Navigating Pregnancy with Shanda Dorff, MD

[00:11] Jennifer Milner: Welcome back to Bendy Bodies with the Hypermobility MD, where we explore the intersection of health and hypermobility, focusing on dancers and other aesthetic athletes. This is co-host Jennifer Milner here with the founder of Bendy Bodies, Dr. Linda Bluestein.

[00:29] Dr. Linda Bluestein: Our goal is to bring you state-of-the-art medical information to help you live your best life. Please remember to always consult with your own healthcare team before making any changes to your routine.

[00:39] Jennifer Milner: Our guest today is Dr. Shanda Dorff, founder of Complex Cares, an organization that focuses on connective tissue diseases, mast cell diseases, POTS, and dysautonomias. Hi, Dr. Dorff, and welcome to Bendy Bodies.

[01:00] Shanda Dorff, MD: Hi, it's a pleasure to be here and thanks for inviting me.

[01:09] Jennifer Milner: Absolutely, we're thrilled to get to chat with you. Yes, we are. Can you start out by telling us a little bit about yourself?

[01:19] Shanda Dorff, MD: I grew up in a small town in southern Missouri where I had no idea that being double-jointed was abnormal, just because I always was around my mom and other family members who could do the exact same things that I could, so it very much seemed normal. I now know that that just means genetic. But I was also a kid that was often very sick and thought there had to be a better way. And so it was at that point, just dealing with a lot of illnesses as a child, that I decided at age 7 that I was going to be a physician.
[01:47] But it was when in residency and rotating with various different specialists who were screening some of their patients for connective tissue disease and hypermobility — in particular, the sports medicine doctors, the dermatologists, and I even did some elective rotation with the geneticists — that I found it kind of strange that when they would ask patients to do various different hypermobility exam type of testing assessments, the patients would look at them almost grossed out. Like, what are you talking about? And to me, it just seemed like, oh, come on. Surely you can do this. Let's just bend it back this way and move on. And the ones I was rotating with would kind of whisper to me, sort of like, you need to get that checked out.
[02:30] So I got to thinking, well, wait a minute, maybe these things aren't as normal as what I was expecting growing up. When I went in for my regular annual checkup with my physician that first year in residency — after I'd had at that point three different people telling me I need to get evaluated for it — I just asked and was told that it was something that would only make a difference if you're going into the NFL. They asked me if I was changing careers. I was never particularly good at football, although I do think it's fun to watch. So I just didn't really give it too much more thought.
[03:01] But then as I got back into working with patients during other rotations, it kept coming back into my mind that, well, maybe there's more to it than just being told this isn't going to matter. So that's when I started reading a lot more into it and working a lot more with it. In part due to my own curiosity, due to what I thought at the time might even be a little bit of selfish reasons — wanting to try to find out for myself, or is there a way that I could prevent a potential problem in myself? But also as I was identifying things — not only what to look out for, how to try to prevent things, how to retrain the muscle memory, retrain the body — I was also applying what I was learning to any patients I felt could potentially benefit from it.
[03:46] Over the years, more and more patients, including sometimes four or more generations of a single family, came to me. And it really just became more and more of a passion. By this point, I had already been found to have the classical type of EDS. I was also really surprised when I was pregnant — and I'd also been found to have mast cell disease — that especially during my pregnancy with my daughter, my second child, whenever I was having a problem or difficulty from the mast cell disease, people would say, well, you're pregnant, we don't know what to do, so we can't do anything. I was like, well, I guarantee that every single patient is the result of someone being pregnant at some time, and I'm sure that I'm not the very first patient who has been pregnant and has had mast cell disease at the same time. They were very quick to say there was nothing they could go from to have any reference. But I knew all the information was there if you would just piece it together. It just maybe wasn't in a single concise source.
And so while I was pregnant, I decided that once I'd had this baby — because it was a very, very difficult, challenging pregnancy — I was going to make sure that others wouldn't be without resources or without the information out there. That's why I started gathering data, research, ideas, notes, things like that before I actually went to construct the paper itself or, for that matter, the Disjointed book chapter. It was because I knew what it was like to feel like you need help, but then others not knowing where to go to get the information to guide them and not wanting to risk doing something wrong and feeling like you were stuck.
So that's why I continued with the passion of working with connective tissue disease and with women's health, prenatal care, and things like that — wanting to make sure to hopefully be able to help many future generations of people worldwide, not even just in my community. And ultimately it was in working with these patient populations, because these conditions involve multiple different body systems, that I found no matter how hard I tried, I was never able to consistently capture everything and feel like I was reviewing everything and answering all their questions within a typical 15- to 20-minute doctor's appointment. I tried for years and I always felt like I was missing something or always having to play catch-up.
[06:35] And so now that I am my own separate entity at Complex Cares LLC, I actually get to feel pretty spoiled because it lets me have as much time as needed to ensure getting to hear each and every person's unique story. Some may have more detours to their life adventure path; others, it may be more of a straight shot. But no matter what, every story is important, every story is unique, and every story deserves and needs to be told. This way, without the time limits that more typical practices have, it now allows me to really get to know their stories.

[07:21] Jennifer Milner: I love that. And I love hearing through your story that it seems like your whole life you've been a problem solver, and that from a very early age you wanted to do medicine. Every time you move forward, you see a problem and you're like, well, that's a problem, let's solve it. I don't feel good, there are reasons, let me look it up, let's solve it. You have this instinctive detectiveness to you, but also an instinctive desire to fill that hole and fill that space. If you see a wrong, you want to right it, or if you see an emptiness, you want to fill it.
[07:57] And it sounds like the research that you've done for yourself, and sort of putting together your own connective tissue disorder program as you moved forward, has really helped you with your own health, but then your health has spurred you forward to help other people. You've seen this hole with pregnancies and postpartum and women's fertility health and thought, well, somebody needs to do something about that — I think it's going to be me. It's so fantastic to meet someone like that, someone who continues to feel that passion and that drive to move forward. And it's great to see how your story brings you to this really interesting place that makes you uniquely qualified to help all of these people.
[08:46] So when we're talking about, if someone is contemplating pregnancy and they have EDS or another connective tissue disorder, what are some important considerations for someone like that?

[09:01] Shanda Dorff, MD: First, I just want to say thank you. Some things for them to keep in mind initially are true for everyone regardless of connective tissue disease — the fact that it's important, whenever possible, to try to make sure that you are at your healthiest before actively trying to get pregnant, as well as trying to minimize and, when possible, get off of or transition to medications that would not potentially be harmful or dangerous to a pregnancy or to the delivery.
[09:38] Also, it's very important to remember that not everyone is able to get pregnant the very first time they try. They only consider it abnormal or want to do further investigation if they anticipate the mom will be under 35 years of age at the time of delivery after a whole year of trying without successfully conceiving, versus they would start investigating after about six months if they anticipate the mom would be age 35 or older at the time of delivery, just from a risk stratification standpoint.
[10:07] But specifically with regards to EDS or hypermobility spectrum disorder, while there is a very wide range of potential complications that can happen, it doesn't mean that one person is going to have all of them. Some people may not even actually fully experience any complications at all, just the potential for them. So it's important to have it on the radar screen but also not let it overwhelm you, not let you feel like it would be impossible, like, I could never get pregnant because of every possible thing that could happen. Because in life, there's an infinite number of things that could happen, even completely unrelated to hypermobility or EDS. We're fortunate in the fact that we don't experience everything altogether. We just want to make sure that we know what to look for, that we are monitoring, that we're working to try to prevent things whenever possible, and that we're working with clinicians who are aware of what can be very helpful for us — so that if something were to develop, it's able to be identified sooner and taken care of sooner, and doesn't risk potentially spiraling.
[11:14] Also, don't be alarmed just by being told that the pregnancy would be considered high risk, because many, many pregnancies are considered high risk. In the US, for example, pretty much anytime a woman has a diagnosis before she gets pregnant, it'll make it considered high risk. And there's a wide range of severity within that high-risk category, some much more severe than others. But even if a woman has a BMI of 30 or above before conceiving — which would technically mean she is obese — even if she has no other health issues whatsoever, they would consider that higher risk because of the higher potential risk for gestational diabetes and things like that. So they just want to make sure they're watching it closer. It doesn't mean that there's going to be a problem. It just means they want to watch closer.
[12:00] Now, there are definitely some conditions that are much higher risk within that high-risk category, but those are usually separate from a connective tissue disease. Also, make sure whenever possible that you get a chance to try to meet with a prenatal provider, whether it be an obstetrician, family physician, or midwife — in various different countries, there are different ones who do the prenatal care and deliveries — and try to do this planning beforehand. You also may want to meet with a genetic counselor to discuss risks potentially not only to the mom but for the baby inheriting the condition.
[12:39] Most types of EDS — but not all — including but not limited to the most common type, the hypermobile type, are inherited with what they call an autosomal dominant pattern. So it doesn't matter which gender the parent is; the baby would only need to inherit it from just one parent in order to have it. But with it being a dominant trait, it's not something that would skip a generation. It's one that if it's there, it's there. I tend to think of it similar to how dark hair is dominant to blonde or red hair. Sometimes blonde or red hair may kind of skip a generation, but dark hair — provided it's their natural hair color, not from some beautiful bottle — wouldn't tend to skip a generation.

[13:23] Jennifer Milner: That makes sense. So it sounds like you're saying, first of all, try to be in good health as you're considering pregnancy — no one's ever going to be in perfect health, and it means something different for everyone, especially for a lot of our listeners. Try to be in good health. Don't panic.

[13:27] Shanda Dorff, MD: Right.

[13:44] Jennifer Milner: Don't let all the possibilities overwhelm you, and consult with some experts, like you said, a geneticist. That seems doable, that seems manageable. Thank you.

[13:58] Dr. Linda Bluestein: And I wanted to ask — in the Disjointed book, we focused on hypermobile EDS and hypermobility spectrum disorder. Would your advice be different if, in the rare case that they're already diagnosed with classical or vascular or one of the other, much more rare forms of EDS?

[14:18] Shanda Dorff, MD: The risk is the greatest by far with the vascular type. And that is one that without a doubt, absolutely essential — not just may want to consider, but absolutely essential — to be meeting with a geneticist or genetic counselor. Not everyone may have access to a geneticist, and I think there are far too few of them out there. But that type not only was the first one they had reliable genetic markers for, so it's much easier to screen for, but it's also the only type associated with dying young. It carries the greatest risk to mom and unborn baby during pregnancy because the uterus itself is a very highly vascularized organ. During pregnancy, you have to have increased vasodilation — where the blood vessels get a little bit bigger — to accommodate more blood flow, because there has to be enough to cross the placenta to feed the fetus. And so when you're dealing with the vascular type, where the blood vessels themselves are the most fragile, and they're having to get stretched out, there is a much greater risk for them to shred or burst, and a much greater risk for uterine rupture.
[15:25] That's why it's very, very difficult for a vascular EDS pregnancy to ever go to full term. Technically, full term is three weeks before the due date itself, but they usually don't allow vascular EDS pregnancies to reach the due date because they know that it puts so much strain on those vessels. The risk for rupture or shredding that could result in loss of both mom and baby is extremely great. That's why they usually try to give some steroids to the mom to cross the placenta and ensure the baby's lungs develop in time, and then try to deliver around 36 weeks if possible.
[16:09] With the classical type, you have a higher risk for uterine rupture, especially if they've had a prior C-section. If they haven't had a prior C-section, then the risk goes down much, much lower — still not anywhere near as high as the risk with the vascular type in general, but it's still there. With the classical type, it's the skin and soft tissue that has a much greater impact. And so if you have a scar across the uterus — and the most common type of C-section these days is what they call a low transverse, which is where they go horizontally across the bottom part of the uterus, just above the pubic bone — as the uterus is growing, kind of going from looking like a flattened-out little balloon to a hot air balloon, that can be stretching out the scars. And ones with the classical type, much more so than individuals with the hypermobile type — though it still happens with the hypermobile type — can have problems with scarring. Not only can it become very widened, sometimes it can become a lot thinner. Sometimes it can become really uncoordinated in how it heals, and the same is true for the deeper tissues such as the uterus.
[17:33] And so if it's stretching out and becoming thinner, and especially if your body starts trying to go into labor, it might end up getting a little confused. It might start thinning at the scar site instead of at the cervix itself. The cervix is that bottom one-third of the uterus that's supposed to be changing when a woman is getting ready to have a baby — becoming thinner, more opened up, getting ready for a baby to pass through. But if all of the body's forces are going right to that scar site, then you can actually tell on ultrasound — and I know this firsthand because I've had it twice — where if they do the ultrasound, the lower uterine segment, right over where that scar site was, the myometrium, which is the muscle wall of the uterus, is gone. You develop what they call a uterine window, where you can literally see through it. If the skin were open, you can actually see through because that muscle is gone. On the ultrasound, you'll no longer see the wall layer of muscle going all the way down across the entire front of the uterus, and whenever the baby moves or kicks, the part without the myometrium just kind of floats on the ultrasound.
And on the operating table, when they open up the skin incision and can see the uterus, when the myometrium is gone, you can actually see through it with your own eyes before you've even cut it, because it's usually down to only about two or three cells deep. I know in the case with my daughter, my second child, I remember being on the operating table and hearing, "Hmm, interesting," and thinking, what? And they said, "I can actually see your daughter moving right through it — you're not supposed to see through solid organs." And then, before they even got a chance to apply the scalpel, they just touched the uterus with their sterile gloved hands to get ready, and it completely split open at the scar site.

[19:59] Dr. Linda Bluestein: Wow.

[20:00] Shanda Dorff, MD: Because it had thinned out so much. And so it actually made me really grateful that I was right there and was already getting ready to meet my now amazing three-nager. But because of the classical type, we knew to expect there could be challenges and problems with the scarring. Now, not everyone with the classical type will experience that. It's just something that they want to make sure they're able to watch and monitor for. That's why in my third pregnancy, they were closely monitoring that segment, and it went really smoothly — so much nicer, so much easier — because as soon as the change happened, they were able to address things right away.

[20:40] Dr. Linda Bluestein: That's fascinating. I've been in many C-sections and I've never seen that before. As you're describing it, I have this visual of the nice thick uterine wall and how it normally looks before they make that incision. That's really interesting.
[20:59] Thank goodness the classical type and the vascular type and the other subtypes are so much more rare than hypermobile EDS and HSD. I just want to reiterate that, because oftentimes when we start talking about those, people will hear little bits of themselves in parts of the story. And for the person that has it — like in your case — it doesn't in a way matter how rare it is, because you have it 100%. But just for the other people listening, just so they realize that those conditions are much, much more rare than the hypermobile EDS and HSD.

[21:37] Shanda Dorff, MD: It is definitely very, very, very much more rare and very uncommon, even for those with the classical type, to experience that. But it's just something to have on the radar screen. That's why it's so important to make sure that whichever clinician is working to help guide and care for the pregnancy knows the patient's health history and feels comfortable knowing what to look out for and understanding what types of risk precautions and monitoring are needed — because that can make such a huge difference. It can really turn what could have been a potentially very tragic experience into a very beautiful, wonderful one, like in the case with my amazing daughters.

[22:23] Dr. Linda Bluestein: Well, that's great. And so what other things during pregnancy should people be watching for, whether they have an actual diagnosis or they suspect they fall into the category of one of these diagnoses?

[22:41] Shanda Dorff, MD: Well, initially, some of the most common things present in ones with hypermobility — whether it be full EDS or just on the hypermobile spectrum — is that you can experience more challenges from instability. The progesterone and pregnancy hormones that are designed to allow for the pelvis to change to accommodate a growing uterus don't tend to say, "We're only going to do this at a certain moment in time." And especially if things are already extra flexible, lax, or hypermobile to begin with, you might start noticing those changes even earlier on.
[23:11] And so you might be needing an SI belt, or sacroiliac belt, because you could be noticing the muscles around the pelvis starting to get sore because the joints in there could be slipping a little bit. Sometimes you can get spasms. Sometimes you can get discomforts. I know for myself, I was actually kind of surprised when the SI joint came out, because I remembered being told in med school that that was one of the few joints in the body that doesn't actually move. I now know that it does move. But it felt like it was just kind of farther back and deeper than what would be in alignment with the ovary — it was actually the SI joint. I was literally able to hear the pop, and it was like the pain instantly went away, despite all kinds of imaging and ultrasounds showing, well, there was no cyst. It was just because of the SI joints.
[24:01] The pubic bones likewise can also shift and move around. So that's where it's helpful to work with physical therapists. Having the SI belts, making sure you might have a need for a back support, because you can notice back spasms, pain, and scoliosis can be worse during pregnancy. You could have more need for chiropractic or osteopathic adjustment as well.
[24:25] Also, there is a higher risk for breakthrough bleeding, which would be like spotting during pregnancy, or potential miscarriage risk. But that miscarriage risk is much more so in individuals with the vascular type — which, as we talked about, is very rare — or in individuals with the classical type, especially if they've had a prior LEEP or cone procedure. Those are procedures where after a woman has had an abnormal pap smear, she might need to have multiple biopsies of the cervix or different treatments to remove any cancerous or precancerous cells from HPV. That can result in the cervix being a little bit thinner, and she may need what's called a cerclage, which is where they put a stitch in there to kind of loop it up almost like a drawstring, keeping the cervix closed until ready to have the baby. They can just remove the stitch, and it works to contain the pregnancy and allow it to be viable and do well.
Also, there's increased risk for swelling. Most don't really have a lot of cardiovascular effects or problems. In fact, it's really interesting that many patients with POTS — and I believe it's around 70% of POTS patients — actually find themselves feeling better during pregnancy because of the increased fluid volume needed to accommodate the growing fetus. Having all the additional fluid volume and fluid flow can be really helpful for them. It can really reduce a lot of their POTS symptoms and sometimes even reduce the need for some of their medications.
[25:54] But even though someone could have EDS or hypermobile spectrum, they also need to make sure they don't risk ignoring or forgetting about some of just the general pregnancy things to be on the lookout for and to let their doctors know about. For example: if you've been noticing and feeling the baby move fine, but all of a sudden you're not able to feel the baby move anymore. If you're having a significant amount of vaginal bleeding — more than just spotting. If you're noticing the water breaking or amniotic fluid leaking early or in large gushes. If you're noticing contractions happening — even if it's when you'd be expecting to have the baby, it's still important to make sure you're monitoring and letting them know and getting to the place where you can deliver. If you're having severe pain, severe headaches, if your blood pressure is becoming dangerously high — those are all things that are really important to be on the lookout for, for anyone who is pregnant, with or without a connective tissue disease.

[26:52] Dr. Linda Bluestein: That's a fabulous list. Such great information. And what about during delivery? What are some of the complications that can occur during delivery, and how do you recommend preparing for them?

[27:05] Shanda Dorff, MD: Well, the first way to prepare — whenever possible — is to try to plan to deliver someplace that has the ability to perform a C-section if needed. Now, it does not mean that someone would have to have a C-section. It doesn't mean that people with hypermobility should just go straight to a C-section, because that is definitely still considered more physically challenging for the body than a vaginal delivery. However, even in people without any known health issues whatsoever, sometimes surprises happen. So whenever possible, try to deliver at a place where if an emergency were to happen, they could do a C-section if needed.
[27:45] But specifically with regards to EDS, connective tissue disease, or hypermobile spectrum, there can be a higher likelihood for a breech presentation, which is where the baby is in the same direction as the mom — head up higher and bottom down lower. Usually babies, especially when the mom has hypermobility as well as the baby, are a lot more able to keep twisting, turning, and moving around into whatever position they want. They're already showing their own personalities early on because the uterus is more flexible and better able to accommodate, and they're more bendy and flexible so they can twist themselves however they're wanting to be.
[28:21] So it isn't necessarily something you can assume — just because a baby happens to be head down at one doctor's visit doesn't mean they're going to stay that way. Likewise, if they happen to be breech, it doesn't necessarily mean they would stay that way. And sometimes even if they do what's called a version, where they're trying to turn the baby to make sure it would be head down — which is the less challenging way to deliver vaginally, because it's a smaller size for the head to pass through than would be for the buttocks — you'd have a higher tearing risk if the baby were delivering with their buttock cheeks first as opposed to their facial cheeks and their beautiful faces.
[29:04] Also, there is increased risk for tearing. Sometimes they'll do what's called an episiotomy to allow for the passage of a baby through the birth canal, where they make a cut to open up the amount of space for the baby to go through. There are a couple of different approaches. The original types of episiotomies were the midline ones, which if you can imagine a clock face, go straight down around the six o'clock position. The biggest challenge with that, especially in people with hypermobility or connective tissue disease, is if it were to extend, it extends straight back — right down into the anus — and can result in what's called a fourth-degree tear. That's the most severe type of tear from a delivery, where it tears all the way through the wall of the vagina into the rectum.
[29:53] If they do a mediolateral one instead, where they're going at about a 45-degree angle, if it were to extend, it will extend out towards the mom's thighs but not down towards the rectum. So it doesn't risk the most severe types of vaginal tears. It can sometimes be more painful as it's healing because it does cut across some of the skeletal muscles there, but it can definitely still heal.
A fourth-degree tear is also a higher risk in general, even without an episiotomy, for ones with the very rare vascular type of EDS, especially if it happens to be a really rapid delivery. And ones with connective tissue disease in general can have a faster delivery, especially after the water breaks — sometimes it's like, all right, free flow, let's go — just because things can stretch a lot quicker and a lot easier. But they don't have as much of the risk of the fourth degree in any non-vascular type. And remember, the vascular type is very rare, which does provide a little bit more reassurance for most.
[31:18] Also, you can have something called PROM or PPROM — and this is not referring to a dance. PROM refers to premature rupture of membranes, where the water breaks before active labor. In the case of PROM, you're full term but your body isn't having the cervix change and contract consistently. PPROM, or preterm premature rupture of membranes, is where the water breaks before you're going into labor and you're not full term yet either. That's more common in ones with the classical type, which is still rare — not as rare as the vascular type, but much more rare than the hypermobile type or just the hypermobile spectrum in general.
[32:04] With regards to anesthesia and analgesia, some patients with connective tissue disease have difficulty being able to find something that works quite right to numb them.

[32:14] Dr. Linda Bluestein: What about in the immediate time after delivery or in the subsequent weeks? Are there certain things that you would tell people to be looking for or doing?

[32:28] Shanda Dorff, MD: Key things are: you want to make sure to be avoiding a lot of heavy lifting, especially for ones with hypermobility, because you don't want to add to the increased risk for pelvic floor prolapse. That's one that can happen in general with hypermobility and connective tissue disease — a greater risk in women, especially after they've had multiple vaginal deliveries. If you do a lot of heavy lifting, that can be a challenge too, and that's why doctors advise everyone not to be doing heavy lifting immediately after having a baby.
[32:55] But in particular with regards to the pelvic floor, there are multiple different aspects of it. Not only does it involve the muscles along the base and the Kegel exercises — which are not strictly just for right after delivery, but are actually good practice to do at all times — you can also develop what's called a cystocele, which is where the bladder kind of falls backward against the front or anterior wall of the vagina, sometimes making it difficult to empty the bladder. You can have what's called a rectocele, which is where the rectum kind of falls forward against the posterior wall of the vagina, making it tough to empty out the rectum or have a bowel movement, because the walls of the vagina are made of defective tissue in someone with connective tissue disease and are not able to be as strong in general. You can also have prolapse where things are actually falling more toward the outside. That's even true for the uterus itself — where it's actually going down rather than the "seals," where it's kind of falling into the vagina.
Scarring can also be a challenge. You can have problems with excess scarring; it can just become a big tangled mess. It can become really wide, it can thin out, it can develop keloid scars. You can have problems with dehiscence, which is where it pops open — kind of like what I described from my own personal experience with the uterine scar from a prior C-section, where it just kind of thinned and thinned because the uterus was stretching out farther and farther in a subsequent pregnancy.
Also — and this is more for the clinician, whether it be a physician, midwife, nurse practitioner, or physician assistant — you want to make sure to avoid pulling too tight with the stitches, whether it be on a C-section, a vaginal suture, or anything. With connective tissue disease, if you pull it too tight, it'll just shred — it tears right through. You want to pull it tight enough where things are able to touch, but not where it feels like it's super glued for life. Pull it where it touches, put them closer together. Sometimes they need to do multiple layers to provide added support, but that way it doesn't risk shredding the tissue you've got. Allow more time for healing — it's more of a slow and steady wins the race approach. You are able to get to the same goal outcomes and get back to the way things were; just remember to have patience and allow your body the time it needs to heal.
[35:36] Also may need to avoid adhesives if the person has an adhesive allergy — whether it be glue, Steri-Strips, things like that.
[35:45] If a person has problems with bleeding or postpartum hemorrhage — and the risk for that does go up in individuals with EDS or hypermobile spectrum disorder compared to the general population. In the general population it's about 3%, versus ones with connective tissue disease, where it goes up to 5%. So still around 95% of the time it is not an issue, which is really reassuring. It's just a slightly higher risk going from 3 to 5 in ones with EDS or hypermobile spectrum.
[36:15] That's where making sure the ones doing the delivery are prepared to be able to manage it comes in. Common management strategies include things like oxytocin, sometimes called Pitocin, usually done via IV — they usually start it right after the baby and after the placenta has been delivered to help the uterus contract down and stop bleeding. Misoprostol, or sometimes called Cytotec, is a little tablet that can be placed in the rectum that the woman absorbs that way to help reduce bleeding. DDAVP is another great option that can be used — a lot of times it's also very helpful with POTS as well, and that's something anesthesiologists are usually very familiar with and have on standby.
[36:58] I have also seen some cases of more prolonged postpartum bleeding that resulted in hemorrhage from the cumulative volume — not immediately after delivery, but over time — in mast cell patients, where they might end up using some transvaginal mast cell medications such as dye-free diphenhydramine, which is the active ingredient of Benadryl, or Cromolyn inside the vagina. But that's one where, if you want to know whether the bleeding or challenge is strictly related to mast cells as opposed to a typical gynecologic or obstetric source: if it's related to obstetrics or gynecology, the Cromolyn or diphenhydramine isn't going to do anything. If it's related to the mast cells, you'll get a response right away. So that makes it really fast and simple to distinguish.
[37:42] But those would not be expected to be the issue immediately after delivery. That would be more for prolonged cases, because immediately after, you first have to make sure the uterus is clamping down, and whether there's an additional tear or bleeding source that might have been missed. You might need to cauterize if you're in a C-section, or have other stitches placed. You can also still use the oxytocin, Cytotec, or DDAVP at the same time as you're taking care of the bleeding source.
And thinking about it more — there's also a potential increased risk for hip, pelvis, SI joint, back, and lower extremity instability just from the delivery itself and from the position you're in when trying to push the baby. That can be a challenge for the knees, hips, and lower back, especially for individuals who are hypermobile.

[38:39] Jennifer Milner: And I think it's worth mentioning again that we are having this conversation with Dr. Dorff about all possible complications that could happen during pregnancy, delivery, and postpartum. So it's important as listeners hear this that they not think all of this is about to happen to them, right? As you mentioned earlier, Dr. Bluestein, you can see pieces of yourself in some of these stories, and that's really easy for us. So it's just a reminder — we are trying to pick Dr. Dorff's brain because she is such a wealth of knowledge. We're not saying this is what's going to happen with every single hypermobile person who becomes pregnant. Most do not have a lot of problems.

[39:28] Shanda Dorff, MD: Most do quite well, and really great. Matter of fact, the majority of pregnancies, even ones with connective tissue disease, the vast majority go beautifully without problems.

[39:42] Jennifer Milner: Which is so great to hear, of course, for anybody who's considering pregnancy or is pregnant. I want to get to breastfeeding, but really quick, I just wanted to ask — because most of our population that listens are dancers or other artistic athletes — are there special contraindications or precautions you would take for exercise with this population during pregnancy or after pregnancy? Or would you not consider that to be very different from other precautions for most pregnant people?

[40:13] Shanda Dorff, MD: I would try to make sure in those individuals that they have good supports for the pelvis when they're doing some of their activities, whether it be with an SI belt or pregnancy belt, things like that. Because not only are they generally very toned — with excellent muscle strength and very skilled in their movement — they are not going from a very sedentary lifestyle to all of a sudden wanting to be doing something very acrobatic. These are ones that are very skilled, very trained. They also know their body very well.
[40:49] But don't be afraid to let yourself have just a little bit of additional support when you're doing some of the movements or activities, so that you can still be enjoying the dance that you love without risking the added challenges from the hormonal influences that could make something go out of joint a little bit easier, that could limit some of your ability to do the dancing you want, and that could potentially cause discomforts or pain too.

[41:14] Jennifer Milner: Excellent, thank you. So moving to breastfeeding — are there any special considerations that people with hypermobility should take during that time?

[41:26] Shanda Dorff, MD: Yes, and a lot of it depends on which type of connective tissue disease you have. For example, with the classical, classical-like, or dermatosporaxis type, there's a much greater risk that the skin of the breast tissue or the areola is very lax or hyperextensible, where it might actually seem like the mammary glands or the breast tissue itself isn't starting until lower down, with just kind of looser excess skin up top. Or you might need to feel like you're kind of lifting things up, or may have the areola more flattened out or not really staying back when a baby is trying to nurse — it might kind of fall forward across the baby's mouth and nose. So you might just need to have a gentle hand placed on the breast tissue, or feed through a nursing bra, so that it works to hold the looser or hyperextensible skin out of the way so the baby is fully able to breathe, nurse, and things like that.
[42:20] Some things that can be helpful if you happen to have a more flattened or inverted nipple are using a nipple shield or using a breast pump first, because that can use a little suction to kind of draw things out. Now, sometimes that can be very tender, especially in the beginning. And many moms — even if it's not their first time breastfeeding — when their body is first starting to lactate and getting that colostrum, it tends to look more like egg yolk coming out before it ends up looking like what they tend to think of as milk. It is pretty sensitive on there and can sometimes be very painful for some, while others are just more sensitive. Your body does work to adjust.
[43:02] But don't be afraid to ask for help, whether it be from a lactation consultant, a breastfeeding medicine specialist, the clinicians who delivered the baby, or the baby's doctor. There are lots of resources out there. You are not alone. There are also many support groups made up of moms wanting to make sure that both mom and baby are able to have a successful breastfeeding journey.
[43:26] Also, sometimes the babies may seem like they're latching on just fine but might not be able to suck as well as needed. Tongue tie can be a challenge for them. And especially if the baby happens to have hypermobility, it may seem like their tongue is able to stick out just fine because things are really stretchy, but they may not be able to suck as well as will be needed to draw the milk out. A way that you can kind of check for that is to put a little finger in the baby's mouth — the baby should immediately and automatically, through a reflex, start trying to suck on it to draw milk out. That is what the baby is supposed to do. If they're not able to have a strong grip and you can easily just slide your finger right out as opposed to feeling that resistance because baby has gripped on and is trying to suck your finger, then you might need to get the baby checked to see if there's a tongue tie. To take care of it, it's actually a really simple, usually in-office procedure where they just use little sterile scissors to snip the little extra tissue that's holding the tongue down a little bit, and then it's free and entirely fine.
[44:38] Raynaud's of the nipple can also happen. Raynaud's — much like when you think of it for the fingers or toes, where it can make things get discolored, sometimes blue or purple, sometimes super pale, cutting off blood flow and causing a lot of pain — that can also happen to the nipple. In particular with the nipple, you tend to notice it either right after the shower, because of the warm moisture of the shower, or right after the baby has been nursing, because the inside of the baby's mouth is body temperature and moist with saliva. As soon as it gets exposed to regular room temperature, it's like an immediate feeling of something really cold coming through, which can constrict the blood vessels. Sometimes that can cause a lot of pain, which can limit the mom's ability to feel comfortable doing the breastfeeding.
[45:19] In those settings, maybe needing a small amount of nifedipine — which is just a calcium channel blocker that's been around for a really long time — can help with Raynaud's there to allow the blood vessels to open up and improve circulation. But you just want to make sure, again, to check with your doctor to make sure it would be a safe potential medication for the individual patient.

[45:39] Jennifer Milner: Excellent. That's a lot of really helpful information on that. I know that with all the work you do with connective tissue disorders, you also work with the different comorbidities that are often common with some of them. So what might some special considerations be during pregnancy for mast cell disease?

[46:00] Shanda Dorff, MD: Key things are: check the safety categories of the medicines you're taking, whether it be mast cell-specific medications or other medications, for both pregnancy safety as well as lactation safety afterwards. Because you always want to make sure you're doing what's safest for you as well as for the baby whenever possible. Sometimes a surprise may happen — not everyone is always able to fully plan every pregnancy, and we fully understand that. But whenever you're aware of a pregnancy, or whenever you have the chance to plan, try to make sure you're having the safest medications. The safety categories are widely available, and your doctors are able to help guide you through those, kind of like how even before a surgery they go through your list of medications and what's safe to take for a procedure.
[46:49] In general, from a medication standpoint during pregnancy, it's best — because you're really not able to get a lot of what they call the gold standard double-blinded studies when someone is pregnant, just from a general medical ethics standpoint, so the studies are usually more after-the-fact observational. Someone had to have the medicine and they couldn't go without it. So you just want to be able to have as low an amount of medication as possible to allow you to remain functional. You may not be feeling like you're on top of the world, but you still need to be able to do the things you need to do, just with the lowest amount possible, because that makes the least chance for it to potentially affect the baby across the placenta.
[47:34] Also, make sure that you have a prenatal and delivery team in place that understands your needs. Make sure they understand, for example, what mast cell disease is. Some do, some don't. Sometimes it's helpful to have multiple people involved working together, just to make sure that if you were to have any type of reaction or problem — whether it be during pregnancy, during delivery, or even afterwards — they're prepared to be able to manage it. Sometimes it may end up needing to be you bringing some of your own medications from home if you have something that's compounded, for example, because not every hospital is able to have that readily available. So it's important to make sure your doctors know if you need things compounded, because a lot of hospitals want to make sure that they're only administering medicines they feel comfortable with and that they know about.
[48:31] Also, if you have a reaction, the severity of the reaction determines how aggressive they need to be with the treatments. If it's something that's pretty mild — maybe just a little more tired, just needing a little bit of a nap — versus if you're having itchiness, rash, irritation: you might just need some additional antihistamine, H1 or H2, maybe needing a steroid, maybe needing epinephrine if you're having anaphylaxis, which hopefully would never happen. But we do know that some people may experience that. So it's always important to make sure the ones in your care team know what symptoms to be on the lookout for and how to address it if something were to happen.
[49:08] And again, most of the time things go very smooth sailing and don't end up needing those additional precautions in place. But it's always better to make sure someone is prepared if the need were to arise rather than trying to figure out what happened and what to do in the midst of a moment when you could be struggling.
Sometimes you may want to pre-medicate before, such as if you happen to know in advance that you might need to have a C-section — for example, if the placenta is blocking the opening of the cervix, which is called placenta previa, and you cannot deliver the placenta first. There are some other reasons you might need a C-section too, but that particular reason is completely unrelated to the connective tissue disease. If you know in advance you might need it, you might want to pre-medicate by having an extra dose of some of your medicines around an hour or so before, to try to reduce the activation of your mast cells and bring them down to a more typical normal response.
[50:10] Also, you can have increased bruising, bleeding, fatigue, rashes, and increased hypermobility and instability. But in this case, it's multifactorial — not only do you have the progesterone affecting it, and the connective tissue disease affecting it, but the mast cells also release an enzyme called elastase 2, which works to make things extra stretchy as well. It can also affect mood and sleep, both in terms of ability to fall asleep, comfort while sleeping, and actually getting truly restful sleep.
[50:41] So it's not simple, but then again, pregnancy itself is not super simple either. And a lot of those things can be affected just from pregnancy itself. Pregnancy is also a very specific, limited duration of time. It is not something that's indefinite. And I think it's really great, because afterwards you get to see the result of all of it. I never really thought feet were particularly cute until I got to see my baby's little feet on ultrasound — I thought they were just absolutely adorable.

[51:20] Dr. Linda Bluestein: Absolutely. And obviously you have an incredible wealth of knowledge on these topics. Most people are not going to have access to you when they're pregnant or be able to get in to see you. So for people looking for someone to help care for them, do you have any suggestions — if they have one of these conditions or one of the comorbidities — of how they could find somebody who would be able to help them in the best possible way?

[51:22] Shanda Dorff, MD: The Ehlers-Danlos Society has a great list of resources. And like myself, I know there are at least in the Twin Cities multiple different OB-GYN and high-risk specialists who have become increasingly more familiar with this, do really well with it, and have been very receptive and open to doing peer-to-peer discussions and things like that.
[52:14] I have no problem also providing individuals with resources. For example, the obstetrics chapter of the book Disjointed is one that I wrote. And there's a bibliography in that chapter that lets you see where in the medical literature a lot of the different information came from. There's also in the Journal of Obstetrics and Gynecology, the article where I was lead author and Dr. Lawrence Afrin was co-author, about management of mast cell disease for pregnancy, delivery, postpartum, and lactation, and we tried to provide some helpful tables and resources to make those available to people anywhere — not strictly those who are able to come see me — to try to have comprehensive resources out there.
[52:56] Because a lot of what I've observed is: if you find clinicians that are really eager to learn and help, but they've been searching for anywhere from 30 minutes to an hour, sometimes two hours, and haven't found what they're looking for, sometimes they'll get frustrated and might stop looking. So that's where wanting to make things really quick, easy to find, and comprehensive comes in.
[53:17] I know you are also one of the wonderful authors of the Disjointed book yourself, and it has been very handy for a lot of — I know some of my patients have really enjoyed your chapters. And even the perioperative management recommendations for ones with Ehlers-Danlos syndrome that you authored — that journal article along with Dr. Pradeep Chopra — is another amazing, excellent resource. It talks specifically about obstetric as well as other surgical considerations too.

[53:46] Dr. Linda Bluestein: Yeah, and just like both of those articles you just mentioned, I think those are things that are not that long, probably, so people could print them out, highlight a few things, and at least have something to bring in. Because you're right — if some of those things are not found super quickly, and we know everyone has limited time, that's a great suggestion for people to look for those resources and take them in.

[54:11] Jennifer Milner: Well, I have learned a lot today. And I'm really grateful for you coming on here. I feel like I've learned that there definitely are special considerations for people with connective tissue disorders who might be trying to get pregnant, during pregnancy, postpartum, breastfeeding, and dealing with all their comorbidities. And that it's certainly something to keep in mind as you're going through the whole process, and it's really helpful to find someone who understands this process and who can specialize.
[54:47] It also sounds like you've recommended some great resources, including the book that you and Dr. Bluestein have both contributed to, as an easy way for people to at least find a place to start. Yes, Disjointed. And I know we talk about that book a fair amount on our podcast. And I just have to say once again that the authors are not making money off of this — the authors have contributed their time and their expertise in order to provide a wealth of information in one space for all things hypermobility-related. So every time we talk about it, Dr. Bluestein is not making a dollar. It really is a great resource because it has amazing people like Dr. Bluestein and like Dr. Dorff.
[55:35] So, finding a book like that, going to the EDS Society's page and finding those referrals — it is possible to find people out there who can help. I do believe that the medical community is moving to a place where they're more amenable to sharing information and speaking with other specialists about a patient they might have in common. I think we are definitely moving into that time. So there absolutely is help out there for people who are looking to go through a pregnancy with hypermobility and the other comorbidities.
Dr. Dorff, where can people find you if they want to get more information? What's your website, your Instagram — what's the best way to find you?

[56:25] Shanda Dorff, MD: So I'm at Complex Cares LLC, which is in Shoreview, Minnesota. Our website is complexcaresmn.com — that's "cares" with an S and MN for Minnesota, dot com. They can call, text, or leave a voicemail at 651-756-9596. And then we also have a clinic Facebook page, though I don't get a chance to be super active on it just because I'm super busy with patient care. On Twitter, I'm @carescomplex, though I'm not able to be on it very often.
[57:12] And then something else I just want everyone to be able to remember — because this was a lot of stuff to try to take in — remember, it does not mean you would experience all these things. Matter of fact, the vast majority go very beautifully without any problems at all. But if you're finding yourself feeling stressed or overwhelmed, I want you to take a chance to look in the mirror and specifically look at your shoulders. Pay attention to the size of them. There is a very limited amount of space between here and here. There is not room to carry the weight of the world on them.
[57:43] Lift up and unburden yourself. There is room for a shoulder to lean on, room for a hug, but there's not room to carry everything there. When you try to do that, it physically can make your neck hurt, your back hurt, be uncomfortable, and can really disrupt your sleep and everything else. Just take a peek at your shoulders. Remember, there's only so much space here. There's only just so much that I can do. It's okay that there's not more space there, because our bodies aren't made that way. Even though they're bendy, they can work to try to hold a lot of things — but we want to make sure we are allowing ourselves to feel that it's okay to say, I need to pause, or I need to take a little break. Or, okay, this is a lot for me right now, I just need to take a breath. And that's just fine.

[58:29] Jennifer Milner: Thank you, that's so well said. And I'm probably going to go back and listen to that about once a week after this podcast comes out as my little daily affirmation. That was beautiful. Thank you so much. We do forget that we don't have to do everything, and it is OK to take a pause sometimes.
[58:46] Well, you have been listening to Bendy Bodies with the Hypermobility MD. And today, we have been speaking with Dr. Shanda Dorff, founder of Complex Cares. Dr. Dorff, thank you so much for taking the time to come on the Bendy Bodies podcast and share your amazing expertise with us today.

[59:02] Shanda Dorff, MD: It was an absolute pleasure. Thank you for having me.

[59:05] Dr. Linda Bluestein: We love chatting with you. Thank you so much.

[59:08] Jennifer Milner: Absolutely. Thank you. And to everybody else, we will see you again soon.

[59:13] Shanda Dorff, MD: Bye.

[59:15] Dr. Linda Bluestein: Thank you for joining us for this episode of Bendy Bodies with the Hypermobility MD, where we explore the intersection of health and hypermobility for dancers and other aesthetic athletes. If you found this information valuable, please share it with a colleague or friend and leave us a review on your favorite podcast player. Remember to subscribe so you won't miss future episodes.
[59:36] If you want to follow us on Instagram, it's @bendy_bodies, and our website is www.bendybodies.org. If you want to follow Bendy Bodies founder and co-host Dr. Bluestein on Instagram, it's @hypermobilitymd, all one word, and her website is www.hypermobilitymd.com. If you want to follow co-host Jennifer Milner on Instagram, it's @jennifer.milner, M-I-L-N-E-R, and her website is www.jennifer-milner.com.
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[1:00:24] The thoughts and opinions expressed on this podcast are solely of the co-hosts and their guests. They do not necessarily represent the views and opinions of any organization. The thoughts and opinions do not constitute medical advice and should not be used in any legal capacity whatsoever. This information is not intended to diagnose, treat, cure, or prevent any disease, as this information is for educational purposes only and is not a substitute for medical advice, diagnosis, or treatment. Please refer to your local qualified health practitioner for all medical concerns.
[1:00:52] We'll catch you next time on the Bendy Bodies Podcast.