Eyes Problems in EDS with Dr. Eric Singman and cohost Dr. Dacre Knight

[00:42] Dr. Linda Bluestein: Welcome back, every bendy body, to the Bendy Bodies Podcast. I'm your host, Dr. Linda Bluestein, the Hypermobility MD, a Mayo Clinic-trained physician dedicated to helping you navigate Ehlers-Danlos syndromes and complex chronic illness. Today I'm joined by Dr. Dacre Knight, who is not only an expert in EDS, HSD, POTS, and mast cell disorders, but is also joining me as a recurring co-host. Dr. Knight recently became the medical director of the UVA Ehlers-Danlos Syndrome Center, which is officially partnering with Bendy Bodies. Today I'm also joined by Dr. Eric Singman, a neuro-ophthalmologist who works at the intersection of the eyes, the brain, and the complex symptoms so many people with EDS struggle to have taken seriously. He's a professor at the University of Maryland School of Medicine and has spent decades caring for patients whose vision problems don't show up on standard eye exams. If you haven't already, be sure to check out episode 180 for part 1 of our conversation. Dr. Singman has held leadership roles at Johns Hopkins Wilmer Eye Institute, the Department of Defense, and Social Security Administration and helped shape national guidelines on disability and brain injury. His work focuses on visual dysfunction after brain injury and the visual impacts of hypermobile Ehlers-Danlos syndrome, making his perspective especially relevant for the bendy bodies community. As always, this information is for educational purposes only and is not a substitute for personalized medical advice. Stick around until the very end so you don't miss any of our special hypermobility hacks. Here we go.

Well, I'm so excited to be back with Dr. Singman. When we released the previous episode, I have to tell you, so many people really enjoyed that conversation and requested that we have you back for a part 2.

Dr. Dacre Knight: Thank you.

[02:38] Dr. Linda Bluestein: So I first wanted to talk about an EDS vision triage to help us figure out when we need a specialist and who might need a specialist. Can you give us maybe a 5-step or so decision tree for who needs to go where, so that we know if somebody has various different red flags or if they need to see a subspecialist? Because I don't think a lot of people realize that there's within ophthalmology, there's cornea, there's retina, we talked about dry eye clinics last time, and also neuro-ophthalmology, which is what you practice. Can you give us an idea of how a person might determine what subspecialist they need to go to?

[03:20] Dr. Eric Singman: Sure thing. First, if they know they have a particular vision problem, like they have a history of retinal detachment, retinal tear, et cetera, they should go to a retina specialist. They should go to the specialist they know about. A history of keratoconus, which is weak corneas, they should go to a cornea specialist. So if they know they have a particular problem, by all means, go to the specialist in that area.

If they have a new vision problem, believe it or not, any comprehensive ophthalmologist is a good start just to go through the eye exam and say, okay, it's not your cornea, it's not your retina, it's not glaucoma, it's not inflammation in your eye, it's not this, it's not that. Once they do that, they can, by process of elimination, if you will, be able to guide you where you need to go.

Now, sometimes they do end up going to a neuro-ophthalmologist. The most common person is: you have a vision complaint. I don't know why. Your eye looks fine. Go see a neuro-ophthalmologist. That's the most common vision problem. That's the patients I get.

On the other hand, they may find other things. Now, sometimes the problems can be subtle, and sometimes even a good comprehensive ophthalmologist might say, I'm not really sure. Perfect example: someone says, "Reading is hard. I get tired when I read. I don't have the reading endurance I used to have." Well, the patient's going to go to an eye doctor and ask, is it my eyes? And the doctor's going to check your eyes and see if it's okay. And then hopefully the doctor's going to ask you, well, why is your reading endurance down? Well, I mean, I just can't seem to remember what I'm reading. Well, that's not an eye problem. That's a cognitive problem. So you don't need an eye doctor.

On the other hand, if you say, boy, my eyes just get so tired. I just feel like my eyelids are coming down. I'm just exhausted. Well, then that doctor might look for some reading problems. The classic one we mentioned last time we were here was convergence insufficiency, where a person has trouble either initiating or maintaining that cross-eyed posture you need for reading comfortably. Once that happens, then you can go see a neuro-ophthalmologist.

Now, clearly you got to be careful. We'll talk about this later, but people have been turning Ehlers-Danlos into cash cows, and they've been turning some of these soft reading findings into big-time cash cows. I just got off the phone with someone about 5 minutes ago, a delightful, sweet person who watched the Bendy Bodies Podcast from last time, and she asked me some really excellent questions. This is an extremely intelligent person, like all my Ehlers-Danlos patients tend to be. Well, she knows what's going on. And unfortunately, it sounds like she may have seen people who have been on the dark side, so to speak.

As far as a triage, there are certain red flags you want to look for. Certain things immediately come to mind. If there is a loss of vision, whether it's in one eye or both eyes, if there's eye pain in one eye or both eyes, if there's double vision, if there is swelling, redness, et cetera, these all are red flags for potentially an ocular emergency. If the eye doctor says your eyes are fine, but you're seeing double, that patient probably goes to the emergency room to make sure they don't have a stroke or a CSF leak or something terrible.

So there are also red flags that most people, in my experience, understand that there's some stuff that just isn't right. And they know that a red flag is when something's amiss. And most of the eye care providers I know, optometrists or ophthalmologists, if they see some of these red flags—vision loss, pain with eye movement, double vision—they're going to probably believe that patient also should get checked out immediately.

The problem is there are some people who say, well, they call me, can I have them see your clinic in a couple weeks? They say, no, you can't. You send them to the emergency room. I just gave them, actually, to the neurologists here at University of Maryland a talk on neuro-ophthalmic emergencies. And so these are some of the things that represent red flags. If something's a chronic problem going on for months or weeks, and if it's getting worse, that could be a portent of an emergency. On the other hand, if it's just stable, but it's just annoying, then that's something that probably is not emergent, but still should be checked out.

So I still think that usually the best way to do it is have a competent, comprehensive ophthalmologist be our gatekeeper, if you will, to look for those things that can and should be referred either urgently or less urgently and take it from there.

[08:19] Dr. Linda Bluestein: And I appreciate that. So you're saying that sometimes reading difficulties would be more cognitive and other times it would be more related to the eyes. And in terms of the examples you gave, are there other ways that we can determine whether it's more of a cognitive or brain problem or an eye problem?

[08:40] Dr. Eric Singman: There are a couple of ways. So let's say convergence insufficiency, which is one of the most common issues that we deal with. We talked about how the fact is that it's your eyes don't want to work together. They don't want to play nice together. Right. And so if you cover one eye by wearing a patch or occluding one eye, and you find you're much more comfortable because your eyes aren't fighting each other, well, you've now developed a quick temporizing measure so you can get through your homework. And second, it gives us a clear idea of probably what's going on.

Now, the problem with convergence insufficiency is there's a gray area between convergence insufficiency and other strabismic problems that can mimic convergence insufficiency, like intermittent exotropia. In other words, some people have a tendency for their eyes to go walleyed, to turn out, but they often have some degree of control. If they have intermittent exotropia, the eye sometimes goes out and we then rate them as good, moderate, or poor control. If they happen to have control that was good and now it's getting less good, that could be a concern that can come with fatigue. It can come with aging, but it also can come with other problems that usually need to be checked out.

One of the biggest issues I see is that someone's given prism in his glasses or her glasses for an exotropia. And the person says, wow, this is so much better. I see great. But now you've relaxed them even more so that they become more dependent on the prism and they get less good control because they're not putting effort in to keep those muscles tight.

Dr. Linda Bluestein: Dr. Singman, you hit on some questions I was just going to ask. Which is great. I think you're already reading our minds where we're trying to go with this, but I will tell you that my knowledge is a little bit rusty from medical school when I first really got my last exposure to eye exams and ophthalmology. Could you give us just a kind of a run-by of the proper convergence evaluation, where we would start? I mean, I know there's lots of things that could be going on, but where would we start, just at a very basic level?

[11:05] Dr. Eric Singman: So the one thing you don't want to do is have someone look at your finger and slowly bring it to their nose. That's the mistake because, one, that's testing both convergence and accommodation—the focusing of the natural lens is accommodation. And two, that's not how it works. Sure, you can test convergence that way, I guess. But that's not going to test convergence in a way that's valuable to anybody.

The way the easiest way to test convergence is to take what's called a prism rack. Very simple. It's a bar with graded prisms on it. And you simply hold it with the wide part out, called the base out, and you slowly run it from a weak prism to a strong prism. And you ask the patient one thing: does this mimic the discomfort you have with reading? Because this causes convergence without causing accommodation, because you're fixing the distance. So you're not changing the working distance. So you can eliminate that variable of accommodation.

So you say, okay, does this mimic the feeling of reading? You get the discomfort and the patient says yes, it's probably convergence deficiency. And you also measure because the prisms are graded—you can actually measure how well they can converge. Now, a young, healthy person should be able to converge 35, 40 prism diopters, which is a substantial amount. I'm not saying they should be able to keep up 40 prism diopters. I don't think anyone can. That'll give anyone a headache.

When you and I converge, let's say we're working at a computer right now. The screen is perhaps what, maybe 2 feet from us or so, right? At least my screen on my laptop. And so I'm converging probably no more than 15 prism diopters to see that screen. Because if you think about the physics of it, when your eyes are looking at infinity, they're parallel. When your eyes are looking at something that's even, you know, 15 or 20 feet away, they only turn in a little bit.

So convergence—unless they're really bad, they should be able to do some work with convergence. And so that's how I like to measure convergence.

And the second trick I do with convergence—as I said, I just test somebody. If someone comes to me and says, my doctor said I have convergence deficiency, I'd be like, yeah. I said, thank you. That's great. Did they ever do a monocular trial? If they tell me no, then I question what the doctor were thinking.

Because the doctor's job is, well, the excuse I got when the doctor didn't do that—I spoke to the doctor, I call, I sometimes call the doctor and say, well, you know, please help me out here. I'm always polite, but they say, well, I believe that two eyes are better than one. So I didn't want to even consider using one eye alone. I said, well, if it's a temporizing measure that helps the patient and they can get through their homework or they get through their workday, wouldn't that be a good thing? And I usually get crickets on the other side of the phone.

[13:54] Dr. Linda Bluestein: Okay. So, and this is a follow-up on that last part. So sometimes you're saying that clinicians don't do a monocular trial, they only do a binocular exam. Is that what you're saying? Then the information you get from that is just kind of limited, right? Presumably. Yeah.

[14:13] Dr. Eric Singman: Of course. Right. The less you do, the less you have. It's that simple.

[14:16] Dr. Linda Bluestein: So from that type of clinician in that setting and in what you're talking about with the convergence evaluation you do, because of course a lot of us do the finger to nose, thinking that might be adequate, but it's obviously not, and to do the appropriate exam, then it sounds like what I had in my doctor's bag in medical school would not be adequate. So this is something that eventually is going to land them in a neuro-ophthalmologist's office. Is that correct?

[14:42] Dr. Eric Singman: I would hope so. Yeah. I mean, as I said, when I teach both optometrists and ophthalmologists about this, I start off and I say, you know, if you really want to measure convergence, you can't measure anything else but convergence. And so if you're going to measure accommodation and convergence, then you're measuring nothing.

And I get some people give me pushback. Well, but the book says we're supposed to have a near point of convergence of 6 inches from the nose. And I say, that's nice to know, but how did that number come about? Did they do it on people of all ages? Did they do it on people who have artificial lenses? Did they do it on people where they can't accommodate anymore? I mean, what exactly are you doing for somebody?

So the bottom line is if someone says, yeah, doc, I have no problem looking at my finger right up to my nose. Great. Great. If it's a negative test, that's wonderful. I'm delighted. But if it's a positive test, then you want to know what's being positive.

[15:57] Dr. Linda Bluestein: Okay. So there's the next step then. And you mentioned this too, just briefly, that there being lots of other possible things—whether it's convergence insufficiency or accommodation, or other things. How do you differentiate from all of those? Is that right?

[16:11] Dr. Eric Singman: Correct. That's my job. Both pediatric ophthalmologists and neuro-ophthalmologists have a bag of tricks that we use to try to explain if there are abnormal eye movements or abnormal eye orientations—if you will—that are causing the problem that's the basis of the patient's complaint. A lot of times it's not.

You know, there are very simple tricks that we use, and the things I use to test the eye are the same things I use therapeutically. So, for example, I might take my watch on a fob and swing it like a pendulum very slowly and ask the patient to follow it. And if a patient says, you know, I'm following it, doc, but honestly, I'm getting a little nauseous from it, that tells me something.

This tests smooth pursuits, by the way. Even if the pursuits are smooth, if someone says you're making me nauseous, pal, then I would say that, okay, well then maybe there's a problem with the smooth pursuit system.

The same way I test saccades, which are rapid eye movements. Just for your listening audience, I'll explain. Smooth pursuits is when an eye tracks something and they're looking at the thing they're tracking and the eyes really don't care about the background. Saccades are where the eye goes from object A to object B. They don't care about the background. They don't care about tracking anything. They just want to get from A to B.

Saccades require you to take fixation off an object of interest—object A—and refixate quickly on an object B. And so pursuits, saccades, and convergence, and divergence, and other ocular motility—those are the things that I like to look for when a person has visual complaints, because if their eyes look fine, but they tell me they have visual complaints, chances are it's going to be in this realm. And if it is, then there are things we can do about it.

If it's an eye teaming problem where the eyes don't want to work as a team, the easiest way to confirm that that's the only problem is to simply cover one eye. Then the eyes don't have to compete with each other. And a person often will be very happy with that. Now, nobody wants to write off an eye. I get that. But if a kid has to go to school, at least they got something.

By the same token though, if somebody covers one eye and says, you know, doc, that improved my reading endurance for like at least double—I went from 20 minutes to like 40 minutes—but then I started getting tired again, well, then you have to ask yourself two questions. One is what's normal reading endurance? And secondly, if the normal reading endurance for that person should be more than 40 minutes, then you're going to want to say, well, maybe there are other problems. And those other problems could be reading hygiene, for example, not even vision.

So I'm a certified low vision specialist, and I can tell you that reading hygiene includes the lighting, the head position, the arm position, the neck position, et cetera. And that is something I also look into because, you know, if I were to show you a picture, if you envision a picture of someone reading in your mind, you'd be picturing someone holding a book in their lap and looking down and reading it. The problem is for Ehlers-Danlos, that's torture because Ehlers-Danlos patients have terrible necks. I mean, just uniformly their necks are bad. So the last thing you want to do is have their reading position down with their chin on their chest, reading down. You want to have them reading in a neutral position. So these are all the things I try to incorporate when someone tells me there's a reading problem.

And by the way, as an aside, I think 40 minutes is a limit on anyone's reading endurance. I like to consider—I tell patients, I don't want you to read for longer than 20 minutes straight. I tell perfectly healthy patients, students, excellent students. I want you to take a break every 20 minutes, not a 4-hour break, although some of my patients would probably like that. But easily, a few-minute break, sit up, get some fresh air, get some sunshine, breathe, get some grass on your feet just for a couple of minutes between 20-minute reading sessions. You'd be surprised how much better people do, just everyone does with that.

[20:27] Dr. Linda Bluestein: Yeah. Yeah. Well, that's a great tip. And thank you. You just pulled another question right out of my mind, which is now, you know, I can just summarize it and make sure I got it correctly. So, as far as reading, in endurance and fatigue, it seems like it is variable from one individual to the next, but generally there are some kind of broad rules too that we just don't want to overdo it. It sounds like a lot of the work that you do in the counseling you provide to patients overlaps in the realm of occupational therapy too, when we talk about ergonomics and things like that. That's great because we know that there is such a great need for occupational therapists in the role of care for EDS patients.

And what I'm also gathering from this is that generally, as far as complaints go, what you hear in these settings is reading fatigue and issues related to kind of the stamina and reading performance mostly. Is that right? There's where those complaints are.

Dr. Eric Singman: That's right.

[21:25] Dr. Linda Bluestein: And then the last thing before we kind of move into the next area that I wanted to touch on—do prisms, so you mentioned the role of prism and how it helps and things like that. Are there occasions where you may be concerned that it's just a band-aid?

[21:42] Dr. Eric Singman: It's always a band-aid. Prisms are always a band-aid, in my opinion. Now, sometimes you need a long-term band-aid, but it's always a band-aid. Prisms are only a temporizing measure.

Like I said, I had this wonderful conversation with this person I was just talking to, and someone gave her prism without finding out why she had double vision. I mean, to me, that's dangerous. I mean, double vision can be caused by anything from something simple to something fatal. And so, you know, you don't just say you have double vision, here's a prism, have a nice day. It just doesn't work like that.

You know, and when I get in—and again, prism is a classic cash cow too. So for example, if I have a patient where they have a strabismus, let's say they come in, they had a small stroke, they had a 6th nerve palsy, which means that their eye drifts in so they're cross-eyed. If it's not too big a prism amount—less than 10 diopters, I never give more than 10 prism diopters, which is not a heck of a lot. These little plastic sheets called Fresnel prisms, F-E-R-S-N-E-L, Fresnel prisms. I cut one out. They cost the doctor around $20. Doctors charge them, they double it up, usually about $40. That's just because of, you know, the way things are marked up—it has to be because of your staff and your time.

But this patient came to me with a Fresnel prism and the doctor told him it was $180, but at least they gave him the Fresnel prism. And then I looked at the Fresnel prism. This had to be a 25 or 30 prism diopter Fresnel prism. And I'm saying, if you ever saw a Fresnel prism, they're made of little lines and they cause rainbows and halos and distortion. So that's why if I give more than 10, you're going to have, you know, you may see one image, but that one image is going to be a clear image and a blurry image. Your brain can't fuse that anyway.

So prisms are a temporizing measure to hopefully let you see single in one direction—primary gaze, front gaze—until we figure out why you have the double vision and what to do about it. I certainly wouldn't build prism into someone's glasses unless they didn't want any other therapy for a permanent and stable double vision. And if I did build—and now there is one caveat to that. Delightfully, you can buy glasses online now for a lot less money.

So the glasses I'm wearing now, I'm a myope—I'm nearsighted. They cost me $9. I get them on some of these online sites. I won't mention names because I get yelled at for that. But I, and I don't work for these companies. I get nothing from them, but there are some online glass sites that are very inexpensive and you can get some glasses with just prism in them. They charge an extra $5 a lens for prism. So you can get prism glasses if you need them for like $20.

The reason I generally don't give them is because by the time 2 weeks are over to order the glasses and get them back into the patient's hands, the prism might not be needed anymore, or they might need a different amount because a sixth nerve palsy is going to get better. You know, if it's an ischemic one from a mini stroke, it's going to get better, or from trauma—let's say head trauma—it's going to get better over 2 to 3 months usually. And so the prism they need at week 2 and prism they need at week 6 is going to be different. You don't make someone spend money for that.

So prisms to me are always a band-aid. They're always a temporizing measure, and their only job is to keep the patient more comfortable and possibly seeing single, if possible, until you find out why they have double vision and what they're going to do about it.

[25:17] Dr. Linda Bluestein: We want to shift gears a little bit to dysautonomia and POTS, because this is obviously something that's very common in this population of people. Can you describe to us what vision complaints make you think orthostatic physiology first?

Dr. Eric Singman: So POTS, which I'm not sure—well, actually, I have an idea why it plagues Ehlers-Danlos patients, frankly. I mean, if I personally believe in the research I've seen is that if their blood vessel walls are as collagen-deficient or problematic as anything else, and it's blood vessel wall stretch that causes the autonomic changes of heart rate and such, I'm presuming that that's part of the reason why they have these dysautonomias related to heart rate because of the baroreceptors in the vessels being problematic.

But that being, you know, that's just my theory. Whether or not that's been proven, I don't know. But what I do know is that when they get orthostasis, they're not going to get enough blood to different parts of their brain. And depending on the fluid—remember that the brain is all connected, right? They're all connected to each other, but there are different pressure heads in different parts of the brain.

If the vertebrobasilar system, which feeds your cerebellum and the occipital cortex, are the ones that get the lower pressure first before the heart rate catches up and gets the blood pressure going again, they're gonna get dizzy. And if they get dizzy, they may even notice some double vision or room spinning. Okay.

And if it gets high enough to the occipital cortex, they're gonna notice a coning down or a dimming down of vision because the occipital cortex is your visual world. That's what you see with. The peripheral vision is more anterior, the central vision is more posterior, and the posterior vision has a secondary and maybe even possibly a tertiary emergency blood flow system so that they lose peripheral vision before they lose central vision. And then as the vision gets better, the vision cones back up. And this could take place over seconds or minutes. That's what they see if it's a posterior circulation.

If it's the anterior circulation, then they might see things like dimming of vision or flashing lights or things like that. So that's harder to be sure of because then we don't know, but that's what they see.

As a neuro-ophthalmologist, my job is to listen to the patient, believe the patient, listen to their complaints and not necessarily let the patient tell me the diagnosis. And the reason I say that is I had a patient, brilliant patient, she was a nurse. She was very new. I mean, she knows EDS better than most doctors out there, I'm sure. And she came in telling me that it was her orthostasis that was causing her to have dimming of the vision in the eyes. And it happened when she stood up. And so that made perfect sense. But then I asked her a little more questions. She says, yeah, I sometimes get dimming in the vision, one or the other eye, when, just out of the blue, even if I'm not standing up.

And so I said, is that orthostasis? And she said, well, I assumed it was orthostasis. We did a workup and, you know, I looked at her and sure enough, I saw a couple of Hollenhorst plaques in her retinas, which means that she was throwing microemboli. They were coming from a carotid, it turns out. Little, you know, calcific plaques of cholesterol and gunk. That's when it shows up in the retina, it gets stuck in the retina. Those are these little bright golden specks of gold stuck in an artery. Now you would never see it without an ophthalmoscope or without, you know, a slit-lamp exam.

And she didn't know she had that. She also had mid-peripheral hemorrhages, which are little dot hemorrhages in the retinal periphery, which is a hallmark of carotid disease. And so she definitely had orthostasis. There's no question she had orthostasis. She knew that she was right. She just happened to have, on top of orthostasis, she also was having TIAs from carotid emboli. And they catheter-rooted her out, which was another story because unfortunately, you know, any surgery in an EDS patient becomes a little more risky and a little more worrisome, right? But I'm, you know, thank God we caught that.

So that's the kind of thing. So I believe the patient, I listen to the patient, I tell everything the patient says to me is important. But it's my job to say, wait a second, is there anything not fitting in this picture so that I can make sure we don't miss something?

And yes, EDS and POTS and orthostasis does cause vision changes, but you got to make sure it's not a huge constellation of changes. It's fairly symmetric changes. It's usually both eyes at the same time. If it's only one eye or the other, that's a red flag for a problem in circulation. And it's usually, as I said, I see more dizziness and dimming of vision than I do anything else.

[30:11] Dr. Linda Bluestein: Well, thank you for that great example. That's something that I talk to people about a lot. If you label your symptoms too early on, then oftentimes we stop looking for answers. We stop thinking about the differential diagnosis. So I'm really glad that you pointed that out because as Dr. Singman pointed out in one of our first conversations, we talked about Occam's razor, but also Hickam's dictum, did I say that right? Where people can have lots of different things going on, especially this population of people. So we don't want to be premature in concluding that all of a person's symptoms are coming from this one particular problem because then we might miss something. So thank you for sharing that.

Dr. Eric Singman: Absolutely.

[30:51] Dr. Linda Bluestein: Fabulous example of that. We're gonna take a quick break. When we come back, we're going to talk about intracranial pressure, CSF leak, and we also have a couple listener questions that we want to be sure to get to. So we're gonna take a quick break and we'll be right back.

[33:10] Dr. Linda Bluestein: We're back with Dr. Singman, and I believe Dr. Knight has some questions for you.

[33:14] Dr. Dacre Knight: So yes, this is really fascinating to me when we start putting all these pieces of the puzzle together from orthostatic intolerance to convergence, accommodation, and all the symptoms and conditions that can go together with EDS related to eyes and neurologic function combined. So one of the other areas too, and we had a recent excellent episode on CSF leaks and EDS, but I think when we tie into that, we would also tie into intracranial pressure, which comes up very often when we talk about neuro-ophthalmology. So I wonder if we can just dig into that a little bit. I mean, first off, we did kind of overview, you know, related to low pressure headaches and things like that that may happen with CSF leaks. But from a very kind of high-level general standpoint, what would be the symptom pattern you may suggest needs to be looked for when we're talking about high or low pressure issues related to CSF?

[34:21] Dr. Eric Singman: So, we get patients who have high pressure, we get patients with low pressure, and sadly, and probably more in the EDS population, we get patients who get both. So the classic IIH patient, the classic intracranial hypertension patient who doesn't have, thank God, a brain tumor or anything like that, is going to be, stereotypically, a young woman of childbearing age with elevated body mass. And we don't know why that causes abnormal CSF hydrodynamics, but it does.

And they come in with, usually, headaches. The headaches usually are worse lying down than up, so the headaches are worst first thing in the morning. Headaches are usually 24/7. They can get a little less as the day goes on because CSF can drain down to the spinal cord. They often have what's called pulse-synchronous tinnitus—also some people call it pulsatile tinnitus—but the correct term is pulse-synchronous tinnitus, where they hear whooshing in their ear. Usually it goes along with their pulse, so it's usually the right ear more than the left. And they also can complain of reduced vision. They complain often that their vision goes dim when they bend over and they—I had one patient tell me she assumed that was her POTS and that was a good thought.

You know, but when you bend over, you would think you'd have increased pressure, increased blood flow to the brain, not decreased blood flow. When I said that to her, she said, oh, okay then. And sure enough, she had marked papilledema, marked swelling of the optic nerves.

So that's the classic high-pressure person. These patients often can also have any cranial neuropathy. They can get a Bell's palsy, believe it or not. That could be elevated CSF pressure, and you need to look for papilledema in those patients. If someone has a bilateral 6th nerve palsy where their eyes cross, or even a 4th, any cranial nerve palsy—so we look for papilledema in those patients. So that's the high-pressure folks.

The low-pressure folks with the spontaneous CSF leaks, or the CSF leak after trauma, or the CSF leak after spinal tap—whatever the reason—those folks are going to feel the best lying flat or even in Trendelenburg position. That's where the bed is slightly slanted, the head's down, the feet are up. That's how they're going to feel the very best. When they get up, they're going to feel miserable. They're going to feel nauseous. They're going to feel headache. They're going to feel out of it. It's a terrible feeling—that's the classic post-LP headache that everyone knows about or may have heard about. And those folks will give you that very different history, very different headache constellation, very different headache environment.

And so that's one way you can tell the difference. Now, then you get the people in between—the people in between are those folks, like I said, often with EDS, where they have elevated brain pressure, but because they have some weakness somewhere in the meningeal system, they can blow and suddenly get a spontaneous CSF leak with either otorrhea—fluid coming out the ear—or fluid coming out the nose, and that's CSF fluid. And they might think it's just a runny nose, for example. Obviously, there are tests we can do looking for that—the beta-2-transferrin test. But the bottom line is that those are the ones who say, and when you speak to them about that, you want to ask them, you know, "How did you feel when you started having that runny nose?" And if they say, "You know, it's funny, my headache got a little better," say, well, that's an important finding because why would your headache get better unless you had high pressure in the first place?

Now, some people get spontaneous leaks without high pressure. So you know, that's it. There are obviously, in either case, if you suspect the CSF leak or you suspect, or you see papilledema from an elevated pressure, you have to work that up. So we do an MRI with contrast.

[38:37] Dr. Dacre Knight: So, and related to that point, when you see papilledema, would you see it on an eye exam every time? And what if you don't see it? And does that mean you need to do a lumbar puncture? And is that going to then be more definitive?

[38:58] Dr. Eric Singman: I tend to be somewhat hesitant with lumbar punctures, only because of the fact that in an EDS patient especially, I could end up with a CSF leak. Now, I'm not saying they're wrong or bad, and they definitely have a place. In fact, I had a patient with elevated brain pressure. They did a lumbar puncture, and the lumbar puncture itself cured her because it left her with a CSF leak that let the pressure stay low or lowered. So she had a CSF leak, and it was a godsend because she was pregnant. I couldn't use any medications. I couldn't use radiation, you know, for X-rays or whatever. So the lumbar puncture was really heaven-sent. But in general, what I do with these patients is, if I see papilledema, then, and I see no brain tumor, I'm pretty sure where we're going with this, to treat the elevated brain pressure, either medically, surgically, or some other way, or a combination.

If I don't see papilledema, I often might still do some other tests. For example, I might get a sonogram of the optic nerves to see if there's fluid behind the nerves, because some of that can show up. I look to see if there are what are called spontaneous venous pulsations at the nerve. That's when you look with a retinoscope—it's a device we use to look in the eye—and you see the vein at the back there pulsate. Most people, 80% of people, have these pretty obviously. If someone was known to have them—because I try to mark it on all my patients whether they're normal or not—if I say I knew this person had SVPs, I marked into my chart a year ago, and now they're coming with headache and they don't have SVPs anymore, I'm going to ask myself, something's going on here.

So sometimes it can be a finding as subtle as that. Furthermore, if they do an MRI and I see some of the hallmark findings of elevated brain pressure, like what's called an empty sella, or if I may see on an MRV narrowed or stenotic venous sinus system, or if I see fluid-bathed optic nerves—I don't make the diagnosis from that, but I'll use those in my thought process.

And ultimately, even before getting a spinal tap for confirmation, I might do something else. I might give a therapeutic trial of the medication that lowers brain pressure, such as Diamox, if they can tolerate it. It's a very unpleasant medication. Most people, I tell people upfront, to kind of lower their expectations in terms of how pleasant it is to take.

And if the medication—the patient says, "Doc, I tell you, I hate this medication. Funny taste in my mouth, the tingling in my fingers, I can't stand it, but it did make my whooshing go away and it did make my headache go away." I'm not going to do a spinal tap on that patient. I have the information I need to work with them.

As far as a low-pressure patient might go, those can be harder. There are generally no ophthalmic findings with low or subnormal intracranial pressure. On the other hand, there are findings with MRI with contrast. The meninges light up pretty well with that, and you might see the cerebellar tonsils sink down in the foramen magnum. For the audience, the cerebellum has these two extensions. They're called tonsils. These are not the tonsils that you swallow with. They're just parts of the brain, and they can fall. They can go below the hole at the base of the brain called the foramen magnum. That's cerebellar tonsillar descent.

The problem with that particular finding is you can see it with high pressure and with low pressure. And you can see it with Chiari malformation also. In fact, that's the definition of Chiari 1 malformation, which a lot of EDS patients get. So when I see that, that adds another complexity.

But if I see someone has classic low pressure, then what we do is I usually send them to neurosurgery for sure. Because they try to find where the leak is and try to stop it up.

[42:43] Dr. Dacre Knight: So just a very quick follow-up before I want to talk about neck and cervical spine issues too that may relate to some of this. You had mentioned the trial of Diamox and patient has, you know, problematic side effects, but they have otherwise improvements, which is kind of a telltale sign. What do you do then? Do you continue them on the Diamox or do you switch to something else better tolerated, or do you just kind of have that discussion with them?

[43:09] Dr. Eric Singman: So first I try to see how much Diamox I need, and if it works well, I ask them, what part of it do you hate? If they say, I hate the tingling, I hate the electricity feeling—they're called paresthesias—I hate the paresthesias, I say, those eventually go away. Let's see if you can handle it. If they say, I hate the fact that I gotta pee every 5 minutes, I say, well, you gotta drink a lot of water and you're gonna pee every 5 minutes and we're gonna hire a porta-potty for you if we have to. But okay. If they have kidney stones, I might limit the Diamox because that can worsen kidney stones. If they hate the funny taste in their mouth, which comes with soda, I say, well, guess what? You're not gonna drink soda anymore. And sometimes you gotta be—it's a little bit of tough love and I get that.

If on the other hand, it's the nausea and the just feeling like terrible, weak, then I have to think of something else. And again, it depends on the dosage. I might go to Topamax, topiramate. That works pretty well too, and that doesn't have that same side effect profile, although it can—you know, the kidney stone issue is still a real issue—but it does otherwise have a less side effect profile.

The way that we talked about before, prism with double vision is a band-aid. Diamox and these other medications for elevated brain pressure are a total band-aid. They are not the cure, and I don't like using them for long-term therapy. I disagree with their use as long-term therapy. They are a temporizing measure to protect the optic nerves if they're swollen, and they're a temporizing measure to give the patient some relief from headache and their pulsatile tinnitus.

Other than that, I take these patients and say, look, we have a couple of options here. The medication is to protect your nerves and make you feel better. It's not the cure. If this patient has a markedly elevated BMI, and we're going to lower that BMI. I've had patients who've gone on some of these new GLP-1 drugs that literally turned their life around. And it has been amazing. I've had patients who tried them and they didn't do so well with them. That's every drug, right?

I've had patients who went to a gym and said, I'm going to do this on my own without drugs. And some of them have been fabulous with it. Great. So if the weight loss can happen, I'll use the Diamox. If I see the weight loss is a real interest and it's possible. If these people say, on the other hand, look, doc, I tried everything. The weight loss just isn't working for me. Then I'm going to go and talk to my neurosurgery colleagues and say, I got someone who's a candidate for a stent.

If the MRV, if the magnetic resonance venogram shows that there's narrowing, or if they have an interventional radiology procedure called manometry, where they go up into the venous system and they show that there's a substantial pressure differential across the venous sinus from one portion to another, that person's a candidate for stenting those venous sinuses. And those persons in our clinic at University of Maryland, we have a wonderful doctor, Jacob Cherian, who's just gifted. And he works with a nurse, Betsy Shearer, and some other people. These people are absolutely gifted. We have a stent program here that I've just—patients just come to me and say, I don't need to see you anymore. Which, and they say it so happily, it just makes me wonder a little. It makes me worry, but I'm—but they honestly do really, really well with stenting.

As you know, maybe your audience doesn't, in the past we used to do shunts where they asked you to drill a hole in the skull. And I have not requested or suggested a shunt in, I don't know, maybe 7 years. I can't think. I just avoid them. The shunts are problematic. Now, those are ventriculoperitoneal shunts. There are also lumbar peritoneal shunts. They have much less of a failure rate, I think, and they're less invasive, but they're still a shunt, still hardware.

So I've been basically, if it's neurosurgical, it's been stenting. Now, there are some patients who can't have a stent for some reason or won't have a stent, and whose optic nerves are being threatened by pressure, and we can't get the pressure down. For those patients, one option is something called an optic nerve sheath fenestration. That's a surgery where we go behind the eye and we take a little window of optic nerve sheath, which is just an extension of the meninges, because the eyes are an extension of the central nervous system. And we take that little window out and out comes the fluid. And the pressure is relieved off the nerve.

Now, not a lot of people like to do this. Some people say that's basically the last, you know, last ditch procedure, or patient has to be losing vision badly. Then there are other people who say, well, why not? And I know there's a brilliant doctor who does these really well in Florida, Dr. Thomas Spoor. He also was in Michigan, and he's just a whiz at these, and he's helped a lot of my patients. We introduced the procedure when I was in private practice in Lancaster County, and we had very good results with it. So I was very happy with it.

There are risks with every procedure, including losing vision. I mean, you're right by the optic nerve. So obviously this is not candy we're talking about. But there has to be an armamentarium of what you can do for patients with high pressure.

[48:17] Dr. Dacre Knight: Yeah, well, thank you. Very comprehensive. And I'm glad you did touch on the GLP-1 agonist because, you know, we hear and we're still learning so much about those, Ozempic and others, and related to weight loss. And that is amusing how your patients come back to you with such relief and gladness. But I think we've related in previous episodes that we talk about how Dr. Mayo once said that the aim of medicine is to prevent disease and prolong life, while the ideal is to eliminate the need of a physician altogether. So right, there you go.

So going back—I mentioned I wanted to talk about you brought it up earlier, talking about the beautiful necks of EDS patients. So here we are talking about now things like cervical spine instability or CCI. You touched on Chiari and things like that. But I just want to kind of add that variable into the mix here and what may change some of the symptomatology that you would find if it's, you know, we kind of worked our way downward, I guess, starting from the eyes and the retina, going back to the tonsils and then further down into the cervical spine. Where do all those symptoms change in that mix?

[49:36] Dr. Eric Singman: So, as I said, you know, the Achilles heel of Ehlers-Danlos patients are their necks. When they have a bad neck, and I get this with my traumatic brain injury patients too, because you can't hurt your brain traumatically without getting neck trauma. They're connected. I just have never seen it personally. You always have some degree of neck trauma.

When the necks are bad, I don't see things getting better. A patient comes to me with visual problems and I ask them, how's your neck? And they say, my neck is horrible. I tell them, I'm going to be very straight with you. I don't think I'm going to be able to get much done, in terms of, let's say, using orthoptic therapy at home or some home exercises on a computer or something for some of the vision problems we see, because that's all they really need. They don't need in-office therapy, in my experience.

I say, I'm not even going to try it with you. I mean, if you want to, go ahead. I'm not going to stop you, but it's not really expensive. You want to fool around with it, but in my experience, when the neck is bad, I just don't get things done.

And, you know, my theory as to why that's the case—and I was just telling the sweet lady who called me today on the phone after seeing your podcast—was that your eye's job is to immediately look at things no more than 15 degrees at most off axis. So if I want to look from, let's say I'm looking at an image of Linda and then I want to look to Debra, that change is enough that I actually see my chin go up and I'm looking. Literally I'm moving my eyes inches. Hey, that's how your brain works. Your brain depends on your eyes to lock onto a target after your neck and head move, the eyes into the correct location. And your eyes do the very fine locking on, very fine tuning.

When someone's neck is bad, consciously or subconsciously or both, they don't want to move their neck or they can't move their neck. And so they're going to try to move their eyes more than they should. As I said before, it's like having a racehorse pull a plow. It's not their job to do that. And the eyes are going to be uncomfortable. The vision's going to be uncomfortable. They're going to have eye strain. They're going to have headaches. And literally, I feel like there's nothing I can do about it because you're asking your eyes to do things that are not their job.

[52:02] Dr. Linda Bluestein: Okay. Well, that's really, really helpful. There's so many things that can go wrong. It's just amazing that people can manage as well as they can, fair bit of the time. We don't have a ton of time left, so I want to get to some of the top red flags that we should be aware of when a person might be getting taken advantage of. You talked about this person at the beginning and you made some reference to the dark side. How does a person know that they're being sold snake oil? What are some things that they should be looking for?

[52:35] Dr. Eric Singman: If the doctor says, if the doctor doesn't ask why the problem is there, if the doctor doesn't explore why the problem is there, if the doctor simply takes the patient's word for it—and I'm not saying doctors shouldn't listen and believe the patient, of course—but if they simply take the patient's word for it without thinking along a differential diagnosis, without making sure there's nothing else underlying, that's a red flag.

If a doctor says, if they only have a hammer and treat every problem like a nail, that's a problem. So if you have a doctor who says, you know, like this woman who called me, who listened to me, she went to a doctor and the doctor gave her prism. I said, well, why did you have—did he ask why you had the double vision? Well, I told him it was after this thing that happened to me and he took it for granted. And I said, but the thing that happened to you, it doesn't make any sense that it would cause double vision. So there must be something else. So he didn't explore that. And that really scared me because double vision could be, as I said, something dangerous.

So one thing I look for is if the doctor doesn't really find out why or really ensure the reason why. Second thing is I look at the doctor's credentials. And physicians take a Hippocratic oath to do no harm. And in my opinion, that includes no harm to the pocket. Okay, because that's harm too. And so if I see someone who says, you can come to me for this therapy, it costs thousands of dollars and you have to buy your stuff from me and et cetera, et cetera, et cetera. And I'm going to say, wait a second, wait, slow down, back the truck up. I got a problem with this.

If something has to pass the sniff test. Patients who are desperate and in pain oftentimes won't take time to do the sniff test because they want to get better. And you cannot blame these people. I would never blame them. You have to empathize with them. But by the same token, you also want them to not go down a garden path and something that's dangerous, as you said before, Dr. Bluestein. And so what happens is I—so red flag is it's going to cost a lot of money and insurance doesn't cover it.

Insurance covers a lot of stuff. It just has to have a medical sound reason for it. I do peer-to-peer review work for utilization review, and I also do peer-to-peer work on behalf of my patients. And I can tell you right now that most insurance companies have guidelines that make it very clear what they cover and why. And for the most part, those guidelines are fairly reasonable. Some of the guidelines I think are a little strict. They're a little always late to get into things. Like I have a patient who really needs GLP-1 to lose weight because it will help her with her elevated brain pressure. And I have to do a peer-to-peer explaining why it's not just cosmetic, it's therapeutic. But I get that.

2 years from now, I will never have to make that call. It's going to be accepted because elevated body mass causes lots of disease, right? So, but right now, it's the same thing. You know, Medicare has an inpatient-only list of things that you could do surgery on, what has to be done inpatient. That list is going to go away because everything's becoming outpatient. But right now there are still procedures that they say should be inpatient. I get that. As we get better. So insurance is slow to catch on, but they do catch on.

So the fact that insurance has never caught on for some of these therapies, whether they're vision therapies or some of these chiropractic therapies that I see, the fact that they've never caught on, despite the fact that these therapies have been offered for years, says to me we're missing the clinical data showing these therapies are valuable. And patients therefore should ask themselves, well, why isn't this covered? At the very least, the patient should ask the doctor, how long has this therapy been around? And if the doctor says, oh, it's been around for decades and the insurance doesn't cover it, say, well, why haven't they covered it? Because they're mean, bad people, insurance companies. Okay. That's one possibility, but it's usually not the right one.

[56:47] Dr. Linda Bluestein: Well, that's super helpful. And I want to ask about mast cell activation and inflammation in the eyes, and then we'll move on to the listener questions before we wrap up. What should we know about mast cell activation when it comes to the eyes?

[57:02] Dr. Eric Singman: Mast cell activation patients are really sensitive to all medicines, number one. So I'm very careful to try to use, if I have to use eye drops in these patients, I try to use preservative-free ones because I just try to lower the amount of different chemicals that mast cell patients get. I just make it my habit and custom to do that.

Second, mast cell patients, because mast cells are involved in allergy and inflammation, mast cell eye patients routinely seem to have dry eye. And dry eye can cause a whole host of other problems, whether it's red eye, eye discomfort, chronic low-level inflammation, blepharitis, which is inflammation of the lids. It becomes a vicious cycle, a world of its own. So I try to make sure I look for dry eye. I ask about dry eye and I try to treat dry eye. I try to treat it with non-chemical methods if I can.

But the way dry eye works is that because dry eye becomes an inflammation that causes the tear film to become a bit of a witch's brew that causes its own inflammation. My first order of business is to put out the fire with anti-inflammatories, whether it's something like cyclosporine or other medication like Xiidra. But put out the inflammation first to improve the quality of the tear film. Once I improve the quality of tear film, then I might go to what are called punctal plugs. There's these little plastic plugs that go into nasolacrimal system. They're easy to put in. They're painless, they're safe, and that prevents the tears from draining down the natural nasolacrimal duct into the nose. That's the natural drain of the tears. By plugging the drain, I increase the volume of the tear film. And that—but I wouldn't do that until I first increase the quality of tear film, or else I'm just making the problem worse.

The other thing with mast cell patients is I routinely try chromolyn sodium eye drops, because they are mast cell degranulation inhibitors. And so those drops, in my experience, usually seem to be tolerated very well by patients and usually do a pretty good job.

If the patient has generalized MCAS, then I might try to treat their eye problems through general means rather than topical means only. And that would be like Gastrocrom—the oral sodium chromolyn. And I've had really good success with that. Sadly, the generic version has been almost impossible to find. And so only the name brand Gastrocrom has been available. The problem is I have patients where only the insurance company would cover the generic, and it's understandable. The generic is much less expensive than the name brand, but I've had to write letters to insurance companies explaining that the generic is simply unavailable. And so they have to cover it. So that's an example of where you try to be an advocate for your patients. But I've been very pleased with the oral Gastrocrom success that we've had in helping the eyes too. I still sometimes have to use topical therapy. I still sometimes have to increase the tear film. But it depends on the patient, their symptoms. If they don't have a lot of dry eye symptoms, but they have a lot of mast cell symptoms, then that's when I recommend Gastrocrom.

It's funny, I've only had to order it 5 or 6 times because usually they have either a rheumatologist or an allergist who does that for them. But some of these patients, you know, sometimes it can be a long time to get an appointment. So yeah, out of sympathy for a patient, if you know it's mast cell and you can tell it's mast cell, you give them the medication.

[1:00:46] Dr. Linda Bluestein: Yeah, it's crazy how it can be so hard to get medications nowadays that are generic. I remember when I was working in the operating room, you'd come in and say, well, what don't we have today? Is it fentanyl? Is it midazolam? Like what do you have? And you have to get creative. But I do sometimes write for compounded chromolyn, which I know can benefit people sometimes too because then they're not dealing with the plastic vials and stuff like that. But of course that can be costly as well.

I do want to move on to the listener questions because I know that they are really hoping to get a couple of answers. So the first one is from Marta and Marta says:

I am very grateful for your podcast. It gave me insight to understand struggles that no doctor earlier connected to before. I have heard you saying that it's possible to send questions to Dr. Eric Singman regarding eye issues. I'm struggling with a change that happened 5 years after LASIK eye surgery—showers of floaters and tiny floating lights that look like glitter. I'm a woman in my early 30s and I want to keep my eye health. The doctor that checked me in Lisbon said my retina detached from the vitreous, but it's stable without any breaks. Now I don't know what I can do if there's a chance for improvement. I struggle looking at the sky and my eyes are very sensitive to light. Dr. Singman recommended to watch out for hitting one's head. Maybe I don't, but I don't know if I can continue my hobbies because she does acro yoga, gymnastics, and aerial hoop. And this also includes being upside down at times and some impact. I would be very thankful if you could pass my information to the doctor or if you could let me know what I could do. I'm from Poland and I don't find any doctors with such expertise as yours. Thank you in advance.

[1:02:29] Dr. Eric Singman: She's not going to like what I have to say.

Dr. Linda Bluestein: Uh-oh.

[1:02:30] Dr. Eric Singman: I'll tell you right now. First of all, the flashes that come with a vitreous detachment, and she seems to describe that pretty well—flashes and the flashes may or may not continue. They usually do settle down some. But the fact that she has LASIK means that she probably was a high myope, which means she was very nearsighted. That's why she would have had the LASIK, probably, I presume. And patients who are nearsighted have thinner retinas and have a greater chance of retinal tears and retinal detachments.

And so, I would strongly recommend that being upside down and impact sports—anything, you know, concussion sports, impact sports—are avoided. Like I said, I hate to say it because, you know, gymnastics is such a beautiful, beautiful sport. It's flying art. I mean, to turn a human body into artwork is a beautiful thing, but I just think it would be a dangerous thing. In terms of looking at a sky, you have to be careful to separate what is normal and what's not.

If I were to have any human being look at a uniformly gray or uniformly blue sky, they will see things floating and moving because they can see their own red white blood cells going in and out of vessels. They can see their own red blood cells moving in their capillaries, and they can see the natural veils, which are like lines of hyaluronic acid that keep the vitreous gel in its place, gently floating around. So anyone will have that. So the floaters—the floater part has to become sort of an acceptance. It's hard to get, it's hard to avoid that.

As far as the flashes go, that's something that needs to be questioned. If the flashes come, let's say, just occasionally, sporadically, they probably can be ignored. If the flashes come every time someone moves their eyes, then you have to worry that there may be what's called vitreoretinal traction. And even though there's not a retinal tear at the pars plana, at the front of the eye, there could be. You have to look for that, especially someone who used to be myopic, used to be nearsighted, because a nearsighted eye is generally a longer eye and has a greater risk of retinal detachment tears.

That person should have very careful, regular retinal evaluations to make sure there are no impending tears. Now clearly, because we can see one cone or one rod activate as a flash, at the cellular level, if there's vitreoretinal traction—traction of the vitreous, because the vitreous is a jelly but it has a skin like a grape and it's attached at the eye. If there's pulling of the vitreous on the retinal periphery, even if it's not breaking it, it still can cause the light flashes. And humans can sense that even if a doctor doesn't see a retinal hole or retinal tear. But that means if it's always in the same place, then the doctor definitely wants to keep an eye on that patient to make sure they don't have some pending tear.

Otherwise, there's not always a lot to do for it. But in that, that patient is having a difficult time, and you don't want to force a patient to choose between the things they love and their health. That's a terrible choice, right? But I would be so careful about impact sports like that. I tell my Ehlers-Danlos patients, if you can stand chlorine in a pool, and many of them can't, I always advocate swimming. I know it's just the least impact. Yoga is wonderful. I don't like yoga upside down, but yoga itself is wonderful. But that I think is magnificent. A lot of patients do great. It's a great exercise. Yeah.

[1:06:18] Dr. Linda Bluestein: So long as they don't push into hyperextension, which can be really tricky because of course they can be really good at it and people ooh and ahh.

[1:06:33] Dr. Eric Singman: Because they're so flexible.

[1:06:33] Dr. Linda Bluestein: Yeah. Yeah. Okay. So the other question is: could you please ask Dr. Singman about his opinion about some rehabilitation approaches used by neuro-optometrists? I'm referring to using the Z-Bell test to remediate spatial deficiencies in non-image-forming retinal processing, and then wearing special glasses to integrate visual and sensory stimuli. I am a hypermobile individual with ADHD and sensory processing difficulties. Helping with symptoms without relying on medication for mild to moderate learning difficulties might sound very attractive, but given the price of the evaluation and treatment, I don't want to spend a fortune if this is not valid or snake oil. Thank you so much for your time. Your podcast is absolutely amazing, super helpful and informative.

Dr. Eric Singman: Snake oil. Next.

Dr. Linda Bluestein: Okay.

[1:07:16] Dr. Eric Singman: Pure, unadulterated, purified, 100% dyed-in-the-wool snake oil.

[1:07:16] Dr. Linda Bluestein: Okay, so Elizabetha, you have an answer to your question. That was snake oil. Okay, wonderful.

Um, as you know, Dr. Singman, we end every episode with a hypermobility hack. Do you happen to have one for us? You've already given us lots of tips, but do you happen to have like a quick win for us?

[1:07:42] Dr. Eric Singman: I think for this hypermobility hack, I would probably say it's okay not to have a doctor who's Ehlers-Danlos literate. Just make sure they're Ehlers-Danlos willing to learn. And most doctors are like that.

[1:07:53] Dr. Linda Bluestein: Yeah, I share that with my patients as well. I mean, it's better to have someone who is willing to learn and listen, right? Rather than just be lost on your own. And patients often find themselves in that situation where they're having to do so much on their own, and that's unfortunate.

Yeah, it is super unfortunate that they have to do so much on their own, but I do see people say things sometimes on social media. I can't believe it. My doctor didn't know how to pronounce Ehlers-Danlos. I can't believe it. I had to educate them about it. And I would say, wow, if they were listening and they were wanting to learn, I mean, we have to know about a lot of things. I think, you know, a lot of people don't realize, they're like, oh, we should spend more time in medical school on Ehlers-Danlos. Well, I, of course, I agree with that statement as somebody who's treating this population of people. But I'm sure, as both of you having been in medical education, everyone's competing for that time with the medical students, and there are just so many things to cover and not enough time to cover it.

So I think if you have somebody who's—I know, I know, Dr. Singman, you've said this multiple times in your lectures, and I've said similar things—if you have a doctor who's curious and willing to learn and empathetic, that goes a really, really long way.

[1:09:03] Dr. Eric Singman: Well, I learned a lot from Dr. Singman today, so I'm very grateful. Thank you.

Dr. Linda Bluestein: Before we close, Dr. Singman, can you just remind us where we can learn more about you and/or where people can get in touch with you?

[1:09:15] Dr. Eric Singman: Yeah, like I said, if you want to, you know, they can—my website is through the University of Maryland. They can look on that site there. And as I said, you know, if patients call me or they can email me with questions, because I got a lot of calls since the last podcast. From the nicest people in the world, just wonderful people. And, you know, as I said, I'm happy to help. Give a little bit of guidance. But most people, I think most of my Ehlers-Danlos patients have shown that they have the resilience, the intelligence, and the savvy to trust their gut and recognize that something is not right.

But if they need a little bit of extra guidance of, you know, where to go with this, they're always welcome to. They have my email and my cell phone. That's fine.

[1:10:20] Dr. Linda Bluestein: Okay. That's so generous of you. I whenever people do that, I always wonder what's going to happen if their phone's going to blow up or what. So well, thank you so much for taking the time to chat with me again today, and I'm so glad that Dr. Knight was able to join us as well. I'm sure that this conversation is going to be so appreciated by all of the Bendy Bodies listeners, and really just appreciate you both.

Dr. Eric Singman: Always a pleasure.

[1:10:32] Dr. Dacre Knight: Thank you so much.

[1:11:35] Dr. Linda Bluestein: Thank you so much for listening to this week's episode of the Bendy Bodies Podcast. If you'd like to go deeper, I share additional education, clinical insights, and resources in my newsletter, The Bendy Bulletin, which you can find on Substack at hypermobilitymd.substack.com. You can also help us spread the word about connective tissue disorders by leaving a review, sharing this episode, or sending it to someone who needs it. These small actions truly make a difference in raising awareness about conditions that are still widely misunderstood.

And don't forget, full video episodes are available every week on YouTube at Bendy Bodies Podcast. As many of you know, I offer one-on-one coaching and mentorship for both individuals living with connective tissue disorders and people caring for them. You can learn more about these options on the services page at hypermobilitymd.com. You can find me, Dr. Linda Bluestein, on Instagram, Facebook, TikTok, X, and LinkedIn, all at Hypermobility MD.

As part of our collaboration with the UVA Ehlers-Danlos Syndrome Center, we also want to share some of their helpful resources. For questions or appointment inquiries, you can contact the UVA EDS Center at [email protected]. Again, that's the letter R as in Robert, UVA EDS Center at uvahealth.org. You can find answers to common questions at uvahealth.com/support/eds/faq.

Our incredible production team is Human Content. You can find them on TikTok and Instagram at Human Content Pods. As you know, we love bringing on guests with unique perspectives to share. However, these unscripted discussions do not reflect the views or opinions held by me or the Bendy Bodies team. Although we may share healthcare perspectives on the podcast, no statements made on Bendy Bodies should be considered medical advice. Please always consult a qualified healthcare provider regarding your own care.

For more information about the Bendy Bodies program disclaimer and ethics policy, submission verification and licensing terms, HIPAA release terms, or to get in touch with us, 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.