ends up being a benign variant, then, you know, uncertainty is not something that many people want to sit well with. And even if that uncertainty is benign, so we don't want to have to put them through that unnecessarily. And in the case of HSD and hEDS, genetic testing is not indicated unless there are some of these other… So it's hard to find by waiting for the right involvement and the involvement of people. So the line going into that, I'm sure you didn't want something like this, you wanted to get involved with it. And I think that's a pretty good, so you don't want that to be the case. So it's going to take a little bit of time. But I think that's a good thing.

Linda Bluestein, MD (30:51.49)

That's great information. And by the way, I'm going to mention another paper that you published. And I will link all of these in the show notes. So if people are trying to scribble down the names or whatever, they will all be there for people to find. You published a paper in 2023 about the overlap between fibromyalgia and hEDS and HSD. And you actually found that people who had both fibromyalgia and either hEDS

were had the most symptom burden and the highest level of comorbidities regardless of whether they had hEDS or HSD which was really interesting to me because you know we've we keep dealing with this which one is more severe a lot of people that are diagnosed with HSD they feel like

people don't take them as seriously and things like that. So I think that was a very useful piece of information. One of the things I think is frustrating for people is that they often get diagnosed with fibromyalgia. I feel like this is really common in my patients. They've been diagnosed with fibromyalgia and then the diagnostic journey kind of stops after that. And do you have any suggestions for people who suspect that there's something more going on who have been diagnosed with fibromyalgia?

Dacre Knight (32:09.811)

You're absolutely right. That can be a big problem. And it's something I've witnessed firsthand too, and just like you describe it. So the fibromyalgia aspect of the condition is something just like we were discussing earlier, where there's overlap of, you know, POTS and the orthostatic intolerance, and just like any of those other things, fibromyalgia can run with it. And what we have seen in the data collected, that was actually the majority of our patients.

also having this condition of fibromyalgia as well. We don't know exactly what's causing it. In most cases of fibromyalgia, it's not clear what is, if any, cause at all. Although we do know from some relatively small but fairly good objective studies that there could be some immune system involvement, there can be some kind of pain pathway signaling involvement and things like that are thrown at us. So...

The problem though that fibromyalgia gets is it's yeah there are lots of misconceptions about it in the community too and that kind of permeates through patient groups as well that it's a waste basket diagnosis or you know something there's no good treatment for it and they only give you that diagnosis if they can't come up with anything else and so it's a big hindrance for our patients because

what I tell patients, if you have hypermobility and fibromyalgia, we really need to address them both. Just addressing one doesn't necessarily resolve the other. And we really want to use every resource we can to improve on your condition. So we don't want to leave any stone unturned about anything that can be helpful. And so when patients get the diagnosis of fibromyalgia and they miss out on the opportunity to find improvement in treatment for their hypermobility, then they're at a major loss there, right? Because that's only half of the puzzle.

And likewise, if they get a diagnosis of HSD or hEDS but miss the aspect of fibromyalgia and fibromyalgia management, then there can still be a lot of those chronic pain conditions that persist. And as much physical therapy as you can try to do, we won't find the expected outcomes that patients want. So we really want to give them all opportunity to improve on both.

Linda Bluestein, MD (34:34.806)

Definitely, and I take it you see the widespread pain quite commonly in your patients. I certainly see that in my patients and that's a really limiting factor for a lot of them.

Dacre Knight (34:45.563)

Yeah, yeah. So yeah, we pulled that together some data on the symptoms of our patients and the widespread pain, whether or not it was actually within the criteria of fibromyalgia was over three quarters of our patients. And the specific diagnostic criteria, as I mentioned, for the full sample of how many hundreds of patients that was, we pulled together at least over 500 was the majority. So it was over 56% of our patients. So

Yeah, it's very prevalent and it's all part of that package of treatment that needs to be addressed in order to find the best resolution.

Linda Bluestein, MD (35:23.158)

Yeah, we'll definitely dig into that a little bit later because I'm gonna hopefully hit you up for some of the questions that were submitted online and kind of do a little rapid fire at the end for some of those if we have time. So yeah, so in your 2022 paper, you had seen at that point 563 patients and then you had seen 773 by the time you wrote the 2023 paper. Again, I love how the research is integrated right there with the clinic and having been a patient.

Dacre Knight (35:35.992)

Yeah, great.

Linda Bluestein, MD (35:53.072)

to fill out all these forms, but it's great, because this is how you get the data, and it's so helpful. So with that first group, you had found that 39% were diagnosed with hypermobile EDS, or you diagnosed them with hypermobile EDS. 48% you diagnosed with HSD, and 13% did not meet the criteria for either. But then in that bigger group of patients, the percentages that were diagnosed with "HEADS were lower, 23%, and 77% were diagnosed with HSD.

Do you have any thoughts about that or can you kind of elaborate on that at all?

Dacre Knight (36:26.539)

Yeah, the first thing that I would suggest and is what I was alluding to earlier is that our patient population has shifted a little bit. Okay, just on who is aware and who wants to be seen here. And usually those who have been around longer are the ones that we've seen first off. And so whatever the explanation is and you know.

Linda Bluestein, MD (36:36.085)

Mm-hmm.

Dacre Knight (36:53.023)

happy to entertain any theories that they're out there, but I'm guessing is that those who previously had the diagnosis were connected to the communities and networks were the first ones to be aware of this information that we had at clinics that were coming in to be seen. So then another thing that has happened too since that time is that we've just seen so many more patients. So when we started, we had, I think it was just one or two half days per week.

Linda Bluestein, MD (37:19.938)

Mm.

Dacre Knight (37:20.887)

And so that was maybe about four, between four to six patients per week. And then it slowed down during the pandemic. So small numbers. So when we were taking small numbers, there were probably a lot of the similar, more of the same diagnoses throughout the first year or two. And then after the pandemic, we were less involved with the coronavirus treatment and taking up a lot of the patient appointments. But then we expanded the clinic

Linda Bluestein, MD (37:26.143)

Mm-hmm.

Dacre Knight (37:50.655)

four half days. So we took a good jump there to about 16 patients per week that we were able to evaluate. Still though, with a growing wait list, it was starting to grow from six months to a year to eventually at one point we had new administration came in that essentially said, all right, we're capping the wait list. We are not taking anyone else because we need to work our numbers down and get through this, which was very unfortunate because patients were calling in. There was being told, no, we can't see anymore patients. So I was doing everything I could to try to kind of keep pushing our chair, our department to find resources and see what we can do. And she was agreeable to help our clinic because it was a great tool for research and kind of education that we're also involved with here to teach medical students and residents about these conditions that often get overlooked.

And so she was very supportive, unfortunately. So this was, came in where the hiring process was involved. We got a PA, she came in to join us, and then all of a sudden we jumped to seeing 30. Now we have access for about 30 to 35 patients per week. And so what that is about, it just immediately wait list was reopened and then has just come way down to now where our access is like a month out or even better, we're only delayed by any other specialist we work with.

So our access is great, but what that has meant is that we've seen so many more patients more recently, right? So if we look at the numbers from the beginning to the end, it's kind of just like an upward bend. And so I think, you know, as other provenance studies may show is that HSD is just much more common than hEDS. And so thereby alone, if we're having many more patients, then that will shift our statistics as well.

Linda Bluestein, MD (39:25.404)

I think that's what that is. I think that's what we're all talking about.

Dacre Knight (39:45.983)

to having a higher number of HSD and the more recent studies that are being done. And just from what I'm seeing myself too, I suspect that in any further research that we're doing, that trend will continue to increase.And for the patients that are diagnosed with HSD and are kind of either they perceive it or they're doctors or maybe. giving them that impression that they believe it's a less serious condition. Can you give any insights or what you tell people if they are thinking that they have hEDS and you tell them that, nope, you don't meet the criteria for hEDS, but you do have HSD and how you actually coach them and educate them on those differences?

Dacre Knight (40:33.711)

Yeah, I think this is really, really important, because this is part of the therapeutic process. First, I mean, to have a diagnosis, right? And then to understand your diagnosis is, you're not going to get anywhere until you get those two things established. Because if you're, and there have been psychological studies about this, if someone's given a decision to be made, and one group is told that they can change their mind, the other group is told they can't change their mind. If you're told that you can change your mind, you doubt something, essentially you're

not going to be satisfied with your care, you're not going to be satisfied with your treatment and your outcomes. So establishing a diagnosis, making it very clear, what are we looking at when we're doing the diagnostic evaluations as clear as possible so that we can make it and then we can move on to the most important part, which is the treatment part. So yeah, it's a great question because even this, just like we're talking about fibromyalgia, having so many misconceptions, there are a lot of misconceptions.

Linda Bluestein, MD (41:25.734)

Mm-hmm.

Dacre Knight (41:33.895)

describing about HSD and hEDS So where do we come from when we are talking about these terms? Well, I think we should go back to where the research is headed right now because it's so, so important. And the first thing I'll do with patients is, at least in our treatment program, is we try to get a poll of understand where patients are in their

journey about how long they've been experiencing their symptoms until they've received a diagnosis. And those numbers have been published there out there, you know, anywhere from 14 years, maybe improving to 10 to 12 years now. And interesting in our patients, I don't know for whatever reason, but it seems like it's always a little bit above that average. So patients, you know, describing they've been 15 years or 20 years, they've been dealing with these symptoms until they actually receive some diagnosis of HSD or hEDS.

And a large part of that, and not only, you know, not to mention is that really unacceptable to where we are in our medical understanding to have someone wait for so long until they receive a diagnosis to be suffering so long, but it also ties into what are the tools we have to make these diagnoses, right? And if we don't have great lab tests, we don't have, you know, a tissue or saliva sample or blood sample that we can take and then make that diagnosis.

have it as prevalent as we just check for anemia. We can check a hemoglobin or a thyroid marker. Then there's reason why there is so much delay. We have these clinical criteria which are helpful, but it takes time, it takes time to find the right person who's even interested in doing it. So where the research is headed is to try to understand what those markers might be, right? So we want to understand and learn if there's something that we can use. If it's a genetic test,

or if there's some other sort of marker that we can take from the blood or saliva. So in order to do that, we have to understand what are the causes of these conditions? What is it that is it one gene, multiple genes, other inflammation or things like that? And in order to do that, that research, in order to expedite that research as quickly as we can to try to improve our diagnostic and treatment flow.

Dacre Knight (43:56.427)

then that's where we designate these groups of patients as distinctly as we can from HSD versus hEDS because maybe there are differences from a molecular level, from a biochemical level between those groups of patients. It really is theoretical at this point because we haven't seen any other major clear differences in symptoms. I mean, we've got some of our studies where we show with the fibromyalgia and things like that, but symptoms can...

be the same. Severity can be the same. And I've seen the whole spectrum from HSD and hEDS patients from one into the next. And so ultimately, it requires the same management and treatment is the same between the two. Eventually, we might get to a point where we understand that there's some of those differences we can test for and we can improve our diagnostics. But for now, it's really most important for research than anything else. So,

We make those labels to help our research studies, but when it comes down to management, it's exactly the same.

Linda Bluestein, MD (45:01.791)

And I'm glad that you pointed out about research, because to me that's one of the...

Most frustrating things, well, we want PCPs to be able to, like private practice PCPs to be able to assess these patients. There's gonna be more consistency if they're seen at a place like your facility where you're seeing such large volumes of this and you are able to consistently make the diagnosis as compared to maybe somebody else who has much lower volume. So I feel like that muddies the waters more.

I actually see, yeah, I actually see not infrequently.

adult patients that have historical joint hypermobility and have signs of hEDS like skin hyperextensibility, easy bruising, tissue fragility, things like that, but they might currently have a very low Beighton score. And that's very challenging since the hEDS diagnostic criteria from 2017 start with the Beighton score. Now you can get another point from the five point questionnaire, but how do you handle a patient like that?

Dacre Knight (46:06.607)

Yeah, so it's on a case by case basis, but you're right. And it could be confusing and frustrating and all the rest. And that's the last thing we want when there's already been so much confusion, right about symptoms and what are the causes. So we want to try to clear it up as much as we can just to get on with the treatment. But I've definitely seen those patients myself is like, for all the reasons that are seen before me, you have a connective tissue problem, right? There's, you know, the gut issues, they

cardiac or POTS and things like that, there's something there. And what it comes down to is we're still in the dark in so many ways about how these things interact and what is underlying it, you know, between mast cell activity and dysautonomia, central sensitization and things like that. But the long story is short, if it's pretty clear that something's there and you can talk to those patients and you can see that all those

things line up and even, you know, regarding the Biden score, whether it's, you know, localized, historical or, you know, even post surgical changes, things like that. We still want to give them the treatment. We still want them to improve anyway we can with any every resource we have available to us. So in that case, just like I said, with hEDS and HSD and now historical or localized hypermobility,

the management is really all the same throughout. So at least our approach. So we still want them to see our specific physical therapists who work with hypermobile patients, occupational therapists and so forth and do the same counseling.

Linda Bluestein, MD (47:46.262)

Yeah, that now that makes sense. My treatment approach is pretty much the same as well, regardless of which of those that I ultimately diagnose them with. So I was fascinated to learn about your EDS Living Program and you published about this in 2023 and I would love to note. So you and your colleagues describe this innovative care model for hEDS and HSD, which

Dacre Knight (47:50.153)

Yeah.

Dacre Knight (47:54.801)

Yeah.

Linda Bluestein, MD (48:16.236)

4.1 trillion with a T annual health care expenditures. So I feel like this model really has the potential to change so many lives if it could at some point be expanded for other conditions. Can you share a little bit about this model? What was challenging about creating it? How has that evolved?

Dacre Knight (48:36.879)

Yeah, so I don't think there's any perfect model. I mean, in some ways, our limitations at our clinic, and I'll just be very forthright about it, is that we see as many patients we can do the diagnostic and evaluation, but we don't have the primary care services. So we don't have the longitudinal follow-up. And that's very important for patients because things change, you know, month to month, if not year to year. And so that's one of our big limitations. But then,

Linda Bluestein, MD (49:04.238)

So that's what about the company. But they have like a business. They have a business and that is the government that has that government that has to prepare for that. So they have to get them out. And each other out is a venture. And I think it has a service to obviously get them out of the business. So they want to have power. So, we're going to do it.

Dacre Knight (49:05.619)

you know, vice versa, and you may share some things like that. Being in the community, you also have limitations because you may not have access to all the resources that we would have in an institution like Mayo Clinic. So it's a balance, and we try to balance as best as we can with the time that we have with patients, how to use it to improve their long-term outcomes. So when we were setting up our clinic and we had long wait lists and we, you know,

patients were waiting or maybe getting turned away, we realized that we have to try to just concentrate and condense down as much of this material information as we can to one, give them the information, education that they need about their condition, but also two, to be able to talk about what their individual needs may be, because it's highly variable. We know these conditions are, there is so much clinical diversity from one patient to the next. And as we say, someone with HSD or hEDS, no two patients are exactly alike. So we have to some way accommodate both. So that is what gave us the idea for setting up this treatment program specifically, so that we could do the diagnostic evaluation as an initial consultation. So we get those patients set up. We know who are the ones that need to do this treatment program, just like I was mentioning earlier about referral to POTS clinic and things like that.

Linda Bluestein, MD (50:20.481)

Mm-hmm.

Dacre Knight (50:31.487)

we refer to ourselves essentially for the EDS Living treatment program, which is a day long treatment program. And the intention of that is to give more of the resource on education behind the conditions that we just can't complete in a single visit. Right. I mean, we spend so much time going through all these red flags and questions about genetic testing and family history and cardiac exams. So,

we needed a way to afford patients that opportunity for the education. So we tie in some of that. And then we put into that also the guidance for some of the standard self-management practices. And what that allows is then for another ultimately final wrap-up return visit with our patients that they've got all this basic knowledge, they've got the diagnosis, and now we can talk here, your testing results, what are your individual concerns, things that you've learned from the

the treatment program that we can attend to now to make sure we tied together as much as we can. So that's the reason that we did it. The challenge is that, you know, in some ways, even one day is not enough, right? And so we still have, you know, questions that come up, which is fine. I mean, we're available to answer those and provide resources as best as we can. But then...inevitably the other challenges too that getting a day it's a it's a quite I wouldn't say intensive program but it's a lot of information so for our patients who have a lot of symptoms fatigue and nausea and stomach issues and any of these treatment programs can be difficult and we do get kind of regular feedback that if this was split up over two days or half days it would be ideal and I would love to do it it's just based on how our

clinic practice is set out to see patients when we can, who's available at times right now, it's in one day. So that's a big challenge. Maybe with time we'll have some ways to improve on that. But I think what you're mentioning too about chronic diseases, a lot of times the patients with chronic conditions, they just not given that attention to answer questions about the disease, about the condition. So they learn more and they learn more.

A lot of things that we take away from that treatment program is that patients come away like, oh, well, I didn't realize that this is something that I could be experiencing or this is another condition. Whether it is maybe it is the first time someone's heard of mast cell or pots. So they know now to be monitoring for that. Because how would you know to ask if you've never even heard of it? So that's why we want to give them that chance.

Linda Bluestein, MD (53:13.922)

Right.

Linda Bluestein, MD (53:17.678)

That's great. And yeah, I can definitely see we're trying to absorb that much information in that period of time, but there's the practicalities of how do you actually do this exchange of information that is so important. And what has been the most surprising thing about the Mayo Clinic, EDS clinic that you found?

Dacre Knight (53:30.96)

Yeah.

Dacre Knight (53:38.343)

Well, I think it's the patient population at first, which I was just referring to earlier, very educated, very well connected. And it's embarrassing to say that when I first was getting to this field, there were so many patients who were educating me in so many ways. But as long as you take it in good humor and as a educational opportunity, it's all for the better, right?

There's so much we learn from our own patients. And in fact, that's the reason why we have set up a patient advisory group from our clinic patients we've seen in our clinic that we meet on a monthly basis now just to get feedback from patients. Because there's so much going on that they're doing, they're learning that we want to know too. I mean, there's two sides, there's two faces to it. So that was an important.

Linda Bluestein, MD (54:32.718)

And you said something at the EDS Society conference that I have often said too, which is you don't necessarily need to go to somebody who is an expert if you, and one of the first rapid fire questions is actually going to be related to this, but if you're PCP, if you don't have access to the Mayo Clinic EDS Clinic, and if you have a PCP though that is empathetic.

wants to learn is willing to work with you and is curious, then that might be able to be, that might be hugely beneficial for you. So, you know.

Dacre Knight (55:04.291)

Yeah, exactly. And that's a very common question we get because as you're saying, because we're limited from doing those primary care services. So patients say, well, now what I've got to diagnose my primary care doesn't know anything about this. And, you know, we remain as a resource there Mayo Clinic resource on EDS that pale in comparison to all the resources that EDS Society has. So we would encourage

them to offer those resources to their primary care and if they are interested to learn. And many of them are, many of our patients do find good primary doctors who are willing to go on the EDS Society website and if not attend conferences, at least, you know, peruse some of the echo programs and things like that they have. And that's the key feature of a good primary care.

Linda Bluestein, MD (55:52.75)

Definitely. And so this is a perfect lead in to the rapid fire questions. I have a few for you. And the first one is what would you like PCPs to know about hEDS and HSD?

Dacre Knight (56:05.115)

Yeah, that's a great question. So first off, I would say that there is treatment because it's so disappointing to hear patients say, well, I went to this doctor and they said, there's nothing you do to treat it. Like, well, the answer is that, well, it couldn't be more from the truth that there are things that we can do to improve on this, to prevent injuries and things like that. So if a primary care needs to know one thing, know that there are treatment resources out there. We generally do see good outcomes and that's what we seek and we should try to.

reach those outcomes that we want to find, keeping patients functional, improve quality of life, and all of those other endpoints.

Linda Bluestein, MD (56:42.794)

And I know you, actually it's interesting that you talked about our different approaches or different practice settings, and it's pretty much as opposite as you can get, because I'm in a community and I really struggle with referrals to different specialists and I have patients that fly in from different places, and so I have no idea what they have locally to them. You have the exact opposite situation, but I am able to follow people over time, which is helpful.

So I see a lot of things that I think, okay, like you said, we learn from our patients, right? We learn a lot from our patients and see what things seem to be beneficial. Do you have thoughts from the patients that you've seen about things like regenerative medicine, prolo therapy stem cell therapy? Have you seen people benefit from that or not?

Dacre Knight (57:29.659)

Yeah, yeah, that's an interesting area because that research is still growing too. I have had patients benefit from it and the advantage that I see right off the bat is that if we're talking about even, you know, regenerative or proliferative therapy, there's still some intervention but it's not quite as that risk of surgery, right, which has much more risk infections and complications and things like that. So that's the benefit I see right off the

Linda Bluestein, MD (57:52.119)

Mm-hmm.

Dacre Knight (57:59.779)

likely an increased safety profile with those types of measures. I mean, we would hope that it doesn't need to come to that point that we can find other modalities that are even less invasive. But if that's the case, I would say that's my impression is that maybe there is this alternative surgery to avoid some of those other surgical risks.

Linda Bluestein, MD (58:18.922)

Yeah, definitely. And what are the things that you have found to be most effective in your patients in terms of treatment? Ha.

Dacre Knight (58:27.527)

Wow, yeah, that's a great question. And I think that's the million dollar question now because once we go through all the diagnostic, we set that, we've done the testing, all those things, now what do we do for treatment? And in some ways, in the back of my mind, I wonder if out in the community and even at our site, if we just need, if there's not enough focus on treatment, even if it's from patients alone who like, I've got a diagnosis, that's all I wanted.

It's like, well, no, there's another piece to this. There's a piece to like, how do you improve with this condition? How do you improve your function and things like that? So don't just stop at getting a diagnosis. You deserve to find improvement in your quality of life. So lots of treatments out there. We do have another research study right now. I mean, so many irons in the fire right now when it comes to research here, which is great. But...

Linda Bluestein, MD (59:16.526)

I think that's a good question.

Dacre Knight (59:24.383)

It keeps us certainly busy. One of them is an outcome study to look and see what patients are reporting they're using and associated symptoms because I think that's huge. We can just talk and talk to patients until the cows come home about all the things about massage and acupuncture and heat therapy and things like that. But we want to have some clinical data to back it up and there's not a lot of data. One thing that I find patients...

Linda Bluestein, MD (59:30.204)

Mm.

Dacre Knight (59:51.807)

Pretty commonly report benefit from though. And so that's probably one of the first things I benefit from or that encourage after the exercise of physical therapy is the heat therapy. Now, autonomic dysfunction can kind of throw a wrench in that, but the heating pads, warm baths, things like that. So that's obviously very safe as long as you're not burning yourself. So that's a good one.

Linda Bluestein, MD (01:00:01.75)

Hmm.

Linda Bluestein, MD (01:00:14.374)

And I'm dealing with an issue right now. And this is a light therapy device that I use that applies heat and also light. And if I find a lot of those things very helpful for me personally. And yeah.

Dacre Knight (01:00:16.436)

Yeah.

Dacre Knight (01:00:24.835)

Yeah, it'd be a surprise how many patients come here and sit next to me in my exam room and they just show me, yeah, I got my heating pad in my purse right here. Like, there you go.

Linda Bluestein, MD (01:00:31.786)

Yeah, right. They're ready, they're ready. What are your thoughts specifically on muscle relaxants? Because, and of course when I say muscle relaxants, I'm not referring to like in the operating room, paralyzing the muscles, but the drugs, the medications that patients typically think of as muscle relaxants.

Dacre Knight (01:00:37.011)

Yeah.

Dacre Knight (01:00:47.294)

Mm-hmm.

Dacre Knight (01:00:50.959)

Yeah, this came up actually at the EDS Society Conference in Dublin, and I was asked this question, and it's a good question because we're all still learning. Again, when I'm talking about lack of data, there is definitely lack of data when it comes to patients with hypermobility who's been given a muscle relaxant as a treatment. And there's some interesting theories behind it. I think both ways. They're very good. I don't

I tend not to try to jump the gun when it comes to prescribing muscle relaxants, although I understand that there's very good argument of how they can be effective. Certainly, so just kind of in a nutshell for the listeners that if the pain related to hypermobility is a result of the spasm because the muscle is trying to counterbalance that hypermobility and the muscle is then working extra, it's causing spasm and pain.

discomfort, then that's the idea how the muscle relaxes or work, which I think is a viable theory. Also counter to that though, being kind of a disclaimer that if you have hypermobility, because the muscles are too relaxed, could that increase hypermobility and increase injury? Yeah, that's a very good theory too. So what it comes down to is we just need to get some more objective understanding trials and things like that to know where does the answer lie. So

In the meantime, I would just go on a case by case basis, patients describe where's your pain, does it hurt here, what are the features of your condition, and then have an idea of whether a muscle relaxer can be useful or not.

Linda Bluestein, MD (01:02:25.61)

Sure. And another controversial or somewhat, I guess, controversial area is bracing. What are your thoughts on bracing? And of course, that can range from, you know, compression type sleeve to like a rigid brace for a joint.

Dacre Knight (01:02:39.223)

Yeah, yeah. So that is an interesting one too. And actually, the first time I came upon the research and application of bracing was from Dr. Chopra. I know he's been on your show recently too. And he's got vast experience on pain management, certainly with EDS and hypermobility. And I know he was at least the times I was listening to him give talks on that as a proponent of bracing, as a kind of a pain remedy.

I think so it's just as he's explaining it, there is a place for the bracing is a pain modality that can be safer than surgery can be potentially safer than medications that have all these side effects. So then there would be the maybe the counter argument from the physical therapist who would say that you know bracing can lead to weakening of the muscle deterioration of the joint stability if it's used for a prolonged period if there's muscle atrophy and things like that so.

want to avoid prolonged bracing to avoid some of that decreased natural stability that's given by the muscles and joints. So again, really the best thing to do ahead would be on a case by case basis. Yes, if we're talking about pain management, it can be very useful certainly to get someone to the point where they're able to be more active. Bracing could be very helpful, but just be aware of their risk with prolonged bracing. So that doesn't come without risk.

Linda Bluestein, MD (01:03:57.59)

Right.

Linda Bluestein, MD (01:04:03.802)

And that's what I find too, that oftentimes if you support a joint, at least in some capacity, they can actually be more active and that can be really, really helpful to improving functional capacity and quality of life. So people wanted to know if you had plans to open another center and how difficult is it to be seen at Mayo if you're out of state?

Dacre Knight (01:04:14.239)

Mm-hmm.

Dacre Knight (01:04:17.683)

Yeah, exactly.

Dacre Knight (01:04:27.363)

Yeah, I think access worldwide could improve and I would love to see access. I mean, we're happy to have patients come from all over as I tell our patient groups at our treatment program, but really what that's a sign when patients are traveling so far is that they just have poor access locally. And so we, you know, we want to make that better. I was in touch with some of our colleagues in Rochester at one point were interested in trying to develop a similar program

Linda Bluestein, MD (01:04:43.893)

Mm-hmm.

Dacre Knight (01:04:56.243)

that's what it really comes down to, is you've got to find those providers and clinicians who are interested in learning about this condition and treating patients with these conditions. And so, I think that's really the main obstacle there, because once it's there, and as I was interested, as I mentioned from the very beginning, if you find those resources, you can use them, and you'll certainly find the patients who want to come and be seen for them. So the development of the program in Rochester, I haven't heard...

much more about it recently. So for now we do take referrals from Mayo Clinic, Arizona and Mayo Clinic Rochester for now. And maybe they just thought that we were able to seek more patients so that there was no need to do that. I don't know, but anyway, how are patients seen from us, for us by out of town? So regularly we get patients from out of town. We do video consultations.

What I'd say about that is our first initial evaluation, though, we do face-to-face because we want to go through all of the things that we can on exam and make sure that we're giving the best treatment recommendations we can. But for follow-up visits, we can do those virtually. It makes it a little bit easier when it comes to travel.

Linda Bluestein, MD (01:06:11.102)

Yeah, definitely makes it easier for people for those follow-ups. And in terms of flares, what do you recommend that patients do if they are experiencing flares?

Dacre Knight (01:06:23.343)

Yeah, so this would kind of come into that treatment category question, I think, pretty well. And what are all the various treatments out there? And it's just dependent on what are the characteristics of the flare? You know, is it just simply pain flare or is it associated with kind of neurological symptoms? Is there some autonomic involvement too? So it's highly variable. Multiple modalities of treatment that can be used just for the same reason. There can be multiple causes of symptoms.

So we would just have to see what is the intensity of the flare, the timing, you know, other kind of typical features that we get on history.

Linda Bluestein, MD (01:07:00.71)

Okay, and last question from the listeners, from the social media post that I made. Do you have a post diagnosis to-do list?

Dacre Knight (01:07:11.303)

Ah, yeah, that's a good one. So that's something that we cover in our wrap up visit usually. And it is usually to the point that we've gone through all those diagnostic steps, it's usually, hopefully, at least, wrapped up pretty well that we've done all the testing we need. So it's not like you need to do a routine echocardiogram every six months. No, we've, you know, we've done all those things that we need to test for. There's not any specific

surveillance that needs to be done other than what you would do routinely with your primary care doctors. So routine lab work, vital science, blood pressure checks, laboratory analysis, and certainly if symptoms change though, then you might need some more immediate attention. So the first thing that's on our to-do list when I'm writing up my notes is to have some sort of continuity of care, at least with the physical therapy. Now primary care, having them be updated

clinical documentation and things like that. It's a good important one too. So make sure they have access to notes and records and they can understand what your evaluation and treatment has encompassed. But the physical therapy, if that can be something that's continued locally or at least access to someone who's knowledgeable. And there are usually more physical therapists out there who are knowledgeable about hypermobility than primary care doctors. And that's something that would direct patients to the EDS Society's provider directory website to see who are the therapists there that they could potentially work with.

to avoid injuries or even see if they do have a pain flare at a joint and it is something that can be ameliorated with this therapy.

Linda Bluestein, MD (01:08:49.17)

Okay, and where would you like to go next with your research?

Dacre Knight (01:08:54.215)

Well, I actually was asked a similar question today. So we're running our treatment program today. So I was with the group earlier. There's lots of things. And just to kind of just a brief summary of the things that we're looking at now. So as I mentioned, we're looking at outcomes, we're looking at treatments, we're looking at sex differences of patients, why there's so many more women diagnosed than men. Even though men are diagnosed, we know this is not necessarily a sex chromosome, but there is some penetrance that is certainly increased for women.

So those are the big ones. And then the biggest probably is this biomarker, so the genetic analysis and things like that. What can we do to improve the diagnostic testing so decrease that time span from symptom onset to when a diagnosis is made? The EDS Society has got the head study running, so if you're not aware of that, I'm sure you've given a plug for this here on your show, but if you're diagnosed with hEDS, I think they've actually already completed their collection, but hoping that there'll be some...

great interesting results that come from that. Or if not, at least, you know, we'll take away some information. So that's probably a very big interesting one for me because, you know, that will kind of clear up some of this confusion too, that permeates about HSD and hEDS if we know more of the background of the biochemical. One that hits home though, on a personal basis, is the question too, that comes up more frequently about the overlap in neurodivergence. I know you had Dr. Echols on your show previously too, about

Linda Bluestein, MD (01:10:19.811)

Mm. Yep.

Dacre Knight (01:10:21.863)

the ADHD, the autism. So it's just incredible how these coincidences or these things that happen in life that's part of our destiny or predestination if that's something you believe in. But our third child was diagnosed with autism and this was after I'd already started seeing patients and started running our EDS clinic. He was also found to have a de novo mutation in his P10 tumor suppressor protein. So

Linda Bluestein, MD (01:10:38.83)

Thank you.

Dacre Knight (01:10:50.271)

That is a rare disease on its own. And it just blew my mind that here I am now, even if I'm not experiencing hEDS myself, but experiencing a family member with a rare genetic disorder. And the same exact thing my patients are doing, going online, researching, where are the centers of excellence? Who's seen these patients? What is the research?

Linda Bluestein, MD (01:10:50.37)

Hmm.

Linda Bluestein, MD (01:11:07.948)

Right?

Dacre Knight (01:11:12.863)

And it just blew my mind. And I went through all the same pitfalls that patients experience about how do I schedule or there's a wait list, it's booking on until 2024. So long story short, that's a very major interest of mine, this overlap of the neurodiversions, autism, and connective tissue disorders.

Linda Bluestein, MD (01:11:34.838)

Yeah, that's a really fascinating area for sure. And speaking of doing online searches, because that's definitely how I got most of my information. I was working as an anesthesiologist in the operating room and had experienced a lot of different problems that kind of led to this transition ultimately. Do you have any tips for people for how to use, you know, Dr. Google in the most successful way? Because it can be.

I mean, I talk to people sometimes about dose and that kind of thing, but there's confirmation bias, there's a lot of challenges that come along with online searches.

Dacre Knight (01:12:11.535)

Yeah, absolutely. Or even online communities too. They can work with you, they can work against you. And I know you're taking a lot of great questions from your social media community too. And it's fantastic that there's these networks there that can support patients and support research and support our understanding. But just on the same side, there's also the kind of pitfalls that come with like increasing anxiety and things like that, or not knowing what is the real condition.

Linda Bluestein, MD (01:12:14.89)

Mm-hmm.

Dacre Knight (01:12:41.459)

So if, you know, I can say for resources, you know, I mentioned earlier too, the Mayo Clinic has some simple guides that are very easy to understand on EDS and HSD, but most of our patients and probably listeners are pretty well past that kind of elementary level of understanding. So the next best really is the EDS Society, you know, the conferences they put together, all the specialists that are there, it's by far the best resource to use.

So if you're not sure about what you're hearing in the online communities, you know, check through some of the resources through the EDS Society. If you still can't find those answers, don't hesitate to reach out to your primary care doctor. If your primary care doctor doesn't know the answer, then maybe they at least know someone where they can ask or maybe direct you to some of those more reliable resources. So no need to sit on something that's weighing on your mind too heavily, causing worrying anxiety. They did.

We have the world of information out there at our fingertips, but just, you know, getting the right kind of guidance on how to use it.

Linda Bluestein, MD (01:13:46.422)

Definitely and I like to end with hyper mobility hacks. So do you have any hyper mobility hacks that you can share with us?

Dacre Knight (01:13:54.275)

Yeah, so yeah, this is always a good one. So when you know, I see patients and talk to them about all the things that they've gone through, as far as the diagnostic process and treatment outcomes and things like that. And what are potential treatment options. So yeah, and just like you pulled out your heating pad there, probably the biggest hack that I've learned out from

benefit of the heat therapy, how it can be so soothing and alleviating in a way if it's muscle spasm that in increasing circulation to the area, the heat is really your friend as long as you know you're not heat intolerant from pots and things like that. So do be careful with you know extending duration in the hot showers and warm baths and things like that. But that's probably if I were to say anything and I can't take credit for it, it's just what I've learned from my patients that what they experience that I in my mind is really the safest and easiest go-to.

is the heat therapy itself.

Linda Bluestein, MD (01:14:57.482)

Sure. Okay, and last question, where can people find you online? And then I guess also, is there anything that I didn't ask you that you wish that I had asked?

Dacre Knight (01:15:09.231)

Yeah, so I am always happy to entertain questions and learn as I said, a steep learning curve as it has been and continues to be. So you can find me on Twitter on X and at KNIDAC. And there is also a website for our clinic, but that's not as much for contact, but there's a Mayo Clinic Connect website where we share research updates and things like that. So

And then there's also a website for our clinic. I don't know if you have show notes, but I can give you those if you want to add those in. Those are some pretty quick and easy links. Any questions? No, I think that we covered a lot and I'm really glad to be here because I think just getting this information out there is half the battle, you know, and then taking away what we can, what research comes up.

Linda Bluestein, MD (01:15:42.167)

Yep.

Dacre Knight (01:16:07.775)

I'd be happy to come back on anytime too if we have some ground baking research that we're ready to share. I'd love to be able to give any of those updates too anytime we can.

Linda Bluestein, MD (01:16:18.018)

I would love that. So consider that you have an open invitation anytime you have updates like that to share. Because I think we all learn differently. So even if a paper is open access, it sometimes really helps to hear from someone like yourself who was involved in the research and can really give us such a better.

understanding and I feel like that can be so beneficial and then we do link all of those things in the show notes so then they can refer to the paper also if they want. But yeah, I would love to have another conversation. Several more.

Dacre Knight (01:16:46.501)

Yeah.

Yeah, absolutely. Yeah, there's a clinical side to it, definitely, from what we're seeing and why we're doing the research. So yeah, I'd love to keep that conversation going.

Linda Bluestein, MD (01:17:00.534)

Yeah, fabulous, fabulous. Well, you have been listening to the Bendy Bodies with the Hypermobility MD podcast. And my guest today was Dr. Dacre Knight, founder of the specialty clinic for treating patients with hypermobility syndromes at Mayo Clinic, Jacksonville, Florida. Dr. Knight, thank you so very much for coming on the Bendy Bodies podcast today and sharing your incredible wisdom and knowledge with us and your experiences. And it's just so great to be able to share this information with the community.