All right, let's go ahead and get started. I want to welcome everybody to AAP Virtual Didactics for today. My name is Sterling Herring. I'm a PGY3 at Vanderbilt. As always, I want to recognize and appreciate those of you who have been affected by COVID-19 more than the rest of us. We recognize that the burden of this pandemic has not been equitably shared. So for those of you who have been professionally or personally affected more than the rest of us, we appreciate you, and if there's anything we can do to support you as your colleagues or as AAP, please let us know. So the purposes of this lecture series are to augment didactic curricula that are ongoing at your home institutions, to offload overstretched faculty, to provide additional learning opportunities for off-schedule residents. We know there are a lot of logistical difficulties in terms of both resident scheduling, clinical responsibilities, and kind of faculty being pulled every which way, and we're hoping to kind of fill in a need there. Also to develop further digital learning resources and to support physiatrists in general during COVID-19. As always, we're going to keep everybody's video and audio muted except for our presenter. If you have any questions, you should be able to see my name up near the top, Sterling Herring. If you double-click my name, you can send me a message, and I will pass that question along to our presenter at an appropriate time, usually that's at the end of the lecture. And if you have any general questions or suggestions, concerns, anything like that, feel free to reach out to Candice at AAP. Her email is there on the screen, or you can find her on Twitter. So without further ado, we're excited for today's presenter coming all the way from Hershey. I say it's all the way. It's all the way from me in Nashville, but all the way from Hershey, Pennsylvania. Thank you for joining us today. Hi. Can you hear me? Yes. Awesome. Thanks so much for having me. I really appreciate it. Thank you for joining us. Let's see if I can figure out the screen share. It says, do I want to stop others sharing? Yes? You do. All right. Here we go. Let's share, and everybody see that? Perfect. Looks great. Awesome. So welcome everyone. Thanks so much for tuning in. I'm Neha Charing. I am actually an attending physician at Hershey Medical Center, and I'm excited to talk to you today about the world of Ehlers-Danlos Syndrome. The title of my presentation is An Unconventional Body in a Conventional World Living with EDS, and I have no disclosures. So I'm going to start with you today because I wanted to bring up a case that actually got me interested in the world of EDS. So I received a referral from orthopedics, and the referral was on a 16-year-old female with ongoing back pain in the setting of mild scoliosis, and as I was reading her chart, I saw that she was having pain for about three years, and her past medical history said that she had anxiety depression, CRPS, painful periods, and then other weird pieces of information I saw in there were that she had seen 20 providers. So the two things that kind of struck out at me were, why is a 16-year-old diagnosed with CRPS, and then why does she have 20 providers on her chart? And that's when the little warning emoji went off in my head, and I thought to myself, should I be concerned? But then I thought, you know what? I'm a physiatrist. It's back pain. No biggie. I got this. And so I forged ahead. So a few weeks later, I met her and her mom, and I got a few more details. I found that the pain started three years ago from a gymnastics injury to her great toe on the left, and she was doing a handstand, and she mentioned that the chair rack was very close to her, and so her left toe hit the chair rack. She ended up being booted for one month, and that's when her pain started jumping from her left leg to her right leg. She was eventually diagnosed with CRPS, and she got to the point where she was unable to ambulate, and she was wheelchair-bound. And so as you can imagine, her and her mom were quite alarmed and ended up seeking a multiple number of care providers. She was eventually sent to Kennedy Krieger for their pain program as well as for their back program. And then fast forward 19 months, she said that she started to be able to ambulate again, and so that's how she actually presented to me. She was ambulating without assistive device, and basically her main complaint was this back pain and diffuse joint pain. And so I really want you all to think, like, how many times have you in clinic got this vague referral, and then you start to dig, and you find out there's all these weird pieces to the history, and you think to yourself. So I chose to stay, and I eventually diagnosed her with hypermobile EDS. And I've chosen to speak with you about this because in my practice, I've actually been seeing quite a few patients with this condition. And while I'll be the first to tell you I'm no expert in the diagnosis, after hearing things like, Dr. Charon, I'm an interesting case, or my unconventional body doesn't suit this conventional lifestyle, or I'm used to providers telling me, Dr. Charon, that they don't know how to help me, so if you can't, I won't be offended. And my favorite is doctors think I'm neurotic and even attention seeking. And to me, EDS fits so beautifully with the world of PM&R. You know, our line of work, we deal with functional and social impairments from any medical diagnosis, and EDS is a great example of a diagnosis that has a broad number of effects on the body and can be quite disabling, and who better to treat that than a physiatrist? And to make it more fitting, May is actually EDS Awareness Month. So EDS is a quite complex syndrome, and it's not black and white. And so today, I really want to just break it down, and I want to go over the basics with you. I hope we can go over the basics of the symptoms, go over a treatment plan that you can be comfortable with, and then also, I just want to make sure you never forget about those zebra cases, because they are out there. We're briefly going to go over the history, etiology, and diagnostic criteria of EDS, but the stuff I really want you to pay attention to and the stuff that I think is really important is what can that clinical presentation look like, and what can your treatment plan look like? I really believe by providing you knowledge on what EDS is and how it may look both inside and out, you're going to gain that confidence and understanding to help create a treatment plan that you and your patients are comfortable with. So if you ask around, many providers don't really know that much about EDS, but it actually is one of the oldest known causes of bruising and bleeding, and was actually described by Hippocrates in 400 BC. He wrote about these nomads who had these very lax ligaments and multiple scars, and the scars were thought to have been a result of cauterization techniques that were attempted to try and stiffen up joints. It wasn't about until 2,000 years later that it finally appeared in medical texts, and a Dutch surgeon in 1657 wrote about this Spanish sailor who had very hyperextensible skin, and this is a picture of George Albers who was that sailor, and in 1991 in the Rheumatology Journal he was actually diagnosed as having EDS. So it took many years to kind of put two and two together, but eventually it was put together. 200 years later in 1892, Dr. Chernogov, who was a Russian dermatologist, actually was the first clinician to describe a case of multisystemic features with linking it to a connective tissue disorder, and in the early 1900s, Drs. Ehlers and Danlos took that information and really began honing in on the disease. They actually did this publication of about a hundred patients detailing all the symptoms of the condition, and in 1936, Drs. Parks and Weber recommended that the condition be termed Ehlers-Danlos. So what is EDS? It's actually an inherited group of disorders that affects the connective tissue. It's inherited from either a parent or due to a genetic mutation, and thus far we have I think 19 various genes that have been linked to the disorder. EDS affects collagen, which is required for normal development of connective tissue and the normal development of bone, tendon, skin, and organs, so it's everywhere in your body. A over here shows what normal collagen fibrils look like, and you can see that they're uniform in size, shape, spacing, and then B, you can see that the collagen is disruptive, it's not uniform, and all that can severely end up affecting the tensile strength of your connective tissue throughout your entire body. So a really simple way of looking at it is collagen is in connective tissue, connective tissue is everywhere in the body, and so if your collagen is disruptive, your connective tissue and subsequently various organs in the body are disruptive, and that can really impact your function. And that's why as physiatrists it's really important that we are on their care. And this is a picture of what that connective tissue looks like holding muscle together, you can imagine that it's everywhere and if the connective tissue is very loose, then your muscle strength is not going to hold your structures together, and you can apply that to bone, joint, skin, and any organ in the body. So in 2017, the International EDS Consortium actually identified 13 types of EDS, and while the condition I had told you has been around for a long, long time, the consortium really felt that the prior nosology had a lot of clinical uncertainty, diagnostic confusion, and just wasn't as clear as it could be. So they did a very comprehensive review and came up with 13 subtypes that now have been recognized, and each of those subtypes have their own diagnostic criteria. So EDS can be inherited and they found that most commonly it's autosomal dominant, and then we talked about already that there's about 19 different genes that have been discovered to have mutations, and the NIH found that the most common types are the classical and hypermobile types, so those are the types that I'm going to focus my talk on today. And the prevalence of classical type is 1 in 20 to 40,000, and the hypermobile type is 1 in 5 to 20,000. So let's get back to my case. You'll recall that she was sent to me for this back pain in the setting of scoliosis, but after chatting with her and her mom, I really found that these odd symptoms of painful periods, CRPS, requiring to see 20 providers, I thought something must be going on, so I started asking some more probing questions. First I asked her, growing up, did you consider yourself double jointed? And she proceeded to show me what she could do with her fingers. I then asked her, can you dislocate your shoulders or knees or sublux them easily? And she did this for me in clinic. I then asked her, can you stand up and put your palms on the floor without bending your knees? And she stood up and did that. And so these points demonstrated to me that she was very hypermobile, and due to her being hypermobile, I decided to test how hypermobile her joints really are. So I used the Byton score, and in 1969, Byton and Horan came up with a scoring system for joint hypermobility. Over the years, there have been many articles that demonstrate it is a valid tool to assess joint hypermobility. It looks at nine different joints where zero is scored as not hypermobile, one is scored as hypermobile, and you can get a total of zero to nine. And the higher the number, the more joint laxity that is noted. Schlager in 2018 did look at the inter-intra-rater reliability for the various hypermobility assessment tools, but because the Byton score is the most common, that's what we're going to focus on today. And they found that there is good inter-intra-rater reliability, especially when a goniometer is used. And so you can really assess what degree of laxity occurs at these joints. They also found that this was a quick and easy test to perform, and it can take less than five minutes to do in your clinic. One downfall in particular is though it only looks at a particular number of joints, and as we've talked about, EDS really can affect anywhere in the body, so this test may be missing some of those hypermobile areas. The EDS Society has the appropriate techniques that we all should be using to perform the test to maintain this inter-intra-rater reliability. A talks about the pinky, and you really should have the patient's forearm and palm on the table and then have them passively extend that pinky. If it gets past 90 degrees, it's considered positive. B, with the arm out straight and the palm facing down, the wrist should be fully bent downward and you want to see if the thumb can touch the forearm. With the elbow, your arm's outstretched and the palm is facing up, and you want to see with the goniometer, does your elbow hyperextend past 10 degrees? The knees are tested by standing, and you're going to lock the knees. And you want to see with the goniometer again, do you get past 10 degrees of hyperextension? And the spine, like we talked about before, the patient stands up, knees are locked, and can you put your palms on the floor? The EDS Society actually has great one to two minute videos that go over the proper technique for each of these tests. And so it's accessible to you and me, and it's also accessible to patients. It's all free. And I highly encourage you guys to test it out before you even have a patient in clinic. It's quick, under five minutes, and it's kind of fun to do too. So now with COVID-19 and virtual visits, I know a question that's been raised is can this be done virtually? And I would argue yes, we can do it, but modified. There are a few key things that you should know. It's obviously more accurate if you do it in person with a goniometer and an experienced person is watching it, but until you can get your patient into clinic, you can visually try and assess the BITEN score and get an idea for how your patient is looking. I would by no means give an official diagnosis or an official score based on my virtual exam, but it can help me for my future visits kind of guide what I need to do in clinic. And another tip is that technique is key. So if you search BITEN score and you just Google pictures about it, you'll see many different publications all with different pictures on how the BITEN score was done. But the technique really helps all of us keep that intra-reader reliability and if you see here on four, they're testing the pinky, but if you are not locking out the joints next to it, you can get a lot more laxity at the pinky joint than if you tested it properly. So this is actually an incorrect method. And the reason I bring that up is because I have a lot of patients that come to me and tell me they've been diagnosed with hypermobility syndrome based on the BITEN score, but when we do the BITEN score based on the EDS Society's techniques, they actually don't meet criteria and you can imagine that does not instill a lot of faith in our patient population if we as clinicians don't all do it the same. So my patient received a score of 6 out of 9, which is indicative of joint hypermobility, but was that enough for me to stop and tell her, yep, you're diagnosed with EDS, you can go home now? And the answer is no. A positive BITEN score implies hypermobile syndrome. It doesn't imply that you have hypermobile EDS. And so BITEN actually noted that the majority of patients with lax ligaments or loose joints actually suffer no articular problems and in fact he said that most hypermobile subjects studied actually feel that being hypermobile is a positive attribute, which enhances their participation and activities. So you can imagine if you're a gymnast and you're hypermobile, that's actually a great thing. You want to be concerned when it turns into non-articular problems. So I started looking for if my patient had some non-articular symptoms that might be associated with this and it turns out she did. She had the loose unstable joints. She had skin that bruised easy, joint hypermobility, dizziness with standing, heartburn, clicking joints, painful periods, joint pain, and then anxiety, depression, and which was interesting to me here was this extreme tiredness and fatigue. And all these symptoms, you know, just don't seem normal for a young girl her age. And if you look at these symptoms, it can lead you in a very different direction when you're coming up with a diagnosis, or if you take a step back and really think about it, it could lead you to that zebra diagnosis. And we all know that abnormal collagen can affect all structures that support the body. So is it really unreasonable for me to think that my patient's multitude of symptoms could be because of Ehlers-Danlos syndrome? And the answer is no, to me it made sense. So what is the diagnostic criteria for me to say that she officially has EDS? And I could do genetic testing, but in the absence of genetic testing, it is clinical. And genetic testing for me has been difficult to get for my patients, and so I tend to rely on the clinical criteria, and that is the Bighton criteria plus the Brighton criteria. So we went over the Bighton criteria, that's assessing those hypermobile joints, but then each of the 13 subtypes of EDS actually have their own Brighton criteria, and the consortium has provided us all what that criteria is. So you can see this is the criteria for the hypermobile subtype, and you can see in criterion 1 up here, there's a box of pictures, and that actually is the Bighton criteria. And if you meet criterion 1, you go on to criterion 2 and criterion 3. So if you follow these boxes, it really assesses for you whether or not somebody has EDS and what subtype they have. This was a really interesting article in the Journal of Physical Therapy and Sport that looked at the efficacy of using the Bighton score as a predictor of the Brighton criteria, and the authors found that Bighton scores could be used as a predictor of arthralgia and joint dislocation, joint dislocation and subluxation in males, and joint arthralgia in females. And their thought was based on the information, and knowing that EDS can have many comorbid symptoms like recurrent sprains, dislocations, early OA and arthralgias, they felt like if somebody had positive hypermobility score with Bighton score, then you may want to implement injury prevention strategies to help and try to reduce the possible negative impacts of having possibly EDS. And that's ultimately improving somebody's function. The Ehlers-Danlos Society found that when you start seeing signs of joint hypermobility, joints that stretch further than normal due to poor connective tissue, skin hyperextensibility, skin that stretches further than normal, and signs of tissue fragility throughout the body, that's when you want to be concerned of EDS. And they found that all three pieces here, joint hypermobility, skin hyperextensibility, and tissue fragility, are in all 13 subtypes of EDS. And it's important to remember that because those kind of guide you to those non-articular symptoms. And remember just because you're hypermobile doesn't mean that you have EDS. When it tips over to some of these characteristics, that's when your radar should go up. And a really interesting piece of information I came across is that it actually takes about 10 to 20 years for these patients to be diagnosed. And you might be thinking like why, why does it take so long? But it makes sense because they have multiple issues and again, it's very common for providers to get kind of bogged on in, are each of these issues united or are they different symptoms? And so it can be really difficult as a provider to put it all together and associate it with one diagnosis. And really, what does EDS look like? And that's the million-dollar question, right? There really is no right answer to what does a typical EDS patient look like. Some symptoms of EDS are visible, things like hypermobility, subluxing, or easy bruising. But some symptoms are not so visible, things like chronic pain, organ rupture, POTS syndrome. And so based on the type of EDS, your clinical picture can look very different. If you have vascular EDS, your picture is going to look really different than if you have hypermobile. So these are two Hollywood actresses that have EDS and you can see that they are vastly different in appearance. And by simply looking at them, you may not know that they have EDS at all. And so it just emphasizes to you that not all EDS patients look alike and you may not even see any visible signs. So I told you at the beginning though that I wanted to help you recognize common symptoms and I realized that EDS affects the whole body, number one, and then number two, there are 13 subtypes. And so really are there common symptoms? Probably not, but the commonality between it all is that it affects your entire body and can affect any organ system. And so I really like this picture because it emphasizes that. And I know this slide looks really overwhelming and by no means is it all-encompassing, but bear with me here. My hope by creating this chart was that you can actually print it out and take it to clinic with you. And if you have a patient that you think might have EDS, you can refer to this to help guide your questions and guide your treatment going forward. Most of us think EDS, you think of joint, skin, pain issues, but I really wanted to draw your attention to some of those unique characteristics of EDS. So for example, you can see under MSK, there's TMJ disease and that can be quite common. You can have piezogenic papules, and I'll show you a picture of what that is in a second. Chiari malformation, when patients have ongoing headache and cognitive dysfunction, Chiari malformation should be considered and interestingly, patients can go misdiagnosed if they don't have an upright cervical MRI. The thought is that when you're standing or you're seated, you have gravity putting weight on the ligaments of the spine, possibly allowing for a positional MRI to help better detect if there is a Chiari malformation. POTS syndrome, that's a form of orthostatic intolerance with the presence of excessive tachycardia and one study demonstrated that approximately 25% of patients with POTS and EDS were disabled and unable to work. They found that the quality of life in POTS patients was comparable to kidney failure patients on dialysis, and so that's really disabling, right? Brain fog, that can occur due to vessel laxity and your blood flow not being as strong as it should be. Kinesiophobia, patients with EDS can suffer from chronic pain and be susceptible to physical trauma and stress, so it's important to ensure that proper psych services are on board when you're treating these patients. One paper demonstrated that 33 to 75% of EDS patients had gut concerns and this ranged from GERD, to gut dysmotility and even significant nutritional deficits. So it's important to keep in mind, you know, with your treatment plan and when you're going over your review systems with patients, you know, you need to check each of these organ systems. These are just some pictures of joint hypermobility. You can see B shows you kind of that TMJ. Skin hyperextensibility. Dr. Nigel Burrows gave this great talk about skin hyper extensibility and the effects that it has on patients at a national conference this past August. He noted that skin is soft, velvety and stretchy in these patients and it's very translucent. He found that because of balance issues, they fall easy and then because their skin is very fragile, they bruise and tear their skin easily. So since the skin integrity is poor, they tend not to heal well, especially in the setting of having nutritional deficits and in fact if you send them for sutures, a lot of times typical suturing techniques actually don't hold on these patients. This is a picture of those piezogenic petal papules and that's a result of the adipose tissue kind of bulging through the fibrous layer of your feet and so it's kind of like a hernia for your feet. This is something you can assess virtually via video. 50% of the EDS population has this which is the positive Gorlin sign where you can take the tip of your tongue and touch your nose. Only 8 to 10% of the general population can do this and again, this is a simple virtual test that can be done. This picture shows you the translucent skin on A over here and you can really see all the like vessels underneath the skin. In the normal population, you shouldn't be able to see that and B is a picture of the atrophic scarring that patients tend to have. This is a picture of Gary Stretch Turner and he actually holds the Guinness World Record for the world's stretchiest skin. So it's been noted that skin hyperextensibility is actually a reliable and reproducible feature of classical EDS and so how do you test for it? Kastori in 2012 found that you can look at the dorsum of the hand, the fourth MCP, or the roller aspect of the forearm and they found that if you get over 1.5 to 2 centimeters, that's considered hyperextensible. One tip for you though is that you should not do it over an extensor service, so like something like your elbow, you don't want to take the skin off of your elbow there because it tends to be stretchier than other parts in your body. Again, this is something you can do virtually. You're not going to grade it by any means, but again, it can help guide your clinic visit for when the patient can come to clinic. This paper discussed survey results of EDS patients and you know I gave you that slide before that showed all these different symptoms that a patient could have, but this study was really looking at how many symptoms really are EDS patients experiencing and the results were staggering, right? So it really showed you that comorbid conditions really affect these patients and it can affect their quality of life and that study wasn't alone. Other studies have shown that patients can have chronic, frequently debilitating MSK pain, they can have fibromyalgia, headaches, migraines, IBS, and so it's really important to check all the organ systems in your patient when they come to see you because there likely is more than one organ being affected. As a physiatrist, we look at the entire picture and so don't forget these areas because it's likely impacting the function of your patient. So it's really not surprising that many clinicians try to avoid seeing EDS patients. It's just for the very reason alone that it can be overwhelming due to the multitude of problems that arise, but I hope that I can share with you some strategies on how to approach these patients because it is doable and these patients are desperate for help and regaining some function and again as physiatrists, that's what we can help with and an important thing to know is that these patients are not looking for a cure. They know better than anybody that there is no cure. What they are hoping for most of the time is just an improvement in their quality of life and that's what we do as physiatrists. So I spent a lot of time kind of coming up with the treatment section and coming up with, you know, what's an easy way that I can approach it with you all today and I know that EDS is overwhelming and when I'm overwhelmed, I try to go to a structured approach and so I thought, you know what, I'm going to use the approach that I use in clinic and I am a huge fan of the Team ISE approach. To me, it's all-encompassing and it allows me to calmly tackle patients and their concerns and so what does it stand for? T is for therapy, E is for equipment, A is for adjunct treatments, M is for medication, I is for intervention, C is for consult, and E is for education. And so we'll go through each of these pieces to hopefully give you a calmer approach to seeing these patients. So therapy. As a physiatrist, I think we can all safely say we would recommend therapy to most patients and I don't think I need to convince you that therapy is a good thing, but if you do need convincing, the EDS Society has this great article talking about how EDS patients have significant disability, difficulties with ADLs, which can all impact their quality of life. They found that patients have positive effects from therapy, including things like strength, stability, education on their disease, and even how to handle their own pain management and currently, they're recommending manual therapy, taping, hydrotherapy, and relaxation techniques all should be employed. But again, as a physiatrist, I feel like we know how to do that pretty well and so for us, I feel like it's really important that we get more details in our scripts because there are many restrictions and limitations that impact EDS patients and can impact their therapy experience and so it's important that we address those things. So when I'm sending someone to therapy, the very first thing we talk about is that I'm not sending you to any therapist. We have to find an experienced therapist, somebody that knows about EDS. We then talk about the goal of therapy, that it's not a cure, but instead it's about educating them on preventative measures and management techniques. We talk about that EDS patients tend to have more gradual recovery and smaller gains over a longer period of time and so that it's a time commitment and as long as they're gonna buy into that time commitment, so will I and if they're okay with that, then in addition to my typical therapy script, which would be like your strength stabilization, I may consider things like low-resistance muscle toning exercises. A lot of times EDS patients tend to have shorter step length, require more time and stance phase, or balance impairment, so maybe I'll do a gait evaluation. If my patient has POTS syndrome, I may scratch my typical script completely because they likely can't tolerate it and we may start back at the CHOP graded exercise protocol. There are even different types of taping that I might employ, whether it be like the rock taping, McConnell taping, and so I think the thing I want to emphasize is being specific with this group is key. You really need to be specific on your therapy restrictions. These patients have very lax ligaments and that includes in their cervical spine, so that means no HVLA, no high-impact activity, no cervical traction. I would avoid over stretching the joints as that can make the ligaments looser. If my patient has POTS, I would be specific in letting the therapist know no sudden head up postural changes as that can trigger POTS and dysautonomia and these are all the reasons that you really want an experienced therapist because you want somebody familiar with these restrictions and also comfortable with adhering to them. Equipment. DME is essential to aid with functional independence and quality of life and as physiatrists, we probably are very comfortable with ordering bracing, orthotics, evals, mobility aids, home and workplace ergonomic evals. But have you thought of how they sleep, right? Have you thought of maybe sleep posture support? Mattress toppers might be employed, body pillows. Have you thought of maybe a blanket lifter? A lot of times the blanket will put patient's feet in plantar flexion and that aggravates trigger points in the calves. And so this might be a simple technique to decrease the morning pain that they're experiencing. Have you thought about the Theracane? The back knobbler? Have you thought about assistive devices for work or school? How about helping them employ voice command? If you Google bracing and equipment for EDS patients, you're gonna get lost in this endless supply of information and it's really overwhelming. But you don't need to be overwhelmed. You just need to listen to your patient and see what are they looking for and go down that route. Adjunct treatment is actually very essential for this group. Lifestyle modifications have been considered one of the most successful strategies for managing pain in EDS. And that can include things from learning how to have a more holistic lifestyle with things like acupuncture, massage, Tai Chi, to considering anti-inflammatory diets, incorporating ginger, curcumin, tumeric in their diet. One question I get asked a lot is about CBD usage. And all I can tell you is that if you search online or ask your patients, you'll only see positive results and positive responses about CBD oil and cannabis products. And anecdotally, I think it's been shown to help compared to opiates. But currently there is no literature out there saying that medical marijuana is used in the treatment of EDS patients. There is a lot of research out there. And so I am anxious to see kind of what comes out of it. When it comes to pain management for EDS patients, there really is lack of evidence out there. But the International Consortium did publish literature on common medication strategies to employ. And it ranges from your conservative measures like topical, Tylenol NSAIDs, all the way to opiates and steroids. And so it's pretty typical of your general population. But a couple of things you wanna be cautious of in comparison to your general population is, for example, EDS patients have known comorbid GI symptoms, right? And so a lot of times they can't tolerate NSAIDs. While it's been used for mild to moderate pain, sometimes we just have to skip right past it. I didn't see any trials testing medication management and neuropathic pain in EDS patients. But I can tell you in my clinic, we do use the common culprits, the SSRIs, SNRIs, TCA, anticonvulsants. But one thing you do wanna be very cautious of is dysautonomia and hypotension. Muscle relaxants can help with painful spasms, but you wanna be cautious because the muscle relaxants can actually increase joint instability and then worsen the pain. And opiates are an option, but there is a lot of evidence out there that long-term treatment can lead to central pain sensitization. And so maybe you wanna consider Tramadol instead, which has serotonergic effects. And a prior study actually on kids with EDS did demonstrate that Tramadol decreased pain and improved physical capacity in kids. Steroids can be used for acute exacerbations, but you just wanna make sure that you're not getting any of the steroid side effects. So on the right here, this was a questionnaire that went out to 79 adult patients with EDS, and they talked about different types of medications that EDS patients were being prescribed. And I just wanted you to see here that in addition to your typical pain and mood treatment, cardiac things are on there as well as pulmonary. And so it just emphasizes that you don't wanna forget about those other organ systems in your treatment plan. An important aspect to pharmacologic treatment is realizing that many EDS patients have nutritional deficits due to their underlying GI and digestive impairments. And so nutritional supplementation is definitely something that's being researched, and you'll wanna consider repleting if it's low. This article actually proposed various doses of supplements for common symptoms that were experienced by EDS patients. And some other things you wanna consider are things like vitamin B6, D, B12, zinc. You wanna replete these things if you find that they're low. It's important to consider various interventions to assist patients with pain, instability, spasm control. And patients tend to have a significant number of trigger points, right? We talked about that the blanket alone can trigger trigger points into your calves. And the thought is that the unstable joints tend to provoke trigger points in the surrounding muscles because they're being overworked for trying to keep them stable. So it's really reasonable to consider trigger point injections and even do joint injections or nerve blocks to assist with pain. But one thing that you wanna consider is that 60% of individuals with hypermobility may notice a poor response to local anesthetic. And so let me tell you a little bit about that. This Danish group was looking at skin strength in EDS patients, and they actually noticed that patients experienced a lot of pain despite conventional local anesthetic attempts. So Ardent Nielsen took that information and subsequently compared the effects of local anesthetic in eight EDS patients versus eight controls. And they found that the effects of intradermal lidocaine were much shorter than controls. So based on that information, in 2003, a survey was completed of 172 hypermobile patients, and it was found that roughly 60% of individuals noticed a poor response and felt local anesthetic for dental procedures in minor surgeries was simply ineffective. And so over time, it's been found that it's really not that uncommon for EDS patients to require larger doses of anesthetic or even different anesthetics. And so as an interventionalist myself, it's really crucial that we pay attention to what has worked for our patients in the past and trialing different medications because you want to improve your outcomes and make sure that they have a good experience with whatever treatment options you're gonna provide. You may also wanna consider chemodenervation or neural lysis or even surgery, right? But it's important to weigh that risk versus benefit ratio. For example, from a Botox perspective, this is a paper that talks about how while we use Botox for pain and dysautonomia migraines, they found that Botox can actually worsen head and neck instability. And they found that it worsened pain and even could cause some serious injury. They felt caution should be used for all Botox injections in EDS patients of the hypermobile type. And they said that muscles involving head and neck stabilizers should be avoided altogether. Some groups are using prolotherapy and there are some articles out there on how prolotherapy can help tighten the ligaments and allow joints to stabilize better. And the EDS Society even talks about it in their recommendations for treatment options. And the thought is that prolotherapy works by initiating a brief inflammatory response, which causes this reparative cascade to generate new collagen and then hopefully improve the strength in the ligaments, joints, tendons. So I feel like the consultation tab is really essential. You know, EDS is not a syndrome that should be treated by one practitioner alone. And I don't think anybody would expect you to treat it alone. It really is a multidisciplinary team approach that needs to be employed. And one of my patients said it beautifully. She said that she really felt like I acted like the quarterback for her care. And I thought that analogy was great because we as physiatrists look at the whole patient and we can help guide the treatment process. And we do it effectively by actively listening to what our patient is experiencing and what are they looking for. And you can use that information to guide your team and grow your team. And the list is endless to help you treat these patients. But one provider that I really feel strongly should be on most EDS patients' cases is counseling services. The prevalence of comorbid psychiatric sleep and fatigue disorder has been very clearly shown in EDS patients. Pain, negative feelings, poor emotional regulation are all commonly associated with that condition. And so it's really important to have those issues explored and treated. And the international consortium actually recommended utilizing CBT therapy in EDS patients. If you have pediatric patients, it's really important to not forget about engaging school resources. The EDS Society has this guide that you can provide to educators as well as parents. And the whole goal is how can we make sure that kids are having a good experience at school? How can we let the educators know what these kids are going through? And how do we make sure that they stay in school? The last part of my Team ICE approach is education. So you wanna make sure that your patient understands your thought process and your plan. You wanna help them understand their disease, things that they should avoid or things maybe that they should start implementing. And for example, yoga is okay for these patients, but maybe high impact sports should be avoided. Maybe they wanna avoid excessive sun exposure because of the tissue fragility. Maybe they wanna avoid hard foods and chewing gum because that can increase the risk of jaw injury or TMJ. If you have a vascular EDS patient, I would probably tell them to avoid playing wind or brass instruments as that can increase the risk of collapsed lung. So it's really important that you educate your patient on their disease because we know that education leads to prevention, prevention leads to decreased injury and improves quality of life. So with COVID-19, I know patients and clinicians are concerned, what do I tell my patients in terms of their health and risk? And the EDS Society has put out a position statement on the risk of COVID with EDS. And in a nutshell, it talks about how EDS itself does not increase your risk of getting COVID. However, there are some key points you need to be aware of. When talking to your patients, you need to go over their baseline symptoms so that they have a gauge of what to assess if there needs to be concern about getting tested for COVID. You need to know that 15 to 20% of EDS patients have baseline symptoms of wheezing and coughing. We talked about 33 to 75% have GI symptoms and that includes things like nausea, diarrhea, constipation. Cough, fever, fatigue, sore throat, those are all common symptoms in adult. So it's important though, you go over these symptoms with your patient so that they have a baseline to kind of gauge if things worsen. The other factor is to pay attention to what are the patient's preexisting health conditions, if they do get COVID. And so for example, dehydration from COVID can worsen POTS syndrome or if you have poor lung volume to begin with, that could increase your risk of pneumonia. So again, education is key. And a few ways I've altered my treatment plan during COVID is really I've started to focus on education and self-engagement in my patient's own treatment plan. You know, some ways I'm doing this is utilizing a lot of the free apps that are out there for mental health, activity, sleep. So activity, Peloton's offering three months free, Nike's offering free. Meditation wise, you've got Headspace, Stop, Breathe, Drink, there's 10% happier. If you even just have the patients Google some of these things, there's a lot of free stuff out there right now that can keep them mentally fit and physically fit. Sleep is really important. There's the CBT iCoach app that is being offered for free right now. It's really important that counseling not be forgotten about, right? So you can do telehealth counseling, support groups, but it's really vital to get them engaged even during COVID because you wanna identify any psychological factors to pain and disability. And it's important to treat underlying anxiety, depression and added on anxiety and depression because of COVID. And so my social worker has been really great in getting me telehealth counseling services for a lot of my patients. So if you need help with that, I encourage you to utilize your resources, your case management, your social work, they're great assets during this time. In addition to all this, make sure that your patient, social distancing it's necessary right now, but that doesn't mean you can't still socially engage whether it be via Zoom or free support group apps. A lot of the support group apps even have like HIPAA compliance. So it's really important that you give resources to your patients. And while we don't have control over COVID, we do have control over how we make use of our time right now. So going back to my patient, I did feel pretty overwhelmed when I saw her, but after hearing her goal of simply wanting to get to a place where she could get to college in the fall, I felt like, you know what, there are things that we could consider. And now it's been about 10 to 11 months that I've been seeing her and we've accomplished the following. So we got her to therapy, PTOD. She was educated on bracing, equipment. She actually did the CHOP graded exercise program as well and did really well with that for her POT syndrome. We got her finger splints, elbow and knee braces to prevent recurrent hyperextension. She actually now does aerial yoga and she found that that has been really helpful for her ligament strength, as well as for her mood. It's gotten her back into society. For equipment, we got her a manual wheelchair so she can have independence when she goes to school. We got her Ted hose. For adjunct therapy, I did get her into acupuncture and she was seeing an acupuncturist, but unfortunately with COVID now, that's kind of been put on hold. Medication-wise, we trialed quite a few medications and we've had a lot of success with Baclofen at night and then Cymbalta during the day. She is one of the patients that is on CBD oils and she swears that it does help with some of her joint pain and so that is something she does employ. For interventions, she's done really, really well with the combo of trigger point injections and bracing. And we've even done a suprascapular nerve block for ongoing shoulder pain that she was having and she had really great results. I wish this consult tab was a lot larger because this looks like I've only done three things with her but we've gotten her to cardiology, endocrinology, nutrition, psychology, genetics, and pulmonology. Cardiology, it's really important to go over, do they have any cardiac issues? Get them a baseline echo, baseline EKG. She's getting treatment for her POTS with cardiology. And so it's really important that consult tab should be a lot larger and it's just really important to utilize your resources and the other providers that are in your institution. And most importantly, education. I really feel like that's one of the most important tabs because the more that you educate your patient, the more that your patient's knowledge of their own condition grows and gives them confidence and independence. And I can tell you my patient is very excited and comfortable with going away in the fall. So in summary, I hope that, you know, this case really didn't scare you away and instead it provided you on some guidance on the basics of EDS, helped you see that no two zebras have the same stripes. And most importantly, I really hope that it helps you realize that if you take a deep look at these zebra cases, they really don't have to be so scary. Any questions? Okay, thank you so much Dr. Sharon, this is Jackie Tillworth, I'm the Marketing Communications Manager for the AAUP. Sterling had to step out because he had a fellowship interview to take before the closing. I don't see any questions coming through for you, but would people be able to reach out to you via email? I can actually, in the group chat, I'll just give you, I'll type out my email here, it's really long. Okay, I can also, I think I just shared my screen so people can see it. Oh yeah, that's perfect, it's right there. Yeah, if anybody has questions, please feel free to reach out, I'd be happy to get you answers if I have them, otherwise find them. Awesome, well thank you so, so much for being a part of this, it's been this past month or month and a half of doing this virtual didactics and we're so grateful for everybody that's volunteered to present. Well, I appreciate you guys having me. Yeah, for sure.

Ehlers-Danlos Syndrome

Connective tissue disorders

Multidisciplinary approach

TEAM ICE

Physical therapy

Custom equipment

Adjunct treatments

Patient education