Why Doctors Miss EDS, POTS, and MCAS with Dr. Dacre Knight

[01:12] Dr. Linda Bluestein: Welcome back, every bendy body, to the Bendy Bodies Podcast. I'm your host, Dr. Linda Bluestein, the Hypermobility MD, and today's episode marks a moment I've been working towards for a very long time. When I launched this podcast years ago, my vision was to create a space where patients and clinicians could learn together, where complex, often misunderstood conditions like Ehlers-Danlos syndromes would finally get the careful, nuanced attention they deserve. I'm incredibly excited to share that this vision has expanded in a meaningful way. Today, I'm welcoming Dr. Dacre Knight, formerly of the Mayo Clinic, who is now the Medical Director of the UVA Ehlers-Danlos Syndrome Center.
[01:43] The UVA EDS Center is officially partnering with Bendy Bodies, and Dr. Knight will be joining me as a recurring co-host twice a month. This collaboration represents a shared commitment to better diagnosis, better education, and better care for people living with EDS, HSD, POTS, mast cell disorders, and overlapping conditions. In this episode, we'll be unpacking why patients with EDS, POTS, and MCAS are so often misdiagnosed and how clinicians can shift their thinking to recognize meaningful patterns earlier. As always, this information is for educational purposes only and is not a substitute for personalized medical advice. Make sure to stay with us until the very end for our hypermobility hacks and many more conversations to come. Let's get started.
[03:09] Okay, I am so excited to be back again with Dr. Knight. How are you doing today?

[03:13] Dr. Dacre Knight: Doing well. Yes, equally excited to be here.

[03:17] Dr. Linda Bluestein: Excellent. So today we're going to talk about the challenges that people have with getting a diagnosis and why it is so hard for people with EDS, POTS, mast cell activation syndrome, etc. — what we often call the triad — why this is so hard to untangle and so challenging for both patients and clinicians really, don't you think?

[03:41] Dr. Dacre Knight: Oh yeah, totally. It really shouldn't be such a challenge. I think we can identify some of the major gaps where things fall short, why it becomes a challenge. And the reasons why it's a challenge for patients makes it a challenge for providers and vice versa. So everything we can do to educate others — which is what we're doing here — will be all to the benefit of that.

[04:08] Dr. Linda Bluestein: Absolutely. Because so many people think there's nothing that you can do, and I don't know about you, but I find that so frustrating because that's not true. There are things. And so we're going to be talking about treatment — you and I are going to have another great conversation in a couple of episodes where we're going to be talking about diagnosis and working people up and some of the big picture problems that we see. For example, I'm sure you see this also: people with EDS, POTS, mast cell related symptoms, etc., they're often labeled as complex, anxious, and/or functional. Why do you think that happens so often?

[04:50] Dr. Dacre Knight: Well, that may be one of the places where we're falling short, because it is the perception that patients with these conditions are too difficult, and providers would simply wash their hands and say, I don't want to bother even attempting to try. And as you pointed out, they may not even make a diagnosis or attempt to make a diagnosis. I hear it all the time that there's no reason to make a diagnosis because there's no cure, so what's the point? And it's just horrible.
[05:23] Truth be told, the reason why these cases become so complicated is because they've just been pushed aside and haven't been addressed head-on. And so every time that happens, it just gets more and more complicated, until someone's finally sitting with a really big complex case.

[05:40] Dr. Linda Bluestein: Exactly. I've often thought that too — that if we were able to diagnose people a lot sooner, a lot of those other sequelae wouldn't happen, or they wouldn't be as severe. And also, by the time somebody's had head pain or neck pain or abdominal pain or whatever for 16 years, it just gets so much harder to tease out. Whereas earlier on, if somebody really did try to look under the hood and look deeper, I think they would have more success. So that's why it's so great what we're doing here and why it's so great what you're doing at UVA. I think that center is going to be so helpful for so many people. It's really exciting.

[06:20] Dr. Dacre Knight: Yeah. And speaking of catching things early, I know we had a show with our partner here, Dr. Ina Stephens, a pediatrician, about why it is so critical to get these kids in who may be affected. Sooner the better, really. That's part of what primary care is too, whether it's with a pediatrician or a family doctor or an internal medicine doctor for adults — they are at the front lines of seeing patients who may be affected with any number of conditions.
[06:49] And bless them for all the things they have to consider and the differential diagnoses they have to come up with anytime they see someone who may be typically in good health but all of a sudden may not be, or it may just be a progression of symptoms getting worse. They do have a very difficult job, but that's why we want to educate as best we can anyone in primary care who may be seeing these patients so they can identify it before it gets worse.

[07:19] Dr. Linda Bluestein: And I imagine you see this as well — people who've tried Western medicine for a little while and aren't getting anywhere, so they give up on it. And I'm not saying that everyone who's not practicing Western medicine is a charlatan, but they might end up trying some pretty expensive things, some far-out there. And so I think that also complicates the picture.

[07:49] Dr. Dacre Knight: Well, why wouldn't they, right? If they're not getting anywhere and there's no progress being made, I have no misunderstanding of why someone wants to continue to care for themselves, and I give them credit for it. And unfortunately, it may be in the wrong direction, or they may end up with someone who's selling them some snake oil. But maybe they do get to the right place, or hopefully get connected to the right person who is knowledgeable. And it doesn't even necessarily have to be a clinician — it may just be someone who has had similar experiences. A lot of our patients are connected on social media and elsewhere. And yes, there are potentially a lot of issues with social media, but there can certainly be some good too.

[08:38] Dr. Linda Bluestein: Yes, there definitely can be a lot of good there. So what do you think medicine gets most wrong about the EDS, POTS, MCAS triad?

[08:47] Dr. Dacre Knight: Well, we're all learning, really. We're all in this together — to take an adage from the COVID pandemic. We are learning about these associations, more so over recent years, and I know you've had some excellent guests on this show who have talked about these. Some years ago we weren't really thinking about the connection there. So I think what we still get wrong is maybe not even fully making those associations. If a patient comes in thinking about EDS, the first thing we think about is EDS. Well, okay — but what other symptoms do you have? And it takes time: is there potentially a role for another comorbidity?
[09:36] I think where we go wrong is not really drawing those associations and not understanding exactly where they relate to each other as cause or effect. This is certainly an area ripe for research, and I'm hopeful that those answers will come with time.

[09:52] Dr. Linda Bluestein: I am too. And do you think our siloed healthcare system is part of that problem?

[09:58] Dr. Dacre Knight: Definitely part of the problem. We have silos in a couple of different ways. There are specialists who sit in those silos, and they're sometimes very hyper-specialized. As our knowledge of medicine has expanded exponentially over many years of study, there is a reason why sometimes these specialists get so hyper-focused in their field that they don't look outside the box, so to speak.
[10:35] There is good reason to have specialists and hyper-specialists — they can certainly add to the care of a patient when we need an expert on a very rare or unusual situation. But we also need clinicians who can act as a bridge and break those silos. Or maybe if those specialists themselves are willing to explore and think outside their area, they can start breaking down the silo effect as well.

[11:07] Dr. Linda Bluestein: Yeah. And I think that's something a lot of people don't necessarily understand — that things are advancing so quickly that if you're not in a certain specialty, it's hard to keep up within a narrow focus, much less something else. So to me, it's more important that you know what you do and don't know and that you know to refer somebody out, or that you're at least open-minded to these other problems and say, let's explore those with these other specialists rather than guessing. You probably see this sometimes when you're reading other people's notes and it's like, oh, I wish they hadn't even said that, because they're outside their specialty and the science can evolve so quickly.

[15:18] Dr. Dacre Knight: It is — and it's not just the science, but the technology and all the tools we have are advancing so quickly that we don't really even know how to use all of them or what to do with all of this information. But I think you're right — at the very least, we should keep these connections, keep an open mind, and keep learning and drawing connections. With time, we can start catching up, although given the way technology and information is growing, I don't know if we'll ever fully catch up. But we can do our best to help patients.

[15:18] Dr. Linda Bluestein: Definitely. And speaking of helping patients better — I don't know if you heard this statistic, but within 3 to 5 minutes of an encounter, the doctor already has the full plan in their head in most cases. They're already mentally writing whatever prescription they're going to write. And if we're in a regular conventional setting with a 10-minute visit, we can't spend an hour, obviously — but we want to make sure we're really looking for patterns, not just checking off boxes. So when you see a patient who comes in with the common things we see — pain, fatigue, GI symptoms, dizziness, brain fog, etc. — are there certain patterns in there that immediately catch your attention, and/or that you want other clinicians to be aware of?

[15:18] Dr. Dacre Knight: That's a great question, because I think that's what we're evolved to do — not only as clinicians, but as human beings — to learn patterns and make observations of the world around us. And certainly as clinicians, we do develop our own internal algorithm with time, and that's to our benefit. You're not ruminating over something for hours and hours, so you can actually help more than one patient. And because we have the experience of seeing multiple patients, there's going to be a variety of different presentations. With EDS especially, as we say, no two patients are exactly alike. So we would altogether have an algorithm of knowing when something is abnormal.
[15:18] Really, the way I approach it — and I still do this day to day — is that I just let the patient talk. And that's one of the hardest things for clinicians to do. There have been studies on this. There's that finding you mentioned about how quickly a clinician forms their diagnostic opinion — and also how soon it is before the clinician interrupts the patient. It's just a matter of minutes. And of course, once they've already created their own mental summary of what it is, it's hard to revise. But William Osler famously said, listen to the patient — they'll tell you their diagnosis. So we really just have to let them talk. That's where we start getting the best clues and recognizing patterns. Even still, we have to be careful not to be too judgmental or to jump to conclusions on any one pattern.

[15:57] Dr. Linda Bluestein: I agree. What about some of the common diagnoses that patients receive before they come see you — whether that was at Mayo or now at UVA?

[16:07] Dr. Dacre Knight: Well, the most common presenting complaints across the board — and we see this in all the studies — are pain and fatigue, in the order of over three-quarters of patients. And if you really get down to the minutia of it, fatigue, although it's very ambiguous, is probably the most common of them all. Then headaches are also very common. So as far as diagnoses go, we see a wide spread across the board.
All of the conditions in the triad you mentioned — EDS, POTS, mast cell activation syndrome — may certainly include pain and fatigue and headaches and digestive issues and all of those other very common symptoms. Hypermobility is an obvious close link to pain, as it may be a cause of pain. For digestive issues, we very commonly see IBS, functional dyspepsia, or what we now call disorders of the gut-brain interaction. For headaches, all varieties, but very commonly migraine-type, tension-type, and maybe some cervicogenic headaches when we're talking about cervical spine or musculoskeletal issues. So those stand to reason as the most common presenting issues.

[17:57] Dr. Linda Bluestein: And what about the people who have been labeled, "oh, it's just anxiety," or "it's just IBS." What are your thoughts on how that might delay meaningful care?

[18:09] Dr. Dacre Knight: I know a lot of your listeners have unfortunately had that issue of delayed care, because that is often what they're told. It's "all in their head," and then they're really at a loss. What do you do with that information? It's all in my head — okay, how do I get it out of my head? I don't even know what that means. And it's not to say — and there are studies on this — that anxiety and depressive issues might not develop with time. Certainly if someone is being brushed aside while on this diagnostic odyssey, who wouldn't have some mental fatigue from all that, and all the repercussions, whether it's anxiety or depression.
But the challenge is that if it's not addressed correctly, if we're not making the right diagnoses and not getting the right treatment and prevention started, then it's just adding to the chronicity of the illness — making it last longer and making it take even longer to improve from. It's adding to the complexity, the comorbidities that develop. So someone being told it's just anxiety — it really is mistreatment. It's malpractice in my opinion.

[19:36] Dr. Linda Bluestein: Yeah. So many of us with EDS do have anxiety — I definitely have anxiety — but that doesn't mean that anxiety explains everything I'm feeling in my body. And I don't know if you have seen this, but I feel like gaslighting has become more and more common, even with people who don't have EDS. I was at an appointment not long ago with a family member who is also a physician — a white male, so has like everything going for them, super fit, all the best possible chances of getting good quality care. And the care overall was fine. But the orthopedic surgeon literally looked at the person's knee MRI and said, "my knee's worse."

[20:25] Dr. Dacre Knight: Yeah. This kind of comment — as my grandmother would say, if you don't have anything nice to say, don't say it at all. If you don't have anything helpful to say, don't say it, because that's certainly not helpful.

[20:37] Dr. Linda Bluestein: Yeah. I was kind of floored by that. And speaking of things that people say — and this can also sometimes be misinterpreted — functional neurologic disorders. I feel like this is an area that's really challenging. There are other functional disorders too, right? Functional gut problems, etc. "Functional" is just supposed to mean related to function and not structure, so it doesn't mean "it's in your head," although it sounds that way. Is there a place for using those labels?

[21:24] Dr. Dacre Knight: I think this is a good question because it causes us to look at our medical practice introspectively — what are we doing, and what do we learn from that? As much as we know, there's still a lot of black space in the outer space of medicine, and that's usually where these functional conditions get lumped in. We haven't identified the cause yet. It's sort of a placeholder as the science improves.
[21:59] Is that good or bad? It's bad in the case where it's a misdiagnosis — it misses an opportunity to find improvements and treatment. But maybe it's at least good in one way, and I'm really stretching here, in that at least we're identifying something's wrong rather than, say, being told your knee MRI looks worse. At least we recognize that there is something wrong. That's a start. But I'm being awfully generous. Do they have a role? I think the answer is yes — there is still a role for unknowns in medicine, and there's a lot that's still unknown. We want to answer those questions and solve those mysteries as best we can, but that takes time.

[22:54] Dr. Linda Bluestein: I think you're right that some people will prematurely jump to that conclusion — "it's a functional neurologic problem" — and then not offer any treatment. I've seen, as I imagine you have, so many people labeled with that and then offered nothing. It's like, okay, what am I supposed to do with that label?

[23:16] Dr. Dacre Knight: Yeah. And those patients may be even more confused than they were to begin with.

[23:22] Dr. Linda Bluestein: Right. I did have one patient who, as I was reading through her chart, was getting progressively more challenged in her ability to walk. And so by the time I saw her, I thought maybe she was going to be in a wheelchair or something. Much to my shock, she walked into the appointment and she seemed actually quite good. So I asked her, "What happened? It sounded like you were progressing towards using a wheelchair." And she said that she was diagnosed with a functional neurologic problem and was sent to a specific physical therapist who specializes in that. And it really worked — the physical therapy really helped her regain strength. I don't know exactly how that person's training is different or what they did differently, but that was interesting, because I've seen so many other people labeled with functional problems and just left, just abandoned. But this one person got connected with care that was really beneficial for her. So that was good.

[24:21] Dr. Dacre Knight: That really is awesome to hear, and I love those stories. Not only does it give you a warm feeling and make me smile, but I think we learn from them as far as how we practice. And what I learn from that is — and you're right, we may not know exactly what this physical therapist did or the specific technique — but I would guess that they've gotten some familiarity and developed their own kind of algorithm, recognizing patterns. They probably see that patients come in with certain things already ruled out, so they don't need to waste time discussing brain MRIs or whatever, and they can get geared into treatments more quickly. They may know that some treatments work better for certain groups of patients based on their presentation. So I would venture to guess they've become well adapted to that.
And that's telling as far as the potential ahead. Just like we were talking about earlier — providers may not want to see EDS patients because they're challenging, but maybe they're challenging just because providers aren't seeing them and haven't allowed themselves the time to develop these patterns and algorithms and know what to do. I would love to share these stories with patients as well to give hope and inspiration. On this show we can probably highlight some of those stories and go through what actually made these patients better, because we certainly do want to inspire hope.

[26:08] Dr. Linda Bluestein: Yeah. Just a couple of days ago, I was seeing a client for coaching — just a virtual conversation — and I was thinking to myself, I have one patient whose story is so similar to yours, going down a very similar pathway of workup for pelvic venous disease. They had even been seen at the same clinic. So I wrote to that patient and said, would you be willing to talk to this other person? And she was like, absolutely, I'd be happy to, because she was much farther along in her journey.
[26:43] So I think you pointed out earlier about social media and how people can really use that beneficially. I've had that happen a couple of times where I thought one person could really benefit from talking to another person. Of course you have to get their permission and all of that. But in one of those cases, it was my very first patient, and I wanted her to talk to this other young person. She wrote down this beautiful thing, and I said, we should probably publish this somewhere. So we published it on KevinMD, and then that led to another thing, and that led to me ultimately interviewing her on the podcast, because I thought, wait, we should have more than just one person listen to this. So I think you're right — these kinds of stories really help us keep hope alive, which is critically important.

[27:31] Dr. Dacre Knight: Absolutely. And I know you know well — I've sent you patients who've had a long history of dance or ballet and got EDS. As we're getting hyper-specialized, there is someone who's specialized in EDS and has an understanding of dance and ballet, so I appreciate that. And those connections are certainly valuable. I've noticed the same thing you have about connecting patients.
I know the EDS Society has tried to act on this as well — health advocacy programs, patient advocacy. We have a patient advisory group that helps inform our practice. And what I've learned through working with them, and from giving talks to the EDS Society's Health Advocacy Program, is that it's very good for patients to be involved in this, as it really is almost part of their care journey. As we say in school, one of the best ways to learn something is by teaching it.
[28:42] All through medical school, we were trying to teach each other various things, and it was helpful — but it's also helpful for patients to teach others as a valuable part of their treatment experience. I'm sure we could do a full episode on social media because you're right, there's definitely some good to it and getting people connected. My ideal world is that there is some platform somehow that patients can be connected. I've got some very knowledgeable patients — I can think of many occasions where they would be excellent resources for certain other patients. And patients are eager to do this: here's my information, let me link up with anyone I can help. I'm so appreciative of that.
[29:30] It's hard though, when you go into social media — it's kind of like the Wild West. It just gets derailed so quickly. Even someone who may be as gregarious as they can be just gets sidelined. It's very difficult, but I would love to nurture that somehow. And I know what we're doing here with the show is at least trying to get the message out there. But that personal connection between others is so critical.

[30:02] Dr. Linda Bluestein: Yes, it absolutely is. And I'm glad you brought that up because I think talking about social media on a future episode together is a great idea. I also want to mention to everyone listening right now that we are going to be addressing all kinds of listener questions. Please send in the questions that you want Dr. Knight and I to answer, because we're fortunately going to be having these conversations a couple of times a month. Sometimes it'll be just the two of us, sometimes it'll be the two of us with a guest. So there will be great opportunities to answer your questions about anything. Go to bendybodiesbodcast.com, submit your questions there, and the team does a great job of bringing the really great ones to our attention. We'll try to get to as many of those as we can.

[30:52] Dr. Dacre Knight: Definitely looking forward to that. And I see it on a regular basis in my clinic — we're discussing the plan of care with patients, and when I ask, "Do you have any questions?" it's almost like deer in headlights. They know they do, but they just wait until they leave and go home and think of all the questions then. So yeah, this is a great opportunity to get those questions out there.

[31:11] Dr. Linda Bluestein: And I find that I'll sometimes answer a question a little bit differently on one day versus another, so even if it's a question that's already been addressed, hearing a different explanation — and you're probably going to explain things differently than I do — makes it worthwhile. So I'm really excited about this collaboration and getting the opportunity to hang out with you.

[31:30] Dr. Dacre Knight: Certainly. I've learned a lot already and I'm very excited to learn more and share more.

[31:35] Dr. Linda Bluestein: Great. We're going to take a quick break and when we come back, we are going to talk about what clinicians tend to miss and some takeaways for patients and clinicians.
[33:41] Okay, we're back with Dr. Knight. We're going to talk a little bit first about the triad and whether that concept is clinically helpful or whether it sometimes tends to oversimplify the reality, which tends to be even more complex than that. What is your thought about the EDS, POTS, MCAS triad?

[34:05] Dr. Dacre Knight: Well, it is an interesting triad, because on first glance we're looking at very disparate things — cardiac, neurologic, tissue and musculoskeletal, and then immune, potentially autoimmune. And how in the world could these potentially be related? But as we would also say in connective tissue disorders, you really can't rule it out — connective tissue is head to toe. So we have to think about every system.
[34:38] And when we talk about these systems, these are some of the largest systems in the body. So it's not too surprising that they may be connected. And downstream of that, they may involve other large systems — gastrointestinal, urogenital. So my initial thought is that these are wildly different but also very big and very closely connected.
[35:11] What would be the natural course of a doctor's thought on it is: how do we potentially link these up in a way that treatment can approach at least two, if not all three? That's ideal for everyone — ideal for the patient, ideal cost-wise, ideal for the provider in terms of having fewer balls to juggle. And usually that's what we're taught in medical school: Occam's razor. Don't add to plurality if you don't need to — more things are likely related to one cause than multiple. So if we can tailor treatment in a way that kills two birds with one stone, that's awesome. That's what I would like to do, and that's what I think is best for patients.

[35:59] Dr. Linda Bluestein: And I'm so glad you said that, because I think so often, people have moved away from prescribing opioids for chronic pain — but I still see some very young people on opioids, and I'm not saying they're never appropriate, sometimes they are, but so often you end up prescribing opioids and then they get constipation and nausea, so they end up getting a prescription for Zofran to offset the nausea that makes them more constipated and gives them a headache, and so now they need something else for the headache. I couldn't agree more that if we can address the root cause — whether it's the mast cell or some other process that's going on — we have such a higher chance of success. Otherwise we just keep playing whack-a-mole and keep adding things just to mitigate the damage from what we're already doing.

[36:02] Dr. Dacre Knight: Well, that is truly an internist or a generalist's biggest nightmare — you see a patient who's got a medication list that's a mile long, dozens of medications all working to counteract the effect of another medication. And that's usually our job: sitting in the middle between other specialties trying to make it all make sense, and it often doesn't.
[37:22] I think listeners may be wondering, so what is that magic that's going to treat all three of those? We don't have that magic pill yet, but there certainly are some examples. Take opioids for treating pain — all right, so we opioid to treat pain, but the negative effect is all those gastrointestinal issues, not to mention a number of others. But if we're looking at treating pain in EDS, there may be some medications that may also be useful in the case of POTS or mast cell activation, and vice versa.
[38:09] For example, take something like low-dose naltrexone, where we see overlap between at least two of those — mast cell activation and pain potentially. That's just one among other examples. Maybe this is another show too, where we talk about treatments in general. But we have to be careful with certain conditions as well — beta blockers, for example, would be first-line in many cases for POTS, but they can exacerbate asthma issues. And some other POTS treatments may make mast cell activation worse.
[38:46] So I think that's how we link it up. And we have to think about any one of these — take mast cell activation: if we're going to treat that, we think about the other systems that could be affected. We may not think it's affecting the gut, and then all of a sudden their IBS improves. So if we keep a wide net of what may be related when we're considering an approach to treatment, we can potentially target multiple outcomes or multiple conditions for a good outcome.

[39:29] Dr. Linda Bluestein: I'm so glad you brought up about people possibly thinking we know about some magic pill that they don't. We're going to talk about treatments in a couple of episodes. I agree. I love low-dose naltrexone — it's something I prescribe a lot. People have heard me talk about it a lot on the podcast for sure.
[39:47] And when it comes to prescribing, and you talk about the internist's nightmare of so many different medications — the deprescribing, the weaning off and stopping things — we don't get training in that. I remember one of my guests quite a few episodes ago, Dr. Heather Tick, who asked me: how many lectures do you remember from medical school on deprescribing?

[40:13] Dr. Dacre Knight: None. Yeah. And there's not a lot of investigation into it either. A lot of the trials and studies are into the effects and benefits of a medication — not "what do we do now that it's no longer needed?" What's the use of a trial stopping a medication for a pharmaceutical company? They don't want you to stop it. They want you to keep going.

[40:43] Dr. Linda Bluestein: Right. We talked about this a little bit earlier, but when it comes to conditions that can commonly masquerade as one part of the triad, are there certain things that come to mind?

[40:53] Dr. Dacre Knight: Yeah. I think a lot of these things do overlap, and the first thing that comes to mind is that we have to stick with first principles — what we have at our easiest disposal of guidelines and criteria that are already established. They're not perfect by any means, but they're there as a starting point. So this is when we go through diagnostic classifications and criteria, whether it's hEDS, HSD, or criteria for IBS or for POTS and things like that. They give us a starting point for what may be truly involved.
[41:41] Having said that, keeping the best interests of the patient front and center, I will still consider potential treatments for someone if they're half a point off the scale of a criteria. This is where the art of medicine comes into play. We just have to use our best clinical judgment — and the best clinicians have good judgment about how far to go outside the borders and how to account for the differences between one patient and the next, because there may be a lot of differences. There are certainly a lot of differences between patients when this criteria is being developed to begin with. That's what makes it a little cloudy. But it just goes back to keeping an open mind about what someone may be presenting with and what may actually benefit them.

[42:39] Dr. Linda Bluestein: We know that it's so common for people to come in with a review of systems that's positive in multiple different systems. How do you decide what to address first?

[42:51] Dr. Dacre Knight: This is a very good question, and it was actually asked at one of the EDS conferences a couple of years ago — I gave a whole talk on it, on how do you prioritize symptoms.
[43:06] Really the first way is whether it's emergent or non-emergent. Is this something we need to address today, tomorrow, or maybe even within hours? It may not even be something the patient is aware is urgent. We don't see those situations so often, and usually it's because our patients are pretty well educated about when something is emergent — they seek urgent help and get to the emergency room or wherever they need to go.
[43:42] But the next step beyond that is to ask: what is impacting you the most? This is a chronic condition, so what is affecting you from doing the things that you enjoy doing, or that you previously enjoyed doing? And you can get any number of answers related to different things — it may be sleep, or back pain, or headache. And then we go down a rabbit hole a little bit, talking about the characteristics of that headache, when it happens. But that's where I would say to start: what is the most pressing concern? What is the biggest issue? That may actually tease out other things too, but let's start there.

[44:45] Dr. Linda Bluestein: And in terms of what clinicians tend to miss — what are some subtle red flags that should prompt clinicians to look deeper?

[44:53] Dr. Dacre Knight: I love the description of red flags, although when I was first in medical school learning about them, they were related to back pain, and it stuck with me. Now that I've come to recognize red flags for other conditions, I've got so many red flags that I need to start coming up with other names for them. But they are the things we'd be concerned about that could impact someone's life the most — whether it's an emergency or increased morbidity or mortality.
[45:34] When we talk about EDS, POTS, or mast cell, they've all got their own red flags. For mast cell, that would be anaphylaxis. For POTS, it would be arrhythmias that can be fatal. And for EDS, usually what we're talking about are genetic red flags — where there may be concern for something identifiable with genetic testing, something that may be inherited and potentially passed on among other family members who may want to know as well. And that may be related to different treatment planning as far as surveillance with imaging and other testing that may be useful to avoid further complications.

[46:19] Dr. Linda Bluestein: And you're right about the abundance of red flags. So maybe a yellow flag might be the saying, "if you can't connect the issues, think connective tissues." When you have somebody sitting in front of you reporting problems in multiple different systems — and you mentioned Occam's razor — what could possibly explain the bulk of these symptoms? It has to be something that's system-wide. Connective tissue, EDS or HSD, could explain system-wide problems. Dysautonomia is clearly system-wide — it controls your cardiovascular system, your GI, etc. And mast cells we know are everywhere as well. So I think that's another piece of it.

[47:13] Dr. Dacre Knight: Yeah, I'll just add to that — I think that's absolutely right. And to the benefit of patients, treating each of those that we can, even though they may have different treatments. That's something to keep in mind.
[47:35] And I'll venture to say that, as much as we can do to try to narrow it down, there is — and we used to love to challenge each other in residency with this — while Occam's razor says narrow it down, there's also Hickam's dictum, which says a patient can have as many problems as they please. And it's true. So as much as we want to link EDS and POTS and mast cell, and maybe there is yet a single common pathway where one is causing the other and we just haven't recognized it yet, we still need to recognize that all of those may potentially be present and may in some ways have different treatments. We need to address all those things we can to provide the best treatment to patients.

[48:34] Dr. Linda Bluestein: I'm glad you pointed that out, because that is definitely something that happens quite commonly — once someone does have the EDS diagnosis, or even if they just suspect it, they start writing everything off to that. But you're right: they're entitled to other diseases too. They can definitely have other things going on. So that's an excellent point. Thank you for bringing that up.
[48:53] And I will link in the show notes the red flags document that I have on the Bendy Bodies Podcast website, so people can see the things that should really make us think about doing genetic testing for some of the more rare types. I'm really glad you pointed that out as well.
[49:09] And speaking of labs — why are normal labs and normal or unremarkable imaging results so challenging?

[49:23] Dr. Dacre Knight: Let's start from the beginning of why we might order imaging or labs to begin with. The obvious answer is because we don't know what's going on. Some of the most common situations where we don't know, like I mentioned — the most common presenting complaints of pain or fatigue — can certainly warrant imaging and lab tests.
[49:51] To answer your question, why labs and imaging may be confusing: there is no specific lab test that can tell us someone is in pain. We can't justify someone's pain with a lab test. So it catches patients in a difficult situation where they may have normal lab tests and are told, well, you don't have any reason for your pain. And how much worse can you get as far as trying to get any clarity on what you're suffering from? That's the first issue and probably the most common for our patients.
[50:33] The next issue is that if there are some lab abnormalities — okay, that's good if we can identify a diagnosis clearly, like an autoimmune disease such as rheumatoid arthritis, which we can diagnose with imaging and lab tests and get on some very good treatments nowadays to decrease the duration of symptoms. But there's also the possibility that these lab tests are abnormal in a way we'd call a false positive, and that leads us down the wrong path of treatments, just delaying things, costing more, and ultimately not serving the patient well.
[51:24] So again, back to the art of medicine — we have to think about the condition the patient is in, what they're presenting with, and then what are these labs actually going to tell us or not tell us, and use our best judgment about what may be useful.
One quick example — I could talk about this all day, because it's what I do every day. One of the screening tests we use for autoimmune diseases is the ANA, the antinuclear antibody. It's a very good screening test. As a screening test, we want to catch all those potential cases that we can. That doesn't mean it has high specificity for any one disease — it just means we're less likely to miss something. We want to not miss. But because it may not be very specific, and ANA is one of those, it can lead to false positives. A lot of patients will say, "My ANA is abnormal," and they get concerned about it. And it's like, well, that's the reason why we do the other autoimmune tests — for lupus, rheumatoid arthritis, Sjögren's, and so forth — because the ANA can only get us so far. It sort of just opens the door, but it often does add to extra anxiety. And that's certainly part of the complication of imaging and lab tests — abnormal results will bring patient anxiety, so we have to be ready to answer questions that may come up related to those.

[53:13] Dr. Linda Bluestein: Yeah, definitely. And we can't see someone's pain on imaging. My mother's hands before she passed — she had horrible arthritis, and she actually had less pain in her hands than I have in mine, even though the imaging looked far worse. The imaging does not tell that part of the story, and that can be really challenging for people.
[53:37] I had a patient come see me once who traveled a pretty good distance. She knew she probably wasn't hypermobile — I wasn't worried about a connective tissue disorder for her — but she had chronic pain. And basically all I did was look at her imaging reports with her. I didn't even have the actual images, but I said, you have plenty of reasons to have pain. And she started to cry and said, "I do?" And I said, yeah, you have all these things going on in your body. Nobody had told her that. So I think oftentimes, as you said, words matter a lot and we should pause and think before we speak.
[54:25] Speaking of pausing and thinking before we speak — we want to give some takeaways for both clinicians and patients. Are there maybe three questions that every clinician should ask when a patient presents with symptoms spanning multiple systems?

[54:43] Dr. Dacre Knight: Yes, there are. And just to think about your patient example there too — I presume in her case she had been told that everything was okay. Is that right?

[55:01] Dr. Linda Bluestein: Yep.

[55:01] Dr. Dacre Knight: And yet she was still trying to emphasize that something's not right.

[55:04] Dr. Linda Bluestein: Right. And I think what happened was, probably there was nothing surgical. This has happened to me too — you know, if there's nothing surgical, some orthopedic surgeons in particular might imply or say, you're fine. But it's like, no, you have pathology there that explains your pain, there's just nothing for me to operate on.

[55:05] Dr. Dacre Knight: Yeah, exactly. I encounter that all the time too. Different specialties have different approaches as far as what they will and won't see. And sometimes surgeons will see patients for conditions that may not need surgery but that they're knowledgeable about — and that's ideal, because surgeons obviously have a lot of knowledge and experience treating not just surgically but knowing about the conditions they treat.
[55:54] As a starting point, a clinician could at least ask — as I mentioned earlier, letting the patient talk — what is the most bothersome thing to you? That's a starting point. It's not going to get them all the way there, as you just pointed out, because the patient told them that and it didn't really go anywhere. But for a clinician at least to grasp what is really front and center — beyond that, we have the whole mnemonic we call SOCRATES: the site, onset, character, radiation, associations, timing, exacerbating and relieving factors, severity. They can navigate from the presenting complaint to get more characteristic features.
[56:51] But I think the next thing a clinician would do well to keep in mind is an understanding of what the patient has gone through to get to them. That's very telling. And in the case of our patients who have gone through so much, I mean, that's an answer as clear as day that there's something unusual here, I can't use my standard algorithm, I've probably got to think a little more creatively. Those two questions are at least a very good starting point: what is most bothersome, and what have you done or been through to get here?
And then, to keep the patient's experience front and center and keep their goals of care in mind — as we talk about shared decision-making, where we're moving away from a paternalistic approach of "I'm the doctor, I'm telling you what to do" — the next question relates to that: what are your goals of care? What do you want to see improve? I think that's a good basic approach, though there are obviously hundreds of questions that can be tied to any of those.

[58:37] Dr. Linda Bluestein: And for patients, how can they describe their symptoms in a way that clinicians are most likely to listen and understand?

[58:45] Dr. Dacre Knight: That's another excellent question — and something that gives patients a lot of hesitation and nervousness about seeing doctors to begin with, through no fault of their own, because they've likely been on a long journey and possibly been misguided and misdirected.
[59:11] I think the best starting point is to have an understanding ahead of the visit: here are the 1, 2, 3 specific items I want to make sure I address. I have patients who come in with notes on their phone or written out, and I love it. It gives me the sense that at least they'll have peace of mind that we covered those things, even if they can't think of all the questions at the time of the visit — at least they get those out there. I highly encourage that. We don't need to burn the midnight oil over it, but at least jot some notes down. It really does serve you well.

[59:49] Dr. Linda Bluestein: Well, this has been a great conversation. As you probably know, I like to end every episode with a hypermobility hack. Do you happen to have a hack to share with us? If not, I have one for clinicians.

[1:00:08] Dr. Dacre Knight: Well, my last one was to trust your gut. So I don't want to use that more than I should. I'd love to hear your hack.

[1:00:21] Dr. Linda Bluestein: The funny thing is, your hack and my hack are very closely related.

[1:00:27] Dr. Dacre Knight: Yeah.

[1:00:29] Dr. Linda Bluestein: So my hack is for clinicians. When you're seeing a patient and you've gone through listening and trying to get a really good sense of what's going on, I think it's very reasonable to ask the patient: what do you think is going on?

[1:00:47] Dr. Dacre Knight: Absolutely. I love it. And I think they may be surprised — they may have a more informed answer than they expected.

[1:00:55] Dr. Linda Bluestein: Right. And I had one of my doctors, a neurosurgeon, ask me once: what are you most afraid of, or what are you most worried about? And I think that's a great follow-up question to "what do you think is going on?"

[1:01:10] Dr. Dacre Knight: Right. And that probably leads into a better understanding for them of what the patient wants to get out of their care. We can do that in a shared decision model, and it's best for everyone.

[1:01:25] Dr. Linda Bluestein: Absolutely. And when you do that — allowing the person to verbalize what they're most afraid of — it allows us to address it, at least. And like you said, when they come in with that list of questions, we know we're not going to cure people. It's not like when they leave they're going to be pain-free. The goal is to improve their quality of life and their functional capacity. But it's really helpful when they come in with those questions, and if as the clinician we've asked those two questions, that really gives the person the opportunity to air that.
[1:01:58] And they might be afraid of something that we can actually say, I feel very confident that that's not the case. For example, I'm sure you've had this happen a lot — they're really afraid they have vascular EDS, and they might be 70 years old with no signs at all of vascular EDS. And so we can reassure them: this does not look at all like vascular EDS, plus you're 70 years old — by now we would really have a good idea about that.

[1:02:24] Dr. Dacre Knight: We can see that. But that's a great example where just being of service to the patients — listening and answering questions — is a huge part of what we do. And that's a very simple thing to do.

[1:02:39] Dr. Linda Bluestein: Yes. And we should be giving them at least that. Well, this was such a great conversation. I loved getting to spend the time with you. And before we go, can you just tell people where they can find you and where they can learn about the UVA EDS Center?

[1:02:56] Dr. Dacre Knight: I would love to have that out there. We will obviously be on this podcast and all the listening platforms it's available on, so look forward to staying connected there. As far as the UVA Health Center, we have a website — and make sure those links are available in the notes — and also a phone number. We take self-referrals and we help answer questions and do what we can to see patients when that's available.

[1:03:27] Dr. Linda Bluestein: Thank you so much for taking the time to chat with me. I know that you're very busy. This is an exciting time over at UVA, and I just can't wait to see how the center evolves. I love having these conversations with you, so I look forward to the next one.

[1:03:39] Dr. Dacre Knight: I enjoyed it thoroughly as well. Looking forward to those.

[1:04:41] Dr. Linda Bluestein: I'm so excited about having Dr. Knight here twice a month to co-host episodes with me and have these great conversations. Please do remember to submit your questions to bendybodiesbodcast.com because we will be addressing as many of those questions as we can. I really look forward to seeing what you want to hear us talk about.
[1:05:00] Thank you so much for listening to this week's episode of the Bendy Bodies Podcast. If you'd like to go deeper, I share additional education, clinical insights, and resources in my newsletter The Bendy Bulletin, which you can find on Substack at hypermobilitymd.substack.com. You can also help us spread the word about connective tissue disorders by leaving a review, sharing this episode, or sending it to someone who needs it. These small actions truly make a difference in raising awareness about conditions that are still widely misunderstood.
[1:05:28] And don't forget — full video episodes are available every week on YouTube at Bendy Bodies Podcast. As many of you know, I offer one-on-one coaching and mentorship for both individuals living with connective tissue disorders and people caring for them. You can learn more about these options on the services page at hypermobilitymd.com.
[1:05:41] You can find me, Dr. Linda Bluestein, on Instagram, Facebook, TikTok, X, and LinkedIn, all at Hypermobility MD. As part of our collaboration with the UVA Ehlers-Danlos Syndrome Center, we also want to share some of their helpful resources. For questions or appointment inquiries, you can contact the UVA EDS Center at [email protected]. Again, that's the letter R as in Robert, UVA EDS Center at uvahealth.org. You can find answers to common questions at uvahealth.com/support/eds/FAQ.
[1:06:22] Our incredible production team is Human Content. You can find them on TikTok and Instagram @humancontentpods. As you know, we love bringing on guests with unique perspectives to share. However, these unscripted discussions do not reflect the views or opinions held by me or the Bendy Bodies team. Although we may share healthcare perspectives on the podcast, no statements made on Bendy Bodies should be considered medical advice. Please always consult a qualified healthcare provider regarding your own care.
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