Day 4: Four Facts About EDS
So listen. I was going to do a whole different thing than what I put on instagram, but then I put in a lot of effort into creating infographics for instagram, sooooooo...we're going to piggyback off of those fun little babies because I have them and they are pretty. #sorrynotsorry
We're just going to dive into each of these facts and go more in-depth than instagram allows. Let's get into it, shall we?
A quick note before we begin- since these are images with text on them and Wix isn't the best about image descriptions, I'll be pasting any text from them within the body of the post before going into greater detail. Because, you know, accessibility is important. Just in case there's anyone who doesn't know how that sort of thing can work, here you go! In this post, it'll always be the first bit of text after an image, within parentheses, prefaced by "IMAGE TEXT:" so it should be easy to differentiate between what's on the image and what's not. As a blanket image description, each of these slides is square with an array of light pink hues with with abstract pink designs and black text.
(IMAGE TEXT: DID YOU KNOW?
FOUR FACTS ABOUT EDS
Fact 1: It's all about that collagen, baby
Fact 2: EDS is highly variable
Fact 3: Diagnosis can take decades
Fact 4: Treatment? Kinda! Cure? nope!)
(IMAGE TEXT: Fact 1: The Collagen's the Thing!
The Ehlers-Danlos syndromes are a collection of heritable connective tissue disorders that alter collagen production.
Since humans have collagen in every part of their bodies (think: skin, bones, gums, organs, blood vessels, eyes, ligaments, etc.), every single body part can be affected by EDS.)
A lot of people are under the misconception that EDS is just an orthopedic condition. I say people, and I guess I do partially mean us regular people, but I'm really talking about doctors here. I've lost count of the number of doctors I've had to explain this to. EDS is a multisystemic condition. We've got collagen everywhere, there's no way around it. Is collagen exactly the same in every body part? No, but there are also 14 subtypes of EDS in which different systems are affected more or less than others, so that's not a great point. The main thing is this- if it were just an orthopedic issue, that would mean it only had to do with our joints. Mmk. So. If I could just tell my body to only create faulty collagen in my joints and normal collagen everywhere else, wow I'd be living a very different life. Think of all the things I could accomplish with that kind of talent! Do I sound sincere yet?
I don't have any problem with ordinary people not knowing about this. I don't have any problem with EDS patients not knowing about this. But doctors? I have a big problem with doctors STILL not knowing about this. Because the medical professionals I've come across who know absolutely nothing about EDS, who've never heard of it, are usually pretty quick to look it up and at least learn the bare minimum. But it's the ones who think they know, the ones who got one lecture about it 40 years ago and haven't thought about it since but are somehow still convinced they know everything, who are the most dangerous. Those are the ones who still say "it's just an orthopedic disease" and refuse treatment. To be clear, even "just" the orthopedic symptoms are bad enough. But to dismiss the rest of it tells me that not only do you not care about your patients, you don't care about science. And that's not what anyone should want in a doctor.
(IMAGE TEXT: Wait- So What's the Big Deal About this Collagen Stuff?
Think of it like glue. You know when kids build little houses out of popsicle sticks and glue? But sometimes maybe the glue is a little past its prime or the lid has been left off, and the glue just isn't sticky anymore?
No matter how much of the old, nonsticky glue that kid puts on, there's no way that house is going to stand up.
Our collagen works the same way. It's just not structured right. So it'll stretch or tear, but it can't provide the structural integrity that our popsicle-stick-house-bodies need.)
This is my favorite way to explain it. Maybe it reminds me of working with elementary schoolers. But it's also pretty accurate. A lot of people ask whether taking collagen supplements would help, and the answer is, unfortunately, not really. When you take a collagen supplement, your body doesn't use it immediately. It doesn't exist in a form that is useful to humans. So when you take it, your body has to absorb it, break it down, and build it back up into something it can use. For most people, that's great! But for people with EDS, our bodies break it down and build it back up the same way we build up any collagen. So we do have more of it. But it's more of the same faulty collagen that isn't really useful. More of that same nonsticky glue.
(IMAGE TEXT: Fact 2: EDS is Highly Variable
While there can be many similarities and overlaps between subtypes, there is also a ton of variation. That means not only is there variation between subtypes, there is also variation within the same subtype- especially hEDS. No two cases are the same.)
Let's disregard hEDS for just a second. That leaves us with 13 different subtypes (including the one that hasn't been classified yet). Some of those are extremely rare and specific. Periodontal Ehlers-Danlos syndrome (pEDS), for example, is the rarest type and predominantly manifests as severe gum inflammation that can result in loss of teeth before the age of 30. That is entirely different than vascular Ehlers-Danlos syndrome (vEDS), which we'll talk about more later in the month, which puts patients at great risk for things like arterial rupture at a young age.
To shake things up even more, let's throw hypermobile Ehlers-Danlos syndrome (hEDS) back in the mix. It's the most common type, probably affecting as many as 1 in 5,000 people.
*As a side note, I have a feeling that what we currently call hEDS is going to be further divided into other subtypes or other conditions altogether sometime in the future. Possibly the very near future. This is not based on any medical data or scientific evidence, just observation. Looking back at research trends and how classification has split in the past, knowing that multiple genes are being identified right now, seeing the massive amount of variation between patients and she sheer number of people who have it, everything is just raising a little flag in the back of my mind that says further classifications changes may be coming soon.*
With a condition as common and nebulous as EDS, it's expected that there's going to be some variation. There's a degree to which some people are disabled or the severity of the symptoms they experience (which is not at all to say that anyone has an easier time with this condition than anyone else; we don't play the suffering olympics here). Sometimes Person A will have horrible GI symptoms and POTS but has never experienced a full dislocation (yet), while Person B has a shoulder dislocation every week, a hernia, and an extensive family history of EDS, but hasn't had many other symptoms that they can connect to the condition yet. They both have diagnoses, they just have very different experiences.
You'll notice I said "yet" a couple of times. That's because...
(IMAGE TEXT: Fact 2: No Really, Very Highly Variable
Additionally, can be a surprising amount of variation in a single person. A case can progress over a matter of months or years; symptoms can appear *or* disappear over time. There can also be variation in one person on a day-to-day basis, or even hour-to-hour.)
One case can change drastically over time. Person A from the above example could experience a full dislocation tomorrow. A new symptom could pop up at any time. No one seems to know why it happens, and when most people ask their doctors the response they get is a shrug and, "well, EDS is weird." You can develop a new comorbidity overnight or it can happen gradually. Or sometimes things just...magically get better?
One of the trickiest things to navigate is the everyday variation. One day you can feel fine and then the next three days you can't get out of bed. Even if you've been able to cope with that yourself, it's a lot for other people to grasp sometimes. If you know someone with a chronic illness, please believe them if they say they don't feel well. Even if you saw them yesterday and they were going on a hike and looked spectacular. Even if you saw pictures of them at a party last night on instagram. Even if you saw them that morning at brunch. Chronic conditions like these can change in a second. We have a lot of practice masking when we don't feel well in order to not inconvenience other people. It's possible your friend really was feeling great at brunch! It's also possible they had an occipital neuralgia flare and felt, quite literally, like they were being stabbed in the base of the skull with an ice pick every thirty seconds (oh no, am I speaking from personal experience again?). I promise, you would not have been able to tell the difference. I also promise, if they tell you they don't feel well, they DEFINITELY DON'T FEEL WELL.
(IMAGE TEXT:Fact 3: Diagnosis Can Take Literal Decades
Some cases are caught early, but the majority go years without diagnosis or treatment. Many people aren't diagnosed until well into their adult lives, some in their 40s or above.
BIPOC, especially Black people, go a much longer time without receiving diagnoses. This is due to both medical racism and misconceptions about EDS. Often, medical professionals do not look for bruising that may not be as immediately visible on skin darker than ivory, for example. There's also that persisting myth that Black people feel less pain than others, and given that pain is a major symptom of EDS, overlooking or dismissing it can unnecessarily lead to prolonged periods of misdiagnosis or lack of diagnosis altogether.)
One reason it can be difficult to get a diagnosis when you're young is that you just haven't had enough life experience to have had the "correct" symptoms to qualify for a diagnosis. The current hEDS diagnostic criteria, while a massive improvement on what existed before (which was possibly nothing?), is deeply flawed. There is a strict set of criteria, starting with a set of joint hypermobility measurements for a very small group of joints, ignoring hypermobility in any others. I haven't mentioned the Beighton Score here before now because I hate it. I understand it. Like, I get its need and purpose. But I hate it. I also know that the diagnostic criteria is being edited and genes are being identified, and the current criteria isn't a forever thing it's just a "best we have right now" thing, which is honestly pretty good. But thinking about all of the people who slip through the cracks because they haven't experienced those things yet and won't be reconsidered if they do experience them because doctors don't seem to understand how it works, makes me incredibly upset.
And that's not even getting into the biases inherent in the medical system. The US healthcare system is racist. It is sexist. It is fatphobic. It is transphobic. It is homophobic. It is classist. It is ageist. I have been yelling about this shit for years and I will not stop. It is an especially big problem within the world of EDS. There's this picture of the typical person with EDS and it looks like...well, me. Thin, young, cis, (very) white woman. And that's bullshit. From the first symptoms I remember, it took more than 20 years for me to get a diagnosis. And I look exactly like what doctors picture when they think about EDS. And I had the time and means to pursue a diagnosis. And I had the privilege to be annoying about it. What the fuck does that say about everyone else's chances?
I feel like a broken record saying this, but we're going to talk about all of this later in the month. I have a lot to say about diagnostics. Like. A lot.
(IMAGE TEXT: Fact 4: "The Cure" is for Music, Not for EDS (Yet)
As of now, there is now cure. There is also no direct treatment.
But that doesn't mean there's no hope!
Symptoms and comorbidities can be treated as they show up. And with the knowledge that we have now and all of the advances medicine has made, at least some predictions about potential treatments can be made for certain patients in advance.
As long as science and research keeps progressing more and more strides will continue to be made to help us in the future.)
I was going to put a whole bunch of The Cure puns in here, but I just don't have the energy. I'd apologize, but I'm almost positive that no one but me would've been happy about that anyway. So...you're welcome?
Anyway! No, there is no cure. There is no treatment. There isn't really anything you can do about your collagen being uncooperative. But there are a lot of resources out there (my favorite is The Ehlers-Danlos Society, in case you couldn't tell by the hundreds of times I've linked to them by now). And as we move forward, there are only going to be more. So I think it's only going to be better and better. I don't necessarily think there's going to be a cure, at least not for a very long time, but I do think there's going to be more comprehensive treatment. And I think that as more genes are discovered, treatment processes can get more streamlined. Maybe we'll be better able to predict the course of this highly variable illness within any given individual. It's a nice thought, in any case.
(IMAGE TEXT: Thank you for reading!)
Whew! If you've made it this far, I truly appreciate it. Thank you so much, really. I hope you got something out of it, even if it was just enjoyment. I certainly enjoyed writing it! Thanks again, and I hope you come back tomorrow. And then maybe the next day? Bring your friends! It'll be a...party? Well, it'll be a something!