When Surgery Isn’t Simple: What hEDS Patients Should Know Before Going Under | Office Hours (Ep 170)

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Dr. Linda Bluestein: [00:00:00] GERD is extremely common in people with EDS or other connective tissue disorders. The connective tissue laxity leads to a weakened lower esophageal sphincter, and we can get these hiatal hernias, which is when you have like parts of the, the upper parts of the stomach actually coming up into the chest.

Welcome back every bendy body to the Bendy Bodies podcast. I'm your host. 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'll be answering some of your most pressing questions.

Pros and cons of wearing a cervical collar to surgery, nitrous oxide, M-T-H-F-R and EDS. [00:01:00] CRPS, EDS and MCAS. That's a lot of acronyms. We'll explain what those are later, of course, um, specific surgeries with EDS and so much more. But first I want to introduce two amazing people to you. Um, if you're watching the video, you're gonna see some new faces here.

We have two of the human content producers joining us today, Tessa and Shanti. We thought it would be fun to give you a little behind the scenes look for this office hours episode, so they'll be popping in to read some of your questions and to keep things rolling. Tessa and Shanti, thank you so much for being here.

Producer Tessa: Thanks for having us. 

Producer Shahnti: Thanks for having us, Linda. 

Producer Tessa: It's a little scary to be in front of the camera. This is new. 

Dr. Linda Bluestein: I think it's great 'cause I don't know that people realize like, how many people are involved in putting this together now. So I think it's really cool for them to get to see some of the faces and, you know, make that, make that connection.

So I'm super excited. Um, be sure to stick around until the very end so you don't miss our special hypermobility hack for the day. As always, this information is for educational purposes only and is not a substitute for [00:02:00] personalized medical advice. Here we go.

Okay, so today we are going to be covering some listener questions and uh, Tessa and Shanti, if you want to read some of these questions, that would be fantastic. So, um, I don't know, Tessa, if you wanna start with the first question and then we can discuss it. 

Producer Tessa: Yep. Absolutely. So the first question is from Adele, and she's talking about local anesthetic resistance and failed nerve blocks.

She says, I'd love it if you could discuss the phenomenon of hypermobile persons being resistant to local anesthetics. I've had terrible experiences where a nerve block that was supposed to control post-surgical pain failed and local anesthesia for a core breast biopsy failed. My dad had problems getting numb for a tooth extraction and had to switch to IV propofol.

What do we know about this? What should people with hypermobility do about it? It makes many medical procedures for me super scary. Doctors and dentists should know about this a lot more than they do. 

Dr. Linda Bluestein: [00:03:00] Yes, that is such a fantastic question and such an important topic, and I have to confess as an anesthesiologist before I started doing this work.

If you told me that local anesthetics sometimes fail, I'd be like, no. They work every single time. But now I know that that is absolutely not true. Local anesthetic resistance is an absolutely real phenomenon. Many people with EDS and HSD report that when they go to the dentist and they get numbed up, or if they get an epidural or a lidocaine injection, it often takes longer to set up.

It doesn't work or wears off faster or they need more medication. Um, this is not psychologic, it's not exaggerated. And we did talk about this a lot with Dr. Audrey Kershaw because she's an oral surgeon, so of course she's using local anesthesia a lot. So, um, I urge people to check out that episode as well.

There are biologic mechanisms at play here. This is not, you know, an anxiety thing. Um, there's connective tissue differences that can alter the diffusion of [00:04:00] the anesthetic through the tissue and reduce local concentration at the nerve endings. Um, there's also sodium channel variants like. The one called SCN nine A Polymorphism, and we're gonna talk more about that in just a little bit because that's actually super important when it comes to persistent pain.

And that can affect the anesthesia, uh, binding and nerve membrane excitability. We can also see things with altered blood flow. We can see abnormal microvascular structure and autonomic dysfunction, and these things can change absorption and metabolism of local anesthetics. Also increased nerve density or sensitization in some connective tissue, patients may lead to atypical pain signaling.

There's also pH differences in tissues that can happen due to chronic inflammation. And local anesthetics are dependent on having the right pH in the tissues. So if you have that or mast cell activity, that also contributes to inflammation that could reduce anesthetic efficacy. Um, what are some things that you can [00:05:00] do about this?

Okay. First of all, communicate early and often. Always tell your dentist, your surgeon, your anesthesiologist about your diagnosis and in particular about your previous anesthetic experiences. So if you've been to the dentist multiple times and they've tried to numb you up and it doesn't work, make sure that they know about that.

Um, also we can use multiple and also alternative techniques. So sometimes we can combine different, um, types of anesthetics. Like we can use lidocaine and bupivacaine, or ropivacaine, or mepivacaine. These are all different local anesthetics. Sometimes that can improve results. Um, sometimes adding something called bicarbonate that can also increase, uh, tissue penetration and help give us better results.

Um, if we use ultrasound guidance, if we're doing regional blocks, um, like a femoral nerve block or a popliteal block, or a Stella ganglion block, or an inner scale block, these are all different peripheral blocks that we do. And if you're using ultrasound, it helps you to get the medication more [00:06:00] precisely in the exact right location, and that will improve precision and outcomes.

Um, also sometimes you just need to take more time. So one time that I went to the dentist, they came back and checked, it was like, Nope, not yet numb. Yet, not numb yet. Um, and if it can take a really patient dentist to keep coming back and checking and waiting some additional time, but sometimes it just takes longer and you don't necessarily need more.

Um, also, epinephrine free formulations can be better for some patients that have dysautonomia or pots, um, because sometimes that uptake of epinephrine into the bloodstream can cause, you know, extreme tachycardia and increase our anxiety and make us feel really crummy. I would definitely recommend that people document what does and doesn't work.

Um, especially if you find something that does work and you find a method that was successful, make sure that you document that. Um, if you're a clinician, make sure that you validate your patient. Um, don't assume that failure is anxiety or that they're exaggerating. You should expect variable responses even within the same [00:07:00] patient at different times or different tissue sites.

Um, the pH of our body changes. At different times. It depends on how hydrated we are, et cetera. Um, you also want to consider different anesthetic choices buffering and how much volume you're using. You could consider adjuncts like clonidine or dme toine, um, so that you can get some prolonged analgesia that way.

And then I also wanna talk a little bit about this SCN nine a, um, gene. So it encodes the Nav 1.7 sodium channel. Um, this is a, you're gonna get a little geeky here for a minute. Um, this is a voltage gated sodium channel that's critical for pain signal transmission. And it's really important because it can cause gain of function mutations, and these can cause pain syndromes such as Ery Al Gia or paroxysmal Extreme pain Disorder.

So if people have paroxysmal Extreme Pain Disorder, they're gonna have extreme pain without much in the way of findings, and it's because of a genetic, uh, [00:08:00] mutation or genetic variant. So we can have congenital insensitivity to pain. So we suspect that there are also polymorphisms in addition to these mutations that may contribute to local anesthetic resistance or atypical pain sensitivity.

So you might have EDS contributing to some changes in how you p perceive pain, but you could also have this s CN nine aging. So, yeah, just wanted to make sure to mention that. I'm gonna mention a little bit more about primary rith myalgia. Um, this is caused by, like I mentioned, the gain of function mutation in this gene and it makes pain signaling nerves fire too easily.

Um, people who have this get burning pain, redness and warmth in their hands and feet, that's often triggered by heat. Exercise or stress. And you can see right away how this could be confusing with mast cell activation syndrome because people who have mast cell activation syndrome get redness, swelling, inflammation, pain, and also is triggered by [00:09:00] heat.

So there's clearly some, you know, overlap in presentations here. Um, these symptoms can be severe and disabling, and patients often seek relief by cooling the affected areas, which can also, um, lead to tissue injury if you overco the tissues. So there's a lot of overlap here. Some people with EDS or Dysautonomia report roal like episodes possibly due to similar small fiber nerve problems rather than a full SCN nine a mutation.

And then let's just talk very briefly before I go on to the next question about paroxysmal Extreme pain disorder. This is another gain of function mutation that can lead to sudden severe pain attacks in various different parts of the body. Attacks can be triggered by defecation or pooping, um, cold or even emotional stress, and sometimes are accompanied by flushing or autonomic symptoms, which of course is again confusing because that has a lot of overlap with mast cell activation.

So we can see this hyperexcitability of pain fibers, but with [00:10:00] different anatomic distributions and triggers. Alright, so these are just important things for us to be thinking about, uh, when it comes to people who have this, you know, reduced, uh, effectiveness of local anesthesia. You know, do you possibly have one of these other things that accompanies that problem?

So thank you so much for reading that question, and I think we have some more. Can I ask. 

Producer Tessa: Personal question and relevant question as you're talking about this. Yes. Um, I'm so curious about, and I think you've talked about this in a previous episode, but I'm so curious about the effect that anxiety has on the efficacy of anesthesia.

Because I, I went to the dentist. I had two, um, like dental surgeries and the first surgery they gave me. A dose of local anesthesia and I couldn't feel anything. It was perfect, but something went wrong in the procedure and it made it so that in the second dental procedure, I got the same dose of [00:11:00] anesthesia, but I was coming in very anxious.

I was like. Shaking. I had barely eaten. I was, I was just nervous for it. And I swear I could like feel everything and I was in immense pain, the whole procedure. Mm. And I'm just wondering like, is that a myth that anxiety affects, uh, anesthesia? 

Dr. Linda Bluestein: That's a great question. I love that question. Um, definitely anxiety affects the way we respond to medications in, in general, local anesthetics, um, sedatives, et cetera.

And so I can definitely see where that could be the. Where that could be the case. And you know, if you're more anxious, and like you said, you were barely eating anything, maybe you were more dehydrated, maybe your pH was different, um, you know, going into the, the whole procedure. So, um, that's the acid-based balance in our bodies.

So if you are not as well hydrated, um, then that's going to affect that. So I can definitely see where that could be the case. Plus, if you have. If you're more anxious and [00:12:00] your HPA or your hypothalamic pituitary axis is like more, um, turned on and activated, then you're gonna have a difference in the neurotransmitters that are active in your body.

So. I can definitely see that being the case, and I can give you another, uh, fascinating example that's very similar to yours. Um, I actually anesthetized for cataract surgery, uh, somebody that I knew very, very well, and it was funny because, you know, we have two eyes, right? So most people have one cataract surgery.

Then the other one, and I know you guys know a lot about ophthalmology because of Dr. Glaucomflecken Fum. Did I say that right that time? Mm-hmm. Close enough. Yeah. Okay. And so, um, I know that you guys know a lot about ophthalmology, but anyway, so I was gonna be anesthetizing this person and they really, really wanted to have a general anesthetic.

And I was like, no, no, no, no. We are not doing a general anesthetic that is gonna be more risky, but we're gonna give you sedation. But she really didn't wanna remember anything, so I. Had this plan and I did my plan and it was great. [00:13:00] And she was super happy. She was like, I don't remember anything. Like the sedation was perfect.

So when she came back for the second eye, I literally looked at my anesthesia record like it was a recipe, and I was like, okay, I'm gonna follow the exact same thing. And guess what? She remembered everything. 

Producer Tessa: Ah, 

Dr. Linda Bluestein: so, so 

Producer Tessa: what's the difference? What happened? So, 

Dr. Linda Bluestein: I don't know. I don't know. Our bodies are just different from one day to the next.

We can respond one way to, um, a medication one day and differently the next day. We recorded a podcast episode yesterday with both of you, and I don't know if you remember that. I was like so itchy during that podcast episode and, um, I've been dealing with this spitting stitch, which we're gonna talk about at the end briefly.

For the hack. But anyway, so I took some diphenhydramine or Benadryl, and every once in a while when I take Benadryl, I get this like dystonia, like, like just, um, weird reaction where I cannot stop moving my body and I get in bed and my husband is like, stop moving around. You're driving me [00:14:00] nuts. Um, but I don't get that all the time.

So, so our bodies definitely respond differently, um, on different occasions. 

Producer Tessa: That's so interesting. Yeah, it, as a patient, I always just want to like, put everything in the hand, put all my trust in the hands of the doctor and in the hands of medicine and I'm like, you know, I, I hope it just works flawlessly every time, but this is the reality of it, is that our body responds differently on different days and yeah, and I think 

Dr. Linda Bluestein: that's a great point.

I think an important point there too is. You're probably not ever going to be over hydrated. So if you go in for a procedure and you know, nor we normally tell people you know, nothing to eat or drink after midnight and your surgery might be in the afternoon. Well, the reason why we do that is because, you know, we've experimented, like in my group, my anesthesia group, we would experiment with telling people, you know, you can have some clear liquids or even a light breakfast.

Um, if you were having surgery at like four in the afternoon, you can have a light breakfast. [00:15:00] So long as it's before, let's say 8:00 AM so that way you have like an eight hour window. Well, we were in Wisconsin. People would come in and they'd be like, yeah, I had a hamburger, french fries, a couple two liter bottles of soda.

And we're like, that's not a light breakfast. We met like a piece of toast. Um, so clear liquids, generally speaking, you can have up until four hours before the surgery definitely talk with your surgeon. Clear liquids are things that you can see through, that you could read like a newspaper through. Um, and also black coffee, black coffee's also a clear liquid.

Something like jello broth. Um, I feel like a lot of times we err on the side of such long NPO or nothing per mouth. Uh, nothing per us, um, is the Latin term for it, but I feel like we err on the side of such long, long times that then people come in super dehydrated and that doesn't serve them well, and it makes us harder, makes it harder to get the IV in and things like that too.

Producer Tessa: Interesting. Okay. Note to self, I'll be hydrating before any [00:16:00] surgery. Yep. Yep. I know we also had a listener question come in actually about a, about a surgery that went that went wrong. Can I read it out to you? Sure. Monica writes in. I was recently diagnosed with Hypermobile Aler Danlos by a physical therapist who helped me understand how much it's affected my health.

Before that I had surgery for GERD and I hiatal hernia, but it failed almost immediately. The hernia came back and my symptoms got worse. I suspect my tissues didn't heal properly because of EDS and that I developed MCAS. As a result, I'm in touch with surgeons who specialize in GERD and they're eager to do a revision.

They think that the original surgery was not performed correctly. The surgeons don't seem to take EDS seriously enough. While my physical therapist is encouraging me to wait on any more surgeries with severe GERD and advanced esophagitis, I'm at a loss. What's your experience or advice on fundoplication and GERD surgery for H EDS patients?[00:17:00] 

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Whether you're shopping for yourself or someone you love, there's so many options to choose from. A portion of the proceeds goes to support EDS nonprofit organizations. For more information, please visit bendy bodies boutique.com. Thank you so much for listening to Bendy Bodies. We really appreciate your support.

It really helps the podcast when you like, subscribe and comment on YouTube and follow rate and review on all audio platforms. This helps us reach so many more people and spread the information to everyone. Thank you so much again, and enjoy the rest of the episode.

So I love this question and I [00:18:00] love Tessa, that you picked out this question because I've actually had a Nissen fundoplication for severe gerd. Um, mine just came outta the blue. All of a sudden, one day you're supposed to have this sphincter at the bottom of your esophagus, and all of a sudden, one day mine just decided to quit and I had outrageously severe gerd.

I would bend over and food would come up and things like that. So this is. Great question. Um, affects a lot of people. GERD is extremely common in people with EDS or other connective tissue disorders. The connective tissue laxity leads to a weakened lower esophageal sphincter, and we can get these hiatal hernias, which is when you have like parts of the, the upper parts of the stomach actually coming up into the chest.

Also if people have dysautonomia, for example, pots and or impaired gastric motility, um, that causes slower emptying and that can contribute to reflux. Um, we know a lot of people with these conditions have gastroparesis or delayed gastric emptying and that can make the symptoms, uh, worse. Also [00:19:00] coexisting mast cell issues can also mimic or worsen reflux symptoms.

Um, you can get a lot of chest pain, epigastric burning and throat clearing. Um, I had so much chest pain multiple times before I had my ni fundoplication. I thought I was having a heart attack. Um, I. I really, really on multiple times. Multiple times I was in my early thirties and I really thought I was having a heart attack.

Um, so things that you wanna consider before having surgery, and this is for anyone who has severe gerd and maybe they're talking to a surgeon, um, not necessarily for Monica. We're gonna get to Monica's question about a redo in just a minute. But things that you want to have done beforehand, um, you wanna for sure have an upper endoscopy and they're gonna look for things like esophagitis, uh, Barrett's esophagus and how big the hernia is.

Barrett's esophagus is a condition where you start to see, um, abnormal changes on the pathology in the esophagus, and those people who have Barrett's esophagus are at increased risk for esophageal cancer. And you do not want esophageal [00:20:00] cancer. Not that you want any cancer, but esophageal cancer is a really bad one.

So if you have Barrett's esophagus, your recommendations are gonna be different than if you don't have Barrett's esophagus. So just keep that in mind. Um, definitely ask if you had biopsies or not, and if so, did they show any signs of Barrett's esophagus? Um, oftentimes if they do, then they want much more frequent monitoring and, uh, you know, keeping a much closer eye on that.

Another thing that you need to have is esophageal mammo tree to check for peristalsis, um, which is really, really important for choosing the right kind of wrap in order to keep things, uh, in the stomach basically separate from the esophagus. Um, this is not a fun test. I had to have this, of course, before I had my surgery.

They put a probe down, um, through your nose or your mouth, I can't remember which, and you're wide awake and they have you swallow and do all these different things and it was not fun. But, um, it's very important that they can get an idea of the strength of, uh, your esophagus because they don't wanna put this wrap in [00:21:00] place at the top of your stomach, um, near the bottom of your esophagus if you're not gonna be able to push food through.

'cause now you're gonna have a different problem. They also often do 24 hour pH or pH impedance studies to confirm, uh, whether you have pathologic acid or non-acid reflux. Um, gastric emptying studies are really important to have if you have early satiety nausea or bloating because. Unrecognized gastroparesis can definitely sabotage outcomes.

Um, you also wanna review your medications 'cause maybe you're taking something like opioids or anticholinergics that can worsen reflux or motility and maybe changing some of your medications might help. You also want to optimize non-surgical management, like elevating the head of the bed, avoiding late meals, using smaller portions, um, limiting alcohol, mint chocolate, and high fat foods.

Um. Sometimes you wanna trial proton pump inhibitors, but ideally those would be taken only for a short period of time unless you have eoe, which is eosinophilic esophagitis. Or if you have Barrett's esophagus. [00:22:00] Um, there's some other indications as well to talk to your gastroenterologist about. Um. H two blockers like famotidine can be very helpful.

And also, uh, you know, walking around after meals and bedtime can really help as well. Definitely wanna treat constipation and consider medications that can accelerate gastric emptying. Those are called prokinetics. Um, also treating MCAS flares with things like H one and H two blockers. Chromelin, if appropriate, can be very helpful 'cause that can masquerade as reflux.

If you are thinking about surgery, you wanna consider things like tissue fragility and hernia recurrence risk, because these are higher in EDS. So you wanna choose a really experienced surgeon in four gut surgeries like this. So there are also multiple different ways to do wraps that can do partial wraps, or they can do a full wrap, which is the Nissen fundoplication.

That's the surgery that I had, and I think I have a picture of that. I'll see if I can dig it up and we can maybe put it in the, uh. In the show notes or social, on one of the [00:23:00] social media posts if anyone's interested. Um, there's also a procedure called the links procedure, which is a magnetic sphincter, which is an option for, you know, selected patients that have normal motility and maybe have a small, um, or no hiatal hernia.

But the data for that in EDS patients is limited. Um, there's also details to consider for repairing the hiatal hernia itself. Um, and you can consider cruel reinforcement, which is, uh, the part of the diaphragm where the esophagus, um, you know, comes through. The use of mesh is a little bit controversial in EDS because of healing and erosion risks.

Um, and also a lot of us do not like foreign bodies and mesh is a foreign body, so sometimes we reject. These things as we're gonna talk about later with sutures and my forehead. Um, so, um, another thing to expect after a fullness and fundoplication is that burping or vomiting may be difficult. And after I had my ni and fundoplication surgery, I was having so much pain, [00:24:00] um, even weeks later, and I did not know what it was from.

And I was talking to my, I was working in the operating room at the time, right? So I was seeing surgeons. All day, every day, including my surgeon who operated on me. And I said to him one day, as we were literally like passing in the hall, I'm like, why am I getting so much pain from time to time? And another surgeon was literally walking by, they were partners or maybe they were walking together and my surgeon didn't say anything, but the other surgeon was like, oh, you have gas bloat?

And I'm like, what? He's like. You're getting gas in your small intestine that normally you would burp out, but because you had this nien, because you now have this tight wrap around your esophagus, you can no longer burp that air out. And so it can be really, really uncomfortable. So the interesting thing is my nien, um, did loosen, it's not completely failed, but I did throw up a few times and my surgeon initially was like, you can't throw up.

That's impossible. Oh yes, you can. [00:25:00] If you have enough of a need to throw up, you can throw up even with a nissin. So I don't know if my nissin partially failed because of the fact that I, you know, vomited a few times I got food poisoning and also probably motion sickness. Um, or is it because of my connective tissues?

I'm not sure which. But right now, knock on wood, I'm in that sweet spot of like, my GERD is perfectly well controlled. I'm not taking a PPI, um, like omeprazole and, and also I don't have gas bloat, so I feel. Really lucky. I have like, just the right amount of, uh, you know, coverage down there. Um, some special EDS considerations in the perioperative period include, um, positioning.

So I wanna be thinking about joint protection. Um, we wanna be thinking about our neck, which we're gonna talk about later when it comes to, you know, do you wear a cervical collar or not? Um, if your skin tears easily, be sure to let them know about that. Or if you have any problems with adhesives. Um, we wanna be careful how we're securing IVs and airways because a lot of people are reactive to tape.

[00:26:00] Um, we mentioned already about local anesthesia resistance, so sometimes multimodal analgesia is important. Um, and also wanna think about things like pots and dys, denomi. Do we need to have more fluids? Um, do we need to get mobilized? You know, slowly Do we need salt or compression postoperatively to reduce orthostatic symptoms?

We also wanna be thinking about wound healing and bleeding. Um, really, really meticulous technique is important for this and discuss scar care and activities, uh, ahead of time with your surgeon. In terms of the postoperative course, um, dysphasia or difficulty swallowing is very common. I remember I was on a clear liquid diet for two weeks, and then after that I could start like slowly progressing to foods and I don't know, I've, I had my nien, gosh.

20 years ago. It was a long time ago. So, um, now the re the requirements might be different, but at that time, clear liquids for two weeks and then slowly progress on your solids. Um, but I remember, like it was yesterday, [00:27:00] being in a restaurant, I ate meat at that time and I ate a hamburger and I could feel this chunk of the hamburger stuck in my chest.

It was so painful, so, so painful. Um, so when they say advance slowly from liquids to soft to regular foods, because you have this tight thing now and it doesn't function like your normal esophageal sphincter does. So if you have fundoplication surgery, if you have one of these GERD type surgeries, just remind yourself to take it really slow and, um, you know, progress very, very intentionally.

You wanna watch for things like persistent dysphagia, uncontrolled gas bloat, um, and one of the tricks that this other surgeon told me was lay on my back and roll, put with my knees up to my chest and roll side to side. And what that does is it helps move things through the small intestines. So probably a good hack anyway, um, just in general for, you know, getting [00:28:00] your GI tract to move.

But in particular, that really helped me when I would get these gas bloat. Pains. So it's interesting 'cause my surgeon was like, I don't know, but this other surgeon says. I think I know what that is and this is what you should do about it. So I got lucky that I asked that at the right time. Um, chest pain is quite common, as I mentioned.

Um, some people do get regurgitation. Uh, weight loss is, uh, quite a common phenomenon. Um, sometimes people get, you know, the wrap might be too tight. It might be, it might slip. You could have an unrecognized motility disorder or a recurrent hernia. Um, we already talked about gastroparesis, but make sure to address that it's really important, otherwise we can get reflux like symptoms, even with a quote unquote perfect wrap.

So who tends to do well versus who tends to do poorly? Um, better candidates are people who have objective evidence of reflux on pH testing. They're responsive to PPIs, but intolerant. Um, they have normal or only mildly impaired motility, smaller hernias, and have realistic expectations. Um, [00:29:00] higher risk for suboptimal outcomes includes people with severe hypo motility, significant gastroparesis, uncontrolled, MCAS, large paraesophageal hernias with very lax cura, which again is that area.

It's acro, kura means cross, so it's the part of the diaphragm where the fibers cross each other, um, or prior for gut surgery. So again, like this person's asking. If you're having a redo surgery, it's definitely, uh, you know, higher risk and less likely to have good outcomes. Um, some questions that you could ask your surgeon include, you know, do I have proven reflux on pH testing?

And what's my motility like given my motility? Would you consider aisin or a partial rep for me? Um, how often do you operate on patients with EDS and other connective tissue disorders? And I would also ask specifically, how many of these surgeries have they done? How will you minimize recurrence with my tissue laxity?

Will you use mesh? And why or why not? Um, what is the plan if I have dysphasia or gas bloat after surgery? And how will [00:30:00] pots, MCAS, EDS, HSD affect anesthesia and recovery? So, um, in conclusion for this, and then we're get, now we're gonna address like. About this particular person that's looking at a redo.

But if you're looking at a first time surgery, um, it's very important to diagnose the GERD very precisely. Um, tailor the operation to the person's motility and manage expectations. Um, a partial fundoplication or optimizing your medical and motility care may outperform a one size fits allness and fundoplication.

Okay, so let's talk a little bit about when aness and fails. So, failure can mean persistent or recurrent reflux. It can mean gas bloat, dysphagia, or wrap slippage or hernia recurrence. Um, in EDS, a number of things can contribute tissue fragility. Poor collagen integrity, or weak, cruel support can greatly increase.

Recurrence risk. Also, if you have high intraabdominal pressure, you can imagine the higher the pressure is inside your abdomen, the more that's gonna wanna push things up into your chest. So if you have [00:31:00] bloating, constipation, chronic cough, vomiting, et cetera, that further stresses the repair. Um, other things include unrecognized motility disorders or gastroparesis, as we talked about already.

Um, when it comes to a recurrent problem, like this repeat diagnostic workup is essential. Um, probably an upper GI contrast study, like a barium swallow. Um, you're probably gonna have another EGD more thermometry, probably more, you know, impedance and pH testing, maybe another gastric emptying study. Um, you always wanna confirm if it's a technical failure or a functional failure.

Um. A technical failure would include like a slip and or a loosened wrap, and a functional failure would be dysmotility hypersensitivity or MCAS. Some of the common failures in EDS include hiatal hernia recurrence due to choral laxity, or poor suture hold wrap, disruption or migration. Too tight or overcorrection can cause outflow, obstruction, dysphagia, and gas bloat, um, underlying motility disorder [00:32:00] or mast cell activation or visceral hypersensitivity that can mimic reflux despite normal studies.

Um, I had an EGDA number of years after my ni fundoplication. I was having problems with chest pain. Still knock on wood now, have been really good for quite a few years, but I had a period even after my surgery where I wasn't doing well and I was having chest pain. And uh, basically after I had the study and they were like, it looked perfectly normal other than the fact that your rep is a little bit loosened.

And they said you have visceral hypersensitivity. I think a lot of people take this personally and they're like, you know, oh, it's a, it's a character flaw or something like that. Um, when we have all of these pain signals coming into our nervous system, our nervous system adapts by. Actually producing more of these neurotransmitters and like looking for pain.

And this is called central and peripheral sensitization. So I definitely have that. Low-dose Naltrexone, as I've talked about a lot on this show, can help a lot with central and peripheral sensitization. It's one of my favorite, um, hacks for that. Okay, back to this person's [00:33:00] particular question. Uh, demon Monica's question.

Uh, first we wanna optimize non-surgical management. We want to adjust our diet and meal timing. We want to do small frequent meals and avoid gas producing foods. We wanna treat underlying gastroparesis with prokinetics. Um, consider pyl Loic, Botox, or something called GPO m. Um, this is a type of surgery that can be very helpful for gastroparesis and.

Shanti has been trying to chase down one of the biggest GPO surgeons in the country. Um, so we're g we keep trying to get him on the show. If you happen to know of another GPO surgeon, uh, let us know because we wanna discuss that. 'cause gastroparesis is such a common problem with EDS and GPO can be, um, really, really helpful.

We've gotten some great reports on that. You wanna manage MCAS with things like H one, H two blockers, chroma, and serin lutein, et cetera. Um, you wanna address pots and dysautonomia for improved g motility intolerance. And also sometimes, uh, image guided dilatation can help if the wrap is [00:34:00] intact. But, um, if the wrap is too tight.

Endoscopic revision techniques might be an option for, uh, those that have parcel loosening or slippage. If you are considering a reoperation, um, a redo fundoplication is technically complex and is going to be high risk in EDS due to friable tissue scarring and higher rates of recurrence. Uh, you wanna consider.

The possibility of a partial wrap, um, for dysphagia or poor motility, um, and select cases. Taking down the wrap or considering a ru and y conversion can be helpful for severe, recurrent cases of reflux or obstruction. You definitely in this case want to seek out a high volume surgeon who's doing lots and lots of four gut surgeries that's familiar with connective tissue disorders, and we're going to have a list later of questions that you want to ask your surgeon beforehand.

So you can get a sense of how familiar they are with EDS and HSD. Avoid mesh reinforcement if possible, and if used select biologic [00:35:00] materials because of erosion risk, you want to collaborate amongst the members of your team, GI surgery, anesthesia, nutrition, pain medicine, and mast cell specialists. You want to consider prehab and postoperative care, um, including wound healing support.

Okay, so a failed ness and does not mean you did something wrong. Tissue fragility and EDS often plays a major role. We don't want to rush into revision. We wanna get objective testing and address motility and mast cell issues. First, advocate for yourself. Find surgeons experienced with EDS or complex for gut reduce surgery.

You wanna keep detailed notes on your surgical reports, imaging, and what has and has not worked for you. Um, there's a few episodes where we've discussed similar topics in. Um, on bendy bodies, and we'll link those in the show notes. Okay, Monica, I hope that helped answer your question and boy did I get long-winded there.

I'm having so much fun with this that I think I got totally, uh, out of track with time and I know y'all are probably getting a little exhausted. So [00:36:00] I think we're gonna actually stop here and we're gonna turn this into a two-part, um, series. On these, uh, surgical and anesthesia related questions. So we'll jump into the hypermobility hack, I promise I am going to cover those other topics that I mentioned at the beginning.

Um, but they'll be in part two, so be sure to watch for part two. That'll be coming up shortly. So today's hypermobility hack is also a bit of a teaser. I was trying to find the picture that I have for my surgeon of my Nissen fundoplication surgery 'cause it's a really beautiful picture and I realized that I have like my medical papers, um, in a folder and it's not organized in a binder like I always tell my patients to do.

We are going to talk about this in a future episode. Um, having a binder is really, really helpful and I've had people say, well, why bother with a binder because my doctor's not gonna look at all of that information. That is not the point. The point of a binder is that you can then pull out and reference important things when you need to have them.

So you have easy access to, you know, an encounter note [00:37:00] or certain imaging findings or lab findings, et cetera. So watch for that in a future episode, and that is your hack for today. Start working on your binder. Okay, so that's it for today's episode. Thank you so much for all your fantastic questions.

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