Hello and welcome to episode 5 of the podcast Tips and Tricks on How to Be Sick.
I’m Eirenne and I will be your host as we talk about navigating the world as people with chronic illnesses, both physical and mental, disabilities, and the ways in which the world isn’t necessarily designed for us.
In this episode, we are going to be talking about Ehlers-Danlos Syndrome, or EDS. There are many types of EDS, but they are considered to be the same family of disorders because they all stem from the same cause: improperly made collagen in the body. Collagen is a protein that forms the latticework that everything in the body gets built upon, but it’s not all the same. There’s collagen in your skin, but it’s different from the collagen in the cartilage of your knees, which is different from the collagen in, say, a placenta.
There’s like 28 different type of collagen across several different genes, and each one has a different effect if there’s an error in the genetic code, resulting in a wide range of possible symptoms for people with some form of EDS.
These can range from relatively mild symptoms like smooth, baby-soft skin that might be extremely stretchy in some people, or easy bruising, or odd scarring, or joints that move too far or in directions they’re not supposed to (this is called ‘hypermobility’) or that dislocate completely or partially, sometimes without any kind of trauma or injury (a partial dislocation is called a ‘subluxation’).
To potentially life-threatening things, like heart and blood vessel weakness that can cause sudden, early death, or instability of the skull on the cervical spine, which can result in the brain actually sliding out of position. Please note that I’m using “mild” here as an indicator of “probably not going to require immediate medical intervention to save the person’s life” and not to say that the symptoms aren’t bothersome or painful or life-changing in many ways.
That huge range of potential problems is why this is considered a “diagnosis of exclusion” — meaning, a doctor needs to evaluate you and determine that your symptoms aren’t being caused by something else first, because having a correct diagnosis is critical to getting the right kinds of monitoring and treatment.
“But Eirenne, I have some of those symptoms; are you saying I can’t say that I have them?”
Nope, not at all! You can absolutely identify if your joints are moving more than they’re supposed to, or if your skin stretches more than most people, or if you bruise a lot, and you should feel free to use any tips or tricks we offer for EDS that you think will help.
But, just know that those symptoms can be caused by other things besides EDS, and it’s important to find out what’s really going on, so you can know how to monitor it and how to treat it. Remember how I said there are different types of Ehlers-Danlos? Each type has a set of common symptoms, some of which show up in some types, but not others. If you look at a list of symptoms and assume that you have one type of EDS because of it, you might be missing another type that could have the some of the same markers, but that needs a different kind of treatment or monitoring test.
“But what about blood tests? Can’t they just test for genetic stuff?”
Well, yes and no. They can run genetic tests — either blood tests or tissue biopsies — but they will only be helpful in some cases. Why? Because not all the genes that could be involved in EDS have been mapped yet. In fact, the type of EDS that I have, Hypermobility type (hEDS), is one of the types without a definitive genetic test available for it. There are tests for the more severe types, the ones my geneticist called “lifespan shortening variants.” That’s going to be stuff like Vascular type (vEDS), that can cause very serious conditions like spontaneous aortic dissection, where the biggest artery in the body literally pulls away and detaches from the heart.
For folks with that sort of serious risk, there are different routine monitoring tests that need to be done (or done more often) to find any problems as early as possible, because it’s pretty much always easier to successfully treat something the faster you find it, before it gets worse — or before it’s too late.
Basically, you know your symptoms and your body, but you probably don’t know all the possible causes of those symptoms, so only a specialist can give you a definitive diagnosis of Ehlers-Danlos Syndrome. By “specialist” I mean a geneticist or a specially-trained rheumatologist. This is a rare disease, and it can be hard to find doctors who know a lot about it. Most of my doctors only vaguely recognized the name when I was first diagnosed, and I see doctors at a major university research hospital! There’s a theory that this is more common than previously thought, because it’s hard to recognize, so there’s a push to get primary care doctors to identify the symptoms so patients can be referred to the right specialists, but it’s still not well known. If you suspect this might be a possibility for you, you could try my approach: print out the [NIH summary of EDS] and physically hand it to your doctor.
And, at least in the US, it can be an insurance nightmare if you don’t get a diagnosis from the right kind of doctor. Your orthopaedic specialist, your cardiologist, the nurse practitioner in your primary care clinic — these all might recognize some signs of EDS, but none of them can give you a diagnosis, because none of them will have the broad, specialized training necessary.
A production note before we dive in: the audio will fade out or become slightly garbled in several places during this episode. I apologize for the poor audio and would like to remind everyone that there is a full transcript available for any sections you find difficult to understand in the audio.
So, now that everyone knows what we’re talking about, let’s get to some tips and tricks for dealing with EDS.
So this is gonna be a little different than our normal interview style, we’re going to have a chat with Laura Henderson today. Laura’s got a podcast called [Audio Diary of a Superhero], whose main character has Ehlers-Danlos Syndrome. As Laura does herself. As I do.
Laura, welcome to the podcast.
Eir, I really appreciate you having me on; it’s always a pleasure to talk to you.
So Laura and I met through Tumblr. The [Ehlers-Danlos tag] there is fairly active and Laura was looking for other people that had Ehlers-Danlos, and at the time, was looking for maybe some positive stuff? And wasn’t finding it. And I’m like, “Hey, I have a blog project that’s all about, like, [being grateful for little stuff] that you’re able to do.” And that sparked off a long series of conversations and we became friends over that. So, Laura, why don’t you tell everybody some of your background?
I’ve been living with Ehlers-Danlos Syndrome for pretty much all of my life, though I wasn’t diagnosed until my freshmen year of college. Academically, intellectually, I’ve done a little bit of everything under the sun: creative writing, psychology, computer programming, biology. Although, once I was diagnosed, I decided what I wanted to do with my life was go into research on Ehlers-Danlos Syndrome.
Okay. So Laura and I have both had this our whole lives, of course, but as she said, she was diagnosed in college and I was somewhat older when I got my diagnosis. So we came at this from a couple of different directions, and I think we’re both going to have a little bit different, sort of, tips and tricks for everybody. So we’re just gonna go back and forth and give you guys our sort of top few things that we think will be the most helpful for you to know if you’re just adjusting to an Ehlers-Danlos diagnosis. So, for example:
One of my favorite things to remind people is The Buddy System.
If you’re going to be doing something that you can do with 1 joint, try to involve more than 1 joint to help protect that one. Say you’re pushing the button on a food processor, and you could do it with 1 finger, but if you can add one or two other fingers, you reduce the stress on that one. If you’re going to pick up something with 1 arm, try to pick it up with both, that reduces the strain on both of your wrists, your elbows, your shoulders and can help minimize the stress and pain and dislocations that can come along with Ehlers-Danlos.
That is a fantastic one. I’ve used that quite a bit, myself.
My number one tip is carry a cane. They make some cute little collapsible canes that you can put into a backpack or stick in your car or a locker or even in your desk. Having one on hand at all time is very, very useful, because you never know when something is going to go out of joint and you might need it.
Very true. Right along with that one, for me, is learn to use your assistive aids before you need them. For example:
If you can still, like, shower and not have a problem standing up, but you think that in the future you might need to be able to, say, shower sitting down? Get a shower chair, learn to use it before you need it. So that you know how to reach the different parts of your body to wash them, you know how to wash your hair, you know how to reach your soap or your loofah or whatever it is you use.
Because it’s way easier to learn stuff when it’s not absolutely necessary, when you have a little bit more leeway. And this way, if it does become necessary, you already have it on hand and you don’t have to, say, wait for it to ship to you and you’re left going ‘I feel gross, I need a shower, I can’t stand up in the shower, and I don’t have a shower chair yet’ so you both are already used to it and it’s on hand and ready for you, if it becomes necessary.
Oh, that is so true. And it can be really frustrating, for example, when you suddenly need to learn to use a mobility device and you’re already halfway out of commission. Because you’re basically trying to retrain your body on the fly, with a handicap. And it doesn’t work out all that well, so it’s better to be prepared.
Definitely so. Don’t think of assistive aids as you “failing” to do stuff. Needing a cane, or a wheelchair, or a shower chair, or anything — these are not ‘oh, you’re failing to be able to do something’. No. You’re giving yourself the freedom to be able to do it without hurting yourself.
Having a cane or having a wheelchair and being able to go out and go to the store, or go to the movies, or go out with friends, that lets you do those things without as much pain or dysfunction or exhaustion as you would have otherwise. Without them, the calculus might come out that you can’t go and do them at all. But with your assistive aids, suddenly some of those options are back on the table. So don’t consider these things failures; they’re not. I know it can feel like it at first, but they really are things that give you freedom back to do things that you want to do.
That’s absolutely correct. That leads me to another tip: adjust your attitude.
Eir mentioned that when she and I first me, she had a blog project about celebrating the little victories in life, and really that’s what you have to do. With an Ehlers-Danlos diagnosis, it can feel like suddenly everything has been taken from you. Everything that you thought you could do, that you thought was going to happen, it can suddenly feel like those are no longer options for you. But, instead of focusing on that, focus on all the things that are still available, that the new diagnosis can’t touch, won’t touch, and embrace what you can do. Love your life, even if it’s not what you thought it was going to be.
Definitely. Definitely. This was something that I touched on in an earlier episode when we were talking about ME/CFS, that you have to maybe narrow your focus a little bit? That maybe you can’t do everything under the sun, but that doesn’t mean you can’t do anything. And being able to focus on what it is that you can do and reminding yourself that it’s not all gone is essential to sort of keeping yourself centered and grounded.
Or even really moderately sane. [laughter]
Also that. [laughter]
And speaking of sane, that reminds me of something else. Consider a therapist. Consider some kind of psychiatric maintenance in your medical, sort of, stable. I know that we tend to collect up doctors over time; when you’ve got something that can affect so much of your body, where you end up with the sort of ‘Pokemon’ aspect of collect all of the doctors? Consider adding some psychiatric to that, too. Because this can really screw with you, and having somebody outside of things be able to say, “No, you’re seeing things right” or be able to tell you when your perception is maybe getting a little skewed, is invaluable.
Very true. And, uh, EDS is a huge life adjustment, and sometimes we all need a little extra help with that, a little extra perspective.
Plus there’s the fact that because this can be such a painful disease — it isn’t for everybody, some people don’t have a lot of pain or dislocation, their EDS expresses itself in different ways — but when it is really painful, having chronic pain can change things in other ways, can change your brain.
Like, there’s [all] [kinds] [of] [studies] about how about [chronic pain literally changes the way your brain works] and that can be an odd adjustment in itself, dealing with those kind of changes. So it’s, [laughter] it’s not ‘all in your head’, as it were. I mean, it is, but it’s not. It’s not something that you’re making up. It’s in your brain, in that yes, your brain is literally changed by it, but you’re not making it up if you’re feeling like things are weird or different after this sort of thing takes over parts of your life.
Yeah. I feel like it’s also important to remember that because EDS is so enduring, and it manifests in such a variety of different ways, and it’s inconsistent even in the same person in how it manifests from day to day? It’s very important to remember that, no, this is real, this is happening to you, you are not malingering, you are not a hypochondriac. Your body is telling you things day to day, and you should listen to them.
Exactly. You have good days and bad days. You have days when you’re gonna tell yourself, “Oh, no, I must just be exaggerating it, it’s not really that bad. See? Look at all the things I can do today!” But then, tomorrow, you might find out that actually now you’re paying for that good day, because you did too many things.
Try to pace yourself. Try to remember that, on the good days, you can’t push yourself and pretend that this doesn’t exist. Because that makes your bad days worse. And on your bad days, remember that good days do happen, and to be gentle with yourself while you’re going through those rough days when it hurts more, or you’re more exhausted, or your brain is all fogged and you can’t think. Try to remember that this is not something that you’re causing to happen, it’s not something you’re making up, it’s not something you’re exaggerating, and the bad days don’t last forever.
No, they do not last forever, thank the Lord for that.
[laughter] Agreed. Thank you Laura for joining us for this episode.
Well, I’m just glad I could speak with you about this Eir. Like, you have helped me out so much in the past, and you know, it’s just good to be able to help you back.
[laughter] I appreciate it; thank you.
If you check the website for this podcast, you’ll find a full transcript of this conversation, plus links to all the things we talked about today.
Please, continue to join me here every month for a different topic and set of tips and tricks. You can find us on the web at http://www.sicktipsandtricks.com, on Twitter @HowToBeSickTips, and on Facebook at SickTipsAndTricks. As always, a full transcript of this and every episode is available on our website.
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Tips and Tricks on How to Be Sick 