Episode 37 – The Relationship Between POTS and Ehlers-Danlos Syndrome (EDS)

00:01 Announcer: Welcome to the Standing Up to POTS podcast, otherwise known as the POTScast. This podcast is dedicated to educating and empowering the community about postural orthostatic tachycardia syndrome, commonly referred to as POTS. This invisible illness impacts millions and we are committed to explaining the basics, raising awareness, exploring the research, and empowering patients to not only survive, but thrive. This is the Standing Up to POTS podcast.

00:29 Jill (Host): Hello fellow POTS patients and peachy people who care about POTS patients. I'm your host, Jill Brook, and today we have an episode of The POTS Basics with our favorite neurobiologist who is working tirelessly to make our lives better. Today we're going to review the basics of Ehlers Danlos syndrome with Dr. Cathy Pederson who is a biology professor at Wittenberg University, a POTS researcher, a POTS parent, and the President and Founder of Standing Up to POTS. Thank you for being here again, Dr. Pederson.

01:03 Dr. Pederson (Guest): It's my pleasure. Thank you, Jill.

01:05 Jill (Host): So Ehlers Danlos Syndrome - why is that so relevant to POTS basics?

01:12 Dr. Pederson (Guest): You know it's really interesting. I think a lot of our listeners either have Ehlers Danlos Syndrome, or they've been investigated for it, or they're wondering if they have it. And I saw this statistic in one of the articles I was reading that’s really interesting that said that 80% of people with Ehlers Danlos Syndrome have POTS. But it's actually not the opposite - so it's not 80% of people with POTS have EDS or Ehlers Danlos Syndrome. But it is really common for people to have that Ehlers Danlos Syndrome and then develop POTS. Ehlers Danlos Syndrome is the group of 13 disorders. So it's sort of like dysautonomia - it's an umbrella term that's talking about a number of different conditions within it. We're going to focus in this episode on the one that's most common for POTS patients, and that's called type III or hypermobile EDS. And this particular form of Ehlers Danlos syndrome is really common, as I said, with POTS, but also in folks that have fibromyalgia or chronic fatigue syndrome or myalgic encephalomyelitis, depression, migraine headache. So we see this sort of in that mix of chronic illness in the way that we see POTS in that mix of chronic illness for lots and lots of folks.

02:35 Jill (Host): And I think some newer research just came out - at least one study - showing that it was a little bit more represented in the patient population with autism spectrum disorders. And so, there's that too. So yeah, it really does keep popping up.

02:50 Dr. Pederson (Guest): That's really interesting. I don't think I've seen that article. I did see an article saying that maybe they have a gene for it. So, virtually all of the other 13 forms of Ehlers Danlos Syndrome, they've identified a gene or a cluster of genes, and that's not the case yet for the hypermobile type that again is affecting POTS patients. But there's a study of a large family that had lots of Ehlers Danlos Syndrome in that family that all seem to share a gene. So it's five or ten years of research really to figure out whether that's generalizable to the whole population. But it's going to be really interesting to see, I think.

03:39 Jill (Host): Yeah. OK, when should somebody suspect that maybe they could have Ehlers Danlos Syndrome?

03:34 Dr. Pederson (Guest): Ehlers Danlos Syndrome affects one in 5000 people worldwide. And again, we see it much more in this chronic illness community, but Ehlers Danlos Syndrome affects a protein in the body that's called collagen. And collagen is actually a really, really important protein that a lot of people haven't heard of. In humans, about 25% of all the protein in your body is collagen.

03:58 Jill (Host): Wow.

03:59 Dr. Pederson (Guest): Yeah. So, in Ehlers Danlos Syndrome, different types have different problems with this collagen. But collagen is found in something that's called "connective tissue." And connective tissues support your skin, collagen is part of your bones, it's found in the lining of your blood vessels, of your organs - so many different tissues. And so, we see that it ends up being a huge problem if that collagen protein is abnormal, right? If it's mutated because of some genetic change. So here's who should think about or wonder if they have Ehlers Danlos Syndrome: it's people that have loose joints, right? Boy, we could take that in a lot of different ways, but I mean literally elbow joints, shoulder joints, finger joints that are lax. Maybe you're double jointed. Maybe people had talked about being double jointed when you were a kid. They’re more loose. You can do party tricks that other folks maybe can't do with those joints. A second one is soft, velvety skin. Ad my daughter did get diagnosed with Ehlers Danlos Syndrome, so she's one of those that has Ehlers Danlos Syndrome and POTS. And the doctor who gave the definitive diagnosis - there are a number who said, "Oh yes, of course she has EDS" - but the one who really said it strongly closed his eyes during the examination and felt her arm. And he said, "Yep if she's not pregnant, she's got Ehlers Danlos Syndrome. I'm like, "What??!!" And he said, "Yeah, the skin feels different because of this collagen, as it turns out, so it's softer and more velvety." And so, you know how doctors are, they sort of run in and run out. And so, he ran out of the room and I had both of my daughters there. So, one has EDS and POTS, the other one does not. I'm like, "All right, ladies. Present your arms. I want to feel the difference." And I'd never noticed it, you know? Lily was probably 11 or 12, it took us a while to get to this one. And, you know, I'd had my girls for a long time. I'd never noticed really this difference. There's a real difference in the softness of that skin. So that soft kind of velvety skin. Easy bruising is another one. Now there's a particular kind of Ehlers Danlos Syndrome that's called Vascular Ehlers Danlos syndrome. That's a little bit different. I'm not going to talk about that one so much, but that Vascular EDS there's lots of bruising, but then they can also end up having an aneurysm or something like that. People with Hypermobile EDS will also have that easy bruising. And so if you're like, "Wow, I don't think I hit that very hard." If you're like me, I bump into things all the time. I don't think I hit that that hard. I don't feel like I should have a bruise. That person with EDS will get the bruise. The other thing, interestingly, and again, I think this is so comorbid with POTS it's hard to tell this stuff apart, but lots of people with EDS also have issues with the gastrointestinal system and cardiovascular system. So cardiovascular of course could be POTS, but think of all the folks with rapid gastric emptying or the opposite of that which is gastroparesis, or they've got irritable bowel syndrome. So many different things. And so again, we know that that's common in folks that have EDS.

07:28 Jill (Guest): Well, and what really stuck out in my mind was when you said what percentage of the body's proteins is collagen?

07:37 Dr. Pederson (Guest): 25%.

07:39 Jill (Host): So, of course it can affect the whole body and it also reminds me, I think it was Dr. Brad Tinkle, who said it – it might have been someone else, I apologize if it was - but I think that he had the little saying, "If you can't connect the issues, think connective tissues." And what was meant by that was, if things are going wrong in all different parts of the body and you can't figure out why would this person have POTS and achy joints and subluxations and GI problems, that then maybe it's time to think about the connective tissues. And so this really helps explain why. And when you think about collagen being too flexible or too soft in the skin or in the joints or in the blood vessels or in the GI tract, it makes sense that all this stuff can go wrong. OK, what would be kind of the hallmark of your daughter's Ehlers Danlos Syndrome? I know you've mentioned to me that she maybe has had quite a few subluxations or dislocations?

08:40 Dr. Pederson (Guest): She has. Lots of people with EDS - the hypermobile type - are concerned about joint instability, meaning that their joints don't connect, and articulate is the way I talk about it in my class, where the bones stay in position, the way that they should. And as a result of that, they can either have a dislocation where the bones completely disconnect, or a subluxation where maybe it goes 25% or 50% out of position, but not all the way. The bone didn't completely come out of its socket, to put it sort of in layman's terms. And so that joint instability - either the subluxation, very painful where it slipped a little bit out of joint but not all the way, or dislocations where they've gone all the way out - are really really common. And unfortunately, as we were talking about with collagen, it's not just found in one or two joints. This is what's making up our tendons and it's making up our ligaments and it's help holding all of these joints together. And so you find this in the extremities in the arms and legs. Sort of famously, I think about contortionists. I think most contortionists probably have Ehlers Danlos Syndrome or something similar, where they pop that shoulder out, they fold themselves around in ways that most of us couldn't do even when we were young, right? They can also have subluxations or dislocations in the vertebral column, and I think the ribs are particularly common. And I'll tell a story on my poor daughter. She's really sweet about all this folks, by the way. She really wants people to learn from the things that have happened to her. But she was probably 12 or 13 years old and my youngest often went shopping with me, but Lily was too ill. She just couldn't go, and I think a lot of our listeners understand that. And so we were out just shopping for food and whatnot and I came back and she's like, "Mom, I gotta talk to you." And I said, "OK hun, what's going on?" And she said, "Mom, I think I'm having a heart attack." Now, she's you know 12, 13, 14, something like that. Heart attack is way down on the differential diagnosis, but she's got this really crushing chest pain. And I knew immediately what it was. We already knew that she had EDS, but I hadn't talked to her about this because I didn't want her to worry about it. And she said, "Mom, I’ve marked on my chest where it is," and she had taken a pen and drawn above and below the rib that had subluxed, but she didn't know what it was. She thought it was a heart attack. So she's Googling, as every good teenager would, and came up with heart attack. And then she said, "But the second thing it could be maybe is that I subluxed a rib." And I said, "Honey, that's exactly what it is. You subluxed a rib." And she hadn't done anything - she was sitting in the recliner, as she often did at that point in her life. And she was just brreathing is what she'll tell you, and all of a sudden that rib just slipped and she had this terrible pain. It took us about 24 hours to get that thing to go back into position and for the pain to stop. And so, we were heating and we were icing and we were putting pressure for her using like a Ace bandage and trying to put compression to force it back into joint seemed to help a little bit, but that was really scary for her for sure. Could be the same sort of thing with the clavicle - that's your collarbone. Some people get it in the jaw. You hear people talking about TMJ - that stands for temporomandibular joint - so they'll have a lot of pain in the jaw. My daughter has a lot of problem with that right now. And so I've noticed in the last year is that her jaw is subluxing that she doesn't open her mouth as much when she talks, and I have trouble understanding her. She talks fast but she doesn't open her mouth very much. So she makes me feel like I'm deaf in my old age. But I think she's trying to prevent that joint from slipping out. Other things that are common spraining the ankle or twisting your ankle all of the time where it seems like it just gives out under you. Buckling of the knees - very common in folks that have EDS. And then some people have - there's a band, it's like fascia on the side of your leg - it's called the iliotibial band. So, if you just go straight down from the side of your hip and it'll snap and it hurts. But it also makes them feel like they can't trust that hip. So that's another thing you can see. All of that goes back to sort of that cardinal sign, which is joint instability because of this darn collagen.

13:21 Jill (Host): And in the patient community, I think what we hear so much from people is the frustration of just everyday things, and especially the one that comes up is just getting on your compression stockings can be really tough on the finger joints or the elbow joints or the rib. And I know we're going to be talking about a few things that people can do, but this is really painful, right/

13:44 Dr. Pederson (Guest): It is. And when that joint is subluxing or if it's dislocating, it’s exceedingly painful for that person. It's all sorts of daily issues become a real problem for people with EDS, especially if they're on the more severe end of that syndrome.

14:02 Jill (Host): So, we were talking about pain and unfortunately I think there is pain from the subluxations and dislocations, but then there's also more pain. Can you talk about that?

14:15 Dr. Pederson (Guest): That's exactly right. And for our listeners, who are like my daughter, who both have POTS and EDS, both of these are related to pain conditions. And so for my daughter, it's hard to know whether her pain comes from POTS or whether it comes from EDS. So a lot of our listeners are going to say, "Yep, I know about these." So, one is muscular pain. Sometimes a technical term they'll call it "myofascial pain", but this is pain in that muscle, sort of like when you have the flu - just that aching of the muscles all the time. Sometimes they're throbbing. They can be really stiff, especially if that person hasn't been up and moving. So maybe first thing in the morning or after they've been sitting for a little while. So, muscular pain is one. My daughter has some of that, but her biggest pain problem has been neuropathic pain. It's a little bit different. She doesn't have a lot of pain in her joints per se, unless she subluxed something. But this neuropathic pain has been a real problem, and for her it's changed over time. So sometimes it was like a shooting pain. She felt like there was electricity shooting down her legs or lightning was hitting her legs. Other times she would describe it as a burning pain, and I think now it's more numbness and tingling. So, she's had changes in the quality of that pain, but the pain itself doesn't really go away, it just changes the way that it feels. What we also see is because of the laxity of the joints - sorry folks - people with EDS as they get older are much more likely to have pain from osteoarthritis. That's the normal sort of joint arthritis that people get as they get older. But because those joints are taking extra pressure and don't have that same support from the collagen, that's even more likely as we get older.

16:05 Jill (Host): And I think that's one reason why a lot of the experts would say, just because you can do those party tricks you maybe don't want to because that is more wear and tear that you might have to pay for later, right?

16:16 Dr. Pederson (Guest): That is exactly right. Exactly, especially the ones that are pulling on the joints, which we're going to talk about in just a second here.

16:24 Jill (Host): OK, in Ehlers Danlos Syndrome is, you know, another one of these super complex things, and as such I think it's another thing that's not that easy to get a diagnosis for, correct? You have to see the right person. It's not just a simple blood test, it's kind of the whole process?

16:45 Dr. Pederson (Guest): That’s exactly right. It's very similar to POTS in that way. So right now we don't have a gene for this particular form, so a blood test isn't going to show anything that's particularly abnormal. Same thing with urine tests, MRIs - again, very similar to what we see in the POTS community, which is frustrating. So, here's how they do it for the hypermobile version of EDS: There are three criteria and I'll go through each of them, but let me just give sort of the overview first. The first criteria is what we've been talking about largely, and that’s that joint hypermobility, and it has to be generalized so it can't be just your pinky finger, right? Has to be really throughout the body. The second thing that they have to be able to show is that they've got a syndrome - that they're showing a variety of symptoms that point to EDS and/or that they've got a family member or a sibling that also has it. So again, we think it's genetic even though we haven't found the gene yet. And then the third one is that the health care practitioner needs to exclude any alternative diagnosis that could explain these symptoms. So you have to have joint hypermobility, you have to show this syndrome, and you have to exclude anything else. Alright. So you ready to jump in?

18:06 Jill (Host): Yes. So, how do they do number one? How do they grade your degree of generalized joint hypermobility and whether it's bad enough to qualify?

18:17 Dr. Pederson (Guest): It's really cute how they do this. [Laughs] So there's something a Beighton scale, and I'll put a link to our web page and I've got all this information on our web page on underlying causes and I'll have a link to this scale so people can go and look it up. It's a 9-point scale and basically what they do is they ask that person to do the "party tricks", OK? And what I mean by that is someone with EDS may find themselves in a weird position that feels normal to them that other people are like, "Whoa, what are you doing there," right? So here would be one: you get a point for bending your pinky finger backwards more than 90 degrees, OK? And if you can do that with the right one, you get one point. And if you can do it with the left pinky, you get another point.

19:05 Jill (Host): I think every listener is doing that right now and checking.

19:08 Dr. Pederson (Guest): I think so, right? Check it out. Absolutely. Get one point for pulling the thumb all the way down to the forearm so you take your thumb and you pull it right down to that arm. If you can touch your thumb onto the arm, you get a point for each side. One point for hyper extension of the elbow, and it's got to be above 10 degrees, and so they've got special instruments that they can use to measure this. But most people aren't hyper extendable, right? It goes straight, it doesn't go past straight. So for each elbow that goes beyond normally straight by 10 degrees would get a point for each side. Same thing for the knees. So they look at the hyper extension of the left and the right knee, each one getting a point. And then the last one my daughter did with such feminine vigor that they laughed and that is taking both palms and putting them all the way down on the floor with your knees being straight. In general, you need a score of 5 out of 9 or more for an adult to get the EDS score. It actually varies a little bit by age. So if you're my age - if you're 50 or more - it can be 4, 'cause we get less flexible with age, even if you have EDS. On the flip side, if you're a kid like my daughter was when she was diagnosed, you need a score of 6 or more, because kids are just a little bit more flexible than adults are. But in general, you need 5 out of 9, for this criterion one. But that's only the first part, OK, and so our listeners may have been doing that as we worked our way through. And if you got more than 5 out of 9 on that, you move on to criterion two. And there are three parts to this. This is hard to do on a podcast. It'd be nice if I had something that you folks could look at, but I'll try to put on my professor hat and do as well as I can here. So we have to have two of the three things. So the first feature, they call it feature A, they have to have 5 or more signs of a connective tissue disorder. So again, folks, you can mark this off in your head as we go through here. That soft, velvety skin that I talked about. If you're not sure if you have it, compare with someone else, right? [Laughs] Second party trick, I had a student do this a few years ago in my class and of course I teach about all these things - I'm trying to help our chronic illness community here - and he could take the skin of his neck and pull it out past where his ears are. And so if you can get a skin flap - and it doesn't have to be that far - a centimeter and a half, just showing that your skin is extra stretchy, that would count as one of these. We need five of these. So we've got the soft skin, the skin flap. If you have striations, like lines on your torso or leg that are not related to something that you know about. So maybe not from having big weight loss or something like that, they've just appeared. That can be a sign.

21:59 Jill (Host): So, stretch marks is that what would be considered also?

22:02 Dr. Pederson (Guest): Yes, stretch marks but not related to pregnancy and not related to weight loss. Yeah, perfect thank you. Any kind of abdominal hernia, except the hiatal hernia. Hiatal hernia is going up into your chest, but if you've got a hernia around your belly button, we call that an umbilical hernia. And then there's an inguinal hernia where the testes drop down into the scrotum having a hernia there. There's also a femoral hernia that people can have. So, one of those would also count here.

22:33 Jill (Host): Do you mind putting on your professor hat and just explaining what a hernia is?

22:38 Dr. Pederson (Guest): Yes, a hernia is where you have body contents moving to a place where it shouldn't be. An umbilical hernia, right around that belly button, you can have an opening in that tissue in the muscle, and some of the intestines can actually poke out through there. And so early on, what people can do is, they're like, "Oh, that's weird. I don't like that." They take their finger, they push it back in, but you really often need to get that fixed surgically. So those are examples of hernias, right? Can't be just one, you have to have a recurrent. So at least two of these hernias. Scarring - really, really common and it has to be in at least two sites. I'll say my daughter, before she was a year old, her fingernails got a little too long and she scratched herself on the back of the neck and she had a scar there for more than a year, just from like scratching an itch. So, it could be from surgery, it could be from something that again most people would not scar from. And usually that scar will be wider, like if it was an incision, it'll be wider than the incision mark was, and it'll be a little shallow depression or trench there. The next one - sorry folks - is something called prolapse of the pelvic floor, could also be the rectum or the uterus. And before Jill asks me, I'm going to tell you what a prolapse is. This isn't very nice, OK. Plug your ears, maybe here listeners. I'll use the uterus as an example. The uterus should be laying on top of the bladder. And what can happen sometimes is the uterus literally falls down - they call it prolapsing - through the vagina and it can actually come out of the vagina. So it sort of inverts and drops down. Can be the same thing with the rectum can do that. And then some of the muscles in the floor of your pelvis can also do that. So that can be a sign of EDS. I don't know that it's a common one, but it certainly would be one that would say, Hmm, I think maybe connective tissue may have some problems here. Dental crowding. So, my daughter, my youngest one is just about to get three wisdom teeth out. My one with the chronic illness got all four wisdom teeth and two 12-year molars taken out. So, she had six impacted teeth and a lot of crowding of teeth in her mouth. Now does everyone who needs braces or has impactions, do they have EDS? No, but it can be a sign. Another one - I had a friend who called this the "gorilla factor". This isn't necessarily just EDS, there's another one called Marfan syndrome that can also be this, maybe some others, where the arm span is actually longer than the person’s height. So, I'm 6 feet tall, as it turns out. So, if I have EDS, maybe my wingspan from the tip of my middle finger to the tip of the other middle finger is 6'1", right, as an example. Ad then the last one - very common in young women, even if they don't have EDS - is mitral valve prolapse, and so for this they want to be pretty strict. It's got to be from an echocardiogram that we're looking at that. But you have to have for this feature of criterion 2, at least five of those: soft skin, the skin flap, these stretch marks that you don't know why they're there, hernias, scarring, weird prolapses, dental crowding, the gorilla factor, and mitral valve prolapse. So if you counted five of those, you're still in the running for EDS.

26:10 Jill (Host): And my understanding is that experts and patient advocacy groups are working on revising these to make them less burdensome because some of them are definitely very objective, but some of them are a little bit hard, like who is to really say if your skin is soft or velvety enough, or what is enough dental crowding? So, some of these are admittedly kind of vague, but I think that the patient and expert groups are getting together and trying to improve them.

26:42 Dr. Pederson (Guest): Yeah, it's really hard when we don't have a good solid diagnostic test. And so you're right, we're clustering symptoms or potential symptoms together to say, Hmm, maybe together. And that's why you need five of them, why you don't have one or two, to say yes, I think that this is a problem. I have to say, when I was reviewing for this, what I had on the website was actually much less stringent than this. This is new coming out of NIH - The National Institute of Health - and so that's what we're presenting here. So, this is the new up-to-date stuff. I'm sure there's more in the works, though.

27:19 Jill (Host): I also just wanted to mention, you know we had kind of talked quickly about the prolapses and how those might be less common, but I actually know a few people who have had that happen, and it was very, very scary. You know, it's not life threatening, but it certainly is shocking when it feels like your insides are coming out of you. But I know not one, but two people whose physicians would not believe them, and they ended up having to wait until it happened again so that they could take a picture to prove to their physician that it had happened. And so that makes me feel fairly disturbed, but I just mention it in case there's people out there, I don't want them to feel alone.

28:04 Dr. Pederson (Guest): Yeah, that's a great point. I think that's shocking, and I think, you know, other folks have talked about not being believed, and why would I lie? This is one of those that who would make something like that up? And it's painful, too. I ought to say, not only is it shocking that it's happened, but it's really painful as it's occurring. But to have to wait, to almost be shamed, like I don't believe you unless I see a picture, seems unbelievably cruel. We should not have to prove things like that at all.

28:36 Jill (Host): OK, so that is two of the three criteria groups. Can you remind us what the third one was?

28:43 Dr. Pederson (Guest): Well, I'm afraid we only got the first one. The second one is really fast, though, and that is you have to have a family member or a first degree relative who meet the criteria as well. That's feature B of this second criterion. Again, we think it's a genetic trait, and we think it's something called 'autosomal dominant', which means that if I have it, each of my children has a 50/50 chance of inheriting that same EDS, in this case. So you have to have a family member who has it or meets the criteria. And then the last one is that you have to have at least one kind of musculoskeletal complication. So that could be musculoskeletal pain - so pain in the bones or the muscles - in two or more limbs, and it has to be daily pain for at least three months. And again, lots of folks in our community would meet that criteria pretty easily, or it could be chronic widespread pain for at least three months. Or those dislocations or subluxations that we were talking about before, where there's no trauma. So the idea that the jaw just slips out when you're talking, you weren't hit, nothing in particular happened. My daughter, when she was younger, her hyoid bone in the neck would sublux when she yawned. And so she'd start the yawn and she'd end up screaming, right? Well, there's no trauma there. It's just that this thing is moved out of place, this bone has moved out of place. And so those are the features that you have to have for Criterion 2. So again, I know this gets messy – Criterion 1 was the joint hyperextension, Criterion 2 is showing you've got a syndrome, right? And as Jill pointed out, this isn't pretty. It's sort of a mess of things. And how do you know how much, right? But they're trying to firm that up. And then Criterion 3 basically says that they've ruled out anything else that could possibly cause all of these symptoms. And so they've done their due diligence and there's nothing else going on that would account for the pain or the subluxations, etc.

30:54 Jill (Host): OK, whew. That's a lot, and I can see where it's such a process. And my understanding is that a lot of patients will undergo the genetic testing to rule out or see if they do have other kinds of Ehlers Danlos Syndrome as well. And so that can be a whole big process. But let's say that somebody does determine that they have it. How can they manage it?

31:18 Dr. Pederson (Guest): So unfortunately, this is also like POTS. There is no magic pill, at least not yet. In this case, we don't have a gene, just like with POTS, right? I mean, both of these disorders are so similar and frustrating in the fact that we just don't have terrific treatments. So a lot of it is ways to live with the disorder. Again, unfortunately very similar to what we see with POTS. So physical therapy can be a big one that we have here and the whole goal of physical therapy is to build the muscles around those joints in a way that's even, so that it supports them. So we don't want one muscle group around the knee, let's say, to be really strong and on the opposite side of the knee it's really weak. That's going to allow those subluxations or dislocations really easily. And so the physical therapist should be assessing where you're strong, maybe where the muscles are weak, and really work on getting an even condition of those muscles around those joints.

32:25 Jill (Host): I think another part of that also is letting patients know when it's not great for them to purposely stretch more. I used to think that stretching was a great habit and that you always wanted to be more flexible, if you could be more flexible, and I wish I had learned earlier that, nope, there can be joints for which stretching is counterproductive, because more flexibility just means less stability.

32:53 Dr. Pederson (Guest): Especially in this population. If they've got EDS or EDS and POTS, you're absolutely right. You do not want to be doing some sort of morning or evening stretches thinking that it's for your health. What you actually want is to tighten those muscles up so that you get more strain on that connective tissue and you hold those joints really, really stable. That is an excellent point. The other thing I'd say there is, boy, we don't want, you know, out running 3 miles. We don't want you running a 5K. You could walk our 5k but don't run it because we want this to be low resistance exercise. So you want to be doing things like biking or swimming or walking, walking your dog is great, but you don't want something where you're pounding the pavement or you're pulling on things. Any kind of a pulling machine or a Bowflex where you're pulling on something could pull the joints of the elbows, the wrists, the shoulders, and be really problematic for folks that have EDS. So you want to avoid that kind of a weight machine. Now pushing might be better, but anything where you're pulling can really be problematic. That includes the rowing machine. So if you look at the Levine Protocol or CHOP came out with a revised version of that. [Transcriber’s note: the Levine Protocol was developed by Dr. Ben Levine, a cardiologist in Dallas, who originally developed the protocol to help NASA astronauts experiencing orthostatic intolerance upon returning to Earth. CHOP refers to the Children’s Hospital of Philadelphia, which modified the protocol. You can find more information on our website - https://www.standinguptopots.org/livingwithpots/exercise ] They really like swimming, which is excellent for people with POTS and EDS. But also that rowing machine, if you've got POTS, that pulling motion back can be really hard on those shoulders and elbows and wrists, and so that should be avoided, if you have EDS. So again, pushing's OK, pulling is not so great. So you want to keep that in mind if EDS is part of it. Anything, like I said, that's high impact, whether it's running, jumping on a trampoline, which can be a lot of fun, right? But think of the impact on the body with that. Anything like that ought to be avoided by people that have this hypermobile EDS.

34:57 Jill (Host): OK, so that's a little depressing. So, the trampolines out, water skiing sounds like a bad idea. All the things that you might do for fun and kicks as a little kid that just take a pounding on your joints, maybe try to lay off. But what if it's too late for you? What if you've already accumulated some of this wear and tear?

35:18 Dr. Pederson (Guest): Or you just have a severe case, right? I mean, it's not necessarily that people did something that made their joints worse, but that they just have more issues with their collagen than others. Yet there are all kinds of assistive devices and braces and things that people can use. You’ve got to be a little careful with some of these and you definitely want to talk to an occupational therapist or physical therapist or the doctor before you use them too much, but braces can be great. Now, my daughter, when she was younger, had bones in her wrists sublux, and you could actually feel it, right? And it was really problematic and very painful, and it seemed like she was sleeping in odd positions. And so I bought her wrist braces and she sleeps in them every night. She does not wear them during the day. Now this can be a great tip for our friends with EDS. A lot of people - think about when you sleep, you sort of wind yourself around, you maybe bend your wrist way back or way forward. You've got pressure on your arm in weird ways. And so keeping that straight while she sleeps has really stopped those subluxations for her. Now, here's where my caution goes: You don't want to wear them during the day because you don't want the muscles to atrophy. It's really important that you're using those muscles and trying to keep them active and healthy as much as you can. So that kind of brace that helps with joint stability at night can be great. Now I know people, though, that have really severe EDS in their fingers. And we had a great episode, I think it was Episode Three with Joanna Behm. [Transcriber’s note: you can listen to that episode of the POTScast here: https://the-potscast.castos.com/episodes/episode-3-occupational-therapy-tips-for-pots-patients-with-occupational-therapist-joanna-behm ] That was one of my favorite episodes because she went through all of these strategies if you're having this problem with POTS, but a lot of it was really towards EDS, do this. If you've got this problem OK, let's figure out how to do this instead. So I would encourage folks that are listening to this and saying, wow, I think I might have EDS, go back and listen to Episode Three about occupational therapy with POTS and EDS, if you have not done it already. But there are splints that you can get for the fingers. And there are a variety of different kinds, so if you have particular joints and when you're cutting carrots for dinner or something like that, you can wear them. There are all sorts of things that you can buy that will help with that. But again, when you can avoid wearing them, you want to avoid it, OK? there are writing utensils, so maybe getting things that are a little bigger in the hand so you don't need as much small muscle dexterity. And less pressure can be a good thing. So anything that would reduce the strain on the fingers or the hands can be really helpful. I want to go away from that a little bit. I had a student who had really bad EDS and was actually in her 20s by the time she was diagnosed with that. And really woke up in pain, terrible shoulder pain, terrible hip pain, and ended up really leaving school for a good part of the semester. Was a champion, did a lot of work from home. But really lived on an air mattress 'cause she couldn't take anything that was less forgiving than that, and had surgery on her hip to try to tighten some things up and get some stability back in that tissue. And she came back to school with a crutch and with a brace for one leg, really trying to support that hip. And she's slowly improving and just got the hip brace off. But it can be bigger braces, is my point, so it could be something for the hip. There are other folks who feel so unstable, maybe with the hips, the knees, the ankles, that maybe not in the house, but when they go out other places, they'll use a wheelchair because they're worried about that collapse and injuring themselves and then having a subluxation or dislocation as part of that.

39:28 Jill (Host): You know, as far as the pain goes, that tends to be - pain management tends to be - a big part of it too, right?

39:35 Dr. Pederson (Guest): Absolutely, and it's tricky. And so again, think about that muscle pain, the neuropathic pain that we talked about a little bit earlier, and it can be hard to shut that off. And so I know my daughter has taken some big big medications trying to get control of that and really not much worked. We had something that worked for about, I don't know, three months. She woke up one day and it didn't work anymore. So, there are lots of different kinds of medications that they can throw at it. Some of them are antidepressants - don't be offended by that. Some of them like Elavil is one. [Transcriber’s note: Elavil is a brand name for the generic drug amitriptyline.] It's an older antidepressant that's been shown to interrupt that kind of pain. So there are things like that. There are anti-seizure medications like carbamazepine that can be given. [Transcriber’s note: carbamazepine is the generic from brand name drugs Tegretol or Carbatrol.] They really just sort of shut down the nervous tissue. They make it a lot less reactive. There’s one called Gabapentin. [Transcriber’s note: Neurontin, Gralise, and Horizant are brand names of gabapentin.] So there are all sorts of things that they can do for pain management, but it's tricky and it's really individualized. But it's worth talking to someone about because anything that you can do to improve quality of life and be able to get yourself to do the things that you want to do is super important.

40:48 Jill (Host): And then I'd also remind our listeners about the episode with Dr. Linda Bluestein, who has the Bendy Bodies podcast, and she discussed a bit about pain medication and other strategies, and she's wonderful. [Transcriber’s note: you can listen to Dr. Bluestein’s episode of the POTScast here: https://the-potscast.castos.com/episodes/episode-6-hypermobility-disorders-and-chronic-pain-with-dr-linda-bluestein ] She really takes just a full on approach, you know, look for solutions anywhere, tie them together, and you know, I think she reminded us that she recommends an anti-inflammatory diet. So that might help some pain a little bit. There's a ton of studies showing that when people are vitamin C deficient, that pain is more severe. So make sure you're not deficient in that. They're starting to learn that processed food and sugar after a very, very brief respite from the pain can actually make the pain worse. So I don’t want to imply that those have huge effect sizes or anything you know, if gabapentin doesn't work, I'm not going to say that giving up sugars will work, but you know, I think it's kind of an all hands on deck situation, where anything you can try is worth it. I wanted to mention one other fantastic resource for pain, which is anything you can find on the Internet by Dr. Pradeep Chopra. He's a pain specialist in Rhode Island. He always wins awards for being an incredible pain doctor and he has some wonderful ebooks, some presentations, and he's just so thorough in keeping up on any little thing that might help EDS patients. So, C-H-O-P-R-A is his last name, Dr. Pradeep Chopra.

42:23 Dr. Pederson (Guest): He is fabulous. And I ought to say, I forgot about Dr. Blustein, but I should not have because she did a very nice segment in there about that pain medication and how to really try to attack that pain. So go back. That was one of the early episodes as well, six or nine, I feel like, and she was one of those who were so grateful early on for people who just trusted us that we were going to do something great with this, and of course it's turned out wonderful. But yeah, she was fabulous as well. So go back and listen to that episode as well.

42:56 Jill (Host): A couple other good resources are the Ehlers Danlos Society [https://www.ehlers-danlos.com] and then chronicpainpartners.com, right? That's John Ferman’s group and I believe that he is very generous with supporting webinars with various experts, and I think maybe you know more than I do about that group.

43:17 Dr. Pederson (Guest): Yeah, I've met John. He is truly committed. So, his daughter has EDS pretty severely and POTS and I think he is a frustrated parent like I am, and he is trying to move mountains for a community to bring awareness to the general public, but also to try to get physicians and other health care practitioners to understand these conditions a little bit better. And then his webinar series is amazing and we've got links to a lot of those on our website as well, where he's again bringing in, like we are, some of these big specialists, big name people to talk about their areas of interest and it's all free, right? So, John Ferman and Chronic Pain Partners have done amazing work for folks in the EDS community.

44:10 Jill (Host): Fantastic. We owe so much to the, as you said, frustrated parents trying to move mountains. That is a lot of basics about Ehlers Danlos Syndrome. Is there anything else we should say about it today?

44:24 Dr. Pederson (Guest): I guess the last thing to say is that this can be a very debilitating syndrome and it's hard for people to understand. They maybe can see the dislocations, but they can't see the other things. And, you know, you're not crazy and you deserve good medical treatment and you need to continue to advocate for yourself, which is easier said than done, as a parent who's tried to advocate for my own child. But just know that there are people out there that are fighting for you that care about you. We may not meet face to face, but work is being done on your behalf. And so, hang in there. Hang in there and do the best that you can do each day. Sometimes that's all we can do.

45:09 Jill (Host): Amen. Dr. Pederson, thank you once again for taking time to fill in the POTS basics. We know that you are very busy, and we really appreciate how you put together these great informative sessions for us. And, hey listeners, I think I'm contractually obligated to remind you that this is not medical, dental, political, relationship, or any kind of advice. Consult your medical team about what's right for your unique situation, but please consider subscribing because it helps us get found by more marvelous people like you. So, thank you for listening. Stay strong. Remember, you're not alone, and please join us again soon.

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