Good evening and welcome everyone. My name is Jordan Burkhardt. I'm the Director of Member Engagement at AAPM&R, and I have a couple of housekeeping slides to go over before we begin tonight's session. So the views expressed during this session are those of the individual presenters and participants that do not necessarily reflect the positions of AAPM&R. AAPM&R is committed to maintaining a respectful, inclusive, and safe environment in accordance with our core values, principles of inclusion and engagement, code of conduct, anti-harassment policy, and all participants are expected to engage professionally and constructively. AAPM&R does reserve the right to intervene or remove individuals who do not follow these expectations. And then this activity is being recorded and will be made available on the Academy's online learning portal. An email will be sent after this activity with a link to bring you to the recording and an evaluation. For the best attendee experience during this activity, please mute your microphone when you're not speaking. And you are invited and encouraged to keep your camera on if you'd like and select hide non-video participants. So this will ensure that speakers are prominent on screen. If you have a question, either raise your hand and I will call upon you when the time comes for Q&A or you can use the chat feature and I will facilitate the Q&A with the chat as well. And the time may not permit the panel to field every question. So with that, I welcome everyone to the Hypermobility Member May session and I will turn it over to Dr. Rudin. Hello and good evening. I'm Dr. Nathan Rudin. I'm a professor of PM&R at the University of Wisconsin School of Medicine and Public Health. And it's wonderful to be here along with my colleagues, Drs. Neha Charan and Isabel Huang. Here to talk about hypermobility syndromes and trying to unravel some of the complexity that is involved in the epidemiology and their diagnosis and the management of hypermobile individuals. So I will go ahead and put up some slides to get us going here. Here we go. And so everyone should now be able to see the slide program. Looks great. Okay, fantastic. So first of all, I do not have any conflicts of interest, commercial relationships or even financial acumen to disclose this evening. I'd like to thank Dr. Jennifer Hankinson, who was supposed to be moderating our session tonight, but unfortunately was unable to make it due to a last minute commitment. So that leaves the three of us winging it a bit. And what we hope to do is get through the content that we've prepared and then in whatever time remains, make sure that we have plenty of time for questions and answers. So we look forward to hearing from you all. Okay, I'm just gonna see if I can get my controls to work. All right, so the objectives this evening. We're going to be a little tighter than the originally presented objectives just because it didn't appear that we had enough time in the session to cover everything that Dr. Hankinson laid out. We are hoping to do a follow-up session next month to give you the rest. But to start with, we want to make sure to educate our attendees about the various kinds and presentations of hypermobility syndromes and provide some insight into what sorts of diagnostic challenges occur and highlight the significance of a multidisciplinary and individualized and patient-centered approach to care for these individuals. Gosh, sounds a lot like what PM&R is good at in the first place. So there's a very common scenario that we see in our clinics and it's seen all over the place. Primary care, orthopedic, sports medicine, pediatrics. A young to middle-aged person, more often but not exclusively female, with chronic joint and muscle pain and chronic fatigue. And these individuals may report joint subluxations or dislocations, clicking and popping. These folks tend to have a soundtrack as they walk around. They make a lot of noise. Rolling or spraining the ankles frequently. Back pain often associated with scoliosis. Anterior knee pain often with lateral subluxations of the patella with relatively minor activity. Knee ligament injuries sometimes at rates unusual for people in the age group we're talking about. Pain in the temporomandibular joints. Locking and popping in the hips often with pain. And these are folks who have had quite a diagnostic journey and may already carry diagnoses of fibromyalgia or mixed connective tissue disease or seronegative rheumatoid arthritis. These are folks who might have a single slightly elevated inflammatory marker but don't have the frank presentation of somebody with rheumatoid arthritis. And in all cases, a history of unusual joint flexibility. And not everybody will just say, hey, I'm hypermobile. Some of them you actually have to ask or you have to assess them and see what is there. And they may say, gosh, you know, I never really thought about that before. It does sometimes happen. There are historical features that go far beyond, though, the initial presentation. These folks report pain and tenderness, often very widespread, hence that fibromyalgia diagnosis. They may report lightheadedness and fainting with orthostatic intolerance, trouble with dizziness on standing, and tachycardia, often worse withstanding. GI problems, constipation and or diarrhea, often already diagnosed as functional bowel syndrome or irritable bowel. Although some people do far worse. They have abdominal pain and bloating and nausea. And some people have frank GI dysmotility that's been objectively documented. Headaches are extremely common. Anxiety, depression and attention deficit hyperactivity disorder are very common. And some people will describe out of nowhere episodes of flushing or rashes or swelling or itching that don't seem to match up with a particular trigger. And sometimes they've gone to allergists and been told, well, this isn't an allergy. Your patch tests are normal. We don't know what this is. So it's a complex group of individuals. And what we want our PM and our colleagues and our medical colleagues in general to start doing is asking this question. When you're starting to see all these tools line up, could this person have an inherited hypermobility disorder? And this is important because. First of all, being hypermobile affects lots of aspects of medical and surgical care. Your perioperative precautions, how you close wounds, how you tie tissues together are affected just as much as the medical side of things. Hypermobility is really common, as we'll talk about, but not well recognized. And we haven't really been teaching physicians to teach this supposedly rare condition. And for that reason, people have what's been called in one article a diagnostic odyssey where they will roam the medical seas from specialist to specialist and they just keep getting bounced from one place to another. They wait an average of more than 10 years for diagnosis of their condition, which often also delays definitive treatment. So this is often a frustrated and frequently self-educated bunch of individuals. Many of them are going to turn out to have an Ehlers-Danlos syndrome. So these are a heterogeneous group of inherited connective tissue disorders, and they are all completely different. There were 14 defined at last count. They're all characterized by hypermobile joints, plus evidence of tissue fragility, which usually includes hyperextensible skin. And as far as we can tell, these are disorders of collagen or associated molecules, and there were 14 of them, 13 with known genetic causes. And of these 14, 13 of them are really pretty rare, with classical type 1 in 20,000 to 40,000 individuals, with vascular type 1 in 100,000 to 200,000 individuals, all the way to AEBP1 related, which is an autosomal recessive and running in about the one in a million range at this point. Really, really rare, but hypermobile type isn't rare at all. In fact, the prevalence is as high as one in 500 in one recent study that was done in Wales, and some people think it may even be higher than that. The prevalence of hypermobility with pain in the general population is thought to be as high as 3%, and hypermobility without pain or disability as high as 10%, with some college age surveys showing rates of between 12 and 28% hypermobile. There's a lot of it out there. But the people who've been diagnosed with hypermobile EDS, one in 500 or perhaps more. Now, while this is the most common EDS, it is also the least scary EDS in that the morbidity is somewhat less than in the other EDSs, but can still be awfully significant. There's a really broad range of outcomes with these individuals, all the way from quite functional and employed and doing well to extremely disabled. Hypermobile EDS doesn't alter fertility, but early labor is possible, and there are increased rates of some other pregnancy complications. It doesn't generally shorten the lifespan, although some people with more severe comorbidities can have shorter lives on that basis. And the molecular basis of hypermobile EDS is currently unknown. We don't know what's causing it. So unlike all the other EDSs where you can send off a genetic screen, we don't have one for this. So the only way to diagnose it is good old H&P, history and physical. It's what they look like and what they tell us. Now, markers are under investigation. The Ehlers-Danlos Society has a large whole exome sequencing study, which should be producing results within the next year or so. And there have been some other very interesting projects coming out of Medical University of South Carolina and elsewhere. But right now, still an unknown, so still a clinically diagnosed entity. Now, just to make things more interesting, there are the people who don't have one of the other 13 EDSs, but don't quite fit hypermobile EDS criteria either. These are folks who are considered to have hypermobility spectrum disorders. They don't quite meet the mark for hypermobile EDS. And they're divided up into generalized hypermobility spectrum disorder with a Bayton hypermobility score of five or more. And then there were people with more localized hypermobility, primarily peripheral and even historical people who, yeah, they were a lot stretchier. But now they're 50, 60, 70, and they're not as stretchy anymore. It's not yet clear whether these disorders are going to stick around. Generally, they're characterized by fewer non-articular features than hypermobile EDS. But the inheritance and the comorbidities and the treatment are the same as for hypermobile EDS. And in the end, generalized HSD may turn out to be the same condition as hypermobile EDS. For example, if you take tissue from both populations and look at the dermal fibroblasts, you see identical pathology in both conditions, where while the structural elements of the extracellular matrix appear to be correctly transcribed, the organization of those elements is completely messed up. It's disorganized tissue, as if you tried to put an erector set together with elastic bands instead of nuts and bolts. Things are not arranged properly and the tensile strength is not there. And both these diagnostic groups have the same pathology under the microscope, suggesting we may not really be looking at two things after all. Anyway, we talk about diagnostic challenges. Why does it take years to get diagnosed with this? Well, the journey can often go like this. First of all, we physicians were all taught in medical school that the EDSs are rare conditions. You're not going to see them. If you see it every once in a while, it might be that zebra once in a while in your career. You're going to send them to the geneticist. Wrong. We now know this is a very common problem. It's not rare. Then you'll have a patient come to a doctor and they will say, well, I'm not a geneticist. So I don't think I should diagnose this inherited disorder. But you diagnose this by history and physical. You don't diagnose it genetically. So that doesn't necessarily hold water. You don't need a geneticist. And then, of course, there's the entanglement of the presentation with fibromyalgia, widespread pain, with anxiety, with depression, with ADHD, which makes things more complicated. And people who aren't familiar with the EDSs as entities may not be able to do that untangling. Often there are assumptions of psychopathology and or symptom magnification. A lot of patients talk about medical gaslighting or medical trauma because they have been told repeatedly, this is all in your head. This is not real. Go away. Go to psychiatry. Leave us alone. But the fact is, they've got real physical problems. And so people get referred from specialist to specialist, and they keep getting the same responses over and over again until they finally find somebody who is willing to say, hey, looks like you probably have this. And even then, they will often say, but since I'm not a geneticist, I'm not going to make the call. The fact is, making the call doesn't have to be that hard. Now, why is this PMNR problem anyway? I mean, why us? Well, first of all, the geneticists have told us pretty clearly, there's not enough of us to go around. We get that. But also, while they're great at diagnosing things, they don't do a lot of treating. But hypermobile EDS and the HSDs are common multi-system conditions that cause significant impairment and disability. Gee, the primary problems, what are they? Musculoskeletal pain and joint problems, peripheral nerve problems and pain conditions, deconditioning, and exercise intolerance. The very bunch of stuff that physiatrists are trained to take care of. Because these patients wind up needing customized rehabilitation programs, coordinated care with an interdisciplinary team. They may need electrodiagnostic testing. They may need injections to treat various kinds of problems. Gosh, all things that we have right in our wheelhouse. And it is certainly within our capacity to diagnose these individuals and to begin effective treatment. So I'd argue that we should learn to manage these individuals just as we do our patients with spinal cord injury and stroke and brain trauma and neurodevelopmental disease and musculoskeletal disorders. Because we're equipped for it in ways that many others are not. How do we diagnose the condition? Now, I apologize for the small print on this picture, particularly if, God help you, you're watching this on a phone. I will be testing you on every item on this list later. Not really, but there are international consensus criteria for hypermobile EDS, as well as for the other Ehlers-Danlos syndromes. And the last version of these was produced by an international consortium and published on St. Patrick's Day, 2017. A new set is due out in 2026. For hypermobile EDS, you need to take a detailed history and you do a focused examination. You can find these criteria easily at the Ehlers-Danlos Society's website, which is ehlersdanlos.com, not ehlersdanlos.org. That takes you to another site. If these folks are hypermobile, but don't meet the criteria, they either have a different EDS or a different disorder or an HSD. But if it looks like something beyond HEDS or an HSD, it may be time to refer to genetics. Now, one of the challenges is that these are consensus-based criteria. They are somewhat flawed and a certain liberality of interpretation may be needed. In particular, one of the criteria is that if there's a positive family history, one or more first-degree relatives should have independently met the current criteria. Well, if you're the first patient in the family to come into the office to be assessed, you're not gonna be able to meet that criterion. So generally, if somebody says, yeah, you know, my mom and my sister can put their feet behind their head and tie themselves in knots, I'm gonna give them the point. But there's a lot of variation there. Not everybody agrees with me. But the bottom line is these folks will demonstrate hypermobility as well as a number of non-articular features, both in history and on physical exam. The biggest piece of the exam and one that's pretty easy to do is the Baton scoring system. And for those of you who aren't familiar, this is a nine-point scale, four bilateral sites, one midline, which is the spine. So first of all, can patients extend the fifth metacarpophalangeal joint from 90 degrees or more? That's one point per hand. Can they get their thumb to their flexor forearm? If that's the case, it's one point per thumb. Can they hyperextend their elbows more than 10 degrees? That's one point per elbow. Now we're up to six. If they're standing up, can they hyperextend their knees 10 degrees or more? That's another two points. And lastly, can they bend forward at the waist with their knees still straight and get their hands flat on the floor? That's the ninth point. You always gotta make sure they're not cheating and bending their knees when you check this one. So the maximum Baton score is nine. And with these criteria, six or more is for pre-pubertal children and adolescents, five or more for men and women age 50, four or more for men or women over age 50. There are other scoring systems as well, but that's the one that's used in the criteria. Now there are a lot of other features you can look at. And as physiatrists, we like to do thorough neuromuscular examinations. Other things to look at are toes. You may see hyperextensibility of the first MTP, for example, often with premature development of osteoarthritis. You'll see 35-year-olds with bunions and hallux valgus with this disorder. Ankle instability, patellofemoral instability. Remember we talked about subluxation, kneecaps being off track. You may see patella alta as well. Increased hip range of motion with hip impingement signs, such as the fader maneuver, for example. And increased shoulder mobility, AP glide of the humeral head with respect to the glenoid, increased internal or external rotation. These are the folks who can wash their entire back without needing a long brush. And for the women, they can zip their own dress, no problem. And many of them are surprised that there are people out there who actually can't do it. There are other things to look for as well, such as hyperextensibility of the skin, translucency of the skin. I'll often do what I call a diagnostic handshake. I'll take their hand and if the skin is soft and the joints feel kind of loose, it's a clue. Then you look at arms and legs, you see that translucency. You pull on the skin a bit and see some hyperextensibility. It's like, hey, they've probably got it. So a lot of people have Frank Livido reticularis. There may be cool purple hands and feet. Many of them have renal phenomenon. Don't try this particular thumb maneuver at home. I nearly fell out of my chair the first time I saw it. Many folks have piezogenic papules on their heels, which is the normal fat poking through the heel fascia under the weight when they're standing up. Many people don't know they have them because who looks at the back of their heels when they're standing? Then long fingers, we have some people with Frank arachnodactyly. And those folks will go ahead, check an arm span, check the eyes, check an echocardiogram, look for Marfan syndrome. Flexible flat feet, incredibly common. Wide atrophic hypopigmented scarring is common. Easy bruising is very common. And for those unfamiliar with the Ehlers-Danlos community, all those zebras are because they've pretty much adopted the zebra as their mascot because they really do feel like the folks who have that condition that nobody is looking for, everyone's looking for the horse when they hear the hoof beats and they get overlooked. So you'll see zebra crutches, zebra clothes. These folks really identify. Now, one thing we did at my institution is develop an algorithm for the evaluation and initial assessment of these individuals. It is a consensus-based but also evidence-based guideline. And it is available at the UW Health website to healthcare providers and networks. UW Health doesn't really want to disseminate it widely among patients, but they're perfectly fine with healthcare providers getting hold of it. So you can find it at this website and the recording will give you the details of how to search for it, or you can email me for a copy if you wish. But what it does is take you through the diagnostic criteria and give you clues at various stopping points along the way. We also developed an online EPIC pathway version of this, which has seen increasing use in our institution with 409 uses of the pathway in the first year of operation, which is not bad considering that that's about 25% of the diagnosis-specific opportunities to use it. So some of the comorbidities of note, and we'll probably have some Q&A about this as we go. Lots of these folks have POTS, postural orthostatic tachycardia, and orthostatic hypotension. And we recommend hydration, electrolyte supplementation, and compression wear. Cardiology referral also comes in handy. Irritable bowel syndrome with constipation and dysmotility. Dietary changes can be quite helpful. Probiotics can be helpful. They are referred to GI if things are getting beyond the ability to control symptomatically. Flushing, rashes, pruritus, and urticaria. Some of these folks have mast cell hyperfunction disorders, which can be evaluated with serologic testing. But sometimes if we really think that's what's going on, we'll do empiric, cetirizine, and famotidine, and get them to allergy. And affective disorders, as I mentioned, are very common. Psychology and psychiatry assistance come in very handy. So we consider other conditions besides the ones we've mentioned, the hypermobile EDS and HSDs, if the skin is very, very lax, if there's recurrent skin tearing or poor healing, if there are periarticular subcutaneous nodules, which are a hallmark of classical EDS, if there's a cardiac murmur, if there are severe varicose veins, if there's a personal or family history of aneurysm, that's really gonna kick up our suspicion for other conditions. If there are histories of valvular insufficiency or prolapse, there's severe kyphoscoliosis, if there's marfanoid habitus, or if there's a history of bowel or uterine rupture. Plus recurrent pneumothorax is also something we'll see. For those individuals, we will do more workup. We will send them to genetics. We will send them to vascular surgery. We'll make sure that we're not missing one of the more serious EDS conditions, which can have very high comorbidity. So here's my friend, Bucky Badger, our fine mascot before and after treatment for his underlying EDS. You may not know that Bucky has to do one pushup for every point earned by the football team at every football game. And man, that shoulder instability really gave him a lot of trouble. But with appropriate treatment, we can get him going again, and you can do the same with your patients. But hypermobility, remember, is common. It's not hard to recognize when you know where to look, and it can connect seemingly unrelated symptoms. The diagnosis can be tremendously validating for the patients. I have a lot of tissues in the office because often these are folks who have been looking for answers to what is going on with them for 10, 20, 25 years. And when they're finally told, yeah, you know, you actually make sense. This all does tie together in a way that we can understand and treat. It is a tremendous milestone for them and very, very helpful. And treatment can be truly life-altering. And you guys can do it. And I hope that you will. I hope that you'll join us as we continue to work with this population. I'm gonna stop my screen sharing here now. And I would like to turn the podium over to my colleagues. Now we have Dr. Naya Charon there. Looks like she has unmuted. Coming to us from Penn State, I'm gonna turn it over to you. Can you all hear me? And to Dr. Isabel Huang of UT Southwestern. Hi, everybody. Can you all see my screen okay? Looks great. Awesome. Well, I'm Naya Charon. I'm an assistant professor here at Penn State Health. I'm Isabel Huang. I'm also an assistant professor at University of Texas Southwestern in Dallas, Texas. And we're going to be talking together about the second half of our talk here. And I really appreciate you all staying awake. If any of you are on the East Coast, it's kind of late, so thank you. All right, here we go. So we have no disclosures. And I wanted to start with this quote, and it's a quote about fear. So the oldest and strongest emotion of mankind is fear. And the oldest and strongest kind of fear is the fear of unknown. And I feel like that quote translates so well to the world of EDS. When we see a patient that's too bendy or a referral that is too complex or too confusing, it really invokes fear. And we as clinicians panic, right? And we start to look like these ladies over here, really scared, we don't know what we're looking at, and we're just uncomfortable. But fear is also a catalyst. It's a catalyst for personal and professional growth. It allows you to step out of your comfort zone. It allows you to build resilience and confidence, right? And so I really hope by the end of this talk that you all will be looking more like these people over here who are intrigued, excited, and don't shy away from something that is just not as common. So our objectives, I know Dr. Rudin went over, but from our perspective, what we really wanted to hone in to today was talking about how to make you feel like you're not alone as clinicians and as providers. We wanna help you feel like you're a part of a group and you're not tackling this by yourself. So I'm going to start with just a little bit of a story on how I got into EDS. I started receiving consults when I first joined at Penn State and I was newly out of residency. And so I got everything nobody else wanted, right? Because I had to fill my clinic. And I got a lot of consults for musculoskeletal pain issues including EDS would pop in there. And I really didn't know much about it. And like Dr. Rudin said, I learned about it, I don't know, in fleeting in residency. But what I do remember learning during residency was that this is the issue that you don't wanna treat. It's the new fibromyalgia. So if you see it, run away from it because these people can't be helped. They have too many issues and there's really nothing you can do to help them. So don't worry about it, pass it on to somebody else and find something more exciting like pain or sports or something like that to get into. So I had this preconceived notion in my head that I would only see these patients maybe once or twice and then send them on their way because there was nothing in my wheelhouse, nothing I could do. I was looking at these cases as if there was only one way of seeing them. And for many of you have probably seen these kinds of pictures, the illusions. I was seeing it only as a skull. I didn't have room to see anything else. So one day I received a consult for this 18 year old female with ongoing back pain. And she was referred by orthopedics because of back pain in the setting of scoliosis. And she only had mild scoliosis, it was 27 degrees but she had been seeing ortho and ortho really felt like there was nothing more that they could do for her. So they wanted to send to their colleagues in PM&R. And oh yeah, by the way, she also has EDS. So I chart reviewed her and I had this again preconceived notion that I would see her, I have to fill my clinic anyway, right? I would see her, I would probably give her some PT and just send her on her way and let her know that, hey, EDS isn't really something I do. I can't really help you. I'll send you on your way. So I had this preconceived notion that she was going to be one of those no help cases and there really wasn't gonna be anything I could do for her but then I met her and I started talking to her and I realized that my viewpoint was very skewed. So of course she hit everything on the biting scale. And as you can imagine, then we started talking a little bit more and she's just wonderful. So we started chit-chatting and of course she hit all those non-articular symptoms that Dr. Rubin had mentioned as well, right? And so she had migraines, she got headaches, she had brain fog, she had GI issues, she had GU issues. Oh yeah, she had many injuries growing up where her skin just wouldn't feel if she tore it. Of course she's got joint dislocations and subluxations and then she's bruising and bleeding pretty easily. And she's got some anxiety but who wouldn't with all those symptoms? And so it was pretty clear to me, okay, this is your EDS. And it also became pretty clear to me that I really don't think that referral of simply chronic back pain was really gonna help her, right? And so I took a step back and I thought to myself, what am I gonna do for this lady? What am I gonna do? And I simply asked her, I said, this is what I do in PM&R. I look at somebody's functional ability. My role is to look at how I can make you more independent and more successful at life. So help me out to understand where are you struggling and what is your goal? And her goal was to get her pain slightly more controlled. And she said to me, look, Dr. Charron, I know you can't take away my pain. I don't need you to. I just needed a little bit more control because in the fall, I wanna go to Drexel because they have this fashion program and I would like to go. I was like, okay. So I thought, okay, I can help you, right? And there are so many articles out there discussing why clinicians avoid treating this population. Like Dr. Rudin said, lack of awareness of the condition, right? Lack of understanding of the complexity. It's overwhelming. That fear component we talked about. We in medicine like protocols, right? And there's really no clear cut protocol for these patients. And so it's overwhelming to know that one size does not fit all. The big thing that I hear a lot, I'm a physiatrist, I'm not a psychiatrist, I don't want to sit there and deal with all the mental health and chitchat and talk to the patient. Right. And then a really huge one is how many of you are scared that if you open your doors to this patient population, that it's going to become flooded. Right. These patients, maybe they just have too many needs that are not musculoskeletal, I can't really help with that. But I would argue, this is what we do in physiatry, right? How many other diagnoses do we see that are complex? We don't shy. We're not going to fix somebody's complete spinal cord injury, but we don't shy away from them. We will help look at their comorbidities, we will help look at their functional impairments and see how can we make that person more functionally successful, more independent and improve their quality of life, right? How about a stroke? The same thing goes there. We can't take away your stroke, but we are going to step up and we are going to help you with what's going on and help you with your new normal. We as physiatrists give people hope. That's what we do. So how do we bridge this gap? How do we go from that road of being fearful to being able to develop a plan and an effective path forward? And that's what Isabel and I want to help you with today. We really figured out that when you meet an EDS patient, or any patient in reality, but really somebody with complex issues, getting to know the patient, getting to know what are their goals, making it really individualized really helps to set expectations for not only the patient, but for yourself as a provider, and helps you create an effective plan. So that is where I always start with these patients. I let them know right off the bat, hey, I can't take away your pain. Most of them are not even remotely shocked by this because they know nothing's going to take away their pain. Many of them come in because they simply want something like a diagnosis. They're not looking for what I assume they're looking for. One of the goals at the helm of it is looking at sometimes simply giving somebody a listening ear, finding out what is the thing that they really want to get out of the appointment. Sometimes it is simply validation and giving somebody a diagnosis, and that is enough to know that they're not crazy, and that they know that there is somebody supporting them if they need help. So the way I look at how to treat these patients after I figured out sort of what are their goals and how can I help, I like to look at this mnemonic, T-MICE, and I'm sure many of you have actually probably heard this. I learned it when I was, I think, doing my boards, my oral boards, and I really, really enjoy this mnemonic. T-MICE, it stands for Therapy, Equipment, Adjunct Services, Intervention, Medication, and Education, and then I put in the center because to me I feel like sometimes it's one of the most important things, especially in physiatry, consult. Who do you have supporting you? Who do you have helping you make this patient's care more effective? So therapy, we all recommend people go to therapy, right? That's sort of the easiest thing, but it's really important to note that not all therapy is the same. Many times with EDS patients, education on how to prevent their subluxations, their dislocations, how to manage them if they do happen, sometimes that is the most important thing. A 2021 systemic review looked at the effects of different physiotherapy techniques and found that there were improvements in pain, proprioception, inspiration, and quality of life. And it's really important to note that when you are sending someone to therapy, to set expectations and also to have a therapist that understands EDS and understands the way that the body is different than the normal population. EDS is not like working with the general population. Many times it's simply starting with like a graded exercise program because if you threw someone into a typical exercise program, they wouldn't be able to tolerate it due to their POTS. Sometimes we have to worry about certain allergies. Many times we recommend things like taping, but many EDS patients can't tolerate the various types of tape. So it's really important to have a therapist that is educated and willing to understand the patient and the goals of the patient so that they can create an effective plan. You do have to really watch out with the EDS population because the recovery is very gradual and many times therapy can take a lot out of the patient. Sometimes they have decreased tolerance to typical exercises. You want to also teach them and teach the therapist to avoid overloading structures. Many times with these patients, they have cervical spine instability or Chiari malformations, tethered cords, autonomic dysfunction. So those are all things that you want to notify the therapist about and you want them to monitor and be safe with their exercises and their various joints and locations as well. Again, the core of therapy is educating the patient. Therapy for these patients can be lifelong. I have a wonderful therapist that works with me and many times she doesn't see somebody for years, but she is there as a lifeline if and when something happens. The second part of our puzzle from Team ICE is equipment. The goal here is to improve functionality and quality of life, prevent lacerations, reduce pain, and reduce subluxations and dislocations. Many people think of the typical things like the ring splints. If you go online on the EDS blogs, ring splints are everywhere. But I would caution you to think about other things as well. This picture in the bottom of the feed is a blanket lifter and that can help patients with ankle instability. It holds the blanket up and stops your feet during nighttime with the blankets going into plantar flexion, which can put a lot of strain on the feet. And so that can provide some relief and rest to the feet at night. Things like metatarsal pads can be really helpful. Hens units, microcurrent units, things like coffee holders or different ADL types of equipment are very, very helpful as well. Clinical guidelines for EDS do encourage the use of orthotics and different pieces of equipment. Many times I will talk to different patients and sometimes get them pieces of equipment preemptively. So for example, that 18 year old patient of mine, she is now in college and she's actually about to graduate, but we got her a wheelchair because we knew that Drexel is a larger campus and she might be needing to conserve her energy to make it to classes. And sometimes she might need a wheelchair. So we have thought of different scenarios throughout her years that might be able to help her conserve her energy so she's able to be functional in the classroom, able to get her goal of finishing her college education. And so again, it's about goal setting and thinking outside the box. So other mobility aids, typical things like canes, walking sticks, crutches, things that might help make somebody's life easier and help them conserve energy for what they really want to do in life. This slide is just to show you that there's an endless surplus of information if you Google it. If you Google bracing equipment for EDS patients, there are thousands of blogs out there. There are thousands of different pieces of equipment. If you have a good therapist and a good DME equipment supplier, many times they'll work together while the patient is in therapy to go over different pieces of equipment, try it on the patient, look at the patient's insurance for what might be the more economical route to get this patient what they need. And that's something that in our clinic, we have established and it's really, really helpful to patients to be able to try things on before they take it home. And now I'm going to pass it on to Isabel here. Okay, so we're going to talk a little bit about adjunctive therapies. What's really fun with treating hypermobility patients is that every patient is different. So you really can tailor, you know, work on your toolkit of what you can offer to these individuals and everybody is a little bit different. But, you know, general guidelines for massage, I typically recommend that they don't do any super aggressive type massages. The masseuse should try to avoid overstretching the individual. I usually suggest not having their neck adjusted. That could be very catastrophic for these types of individuals. Because once that rubber band is stretched out for that rubber band to go back, it takes a lot, a lot of time. And once a joint becomes a problem joint, it becomes a problem joint for quite some time. So really want to avoid being too aggressive with massage. As Neha mentioned, a good therapist is really important. So when you're starting out to see patients, I would try to identify a group of therapists or even one therapist that's willing to kind of understand and learn about this population. There's a couple of different sources online. Amazon has lots of great books, exercise books geared towards hypermobility. There's also an online app. It's called the Zebra Club app that's made by somebody who has hypermobility. I really would love a physical therapist to actually create some app like this. I'm trying to encourage my own physical therapist to do that, but it's still on the to-do list. So having a therapist that understands EDS is really important. So pain medication. I actually find that most of my patients don't come in looking for opioids, which is really refreshing because I think they've already gone down that path. A lot of them have, and they find that the higher and higher they go with opioids, it doesn't tend to help with their pain. And if anything, it makes their IBS or constipation issues worse, and that usually makes them feel worse. So fortunately, this is not typically a group that does a lot of opioids from what I've seen. I often suggest actually low-dose naltrexone. This medication at lower doses, high doses, we call it Narcan. Low doses, we call it low-dose naltrexone. And I usually do a very slow wean-up for these patients. So 0.5, I increase it by weekly, and the goal is to get to 4.5 milligrams. They could take it at nighttime. Sometimes if it causes migraines or sleepiness, that usually goes away in about a week or two. If they have very vivid dreams, they can try taking it in the morning to see if they can tolerate that better. But I find about 40% of the people do pretty good on it, and the ones who do good on it, it's like a magic drug. It just really helps qualm that inflammation that they have. It actually really can help with their pain, which is shocking because we would think that we can't help with their pain, but in some cases, we actually can help with the pain. Now, if the patients don't do well with it, I just say, okay, we'll just stop it. We tried it. But you kind of don't know until you try it for these patients. They can't be on other opioids if they're on this medication. So that's another good way to kind of convince people to try to wean off their opioids. So hey, maybe we can try the low-dose naltrexone. CBD can be good for some individuals. Sometimes people are sensitive to CBD, so it really just depends on the patient. Counseling and biofeedback is also important, but I tell the patients that it's not that I think the pain's all in your head because they've been told this time and time again from many, many physicians, but this is just us trying to help your brain understand the pain better and tolerate it better. Acupuncture can be very helpful. And I would say even sympathetic or a parasympathetic nervous system, modulating that can be helpful too. So there's lots of different ways to do that. Reiki, meditation, being with your friends, church groups, just telling the patients like, hey, you need to also work on your stress management, however that looks for you. Dietary and nutrition is actually really, really big in my practice. And I think when we see hypermobility patients, we all kind of practice a little bit differently. For me, dietary and nutrition is the most important thing because I feel that when I see my patients, that their inflammation and their problems, their pains, tend to come from the gut. When their GI issues get better, their pain tends to reduce dramatically. And that's still another area that needs to be researched. I think that's really also what's so exciting about seeing hypermobile patients is because this is the final frontier. This is another group of patients that we really don't know yet. The clay is still unmolded. There's need for hands to mold this clay, to do research, to figure out what's going on with these patients, and to get an algorithm and a treatment plan to actually help them with their pain. So I think that's a really exciting aspect about hypermobility patients. Of course, workplace evaluation and school accommodations. Do they need access to drink water as needed? Do they need access to more time to get to their classes? These little things, you know, many other doctors may not understand hypermobility. If you're their hypermobility doctor, you can at least put those in place so that they can still be functioning members of society, go to school, go to work. And that's enormous for these individuals. Next slide, please. So medications, I talked a little bit about this as well. I, again, do a multidisciplinary approach for medications for pain. Oftentimes, I'm trying to get at inflammation management, which is why I think that the lotus naltrexone works so well for this population. I also do a lot of GI management. Up until a certain point, I do try to help direct them to go to a GI doctor to say, hey, we need to make sure that you're regular as much as possible. You know, there's motility agents you could talk to your GI doctor about, make sure you don't have any ulcers. But a lot of times they have irritable bowel syndrome, constipation, diarrhea, and oftentimes that can get a lot better with nutrition. There's also supplements that tend to suggest to my patients as well. One of them is colostrum. The other one is L-glutamine. Both of these we know help improve the tight junction cells in the intestines, which help reduce the inflammation coming from their GI system. POTS management, oftentimes we work closely with neurology, cardiology. I think POTS is also another area that PM&R doctors could be really great at managing because, again, it affects so many systems. And we're all about quality of life. People who have POTS do not have a great quality of life. And it's not in their head. We know that people who suffer from POTS have an inefficiency with their body's ability to preload the heart. There's actually now studies now, which I love telling my patients about because they love to know facts about their body. I said, you're feeling tired because your body's not able to take that blood and put it into your heart. So when your heart beats, it's not actually getting enough blood flow going forward. So, again, autonomic dysfunction, in my opinion, all stems from inflammation. There's a lot of inflammation. There's this vasodilation issue going on with their vessels, so they're not able to bring that blood return back to the heart. Asthma, allergies, all these are things that we can kind of help guide and help manage. I don't do all the allergy managements, but we, like Dr. Rudin mentioned, I will put my patients on antihistamine because we find that this seems to be helpful for our patients, again, with this inflammation issue that they're dealing with. Oftentimes supplements can be helpful to help stabilize their mast cells, supplements like luteolin, quercetin. Sometimes I will prescribe oral cromalin to help with their histamine in the GI system. Hormonal changes, again, this is something that working with an OB-GYN that's familiar with this can be helpful. Oftentimes I'll see patients become symptomatic with hypermobility issues, meaning that they have pain, inflammation, around time periods of where hormone changes occur. So puberty, postpartum is very often I see issues. Usually they do actually better during pregnancy because there's an immunosuppressive nature, a state when you're pregnant. But postpartum is usually when they have a surge of their symptoms and also menopause is another time when they have a surge of their symptoms. So we just don't quite know yet. At my university, we're hoping to study the hormones and the way it changes and how does it correlate with individuals' symptoms. But we know something's going on with the hormones. That might be why more females are more symptomatic being hypermobile than males. And then mood and sleep and PTSD, like I mentioned, really important to manage as well. We can give medications to help manage their symptoms. But like I said, in my practice, I tend to try to help treat the root cause, which I think is inflammation. Inflammation of every organ system. Okay, next one. And if you love interventions, this is again a great group to understand. There's a plethora of interventions you can try to help these patients with their symptoms. Nerve blocks, trigger points, joint injections. I will say in my clinical experience, sometimes the younger individuals with the steroid injections don't do as well as sometimes it can make their joints a little bit more lax. But the older individuals who may have still ongoing joint pain and actually much more prevalent arthritis do better with a steroid injection. So again, young patients, maybe PRP may be a better option for them, or some sclerosing therapy with dextrose can be helpful for them as well. Unfortunately, insurance doesn't always pay for it, but that sclerosing therapy can kind of help stabilize their joints a little bit better. Botox can be helpful for these individuals, especially with the migraines that they suffer from. But again, being cautious not to overdo the Botox because then it can lead to weakness, which can lead to compensations and muscle groups that may not be helpful for their bodies. Sympathetic ganglion blocks can be helpful, and these individuals do have a higher risk of having tethered cords. So finding a surgeon that's familiar with tethered cord releases, again, this is very hard to find. There's a few surgeons around the United States that do this kind of surgery, but if they have this issue, which is bowel or bladder issues, numbness, weakness in the legs, not going away, that might be a reason to look at tethered cord as a problem. It's a very hard problem to diagnose because as an adult, they're no longer growing. So for children, you can see there's actually a pulling on the cord, but as adults, it may be hard to see because we're not really growing anymore. It's oftentimes imaging and symptom evaluation to see if this is an issue. Next slide. So consulting, finding your consults, finding who's in your team is enormously important. Next slide. So we just wanted to put in sort of what our different systems look like. So at Hershey Medical Center, and this actually has changed, one of the things that I found is that you have to be flexible and things are constantly evolving. So in our institution, we have really good OB, cardiology, GI, home. We've had genetics, and then like Dr. Rudin mentioned, they just don't have the manpower. So genetics at our institution now has completely shut their door to seeing UBS, which is just super unfortunate. It's really helpful to develop your network and then continue to work on that, continue to expand, and sometimes you have to go outside the institution. Yeah. So this is just a compare and contrast. Again, it's also changing all the time. Our allergist just actually sent a message and was like, please stop sending patients to see me unless they're anaphylactic. I do counsel my patients and tell them I don't, a lot of times the testing for, we say mast cell activation syndrome, which a lot of our patients think that they may have or histamine intolerance issues, they're very hard to diagnose unless you're in a flare, the lab testing may not actually be positive. So I do warn them that the lab testing may not be positive. Sometimes they're still very insistent on seeing the allergist and I will oblige and refer, but now our allergist have told us that they no longer want to see them unless they're anaphylactic. So I had just had to counsel the patients about that. We've tried to help prep our patients because in the appointment, you know, they've waited a long time and this is why we're doing this members community group. I reached out to Dr. Hankason to see if we could revive it and get some more excitement and interest in seeing hypermobility patients because we need more doctors seeing them. And these people need somebody to go to or just tell them what their diagnosis is. So we at UT Southwestern, we've come up with a webinar. The patients get this with handouts and pamphlets and reading material before they actually come to see me. And I think it's great because they have more time to digest that information. We don't tell them that if they have the diagnosis or not, but we just kind of go over roughly. This is typically what we go over when you come to the appointment. That way they're a little bit more prepared with the interview when I see them. This is just an appointment intro video. I don't really think we need to play this because it's just me talking about like, hi, I'm Dr. Hwang, information for my patients. So lots of books, books for children even to understand their issues. Two Flux with Fuqua is a very good exercise book that I have my patients say, hey, just look at it. If you've got your shoulder problems, go to that shoulder chapter, because sometimes they may not have a therapist in that area close to them that can see hypermobility patients. So they got to have some kind of resource to refer to. I really enjoy the book Disjointed. It's written, I love the dysautonomia chapter. So lots of good reading suggestions and we always need more. So fortunately, Dr. Gutierrez, she's the chair of UT San Antonio, has incorporated the first ever hypermobility book chapter in Essentials of PM&R, which is great because we need to get this subject on everyone's radar, that this is a topic that PM&R people, PM&R doctors are perfect to take care of. So yeah, again, managing is like having a strong herd of zebras. And oftentimes I feel like I'm in a safari because there's honestly so many zebras around me. But having a core group of providers, we can be that core group of providers. I think if we got our community group stronger and spread the word, I think that would help everybody out. Oftentimes I have patients that move to a different state and they ask me, do you know anybody that sees EDS? And I'm usually like, no, I know someone in Pennsylvania. There's nobody in between and it's so hard for these patients. So we would love to see this community grow and we love to help foster it as well. So thank you so much for everyone's time. I know it's so late. Thank you so much, everyone. We have a couple of comments that I'm going to read from the chat. Dr. Frick said, Dr. Wang, publishing a list of these meds for these issues would make for a tremendous article, which Dr. Rudin responded to getting some thank yous for your presentation as well. If there are anybody, or if there is anyone rather who does have a question, you can either put it in the chat or you can raise your hand and I can unmute you or if you just want to unmute yourself. Yes, there needs to be more research. And like I said, this is like clay that's not yet molded. And that's, what's really exciting about taking care of these patients. I have in the book chapter and essentials for PM&R, I do have lists of supplements and I do have research that supports what it does actually in the body. Now, how it pertains to hypermobility, I only have clinical experience at this time, but we hope to do more studies and we have hopefully funding for more studies for this. So I'm going to read the question just for the recording. Yeah, here we go. Any guidance on getting coverage for soft good type orthoses, which can be beneficial for these patients, but aren't often well researched or covered by insurance? Yes. Well, we probably all have something to say about that. Yeah. Orthoses for these individuals, for the soft goods, I'm assuming you're talking about things like the body braid or some of the other, the shoulder retraction orthoses and some of the other things we see. Even some of the more permanent products like ring splints are difficult to get covered. You usually have to write letters of medical justification, but more often than not, people are either paying for these out of their own pockets or using FSA dollars, which usually do handle most of this stuff. But Neha, Isabel, any other thoughts you have? Yeah. I was going to say the same thing and don't shoot me, but Amazon. Yeah. Etsy and Amazon. Actually, there's people on Etsy who make the ring splints and they're much, much less expensive. It may not be like the, I don't know, titanium, whatever they make out of the ones, but at least they have something. It's a lot more affordable. Yeah. They'll even 3D print plastic ones to measure in some cases. Yeah. Sometimes, well, a lot of times these patients are actually making different types of braces and then selling them as like Etsy because they found that it works and helps. And so that's a lot of times. The next question for dextrose prolotherapy, sorry. Do you do a one-time injection or several over a few weeks? I can chime into some of that. So we will do, it depends on the patient, number one. But many times it is a few injections over a few weeks for us. Yeah. I agree. And I have no prolotherapy practice. We simply don't have any coverage for it whatsoever. At this time, we're still wondering how well prolotherapy is going to work in this population, given that you're trying to create new tissue, you're going to create new hypermobile tissue. I don't know how well it works. I would defer to my colleagues who have more experience with it. What are you finding? It helps. It helps, especially in the joints where you can't really strengthen. Like it's very hard to strengthen that patella. It's very hard to strengthen those fingers. You know, there's just not a lot of muscle groups to cover that area. And that's when I would say prolotherapy to try that. And, you know, with therapy as well, with physical therapy as well. Hey, the next question is for Dr. Charon. You mentioned getting a wheelchair for your young college age patient. Do you usually order a power assist wheelchair versus manual wheelchair? And if power assist, do you have difficulty getting that approved for this diagnosis? Oh, no, but you do have to, I don't, I don't put hypermobility as the diagnosis. So many times these people have things like POTS and autonomic dysfunction, et cetera. So you do sometimes have to think outside the box, but there are very good lightweight power assist wheelchairs out there. And I found it can be really, really helpful. But again, it depends on the patient. It depends on the situation. Yeah, it really depends because when you brace or put them in a wheelchair, you know, then they're using their arms and their arms give out, you know, it just depends on the person. Again, I think the biggest thing is getting to know your patient and what is their goal. Yeah. For, for those, there are people who, for whom I've ordered wheelchairs, who have done very well. They use it as an adjunct. They're up and moving when they feel like they can be up and moving. But then there've been a few who, once you give them the chair, never get out and they become very, very deconditioned. You have to be very careful screening and yes, goals are everything. What are you out to achieve? And can you define functional goals that you're going to be able to measure progress toward? The chair should be a means to a definite functional end. When it is, it's incredibly powerful. If it's just for pain relief, I find it doesn't work. Next question is what diagnoses would you use for getting neurotoxin covered for these patients? Botox. I have a ton of patients with that. Sometimes some dystonia as well. Dystonia. Yeah. Migraines. Yeah. Migraine or dystonia. And with the caveat that if you're using it, you want to make sure not to weaken too much around a hypermobile structure, particularly in the neck region. We've seen some people have problem before. A lot. So when I do migraine Botox and with this population, many times I'll avoid the cervical location, but there, if you, so I'm a DO, so a lot of times we'll palpate and you find their TMJs are very sensitive. So if you inject their TMJ instead of the occiput area, many times it gives them a lot of relief. So we do that as well. Yes. Yeah. Masseters. Um, okay. Uh, Dr. Patel, I am going to read your question just for, um, cause we're running low on time. Um, osteogenesis imperfecta. We learned in pathology, they have a blue sclera in my experience PTS with EDS hypermobility also have a blue tinge to their sclera. Do you notice the same? Yeah. Yeah. Yeah. I think it's because their tissue is thinner. So you may be able to see their vascular structure, um, better. And then there's a certain kind of, I think the brittle cornea type, they tend to have a very blue scale sclera. Um, but I wouldn't say every EDS patient has a bluer sclera, but yeah. But, uh, but since you're academic too, we need to start including them in the literature as it's never been included before. I agree that this is a wide open frontier to, to publish it. I have unending, if I had time, I've on any amount of things to publish. I just am overwhelmed with patients, you know? So we need more people to see these patients, see these people. That's a good tag. That's a good tag to take, you know, Hey, help us out so we can write books. You know, that's, it's a good point. We all need a right. We all need to publish. We all need to research. We only see these patients. I think the other thing, you know, us in PM&R, we are a pretty friendly bunch, right? And we like to work together. So what I found, one of the best things that has happened since I entered this world is the connections you make. Um, I thought in residency, I wanted sports med, you know, the typical, right? And those areas have already been carved out and you kind of either just fit into that box. Um, it's very difficult to spread your wings and fly, right? This is a great area where you can just really meet people, find your interests, find why PM&R and what part of function works for you. And you can make that, you can hone that, um, with this population. And they are a very grateful bunch of people. Yeah, very, very. And you don't have to do it all alone either. That's very important to understand besides the team that you build at your own place and the connections that you make, um, you engage primary care. And one of my big projects here at Madison is to teach primary caregivers how to recognize and maybe even do some diagnosis on these individuals. Um, there's no way that they're going to make me obsolete by doing that because they have so many questions. There were so many things we need to answer as physiatrists, but it's, we're just starting to see a glimmer of, Hey doc, I used your clinical pathway. This patient's got it. I've taken their thus far. I need some more help. What can we do? And just the network expands and you have more people on whom you can call as well as more who want to talk to you. So, uh, it's certainly doesn't have to be a lonely enterprise. It's funny, but you know, Neha, you talked about how, you know, these are the people supposedly nobody wants to treat. Um, it's incredibly rewarding to help them. And the very first patient that came into my office with EDS, I said, what's going on? She said, well, I have EDS type three, which is the hypermobile type. And I said, what's that? She said, well, you're going to know when I'm through with you. And by God, she was right. Um, by the time we got done with that visit, uh, I had two other doctors to call and it just kind of took off from there. So it's, uh, this can be a real springboard for a career. A lot of times in medicine, we complain that patients don't take initiative and rely on us as clinicians to, you know, give them the answer. This patient population, they are invested with you. Many times you're learning like Dr. Rudin said from them, um, and they're okay with that. And so one of the biggest wake-up calls I had when I was treating this population is I don't have the answers and that's okay. They're not looking for all the answers. That was a me problem. Um, they just want someone along the ride that is willing to listen and be on that journey with them. And if you can take a step back from your ego and realize like, I don't have the answers and that's all right. It's a great population to work with because they will not leave you high and dry. They will be on it with you as well on the educational journey. Um, and they're so grateful to just have a listening ear and somebody trying to help. All right. Well, we're coming up on time. Are there any other questions that people would like to ask? I do not know this week's Powerball number. Well, I would like to thank everyone for attending. Thank you to our, um, presenters for sharing their expertise and wisdom. And, um, Member May is continuing throughout the rest of the month. Um, every session is open to any AAPMNR member. So feel free to check out other sessions. And we will be posting the video, um, soon to both our online learning portal and to the member community. Thank you all so much and have a wonderful night. Thank you.

hypermobility syndromes

Dr. Jordan Burkhardt

Dr. Nathan Rudin

Hypermobile Ehlers-Danlos Syndrome

hypermobility spectrum disorders

PM&R role

individualized care

interdisciplinary care

patient goals

quality of life

alternative therapies