What No One Tells You About Pregnancy with EDS | Office Hours

[00:24] Dr. Linda Bluestein: People who have hypermobile EDS and HSD are definitely at increased risk of postpartum depression, anxiety, and PTSD. And unfortunately, these conditions are not always sufficiently recognized. Welcome back, every bendy body, to the Bendy Bodies Podcast. I'm your host and founder, Dr. Linda Bluestein, the Hypermobility MD, a Mayo Clinic-trained expert in Ehlers-Danlos Syndrome. Dedicated to helping you navigate connective tissue disorders and live your best life. In today's Office Hours episode, I will be answering some of your most pressing questions. We will be talking about pregnancy, surgery, and tissue fragility in hypermobile EDS and HSD.
[01:22] If you're watching the video right now, you are going to see some new faces. We have two of the wonderful human content producers joining us today, Tessa and Shanti. We thought it would be fun to give you a behind-the-scenes look for this Office Hours episode so they will be popping in to read some of your questions and keep things rolling. Thank you so much, Tessa and Shanti, for being here and also for wearing your VIP Bendy Bodies merch.

Tessa: Thanks, Dr. Bluestein. Thanks for having us back on.

[01:50] Dr. Linda Bluestein: Of course, of course. Um, stick around until the very end so you don't miss any of our special hypermobility hacks. As always, this information is for educational purposes only and it's not a substitute for personalized medical advice. Here we go. So we're going to start with a question, I believe, on pregnancy and childbirth. Do one of you want to read that question for us?

[02:12] Tessa: Absolutely. Yeah. So this is coming from a listener named Carrie, and they write, could you do an episode on pregnancy and childbirth? I had 4 pregnancies where I knew something was off, but at the time there wasn't much information. Recently I came across new research that describes almost everything I experienced, and it was such a relief to realize I wasn't being dramatic or imagining things. I'm also a medical student wondering what specialties are best for people interested in connective tissue disorders. I'm leaning toward rural family medicine for the multi-system training and access to care, but would rheumatology or immunology be better? Thank you so much for your podcast. It's so great to get reliable scientific information from a reputable source. One out of my four kids has hEDS, and I'm putting the pieces together for the others, my husband, and myself, all thanks to your show.

[03:03] Dr. Linda Bluestein: Okay, well, I love this question and thank you so much, Carrie, for the extremely kind words. And I'm so glad that the Bendy Bodies Podcast has been helpful for you. I wanna address the second part of the question first about specialties because this is a question that as a medical student is super relevant for you. I do get contacted by a lot of medical students or people who are thinking of going to medical school. So I know there's other people that share your question, but also I think it's helpful for people who are looking for clinicians and providers for them to know a little bit more about the different types of specialties as well.
[03:37] So first, we wanna think about family medicine versus internal medicine versus pediatrics as a like, primary type of training program. So these are 3 different types of training programs that people can go into after their residency. If you do pediatrics, then you'll be treating children. Some pediatricians will treat, you know, late teens or maybe even early 20s, but in general, you're treating 18 years and under. If you do internal medicine, then you'd be treating adults. And if you do family medicine, you're treating children and adults, and you might even do some, um, deliveries and things like that. So you can actually treat like entire families. So people who want to treat an entire family, two kids, both parents, um, et cetera, would lean more towards family medicine. But people who want to do more strictly adults might lean to more towards internal medicine. And people who wanna really work exclusively with kids would lean towards pediatrics. So those are 3 types of primary care residencies that people can choose.
[04:37] And then after they do that, they could do subspecialization in rheumatology, immunology, or genetics are 3 that we wanna be thinking about. So you could be, you know, like an internist who does rheumatology. So you would be an adult rheumatology specialist dealing with adults who have possible autoimmune conditions. If you're an adult geneticist, then you would be dealing with adults who have possible genetic conditions, but you could also be a pediatric rheumatologist, pediatric immunologist, or a pediatric geneticist. Another important consideration is something like PM&R, which stands for physical medicine and rehabilitation. And those kinds of doctors work with a lot of, you know, rehabilitation from stroke. They do a lot of, like, kind of biomechanics of the body, but a lot of them also will do interventional pain medicine. So if you think that that's something that you might want to do in the future, if you did, for example, internal medicine and then you did immunology, you would not be able to then do interventional pain management unless you did another fellowship or something like that. Whereas physical medicine and rehabilitation doctors often will do interventional pain management, and they will get some of that in their training. Oftentimes they will do a pain medicine fellowship, but that just depends on the person.
[05:56] If you want to think about being a geneticist, those type of doctors are fantastic when a person has some of the more rare things in their clinical picture, and they need like a really deep dive into some of the rare subtypes of EDS or some of the other more rare genetic conditions. So we know that all of us are affected by our genetics and our environment, but really it's, it's that combination that gives us our phenotypic presentation or our clinical picture. And with some people though, we have a suspicion of a stronger genetic component than with others. So the more kind unusual things that you have in your history and in your family history, the more likely it is that a geneticist will be helpful.
[06:36] And I do have to say that overall, I have a lot of disappointment over genetic testing results. I find them to be rarely helpful. Um, but I do think that over time we will be getting more and more information in terms of genetic testing. So we will be able to, you know, hopefully learn more from people's genetic testing results. But just so people are aware, it is extremely common in my practice for people to get genetic testing. It might even be whole genome sequencing where they look at the entire DNA. And it's not uncommon for it to be negative, even in people where I would expect to be finding some really significant, you know, mutations, if you will. So geneticists play a very, very important role, especially when things are, you know, a little bit more out of the norm. But some of those other professions can be super, super helpful for more of the management side of things related to hypermobile EDS and HSD.
[07:32] The physical therapists are of course critically important in getting back some movement and things like that. But if you're an internist or a pediatrician or a family medicine doctor or an immunologist or a rheumatologist, you can play a really, really crucial role in working up these patients because we wanna make sure we're not missing any confounding variables. Okay, so hopefully that helps answer the first part. Well, I guess it was technically the second part of her question. Does that make sense, Tessa?

[08:07] Tessa: Absolutely. And I'm actually— I'm curious, as we're talking about, um, you know, the different specialties and folks in medical school who personally have experience with EDS, I'm curious how much hypermobility and EDS got talked about when you were in medical school. And, you know, as we're seeing so many more people kind of putting the puzzle pieces together of how much overlap there is with hypermobility and other conditions. I'm curious if there's maybe a push to get it talked about more in universities and in like med school curriculum.

[08:44] Dr. Linda Bluestein: Good question. It wasn't talked about at all really in my training. I think we had like a sentence or two about Ehlers-Danlos. And you know, you're taught about the really, really extreme cases. You know, you're shown pictures of somebody pulling the skin of their neck, like, you know, like these circus tricks, right. They can pull it, they could pull it all the way out and put their skin like over their face or something. So, you know, you're, you're taught these like really, really extreme examples. And otherwise there was really hardly any mention of it.
[09:11] Now, I graduated from medical school quite a few years ago, so I'm hoping that it's better now, although a lot of the medical students that I've talked with say that there's, there's more conversation, but we certainly have a long ways to go. So I really appreciate you asking that question. And then the other thing that I'm thinking of in Carrie's question is, you know, she has 4 kids. And hypermobile EDS and is a medical student. So I should also say kudos to you. That's amazing. Um, I would also, you know, really keep in mind that you have to pace yourself and medical school is extremely demanding. So although there are some things that have been put in place since I went through medical school, like some restrictions on work hours and things like that, it's still extremely demanding.
[09:54] So, you know, you always have to be thinking about what do I need in order to keep my own health, because it doesn't do you any good to get through your entire training and then find that you can't do the job that you wanted to do. I actually have a, I'll call her an acquaintance, 'cause I really only spoke to her one time, but she's an anesthesiologist. And then after she went through her entire anesthesia training, she did a cardiac anesthesia fellowship, which is, you know, another year or two. And then on top of that, she did a pediatric cardiac anesthesia fellowship because when children are coming for pediatric, you know, cardiac surgery, they're very high risk. These are kids with congenital heart defects and it's really, really challenging and they're very high risk of complications.
[10:40] Anyway, she went through all that training, never worked a single day as a pediatric cardiac anesthesiologist. So I think it's also really important when you're considering what kind of specialty you want, having realistic expectations for yourself in terms of how hard is it going to be for me to stand for an entire day? What kind of accommodations might I need? How much am I going to need to be in control of my own schedule? Need to be able to sit down while I'm talking to patients? You know, because something like for me, that was a limiting factor when I came back to work and was going to focus on people with connective tissue disorders was the fact that I couldn't wear lead. Wearing a lead apron is very painful for my back. They're very heavy. And so, um, when I was coming back and trying to decide what I was gonna do, I knew that interventional pain management was really not a good option for me, even though I was trained in how to do that. So as an anesthesiologist, like, I knew how to do that, but I also knew that I needed to set myself up for success.
[11:40] So that's why I decided to work for myself and not work for somebody else who might tell me, you need to see 6 patients in an hour. So hopefully, Carrie, that helps. And, you know, again, I just think it's important to think about where you're at physically now and what's a realistic game plan for you going forward and making sure that you're not spending more time in training than you need to, especially because, you know, none of us know what our future is going to look like. So, Tessa, does that make sense?

[12:09] Tessa: Absolutely. Absolutely. And I'm curious, did you know that you had EDS before you were in medical school?

[12:15] Dr. Linda Bluestein: No, I did not. I was not diagnosed with EDS until I had worked as an anesthesiologist for a couple of decades almost. So it was much, much later in life that I was diagnosed. I did know though that I had a lot of physical challenges. I knew that I had a latex allergy, so I would get like hives on my hands when I was doing my surgical rotations. And sometimes I would have respiratory problems from exposure to different things. I knew that I had asthma, allergies, eczema, like all of those things. I knew that I had a lot of musculoskeletal problems.
[12:50] When I was trying to decide what residency I wanted to do, I knew that a surgical residency was probably not a good option for me because standing on my feet all day long was going to be challenging. So I chose anesthesia in part because I wanted the fast pace of the operating room, and I liked the idea of being in the operating room, but I also needed to be able to sit down, you know, at times. And it gave me the ability to work with my hands and also have a little bit more, you know, control over, over that whole standing piece and, and wearing lead, which was really challenging.

[13:27] Shanti: Dr. Bluestein, can I ask you a, a very personal question then? Since Carrie wrote in about, you know, pregnancy and childbirth, you weren't diagnosed until later and you of course are a mom. So what was childbirth and labor like for you then?

[13:43] Dr. Linda Bluestein: Yeah, I love that question and I'm happy to share. I'm, I'm, I'm pretty much an open book, so I'm happy to share about that. So I had 2 pregnancies and 2 deliveries and they were challenging, especially the first one. In my first pregnancy, you know, even starting with the first trimester, I mentioned just now that I had a lot of allergies and I was having so much difficulty. It was in the summer, so I was having, you know, so many allergic reactions and my doctor did not want me to take any of the second generation antihistamines. That left us with diphenhydramine or Benadryl. So I had the choice of, you know, I'm working as an anesthesiologist, so I can't take Benadryl during the day when I'm at work. So I would take it after I got home, and I was sleepy pretty much anytime that I wasn't working. I was pretty sleepy because I was taking so much diphenhydramine.
[14:28] I wasn't aware of Cromolyn at that time, or I wasn't, you know, nobody had prescribed it to me for many, many years. So that was not something that was in my picture, although that might've been a good choice. I was also not on low-dose naltrexone at that time. Which is something that often people are actually given for infertility. So we had some challenges in that first trimester dealing with a lot of my allergies and things like that.
[14:56] And then going into my second trimester, I started getting a lot of tachycardia, but I didn't know that it was tachycardia. I just started feeling really, really short of breath and it would actually wake me up from sleep. So I would be sound asleep and I would wake up like panting and feeling like, you know, I just couldn't get enough air. And I have asthma, but it felt very different from my asthma. So they did a Holter study, which is where you wear a special patch to look at your heart rate and rhythm for a period of, you know, probably several days. And they saw that my heart rate was going up like to 200 while I was sleeping. And I was having these episodes of, you know, crazy, crazy high heart rates.
[15:36] So they sent me to a pediatric OB specialist in Marshfield, Wisconsin, because I was living in Wisconsin at the time. And she was great, and she diagnosed me with dysautonomia of pregnancy, and she put me on a beta blocker, atenolol, which is a very old drug, so they know it's quite safe in pregnancy. But then they told me that a lot of the pediatricians would not want to deliver a baby on beta blockers because if the mom's on beta blockers, and of course you have the shared circulation while you're pregnant. So the baby's being exposed to the beta blockers, but then once the baby is delivered, they no longer have that exposure to the beta blockers. So they kind of can go through a period of withdrawal like a regular person can when they stop beta blockers. So it's a little bit trickier for the pediatricians. So some of the pediatricians were not comfortable handling that. So I had to kind of think through a little bit more carefully who was going to be the pediatrician for, for my first child. And actually the second, the same exact thing happened in my second pregnancy. Still ended up with the tachycardia and ended up back on Atenolol. I was able to go off of it in between.
[16:39] And then I also developed, they did an echocardiogram to look at my heart to make sure that I wasn't like in heart failure or something. And they found what they thought was a mass inside my heart. So I had to have a follow-up study for that, a transesophageal echo where they put the probe actually in your esophagus to look at your heart. And when they were doing that study, because I was pregnant, um, and my blood pressure ran so low, they gave me a tiny bit of midazolam, which is a benzodiazepine, and they gave me 50 micrograms of fentanyl, which is an opioid that dropped my blood pressure down to like 70. So then they were worried about the perfusion to the fetus. So they said, that's it, no more drugs. So I basically had the procedure awake. I remember the whole thing. Um, but the good news was they found that I did not have a mass in my heart. It was an artifact. So that was great.
[17:33] But then I got in a car accident while I was pregnant. And so I had to go to the hospital, was in some preterm labor, had some back pain, had an epidural steroid injection. I had quite a few things happen while I was pregnant. Fortunately, my back pain got a lot better after I delivered the baby. But after I delivered the baby, then I had— I was on bed rest. I should back up. I was on bed rest for a while. Then I had the baby and I started having terrible tachycardia again. My asthma was was just terribly out of control. I was having vertigo episodes.
[18:06] Wow, this is really sounding bad. Um, but, uh, but anyway, so they worked me up and found out that I had initially that I was Graves' disease, but it turned out it was postpartum autoimmune thyroiditis. So my immune system was attacking my thyroid. So I already had these autoimmune conditions, and now they of course like flared up even more after delivery. They did a workup for heart failure. I was not in heart failure, but I did have to go on some stronger medications for my asthma. And basically with my second pregnancy, a lot of those same things happened, but all in a smaller scale. And I did not have a car accident in my second pregnancy, thank goodness.
[18:44] So I did have some challenges in my, in my pregnancy. I had— I did have epidurals for both, although this first epidural was so late that I really didn't get any effects from it. The second epidural started out really one-sided, but then they were able to redose and get it to be, you quite symmetrical. So that was good. So yeah, those, those are my pregnancy stories.

[19:05] Shanti: My goodness. I mean, that's a whole lot. And as you're talking, I'm just— I think it gives me so much empathy for— you went through medical school, you had an understanding of bodies in general and medical health, and you're able to understand what's going on as, as all of these things are happening, and you're able to advocate for yourself using the language that the doctors do. And I think as You're talking, it just gives me so much empathy for the mothers and mothers-to-be who don't have this medical background, who are going through some of the same complications and having to learn all of it from the ground up, having to teach themselves, you know, through resources like this podcast. And I mean, just wow.

[19:52] Dr. Linda Bluestein: Yeah. Yeah. That's absolutely true. And I was working as an anesthesiologist at the time, so I was on my feet all day, every day in the operating room. And I know I said is that I didn't want to go into a surgical residency because I didn't want to be on my feet. But you're still on your feet a lot as an anesthesiologist. And I was actually on call the weekend that I went into labor with my second child. I literally worked right up until when I had him. So yeah, it was, it was kind of a crazy time now that I think back.
[20:19] But pregnancy with EDS or HSD is often— I don't want to say uneventful, but it can be as uneventful as people who don't have those conditions. But there are a lot of things that we need to be thinking about in terms of some of the different phases. So like in the preconception phase and antenatal planning, there's some things that we should be thinking about. So I can go ahead and go into some of those if you want.

[20:45] Tessa: That would be great.

[20:46] Dr. Linda Bluestein: Okay, cool. So in the preconception phase, so before you are even pregnant and when you're thinking about pregnancy, it's a good idea to be screening for POTS or dysautonomia. You can be screening for mast cell activation. I know a lot of people really struggle to get a proper workup and diagnosis, but even if you just listen to this podcast, subscribe to my Substack newsletter, start getting some information, and have a suspicion yourself that this is something that might be affecting you, at least that's information that you have that you can share with your team. You can let them know that you suspect that you have a mast cell disorder. Hopefully they will, at least if they don't have an understanding of it, at least maybe they would be willing to learn about it, um, or else you can find somebody maybe who is willing to learn about it.
[21:32] It's important to think about possible spinal instability, instability in the temporomandibular joint or other joints, because that can be impacted by pregnancy. Um, it's important to be thinking about pelvic floor weakness because that's something that people can have even if they've never had a pregnancy. Um, you also want to be thinking about your medications. What medications are you taking? What kind of safety is there in terms of pregnancy and breastfeeding? Because unfortunately, we haven't done a ton of studies, and it's understandable why, because, you know, there's, you know, risk involved in doing any of these studies on medications in pregnancy. But there's varying levels of data for different medications in pregnancy. So it's a good idea to review your medication list and maybe look to see what pregnancy category they are. So you kind of have a sense of what medications might need to be changed when you are pregnant.
[22:21] You also wanna think about physical conditioning. So when I have patients and clients that I'm working with, if they are already in really, really having a tough time, if they're already spending a lot of time in bed or something like that, then I encourage them to put off pregnancy until they can get in better shape because pregnancy is very hard on the body and a lot of physical changes happen. So you wanna think about, can you do some, you know, gentle core and pelvic stability programs, aquatic therapy, can be very helpful. Uh, Pilates is wonderful, and there's even some prenatal Pilates classes, um, that can help reduce, uh, joint pain later on.
[23:02] You also may be thinking about your nutrition. You wanna be making sure that you're getting adequate protein and micronutrients like vitamin C, copper, and zinc. These things are very important for collagen synthesis. You also wanna think about your autonomic nervous system, and that's the part of your nervous system that controls all the things that we don't think about— our heart rate, our blood pressure, temperature regulation, gastrointestinal function, et cetera. And if that autonomic nervous system has difficulty, for example, with upright posture or orthostatic intolerance, doing something like graded exercise might help. So sometimes using a rowing machine or a recumbent bike can be very, very helpful. Start low, go slow. That's the biggest thing to do is to start really, really low and work your way up very, very gradually. And you might need to have zero resistance at all on the recumbent bike.
[23:47] You also want to think about if compression garments might help you and/or liberalizing your fluids and sodium or electrolytes. In terms of pregnancy itself, of course, we want to be thinking about dysautonomia. As I mentioned, I had a lot more orthostatic intolerance. I would be walking through a grocery store and suddenly my heart rate would go crazy high, but I didn't feel it as palpitations. I felt it as shortness of breath. So even if you don't have dysautonomia before pregnancy, some people develop dysautonomia during pregnancy.
[24:24] We also want to be thinking about our musculoskeletal system and our connective tissues. During pregnancy, we have more relaxin, which is a hormone that increases joint laxity. This hormone can impact the amount of mobility that we have at every joint in our body, and in particular, it can affect our pelvis, which of course is very much impacted by the developing fetus. And so things like our sacroiliac joint, which is in our, in our back, and those are the, the bones that you kind of feel on either side of your sacrum where it joins your ilium. So those sacroiliac joints normally only have a couple degrees of movement, like 2 to 5%. In people with EDS and HSD, they can have a few degrees more movement, but they're really not supposed to have much movement. And in fact, most doctors don't even think of the sacroiliac joint as having movement at all because the amount of movement is so little, especially when you compare it to other joints.
[25:18] But in pregnancy, because of relaxin, we can have even more movement in that SI joint, and that can cause pain for a lot of people. So sometimes having something like an SI belt can be really helpful, and also things like pelvic floor PT or orthopedic PT can really help with pelvic pain and low back pain, which are common things that we see in pregnancy. We also want to think about the pubic symphysis, which is where the two sides of the pelvis meet in the front. So that's what you normally think of as your pubic bone is actually a joint. And that, if you think about where your bladder is, so this is just below your bladder, it's that bone that you can feel in the front, and that is where the two sides of the pelvis meet, and that is an actual joint. And of course, it's not normally supposed to have much movement, but it can have some movement, um, during pregnancy, and it also is under more stress and strain during pregnancy.
[26:09] As I mentioned, any joint in the body can become more unstable during pregnancy, but we especially wanna be thinking about the cervical spine because of the fact that the cervical spine is so important, um, for other things like pelvic floor function, but it's also super important if you end up needing a C-section, for example. The anesthesia team will need to know about any instability that you have in your cervical spine. So if you do have neck pain or any kind of sensation of clicking or clunking or bobblehead or anything like that, or neurologic symptoms like numbness and tingling in your upper extremities, very important to discuss that with your doctor.
[26:49] If you have numbness and tingling in your upper extremities, that can come from a variety of places. So if you have it in your hands, for example, that can be carpal tunnel, that can come from your wrist, but it's also possible to have numbness and tingling in your upper extremities coming from your neck. So very important to get that evaluated. You also want to be thinking about cervical spine and TMJ precautions, the temporomandibular joint. So some people, um, actually have clicking and clunking and dysfunction of their TMJ. As we've talked about on other episodes, I encourage you to go to bendybodyspodcast.com and look for those other episodes. And it's really important to alert the anesthesia team to any kind of, as I mentioned, cervical instability, but also any kind of problems that you have with your jaw, whether it's painful, clicks, clunks, feels like it comes out of place, if it has open locking, closed locking, et cetera. Those are very important things for the anesthesia team to be aware of.
[27:45] We also wanna work on proprioception. During pregnancy so that we can help prevent falls. We know that a lot of people with EDS and HSD have problems with their proprioception, or knowing where their body is in space without looking. If you have problems with that, you can work with a physical therapist, or you could probably even find some online exercises that you can do. But it's very, very important to prevent falls. Another important thing to think about is vascular fragility. Are you somebody that has easy bruising and prolonged bleeding? And if so, is it just a little bit or is it a lot? If it's quite significant, then you definitely want to be thinking about, you know, do I have some kind of platelet function problem? Do I need to be checked for von Willebrand's disease? You could discuss this with your obstetrician because of course they will want to know if you are at increased risk of postpartum bleeding.
[28:37] So definitely be thinking about, you know, do you seem to clot normally? Normally, or is this something that you struggle with? If you do struggle with it, and if you are discussing this with your OB, and if you do suspect that you have EDS, or even if you're diagnosed with hypermobile EDS but you suspect that your bleeding is even more than most people, definitely bring this up with your obstetrician and point out to them the link with EDS, because they might not be aware of that. In general, during pregnancy, especially if you have vascular fragility, we want to avoid unnecessary invasive procedures. We want to handle tissues very, very gently, and and use minimal traction on tissues.
[29:15] Okay, we're going to take a quick break, and when we come back, we are going to talk about labor and delivery for people with EDS and HSD. We'll be right back.
[30:41] So next we're going to talk about how EDS and HSD can impact labor and delivery. Studies have shown that in labor, some people with EDS and HSD experience more rapid labor. Some people do progress more slowly, but in general, the data seems to suggest that people can have rapid labor. So definitely take that into consideration when you're thinking about where you plan to deliver your baby, where you live. Do you have a ride set up ahead of time? Is your primary driver somebody that may or may not be available? Should you have a backup driver? You know, you might need to call an Uber or something, but definitely make sure that you have like a plan A, B, and C just in case you have a rapid labor.
[31:28] If this is not your first pregnancy and you had a rapid delivery with your first pregnancy, the second pregnancy and third pregnancy could be even faster. Some people do have cervical insufficiency or problems with their cervix staying closed. And if so, they might consider having a cerclage placed, which helps to keep that cervix closed. And they might need close surveillance if they've had prior mid-trimester loss. Definitely want to be prepared in the event of a hemorrhage because some people do have more bleeding at the time of delivery. And there's something called tranexamic acid spelled T-R-A-N-E-X-A-M-I-C acid, and then also desmopressin. These are two different prescription drugs that can be given by the anesthesiologist or by the nurses on the OB floor. And desmopressin is spelled D-E-S-M-O-P-R-E-S-S-I-N.
[32:21] You want to have those medications available if your bleeding risk is elevated. So you could discuss that with your OB ahead of time. Let them know that you are particularly concerned about excessive bleeding and that you heard that these are some medications that can be helpful. There are some small hospitals that might not actually have these medications. And if you are thinking of delivering with like a midwife or something at home, of course they would not have access to those medications either. So these are just important things to be thinking about in the planning phase as well.
[32:52] What are some of the anesthesia and surgical considerations? Definitely make sure that, uh, your hypermobile EDS or HSD diagnosis or suspicion is clearly communicated with the anesthesia team. As I mentioned earlier, we wanna be thinking about the possibility of a difficult airway, whether you have cervical instability, TMD, TMDD-type problems, temporomandibular dysfunction, or if you have other issues with your pulmonary tree, maybe you've had problems with your, with your trachea before, maybe you have problems with your vocal cords. These are all important things for them to be aware of.
[33:28] Also, when we're thinking about spinal or epidural anesthesia, we wanna be thinking about dural fragility. So all of us as anesthesiologists are very aware of the fact that you're putting the needle for a spinal actually through the dura. But if you're doing an epidural anesthetic, you're stopping the needle before you get, um, to the last layer. You're putting the needle in the epidural space, and that's where you're injecting the medication. But people who have EDS and HSD may be at higher risk of dural puncture, and they also are at higher risk of spontaneous CSF leaks. So these are important things for the anesthesia team to be aware of.
[34:04] Also, poor tissue integrity can contribute to increased bruising, slow healing, and tearing of sutures. Um, you wanna make sure if possible that you are working with an anesthesiologist who's familiar with connective tissue disorders. You probably won't know ahead of time who your anesthesiologist is going to be for your actual delivery though, because it's going to be whoever's on call. But maybe you can have a pre-op consultation or a pre-delivery consultation with the anesthesia team so maybe they can flag your chart so that when you do go into delivery, they know that you had brought this up beforehand.
[34:39] Um, you also wanna have a backup plan in place for local anesthetic failure because a lot of people with hypermobile EDS and HSD are resistant to local anesthetics. So, it's very important to be thinking about what your prior experience has been with local anesthetics. So, lidocaine, mepivacaine, bupivacaine, novocaine, et cetera. Um, and make sure that you share that with the team. If you've had problems with local anesthetics being effective, make sure that they know that you've had those problems so that they can have backup plans in place.
[35:11] For positioning, you want to use neutral joint alignment whenever possible. You want to limit the amount of hip abduction, abduction where the hips are going out, although, you know, you have to have your legs apart in order to deliver the baby if you're doing a vaginal delivery. But we want to avoid prolonged positioning in that lithotomy position if possible. In terms of the perineum, which is, you know, like your saddle area, we want to If possible, avoid episiotomies or minimize the amount of episiotomies. Spontaneous stretching and gentle stretching, if it's a vaginal delivery, is preferable. So kind of slower progress can be very, very helpful.
[35:53] The obstetrician can help with that by helping stretch that perineal tissue very slowly. That tissue is very fragile and if you get a big tear, then that can heal very, very slowly and can be very problematic. Traumatic. So, you know, they're gonna need to use their judgment as to whether or not to do an episiotomy, which is where they cut that tissue down there to allow enough room for the baby to come through the, the vaginal canal and the perineum. So sometimes they do need to do an episiotomy and sometimes that's better than having a big tear, but it's something to discuss with the obstetrician or the family medicine doctor if that's who's delivering your baby. Discuss that with them in advance.
[36:30] Postpartum recovery. So it's possible for people to have delayed healing. We want to sometimes consider using longer lasting absorbable or interrupted sutures and low tension closure techniques. We can add topical adhesives if needed and also be thinking about if you've had trouble with sutures before, and we're gonna be talking about this in a future episode. I know I mentioned that when I had my forehead surgery, I had spitting sutures. If you've had spitting sutures, see if you can find out what kind of sutures were used in that surgery and make sure that your delivery doctor is aware of the fact that you had spitting sutures with, uh, whatever type of suture material that was.
[37:05] You also wanna be thinking about your pelvic health because people with these conditions have a higher risk of prolapse, um, a higher risk of pelvic girdle pain and incontinence. So you want to begin pelvic floor physical therapy early. As I mentioned, joint instability can worsen during, uh, pregnancy and then it can normalize later on. You may need bracing or supportive wraps for unstable joints, and we can definitely see flares of pain and dysautonomia.
[37:35] Okay, so there's also some cardiovascular considerations or blood volume stabilization in POTS. So breastfeeding actually can stimulate oxytocin and prolactin, which promotes mild fluid retention and venous return, and can actually help people with POTS. Um, frequent feedings can help stabilize hemodynamics and reduce orthostatic symptoms in some people, but very, very important to maintain hydration and electrolyte intake. You want to consider compression garments, especially if you're sitting for long feeds. If you have vascular and skin fragility, you wanna be using a lot of positional and pillow support to minimize traction on your shoulder and neck joints. You wanna be aware of any easy bruising or subcutaneous hematomas that you have around your breasts or nipples and use gentle latch techniques and use, definitely use lactation consultants to help support you and reduce any complications.
[38:28] Um, you wanna avoid aggressive breast massage or pumping because that suction pressure can actually rupture superficial vessels. In terms of MCAS, um, you wanna continue mast cell stabilizing medication if they're lactation safe. So definitely discuss with the lactation consultants and with your doctors, um, what medications might be able to be continued. For example, cetirizine and cromolyn. Um, you wanna track any reactions in your infant if you are using either medications or supplements.
[38:57] Okay. So what about for the infant and genetic counseling? Hypermobile EDS and HSD are considered to be autosomal dominant, so each child will have a 50% chance of inheriting the condition. Although expression varies widely, we know that people with the hypermobile EDS or HSD phenotype can express in a wide variety of ways. So just because somebody has hypermobile EDS and looks one way doesn't mean that somebody else with hypermobile EDS is going to look the same way. We all know that babies have more joint laxity than we do as adults. So we wanna keep that in mind and also be watching for the potential for easy bruising. If you do see easy bruising, you want to document that and bring it to the attention of your baby's doctor early on.
[39:44] There have been some very, very unfortunate cases where parents have been accused of child abuse because their child has had, you know, injuries and things like that that are disproportionate to the trauma that the child has faced. So, if you have concerns about disproportionate injury or easy bruising, definitely bring that to your child's care team. You want to make sure that your child's caregivers and clinicians know that they need to handle your child with lots of care. So, we need to handle all babies with care, of course, but babies that might have hypermobile EDS or HSD might be more vulnerable to injury, so we need to be especially careful with that.
[40:22] So Carrie and all the other women out there who are considering pregnancy and/or are currently pregnant, I hope this information is helpful. And we are going to go on and talk now about mental health and social support. I will share also that I had postpartum depression with my second pregnancy, and it was really, really rough. People who have hypermobile EDS and HSD are definitely at increased risk of postpartum depression, anxiety, and PTSD. And unfortunately, these conditions are not always sufficiently recognized.
[41:01] I know in my case, it was actually my OB. I was working with her in the operating room one day and she had been on vacation. She came back from vacation and I was, you know, on the other side of the curtain, 'cause I was her anesthesiologist. And she literally looked at me and she said, you're not okay. Like she knew immediately that I was not okay. And she was the first person to recognize that. And I had been suffering with the postpartum depression for a while, but thank goodness she pulled me aside and talked to me and helped me seek help, basically.
[41:33] So these things are very, very common. It's very common for people to experience changes in their mood, increased pain, more fatigue, more problems with their autonomic nervous system. And these can be very, very difficult because now you have this new infant, everyone expects you to be super happy and cheerful, yet you feel cheerful and your hormones are all out of whack and your neurotransmitters are all out of whack. And so it's really important to get help so that you can start to feel better and be the kind of mom that you probably want to be. For me, once I got started on an antidepressant, things turned around very, very quickly, which was huge. So definitely important to incorporate routine mental health screening and early referral so that people can get the help that they need.
[42:22] We also want to have trauma-informed obstetric and postpartum care because a lot of people experience medical trauma. We know with having hypermobile EDS and HSD, but especially people can experience trauma, you know, throughout their pregnancy or during labor and delivery. Caregivers are often in a big hurry and they might not communicate well and/or, you know, send some bad messages. So we want to make sure that we're addressing all of the mental health needs of people who have just gone through this massive experience of delivering a baby.
[42:56] So I'm gonna share some, uh, really helpful resources. Go to bendybodyspodcast.com to check those out. There's a fantastic article by Perazzo et al. where, um, in 2024, they gave some, uh, really great guidelines, evidence-based clinical guidelines for childbearing, um, with hypermobile EDS and HSD. So definitely go to bendybodyspodcast.com to get that information. And some of the take-home themes that I want you to be aware of.
[43:24] Number 1, be prepared. And we don't wanna pathologize labor and delivery in EDS and HSD. Most births are actually routine, especially when we can predict the potential issues like bleeding, positioning problems, anesthesia, autonomic regulation, et cetera. When we are thinking about those things ahead of time, we can often and have really nice pregnancies and deliveries.
[43:52] The postpartum period is its own protocol. We wanna be thinking about the biology of healing because you're always going to have some kind of tissues that need to heal. We wanna be thinking about, you know, tailoring our suture choices, tension-free closures, early pelvic floor physical therapy, and lactation support. We wanna be thinking about hydration and hemodynamic care that will continue after birth. Regular fluid intake is important, compression and breastfeeding related volume retention can improve stability of POTS.
[44:24] Teamwork is crucially important here. Make sure that your obstetrician, your anesthesiologist, physical therapists, and lactation consultants are all on the same page and that they're all aware of connective tissue disorders. I strongly encourage that you share that article, that Perazzo et al. article, with them. Share this podcast with them so that hopefully everyone is on the same page and they can work in a collaborative fashion.
[44:47] Okay, Carrie, that was a lot. I hope that that was helpful. So as you know, we end every episode with a hypermobility hack. I do have a hack to share today, and this is coming straight out of a lecture that Dr. Brooke Winder and I did last night for a place called Pelvic Floor University. We gave this talk last night and some different resources were shared, and I want to share those with you. So one is called My Pelvic Bra, and we will have links to these on the bendybodyspodcast.com website. So one is called My Pelvic Bra. Another one is called SRC. Another one is called Love Steady. And the last one is the EMSELLA chair, E-M-S-E-L-L-A.
[45:29] So the first 3 things are external support devices that you can use to help support your pelvic floor. And the last one, the EMSELLA chair, is something that you can sit in and you can have therapeutic sessions over a period of days to weeks, and that can help strengthen your pelvic floor. So that's your hypermobility hack for today. And thank you so much to Pelvic Floor University for inviting me and Dr. Brooke Winder. We had a fantastic time last night talking about hypermobile EDS, HSD, mast cell activation syndrome, and dysautonomia, and how they impact sexual health and the pelvic floor.
[46:01] And I have one other resource to share with you. As we showed you at the very beginning of the show, I know for sure Tessa is wearing her VIP Bendy Body shirt and Shanti ideas too. They're going to show them to you real quick.

[46:17] Tessa: Yay! Representing.

[46:18] Dr. Linda Bluestein: Okay, I love it, I love it. I think I told you that when I got mine in the mail, I like ripped open the package and immediately put it on because I was on my way to the airport. So I like ripped it open, put my shirt on, and changed what I was wearing immediately. I love it. It's so comfy. I love the material. I wear it all the time.

Shanti: Yeah.

[46:32] Dr. Linda Bluestein: So definitely let us know if you want some of these really, really cool VIP shirts. And in the meantime, you can check out all kinds of cool bendy bodies and hypermobility and EDS merch on the Bendy Bodies Boutique, which you can access through hypermobilitymd.com. You can mix and match designs to make things your own. There's home accessories and all kinds of goods. Thank you so much, Tessa and Shanti, for being here today with me and being on camera so that we can do this episode together. It was lots of fun.

[47:04] Tessa: Thanks so much for having us back on. We love doing these.

[47:07] Shanti: Yeah, it's super fun. And, and thanks to the fans for writing such good questions and for, for all of your great information to them.

[47:14] Dr. Linda Bluestein: Absolutely. To all the listeners, please keep the questions coming. They're fantastic. Yes, we love hearing from you.
[47:20] Well, that's it for today's episode. Thank you so much for all your fantastic questions. Please keep sending them in to bendybodyspodcast.com for a chance to be featured in a future episode. You can help us spread the word about connective tissue disorders by leaving a review and sharing the podcast. This really helps raise awareness about these complex conditions. Did you know that I offer one-on-one support for both clients and healthcare professionals? Whether you're living with a connective tissue disorder or caring for someone who is, I've got your back. Check out my coaching and mentorship options on the services page at hypermobilitymd.com.
[47:52] You can find me, Dr. Linda Bluestein, on Instagram, Facebook, Twitter, or LinkedIn at Hypermobility MD. You can find Human Content, my producing team, at Human Content Pods on TikTok and Instagram. You can find full video episodes up every week on YouTube at Bendy Bodies Podcast. I'm so glad you're enjoying the Bendy Bodies Podcast. We love bringing on guests with unique perspectives to share. However, these unscripted discussions do not reflect the views or opinions held by me or the Bendy Bodies team.
[48:18] Although we may share healthcare perspectives on the podcast, no statements shared on Bendy Bodies should be considered medical advice. Please always consult with a qualified healthcare provider for your own care. To learn about the Bendy Bodies program Disclaimer and Ethics Policy, Submission Verification and Licensing Terms, and HIPAA Release Terms, or to reach out with any questions, please visit bendybodyspodcast.com.
[48:38] Bendy Bodies Podcast is a human content production. Thank you for being a part of our community, and we'll catch you next time on the Bendy Bodies Podcast.