Hidden Causes of Abdominal Pain in EDS with Dr. Pradeep Chopra

[00:54] Dr. Linda Bluestein: Welcome back, every bendy body, to the Bendy Bodies Podcast with your host and founder, Dr. Linda Bluestein, the Hypermobility MD. This is going to be a great conversation with Dr. Pradeep Chopra. This is part 2 of our gastrointestinal problems and abdominal pain series. This is going to be really important because so many of us have suffered from abdominal symptoms that have been really difficult to diagnose, and we are so incredibly fortunate to have Dr. Chopra back on Bendy Bodies.
Dr. Chopra is world-renowned for his incredible work in EDS and HSD, POTS, mast cell activation syndrome, CRPS, and central sensitization disorders. Dr. Chopra is a Harvard-trained, board-certified pain medicine specialist with over 25 years of experience. We will link all of Dr. Chopra's prior episodes in the show notes for easy access.
[01:43] As always, this information is for educational purposes only and is not a substitute for personalized medical advice. Here we go.
[01:53] Okay, well, I am so excited to be back with Dr. Chopra once again. Dr. Chopra, I think that maybe you get the prize for the most guest appearances. I probably should have added this up before this conversation, but it's probably 7 or 8 or something like that now. So we'll link all of those in the show notes, of course. But thank you so much for coming and talking with me again today.

[02:13] Dr. Pradeep Chopra: Thank you very much. I really enjoy coming on here and talking.

[02:19] Dr. Linda Bluestein: Of course, of course. Last time we talked, we were talking about the GI tract, and we started with the upper GI tract, and we even started with the teeth, which was a very interesting conversation. We kind of left off with compression syndromes. We got through the esophagus, we got through the small intestine, and that's pretty much where we left off. So today we're going to try to cover the rest, which is kind of a lot. We'll see how well we do.
[02:46] We want to talk about the large intestine and we want to talk about things like prolapses and also abdominal wall type problems, things that can happen in the vagina and in the pelvis. So I thought maybe we would start with the colon and the large intestine. Does that sound okay with you?

[03:07] Dr. Pradeep Chopra: Sure, yeah, absolutely. We can do that. So we are still inside the abdomen. We talked about all the compression syndromes and the small intestine. For our listeners, our intestines are about 20 feet and 10 inches long, and the small intestine is the major part of it.
[03:33] The part about the intestine that we need to understand is it's mobile. It moves because it has to move food along, just like a toothpaste tube. And when it slows down, you start having problems like bloating, pain, and then you can alternate between constipation and diarrhea. And then of course there's absorption. So people have absorption issues because of this slowing down of the movement of the intestines.
[04:07] One of the things that people with EDS can see is what is called visceral ptosis. Ptosis being spelled as P-T-O-S-I-S. What that means is that it drops down. My concept is the intestine has 3 layers of walls on it, it has muscles on it. But what if the muscles were thin? What if the intestine itself was thin? The weight of the food and the fluids would make the intestines drop down, and that's called visceral ptosis. Viscera means whatever's inside the abdomen. So we'll talk about the intestines dropping down, and then we'll talk a little bit about kidney ptosis because the kidneys can drop down also.
[04:56] Usually the problem that we see in patients with ptosis — that is, dropping down of the intestines — is with the transverse colon. So the colon goes up from one side of the abdomen, that's called the ascending colon, and it goes up the right side. Then it goes across, which is called the transverse colon, and then it goes down, which is the descending colon on the left side. Now, the transverse colon is kind of loose. The ascending colon and the descending colon are fixed, but the transverse colon kind of is in there flapping around.
[05:33] Normally, it'll descend a little bit with the weight of the food and fluids, but in EDS, because they have loose connective tissue, it can descend a lot more. I've seen X-rays of patients where the transverse colon is so loaded that it falls into the pelvis. When it falls into the pelvis, obviously there are intestinal issues and extraintestinal issues. Intestinal issues being bloating, diarrhea, constipation, et cetera. But if it's really loaded and it pushes into the pelvis, it can press on the bladder and the rectum. So now they have another reason for constipation and frequent urination.
[06:15] I don't know how common this is, but it is known to happen in EDS. Obviously these conditions have not been studied in great detail, but it's just something to keep in mind in patients with EDS with abdominal issues.
[06:34] One of the things that is thought about is that these patients have what is called dolichocolon. Dolichocolon means that the gut is elongated — really long and thin-walled. So the weight of the food and the fluids makes the transverse colon drop. It doesn't happen to the ascending colon because it's fixed, and the descending colon is fixed. But then the last part of the descending colon is called the sigmoid colon because it's S-shaped, and that is also loose and kind of flappy.
[07:11] This is a problem that I suspect I see quite often, because what happens is the sigmoid colon is the last part before the rectum. And when it gets loaded, it kind of falls over and can cause severe constipation. The only way I know this is because patients will tell me, like, if they shift to one side or the other, they can have a bowel movement. And so that tells me it's a mechanical problem. The diagnosis is pretty simple — you can do it by X-rays and a dye, and you can see where it is.

[07:47] Dr. Linda Bluestein: Do you have to be standing up for the imaging in order to show the effect of gravity?

[07:53] Dr. Pradeep Chopra: Correct, exactly. But here's the thing. Oftentimes I've asked patients, do you want me to do something about it? Because it might involve surgery and we don't know how effective it is. And most patients are quite used to it and they're like, okay, no thanks, no surgery. But it's something to think about — that this constipation is a kind of mechanical constipation, and adjusting your position or sides can help.
[08:21] So on that subject of ptosis — ptosis meaning dropping down — the organs in our body like the kidneys are kind of fixed at the back of our abdomen, kind of plastered there. But in patients with EDS, they can have ptosis of the kidney, so the kidney can drop down. The official term is nephrotosis, but it's also known as floating kidney. The diagnostic criteria is that if the kidney descends more than 5 centimeters — which is about the height of 2 vertebrae — when you move from a lying position to a standing position, then it's considered a floating kidney.
[09:13] Now, most patients don't have any problems, the reason being that we have 2 kidneys. So if one has a problem, the other kidney takes over the job. Oftentimes the kidney is working fine. But if there is a problem, it's usually a very sharp pain in the flank. That's where the kidney is. This pain is called the classical loin-to-groin pain. It gets worse when you stand, obviously, because that's when the kidney drops down. It does cause nausea and vomiting. And because the renal artery gets constricted — because the blood flow to the kidney gets constricted — it can cause high blood pressure. It can cause bleeding in the urine. And there's a kind of heaviness in the abdomen on that side.
[10:07] Now, we don't know if people who have floating kidneys have it on both sides or just one side. But it's something to think about if everything else has been ruled out.
[10:20] And I wanted to put in a little disclaimer here. Just because somebody has EDS, don't only think about fancy abdominal problems. You can have normal other problems like an appendicitis or a cholecyst/gallbladder problem or something like that. I don't want people to think, oh, I have pain in my loin, and automatically assume their kidney is dropping down. It could just be a kidney stone.

[10:48] Dr. Linda Bluestein: Right, right. And things like diverticulosis that are more common in people with EDS — something like that is going to be much more common. I was going to ask you, as you were talking about renal ptosis or nephrotosis, my husband is a urologist, so he's operated on lots and lots of kidneys, and I don't recall him ever mentioning that he has done any kind of fixation surgery for kidneys. Have you had patients that have had to have surgery for that?

[11:17] Dr. Pradeep Chopra: No, I haven't. And here's the thing — I suspect it's commonly missed, number one. Number two, I think in most cases it's probably asymptomatic because these people are born with it and the blood vessels and the ureter and everything have adjusted to it. I don't know. I haven't seen this. I don't think I've ever seen it.

[11:40] Dr. Linda Bluestein: Okay.

[11:41] Dr. Pradeep Chopra: The only ptosis that I've seen is the sigmoid colon causing constipation. That I have seen.

[11:47] Dr. Linda Bluestein: I've seen that too.

[11:48] Dr. Pradeep Chopra: Yeah. But it's something we should mention — since we are discussing abdominal issues, it's just something to keep in the back of the mind, especially for our physician listeners, that this can be an issue.
[12:02] One of the things that is a big concern, especially in patients with vascular EDS, is bowel perforation. I just want to make sure that this is not limited to patients with vascular EDS. In Ehlers-Danlos syndrome, there is an overlap of symptoms and overlap of pathology. I know patients with vascular EDS are prone to having vascular anomalies like aneurysms. But that doesn't mean that a patient with hypermobile EDS can't have an aneurysm. That is still on the table. And so bowel perforation or rupture is — I'm not sure it's common, but it is seen more often in vascular EDS, and it can also be seen in hypermobile EDS.
[13:02] It is a serious emergency condition. What happens is there's a hole in the intestine, or part of the intestine ruptures. The contents of the intestine come out — bacteria, food, or bile. These patients present with severe, sudden abdominal pain. It's very sudden, it's very severe, and of course because there's infection, there's fever. And because there's infection and inflammation, the abdomen becomes really tender and rigid. They start to breathe faster, and there's fever and nausea and vomiting. But this is an ER thing, completely an ER thing. If you have any of these symptoms, head to the ER, and they can diagnose it pretty easily.

[13:49] Dr. Linda Bluestein: And that could also be related to — not to bring this up already again — but a ruptured diverticulum from diverticulosis, right? Even in a quote-unquote normal connective tissue person, one of the causes of bowel perforation is a ruptured diverticulum. But that's super important to point out, because I think sometimes it's really hard for people who have abdominal pain on a regular basis, and then they get an episode that does seem more extreme than usual, but they're not sure — do I go to the ER or not? Because they've been gaslit so many times.
[14:25] So I think it is very important to point out what are things that you should definitely go to the ER for. If you have that sudden onset or significant worsening of abdominal pain, especially if it is associated with a fever, sweating, or chills — those are some things to be very, very concerned about.

[14:46] Dr. Pradeep Chopra: Absolutely. If it is sudden onset, there's fever, there's chills, head to the ER, no question. It can be from diverticulitis, perforation from diverticulitis — so that's a non-EDS reason plus an EDS reason — and then there can be a rupture. Most ER physicians are really adept at diagnosing that.
[15:11] On the large intestine, I don't think we have anything else to go through right now. Moving into the pelvis, there is something called pelvic congestion syndrome. I suspect it occurs more often than we diagnose it. We know that in people with EDS, they tend to pool blood, and a large part of this pooling is in the abdomen and the pelvis, especially when they stand. The veins in the pelvis are big, huge veins, and so they tend to pool a lot of blood there.
This causes — classically they will present with chronic pelvic pain. It's not sharp, it's kind of a dull, achy pain. The typical point is that it gets worse with being upright. So when you move from a supine to a standing position, it gets worse. And of course, when you lie down, it gets better. They can have varicose veins in their pelvis, the vulva, the legs. There is typically a very heavy, boggy feeling, like there's something loading into the pelvis. It may be associated with lower back pain because it's tugging on the structures attached to the lumbar spine and putting pressure on the pelvic floor. But the main one I want to stress is this pain comes on with being upright and gets better with lying down. That's the classical symptom. And obviously these patients also have POTS.

[17:04] Dr. Linda Bluestein: I did want to talk a little bit more about diverticulosis, because it's common in the general population, but it's so much more common with EDS. Again, it can affect your dietary requirements — the foods that you might be recommended to eat. And it's my understanding that especially in classical-like EDS, the prevalence of diverticulosis is a lot greater. There was a study recently that showed 80% of patients who had classical-like EDS type 1 had diverticulosis. And two-thirds of those people experienced spontaneous intestinal perforations. Speaking of nonvascular types of EDS having intestinal perforations — that type of EDS is quite rare, but I just wanted to point that out.

[17:56] Dr. Pradeep Chopra: That is huge. 80%?

[17:59] Dr. Linda Bluestein: 80% in this study.

[18:03] Dr. Pradeep Chopra: 80%? Wow.

[18:03] Dr. Linda Bluestein: Yep. I can include the study in the show notes so people can see it.

[18:10] Dr. Pradeep Chopra: That is huge.

[18:12] Dr. Linda Bluestein: That's in classical-like EDS type 1.

[18:16] Dr. Pradeep Chopra: I think we can go on to neurological causes of abdominal pain.

[18:21] Dr. Linda Bluestein: Okay.

[18:22] Dr. Pradeep Chopra: So in the neurological causes, let's talk about the spine. We'll start from the top of the spine with the cervical spine and talk about craniocervical instability, which means instability of the neck. What that can do is cause compression of the spinal cord, and it causes compression of the arteries and the veins, as well as the nervous system, including the famous vagus nerve. Now, the vagus nerve plays a really important role in digestion, and so any dysfunction of the vagus nerve can cause GI problems. So yes, instability of the cervical spine can contribute to GI problems. But I don't know if I've seen a patient who had craniocervical instability, had a fusion done, and all their abdominal issues went away. It could be contributing to it, but may not be the only reason.

[19:22] Dr. Linda Bluestein: Have you seen people where their GI symptoms got substantially better after they had their post-fusion surgery?

[19:29] Dr. Pradeep Chopra: Yes, I've seen them — better, but not gone. There are other reasons why they would have GI issues. So that's one of the things to think about.
[19:40] Autonomic dysfunction like POTS can also contribute, because remember the entire GI tract moves on the autonomic nervous system. The sympathetic nervous system covers pretty much the entire intestine, whereas the parasympathetic nerve only goes up to the transverse colon. So people with POTS or autonomic dysfunction, dysautonomia, can also have lots of GI issues.
[20:10] And this is the confusing part, Linda — when a patient comes in with all these abdominal issues, you're left wondering, is this MCAS or is this POTS? What is causing this? And it's probably a few of them, and you just have to chip away at it one by one.

[20:30] Dr. Linda Bluestein: That's where it's really tricky, with the symptom overlap between the connective tissue disorders, mast cell disorders, dysautonomia — the symptom overlap is so huge. But at the end of the day, the big thing is what are you going to do? How are you going to help the person feel better? Knowing exactly the cause would be nice, but sometimes it's really figuring out what is the right thing — I'm thinking of a toy where you pull a string and it starts to spin — what is the thing that's going to have the greatest impact?
[21:04] So we would love to hear your hacks, because your hacks are very popular. As you're talking about these things, if you have additional hacks, be sure to throw those in.

[21:16] Dr. Pradeep Chopra: Here's the thing. Like we talked about, different issues can cause different problems in the GI system. In my own mind, I try to differentiate: if it's painful and when you feel the abdomen it's tender everywhere, I think more of inflammation in the GI tract. If they have bloating and it's not as tender when you feel around the abdomen, I think more of, okay, maybe this is dysautonomia. But at the end of the day, it's both of them contributing.
[21:54] The only thing with MCAS is that MCAS is a pretty nasty condition to have. It can cause a lot of abdominal pain — not "does," but "can" — because mast cells like to accumulate at the surfaces of the body, and one of the surfaces is the intestine. They cause inflammation of the lining of the intestine. And what the intestine does when it's inflamed is it doesn't like anything touching it. So when you eat food, it stops moving and then it starts to bloat up, and then you wind up with diarrhea, constipation, and severe cramping abdominal pain because now you have an inflamed gut that's trying to push food along. That's predominantly MCAS pain.
[22:50] The diagnosis of mast cell depends on other things, but as far as the abdomen is concerned, it's quite painful. For us physicians, when we feel around and they have a diffuse tenderness everywhere, I think more of MCAS than dysautonomia.

Dr. Linda Bluestein: Okay.

[23:09] Dr. Pradeep Chopra: I don't know if you've discussed this before, but tethered cord can cause a lot of GI issues.

[23:20] Dr. Linda Bluestein: We have not discussed that before. Interesting. Tell me more.

[23:44] Dr. Pradeep Chopra: I have seen this, and it has been surprising because oftentimes I tell patients, hey, listen, you're going to see your bladder symptoms improve, your back pain and leg symptoms improve. And then lo and behold, they come back and say, my GI symptoms got better. Not everyone — I don't want to sound like that is the only reason — but I have seen this before.
When I did a search on this, I found that yes, it's possible, because after all, all your nerves pass through your spinal cord. And if your spinal cord is being stretched, it's going to affect everything. So expect to see some improvement in the GI system after a tethered cord release. The tension in the spinal cord causes a disruption of the signals going up to the brain and control of the bowel and bladder function. And when you release the tethered cord, that function gets better. I know for a fact that bladder function improves within 24 hours, as soon as they wake up. But GI symptoms also get better — like constipation and all that — after tethered cord surgery.
[24:35] So one more reason to have that released. But again, tethered cord may be just one of those dots in the whole conundrum of GI issues. You have autonomic dysfunction, mast cell activation, tethered cord, craniocervical instability, and a whole slew of other things that can contribute.

[25:05] Dr. Linda Bluestein: Don't you think — at least based on my personal and clinical experience — that it also is not necessarily that same exact combination of things on any given day, every day? Some days maybe one thing is playing a bigger role, maybe some foods that you ate and your mast cells are unhappy. And then another day, maybe there's something else that's more predominant. I think there's a lot of variability from day to day in a lot of our symptoms because there are so many different contributing factors. It's a constant moving target.

[25:41] Dr. Pradeep Chopra: Absolutely. That's why we don't look at diseases as a straight line. Some days are worse than other days, and some symptoms are worse than other symptoms. Someday your MCAS might be out of control and someday your POTS is out of control. The way I look at it in terms of treatment outcomes is more good days than bad days, and then finally, even the bad days are not as bad or are manageable. It's never a straight line. It's always a wavy curve.
[26:23] So, as we were on the subject of inflammation of the GI tract — the GI tract is loaded with nerves. In fact, there are so many nerves in it that it's called the second brain. And this inflammation eventually gets to the nerves, and the nerves are going to get inflamed.
[26:40] The GI tract actually has its own nervous system. We have a nervous system that we can control — I can scratch my head, that's under my control. Then we have the autonomic nervous system that controls autonomic functions like breathing or heart rate. But then the GI tract has its own nervous system called the enteric nervous system. What happens is that constant inflammation from MCAS or whatever can cause inflammation of these enteric nerves in the GI tract. It's quite a nasty condition. I've seen it on many occasions.
[27:25] What happens is they'll eat food and they won't have pain right away, but they'll have pain after, say, an hour or 2 hours later. That's when the food, as it moves down the GI tract and hits that sensitive area, is felt. It's called visceral hyperalgesia, and it's really obviously quite painful.
And then there's cross-sensitization. For example, if you have pain from the bladder, that inflammation of the nervous system in the bladder can cause cross-inflammation — or crosstalk, as it's called — with the nervous system in the GI tract. And so that can cause GI pain also. That's called cross-sensitization. Different organs, as they cross paths in the spinal cord, cause these pain signals to be mixed up. And other structures can trigger pain.

[28:24] Dr. Linda Bluestein: Yeah, I was diagnosed with visceral hypersensitivity. I was getting scoped and my doctor said, well, if we don't find anything, then the only explanation — this was before I knew really anything about EDS or MCAS — would have to be visceral hypersensitivity. I remember at that time I was having difficulty with swallowing any kind of water or food that was a little bit warmer or cooler than normal. I would get pain in my esophagus as the food came down. And fortunately, knock on wood, it's way, way better now.
[29:08] But I think a lot of people probably fall into that spectrum of at least some degree of visceral hypersensitivity. It might not be the primary cause of their symptoms, but they might be more aware of what's going on in their GI tract. I feel like I have family members or friends who have no clue what's going on in their body at all. Relative to me, I can feel everything, and they can feel nothing.

[29:33] Dr. Pradeep Chopra: That's true. I've always wondered about that. People with EDS live in a different universe from people without EDS. They've had pain for so long, they think it's a normal part of their life. They're lightheaded, and that's just a normal part of their life. Oftentimes kids come in and they're like — you're not supposed to feel dizzy when you stand.
[29:57] The other thing is that your friend in the GI system is the vagus nerve. We love the vagus nerve. It's a really long nerve, but unfortunately, for some reason, it doesn't go all the way. It stops at about the 2/3 and 1/3 point of the transverse colon. It provides innervation to the upper GI tract — the foregut.
[30:26] Patients with dysautonomia — which is dysfunction of the autonomic nervous system — have low vagal tone. That means their vagus nerve is not working as well, or they can have a damaged vagus nerve for whatever reason, which can cause gastroparesis. Now, gastroparesis is slowing down of the stomach, not the intestines. That's where the vagus nerve supplies.
[30:51] There is a lot of work going on in terms of vagal nerve stimulation and stimulators. There are implantable stimulators and some external stimulators. And there is one drug I know of — it's called pyridostigmine, or Mestinon. It doesn't really have a lot of side effects, but you have to give it in pretty high doses to get the vagus nerve work done. But it's a good drug for POTS and GI issues.

[31:36] Dr. Linda Bluestein: I've had some pretty good results with it.

Dr. Pradeep Chopra: Yeah?

[31:36] Dr. Linda Bluestein: I've definitely had some patients do really well with it.

[31:40] Dr. Pradeep Chopra: Patients like it, but I just wish it solved the problem entirely.

[31:47] Dr. Linda Bluestein: Right, yeah, of course. And I am aware that we need to take a quick break in a second, but I want to ask you one quick question before we do. When you were talking about the vagus nerve and those of us with EDS and dysautonomia and whatever combination of things we might have — is this also why so many of us startle so easily? I have family members who, like, I'll surprise them and they're just like, "What?" My husband has walked around the corner before and I jump and he's like, "I live here."

[32:32] Dr. Pradeep Chopra: A person with EDS has, say, dysautonomia. Dysautonomia is dysfunction of your autonomic nervous system, which means your sympathetic nervous system is revved up, and the sympathetic nervous system is the nerve of flight or fright.

[32:45] Dr. Linda Bluestein: Mm-hmm.

[32:46] Dr. Pradeep Chopra: At baseline, people with POTS, their heart rate is often in the high 90s and even getting into the hundreds, which means you're already in a fight-or-flight state.

[32:58] Dr. Linda Bluestein: Mm-hmm.

[33:00] Dr. Pradeep Chopra: So for example, for me, if I look at a snake, my sympathetic nervous system is revved up — I'm in fight or fright. And now somebody in the back comes and says, "Boo," I'm going to jump out of my skin. That's the thing with people with dysautonomia: they're already revved up, and all it takes is a little bit of a startle to send them over. And someday we may have an awesome vagus nerve treatment.

[33:34] Dr. Linda Bluestein: We're going to take a quick break. I want to make sure that we talk about abdominal wall type problems and also issues with the lower ribs. We'll also come back to pelvic floor and mesenteric ischemia. So we are going to come right back after a quick break.
[35:30] Okay, we're back with Dr. Chopra, and we are talking about a whole host of things that can go on in a body with a connective tissue disorder like EDS that can contribute to abdominal-type or gastrointestinal-type symptoms and can make the picture quite confusing. We've covered a lot of things already. Do you want to move on to the abdominal wall and lower ribs, or do you want to go to a different area next?

[35:58] Dr. Pradeep Chopra: I like to focus on the more commonly missed reasons for abdominal pain. Costochondritis — which is inflammation of the rib, or pain from the rib — is common in EDS. In fact, it's so universally common that a lot of people with EDS are like, "Yeah, it hurts." You press on their rib and they'll say, "Yeah, it hurts," but they don't really care much about it. But if the lower ribs — the lowest-most ribs — start to hurt, it almost feels like abdominal pain.

[36:33] Dr. Linda Bluestein: Hmm.

[36:34] Dr. Pradeep Chopra: Diagnosis is pretty easy, and that's why a physical exam in abdominal pain is so crucial. If they complain of pain in their abdomen and you palpate around and then just push on the rib, and they go, "Ouch" — then I ask them, is this the pain you're complaining about? And then that's from the ribs — costochondritis.

[36:54] Dr. Linda Bluestein: And so is that usually costochondritis or is that sometimes slipping rib syndrome?

[37:02] Dr. Pradeep Chopra: Slipping rib syndrome — I think of that more as a rib subluxation. Rib subluxation can cause pretty severe chest wall pain, and that's almost like you're getting a heart attack. It's that bad. But costochondritis of the lower ribs can mimic abdominal pain. When you feel around and apply light pressure on the lower ribs and they complain of pain, I'll make sure — is this the pain that you're talking about? Obviously you have to at the same time look for other common reasons for abdominal pain, make sure you're not missing something else. But it's reproducible pain. That's what I'm trying to say.

[37:03] Dr. Linda Bluestein: Yeah, that's great, because costochondritis is definitely super common. This is something I have also had — I haven't had all of these things, but I've had quite a few. With costochondritis, what can we do about that?

[38:11] Dr. Pradeep Chopra: It's a tough one. The first thing, as in treatment for any pain, is you have to know the cause of the pain. The reason for the pain here is rib inflammation. Between the ribs there are muscles — actually 3 layers of muscles. When the ribs get inflamed, the muscles also tighten up a little bit, and so they have pretty severe pain there.
[38:37] The question I cannot answer is why people develop costochondritis. This is not an EDS thing — I've seen this in non-EDS patients as well. It's predominantly common in women, and they'll have this severe excruciating rib pain. What I'll do is go in and inject steroid locally over the rib. I haven't done it in that many EDS patients, but I have done it in non-EDS patients and they do get a good response. It's not a very fun experience getting shots on your ribs, but you do it over a few visits and they start to get better. But why they have inflammation of the ribs? I have no idea. I've looked at the literature and nobody really knows why.

[39:26] Dr. Linda Bluestein: Is it related to more motion of the ribs, even if it's not like a full subluxation? I mean, it's something that's common in the general population as well, so.

[39:35] Dr. Pradeep Chopra: Here's the thing — the lower ribs don't really move that much. The ribs on the right side cover the liver, and on the left they protect the spleen. There are some floating ribs, but honestly, I don't know.
[39:52] The diagnosis — it gets worse if they, for example, take a deep breath or cough. A big hearty cough will make the pain worse. And obviously it's reproducible, and that should make you think of costochondritis. I just want to say it's a very benign thing. It's nothing to worry about as long as everything else has been ruled out. Almost all patients I've seen at least have tenderness of the rib, and if you press on it, they go ouch. But other than that, they're okay with it, but it can sometimes mimic abdominal pain.

[40:30] Dr. Linda Bluestein: Mm-hmm.

[40:32] Dr. Pradeep Chopra: The other pain I wanted to talk about — two kinds of pain. Let's talk about the first one, which is called ACNES. The full form is ACNES, or also known as anterior cutaneous nerve entrapment syndrome.

[40:50] Dr. Linda Bluestein: Is it compartment or cutaneous?

[40:51] Dr. Pradeep Chopra: Cutaneous, sorry. Yeah, my bad. So the nerves to our body travel along the sides of the body. They come from behind and then sort of travel along to the front. In the front, you have the rectus abdominis — the six-pack muscle. When they reach the border of the six-pack muscle, there's a tiny little tunnel, and they go through under that tunnel and into the muscle and then branch off. For whatever reason, this tunnel can become narrow and it compresses the nerve. These patients present with abdominal pain — it's pretty severe.
[41:41] The characteristics of this pain are that it's kind of localized. They'll say it's mostly in the upper abdomen, and it can be in the lower abdomen also. I've seen this in both non-EDS and EDS patients. In the non-EDS patients, it's usually because of trauma — boxers, wrestlers, plumbers. I've seen it in plumbers. What they do is work on some plumbing with something kind of pushing into their abdomen, and eventually they start to develop the narrowing, the tunnel gets narrowed down, and they develop this pain.
Sometimes surgery can cause it. You don't see it in cholecystectomies nowadays because we mostly do laparoscopic, but you can see it in hysterectomies or cesarean sections. You can see it after pregnancy. The abdomen kind of bloats up, then goes down, and the nerve entrapment can appear.
[42:58] There are a couple of ways to diagnose it. One is you simply start pressing on that edge of the rectus, the six-pack muscle. You press on the edge and you find a tender spot. The other is you have them do a little partial sit-up, and you'll find that exact spot — it'll be one specific spot that hurts. There is something called Carnett's sign, where you lie back and then lift your leg up, which tightens the six-pack muscle, and again you can find a tender spot.
[43:33] The diagnosis is kind of difficult — you have to go in and find that exact tender spot and inject a tiny bit of numbing medicine. And if it takes away the pain, then you know that's what it is. That's kind of hard to do.

[43:55] Dr. Linda Bluestein: You were maybe just going to say what I was going to say — which is if they're not sensitive to local anesthetics in the typical way, then that's going to confuse your result as well.

[44:04] Dr. Pradeep Chopra: Oh, of course. You'd have to check with the patients if they are responsive to lidocaine or not. A lot of EDS patients do not respond — they're insensitive to lidocaine. So I generally use Marcaine or bupivacaine. Nowadays, if I'm doing any kind of injection on any EDS patient, I go straight for Marcaine. It's just much better that way, you're not confusing things.
[44:32] But yes, you can inject a local anesthetic like Marcaine or lidocaine and see whether they get relief from it. And it has to be pretty significant relief. Sometimes you can repeat it just to be sure.

[44:42] Dr. Linda Bluestein: And then what do you do if that test is positive?

[44:52] Dr. Pradeep Chopra: We've tried everything. The literature says weight loss and all kinds of things. But at the end of the day, they need a surgical release. That's the only thing I have seen that helps.

[45:05] Dr. Linda Bluestein: I was just going to say, I bet it's not easy to find a surgeon to do that.

[45:09] Dr. Pradeep Chopra: Yeah. I don't blame them because it's kind of hard — there are these fine nerves, and you have to identify which nerve to release. It's difficult. But yes. The reason I bring up ACNES or anterior cutaneous nerve entrapment syndrome is because it's not all that rare. Easily, if you press on the edge of the six-pack muscle, they're very tender there. They do a sit-up and you press and it hurts a lot. You can have them raise their leg and press — it hurts — and it has to be a very localized pain. And then of course you can numb it up, and if it works, then you're sure it's ACNES.

[45:59] Dr. Linda Bluestein: With the costochondritis, now I understand that's an -itis so that's an inflammatory condition, and there you're using the local anesthetic and steroid. Here you're talking about using just a local anesthetic, which it sounds like is diagnostic. But is there any therapeutic benefit? Like if you did that a few times with some frequency, would that potentially help with their symptoms, or is it just for diagnostics?

[46:25] Dr. Pradeep Chopra: It's only for diagnostics. I have tried radiofrequency ablation of that cutaneous nerve.

[46:35] Dr. Linda Bluestein: Oh, really?

[46:38] Dr. Pradeep Chopra: But I have to admit, I've stopped doing it because it's just not that easy. It's very difficult to identify the nerve. If it's against a bony landmark like we do for other issues, then you know where the nerve is. But here it's kind of floating around, so we don't. I did it years ago and wasn't impressed, so I stopped doing it. I think surgical release is the best way.

[47:07] Dr. Linda Bluestein: What about abdominal migraines? Is that something you see fairly commonly?

[47:12] Dr. Pradeep Chopra: I have seen some. When I'm looking at a patient with EDS, when they come and tell me they have abdominal migraines, I kind of leave it to the neurologists who are already treating them for it. But these patients present with very severe, sudden-onset abdominal pain — cramping, usually around the belly button area. They have nausea and vomiting. They can alternate between diarrhea and constipation, but they have a headache at the same time, and that's what makes it an abdominal migraine.

[47:49] Dr. Linda Bluestein: So do you have to have a headache at the same time?

[47:52] Dr. Pradeep Chopra: Not have to, but they often do. And they also have sensitivity to light and sound, just like a migraine. That's one of the reasons why they call it abdominal migraine. But the usual migraine medicines don't work for this. It's more common in children. I haven't seen it in adults, and oftentimes it goes away — they'll say, yeah, when I was younger I got diagnosed, but it's gone now. So I don't know if it's another condition they grow out of and it's just being labeled as abdominal migraines. But the usual migraine treatments like triptans and CGRP antagonists don't work.

[48:29] Dr. Linda Bluestein: And what about CRPS? Can you get CRPS of the abdomen?

[48:38] Dr. Pradeep Chopra: Yes, you can. CRPS is complex regional pain syndrome — an awfully painful condition. For the longest time, we thought you could only have CRPS in the limbs. In fact, up till recently it was said it happens only in the arms or the legs. But we've seen patients develop CRPS of their abdomen.
It presents classically with CRPS symptoms. They have pain to soft touch, known as allodynia. They may or may not have color change — one of the diagnostic criteria of CRPS is color difference, which is easy to see in an extremity, but in the abdomen it's a little more difficult. There also has to be a temperature difference.
[49:30] In the past, the standard criteria for diagnosing CRPS was the Budapest criteria — it is still the Budapest criteria, but there is now also what is called the Valencia consensus. In the Valencia consensus, they ironed out a few kinks in the Budapest criteria. For example, if you have CRPS in both legs, you measure temperature in a different, unaffected part of the body. So if you have CRPS in the abdomen, you measure the temperature on the abdomen and then on, say, the forehead. The criteria is there should be at least a 1-degree difference — in reality you often see 5 to 10 degrees of difference.
[50:25] But yes, you can have CRPS of the abdomen. It's a lot like post-herpetic neuralgia. They'll come in with their shirt off, and it's very painful even to be touched.

[50:38] Dr. Linda Bluestein: And if someone has CRPS of the abdomen — because, as we know, CRPS can travel, starting in one extremity and then moving to other parts of the body — do you know if CRPS of the abdomen more often follows CRPS of extremities, or if it can just start in the abdomen itself?

[51:02] Dr. Pradeep Chopra: Most times that I've seen CRPS of the abdomen, it has been after trauma. I had this patient who developed CRPS. They had put EKG leads on his chest and his abdomen, and without realizing it, he was put through an MRI scanner.

[51:21] Dr. Linda Bluestein: Oh no.

[51:22] Dr. Pradeep Chopra: He had all these burn marks, and that resulted in him developing CRPS over that area. So it's usually after a trauma that you'll see this. It's become a lot less common now because most abdominal surgeries are laparoscopic — tiny incisions, not like in the old days when they would slice the person open. So there's less nerve damage. But you do still see it. Car accidents, especially when the airbag explodes and hits the abdomen, can cause CRPS symptoms.

[52:01] Dr. Linda Bluestein: Interesting. And what about bladder problems? What can happen with the bladder that we should be aware of?

[52:09] Dr. Pradeep Chopra: Let's talk about bladder pain. Often it's lumped into interstitial cystitis. We really don't know why people develop interstitial cystitis. There are lots of theories, but it basically boils down to inflammation of the lining of the bladder. Some people have even called it fibromyalgia of the bladder. When a condition has many names, that usually means none of them are quite right.
[52:46] The bladder pain — they have inflammation of the bladder, often diagnosed as interstitial cystitis. But the keyword is cystitis, which means inflammation of the bladder. I've talked to gynecologists a lot, and they all think it can be MCAS. But I haven't been able to convince anyone to put cromolyn in the bladder.

[53:18] Dr. Linda Bluestein: Yeah, I tried to talk to my husband about that. In fact, one of our colleagues from Masterminds — we were having dinner at her house, and she tried to convince my husband. He was still in clinical practice, and she was like, "Oh, come on, just try it."

[53:35] Dr. Pradeep Chopra: I mean, you can drink it, you can put it in your eyes — why can't you put it in the bladder?

[53:39] Dr. Linda Bluestein: So you have tried it? No?

[53:42] Dr. Pradeep Chopra: Oh, no, I haven't. I've tried convincing the local urologist, and they all agree. But hospital policies and all sorts of things get in the way.
But yes — you can think simply, okay, well, you're taking cromolyn, you're drinking it, it's a liquid, it'll eventually get into the blood and get into the bladder. But here's the problem with cromolyn: it doesn't get absorbed that well. Its absorption is not that great. So I don't think a lot of it gets into the bladder. Now, ketotifen might be a different drug that could help.
[54:24] Most people who treat interstitial cystitis put patients on a kind of low-histamine diet to avoid irritation of the bladder, or they think about the spasm of the bladder muscle and try to distend it — loosen up the muscle. They do what is called a DMSO instillation, where they expand the bladder, hoping the muscle will stretch out. I think it does help. But bladder inflammation is common in EDS, and if it's an EDS patient who has obvious signs of MCAS, I attribute that mostly to MCAS.

[55:12] Dr. Linda Bluestein: Yeah, I do too. And I've had some really good results with resolution of bladder symptoms from addressing their MCAS, for sure.

[55:23] Dr. Pradeep Chopra: On the abdominal wall pain, I just want to talk about abdominal muscle spasms. It's usually after a trauma, like surgical trauma — a hysterectomy or a cesarean section or something where there's been a long incision. Think of the abdomen as a bag made up of muscles all around it. Some of these muscles, when they are sliced open and then heal up, don't heal up that well, and there are localized spasms of these muscles. Most of the time I've seen it in the lower abdomen — after an open inguinal hernia surgery, or hysterectomy, or cesarean section.
[56:08] Again, it's the same thing — you have them do a little sit-up and you press on that muscle. You can literally feel muscle knots there. In the old days, I used to do lidocaine trigger point injections — numbing medicine injections. But now I just skip that step and go straight for Botox.

[56:31] Dr. Linda Bluestein: Oh, interesting.

[56:31] Dr. Pradeep Chopra: It does help. Because it's not inflammatory — it's just clear muscle spasm. So I might do a few trigger point injections with a local anesthetic first, and if they have a decent response, then I go on to Botox injections for these muscle spasms. So yes, patients with abdominal pain can have outside-the-abdomen pain as well as inside-the-abdomen pain.

[56:56] Dr. Linda Bluestein: Okay. And I want to make sure that before we wrap up, I touch on a couple of things we haven't hit yet. So let me tell you what those are and you can pick which one we do first. I think you and I had talked a little bit about rectal evacuation disorders; food intolerances, which we've kind of touched on; MCAS, but we haven't talked more specifically about some of those. Pelvic floor dysfunction, endometriosis, ovarian cysts or torsion, and dyspareunia-related pain. That's the list you had given me before we started. Are there some of those we can try to touch on in the last few minutes here?

[57:35] Dr. Pradeep Chopra: Sure. Just before we go into that, I just want to say that endometriosis is very, very, very common in EDS. And that's one thing — if you are having pelvic pain, don't miss out on endometriosis. When you have your cycle, the pain gets extraordinarily worse. That's one thing to look at.
[57:59] Rectal evacuatory dysfunction is when patients are trying to have a bowel movement and they can't empty it completely. They have pain and difficulty emptying completely — they might go partially, but not all of it. It can be for different reasons. It could be because of weak pelvic floor muscles, or like I said, the sigmoid colon getting loaded and flipping over, which can cause some of this. And just chronic constipation can cause rectal evacuatory dysfunction. They figure this out by doing a manometry — they put a balloon in there and look at the pressure.
[58:50] Vaginal pain in EDS is actually very common. The thing is, a lot of women don't really want to talk about it and don't bring it up. So I sometimes bring it up. A lot of it, I think, is related to MCAS. Like everything else, the lining of the vagina is a lining and it gets inflamed. That causes inflammation of the vaginal lining.
[59:15] It can also come from pelvic floor dysfunction. The pelvis is like a ring, and the pelvic floor is a little floor on the bottom of that ring. If the ring is not steady, then the pelvic floor is being stretched, and that can cause pelvic floor spasm and vaginal pain.
There are also other reasons that can cause issues — like recurrent urinary tract infections that can cause pain in these patients. When that happens, I think of tethered cord syndrome, because the sphincter to the urethra is not closing properly and organisms are going up the wrong way. But it can be for other reasons. Sometimes bladder interstitial cystitis or bladder inflammation can get misdiagnosed as recurrent urinary tract infections.

[1:00:36] Dr. Linda Bluestein: Lots and lots of reasons. I had a period when I was in residency where I had these horrible episodes of abdominal pain, and then in between I would be relatively fine, but they were trying to work me up for all kinds of different things, including porphyria, and all the testing came out negative. And fortunately now, knock on wood, I don't have this problem anymore.
[1:00:59] This was such a great conversation because I feel like so many people — well, it's like with me, right? I was told if we don't see something structural, then the only thing I can think of that could be going on with you is visceral hypersensitivity. And we just went through this exhaustive list of all kinds of other things that can be contributing to abdominal pain, bloating, constipation, diarrhea, all kinds of GI symptoms. This was such a great conversation. I really appreciate you taking the time to go through all of this and teach us so many things that we should all be thinking about for EDS and HSD. Thank you. It was great.

[1:01:38] Dr. Pradeep Chopra: There must be at least another dozen conditions — not quite related to EDS, but that can cause abdominal pain. People with EDS can have non-EDS pain conditions as well as EDS-related pain conditions.

[1:01:50] Dr. Linda Bluestein: Right. Do you want to rattle some of those off right now, so that people — because otherwise I think they're going to email me and be like, wait, that was a cliffhanger.

[1:02:03] Dr. Pradeep Chopra: Okay, so you can have ovarian cysts or ovarian torsion — that can do it. Pelvic floor dysfunction — we talked about that. Volvulus or intussusception. Volvulus is when a part of the intestine just kind of twists on itself, so the blood flow is restricted and it becomes really painful. Intussusception is when parts of the intestine go into each other.
[1:02:29] One of the things I wanted to say was that people who have continuous abdominal pain are easier to diagnose than, like, what you had — it came and then it went away. By the time you get to the doctor's office, there's no pain, and so it's hard to figure out what's causing it. But if you go in there with actual pain, you can sort of figure out what's going on. I suspect some of these people might have volvulus that twists and then untwists.
Then there's intussusception. MCAS is a really big reason for GI pain. Food intolerances — that's like five podcast episodes' worth of content. Why people with EDS have food intolerances, we're not sure, but there are lots of food intolerances that can cause this.
[1:03:28] Then you have visceral and neuronal crosstalk that can do it. Rupture of the uterus, especially in pregnancies — uterine rupture. I haven't seen it, I haven't even heard of it, but it's something to keep in mind.
[1:03:46] Retroperitoneal hematoma — a blood vessel will break in the back of the abdomen and cause a collection of blood there. Mesenteric ischemia or dissection can cause it. There's a part of the intestine that has a flap — let's call it a flap — and blood vessels travel to the intestine through this mesentery. Sometimes those blood vessels can get kinked or blocked, or they can rupture, and so there's no blood flow to the intestine, and they have abdominal pain.
[1:04:30] And then hernias — there's a weakness in the abdominal wall and the contents of the abdomen are poking through it. If you don't fix it in time, food matter can get stuck in it or it can twist on itself.

[1:05:02] Dr. Linda Bluestein: Get incarcerated.

[1:05:03] Dr. Pradeep Chopra: Yeah, incarcerated. I haven't seen that lately because doctors are pretty good at diagnosing hernias and catching them early on, but it can happen. Those are some of the outliers of abdominal pain in EDS.

[1:05:23] Dr. Linda Bluestein: And we know hernias are more common in EDS. Some of these things are common in regular people, but some of them also occur more commonly with connective tissue disorders.

[1:05:33] Dr. Pradeep Chopra: Recurrent hernias is in the diagnostic criteria for hypermobile EDS.

[1:05:36] Dr. Linda Bluestein: Right, exactly. Well, very good. Thank you so much. I was taking notes as we were talking, and I'm going to recommend about 8 other episodes that people are going to want to listen to based on this conversation, because they're going to be like, wait, I want more information about XYZ.
[1:05:54] So they're going to want to check out the 2 episodes that we did with Dr. Theoharides, where you were the guest host for the second one. And I've interviewed Dr. Irwin Goldstein and Dr. Andrew Goldstein about perineal issues and pelvic problems. Dr. Kelly Caspersen about urologic problems, including interstitial cystitis. I interviewed Dr. Zach Spiritos, who's a neurogastroenterologist, about gastrointestinal problems. And then the last one I wanted to mention is the episode with Dr. Adam Hansen where we talked about slipping rib syndrome. We'll link to all of those in the show notes as well.
[1:06:33] This has been such a great conversation, and I'm so grateful to you for taking the time again to chat with me and teach us so many wonderful things that all of us should know about for EDS and HSD and this complication that we might experience and that can be easily missed.

[1:06:55] Dr. Pradeep Chopra: Thank you for having me on. It's a pleasure.

[1:07:53] Dr. Linda Bluestein: Well, I am so grateful for Dr. Pradeep Chopra coming on the Bendy Bodies Podcast once again to share his infinite knowledge with us. It was so great to talk about this part 2 of gastrointestinal problems, and I hope that you will also check out the other episodes mentioned during this conversation. I had a great conversation with Dr. Zach Spiritos, 2 conversations with Dr. Theoharides, and so many others that we will link in the show notes — I definitely urge you to check those out so you can take an even deeper dive.
[1:08:23] And thank you so much for listening to this week's episode of the Bendy Bodies with the Hypermobility MD Podcast. You can help us spread the word about joint hypermobility and related disorders by leaving a review and sharing the podcast. This really helps raise awareness about these complex conditions.
[1:08:39] Did you also know that I offer one-on-one support for both clients and healthcare professionals? Whether you're living with hypermobility or caring for someone who is, I've got your back. Check out my coaching and mentorship options on the services page of my website at hypermobilitymd.com. You can also find me, Dr. Linda Bluestein, on Instagram, Facebook, TikTok, Twitter, or LinkedIn at hypermobilitymd. You can find Human Content — my producing team — at human content pods on TikTok and Instagram. You can also find full video episodes every week on YouTube at Bendy Bodies Podcast.
[1:09:12] A quick note: while I'm so glad that you're enjoying the Bendy Bodies Podcast and we love bringing on guests with unique perspectives to share, these unscripted discussions do not reflect the views or opinions held by me or the Bendy Bodies team. Although we may share healthcare perspectives on the podcast, no statements shared on Bendy Bodies should be considered medical advice. Please always consult a qualified healthcare provider for your own care.
[1:09:36] To learn about the Bendy Bodies program disclaimer and ethics policy, submission verification and licensing terms, and HIPAA release terms, or to reach out with any questions, please visit bendybodiespodcast.com. Bendy Bodies Podcast is a Human Content production. Thank you for being a part of our community, and we'll catch you next time on the Bendy Bodies Podcast.