So you wanna know what Ehlers-Danlos Syndrome is – Simply put Ehlers-Danlos Syndrome, or EDS is a defect in the collagen. Collagen is the glue of your body, it’s found in your skin, organs, and connective tissues, virtually everywhere. Those with EDS just don’t do collagen right. How the collagen is affected varies by the type of EDS the patient has. I have Ehlers-Danlos Syndrome Hypermobility, or HEDS. In my case the faulty collagen mainly affects my tendons, ligaments, and skin. It also affects my gastrointestinal tract, and minimally my vascular system.
I bet you wonder what exactly that means? When your collagen in faulty it effects the body in a variety of ways, with EDS it varies according to what type you have. In HEDS the faulty collagen causes our joints to be loose, and prone to dislocations and/or subluxations. I even dislocate joints when sleeping. The easiest way to explain this is to think of the tendons and ligaments that hold your joints together as rubber bands. In a normal person these rubber bands stretch nicely, but still hold the joints tightly in place, they stay nice and tight as they stretch out, and in. In HEDS, our rubber bands are loose. They stretch out wonderfully, they just don’t stretch back to hold everything together. The severity of this varies greatly from person to person, even within families. EDS is inherited, but it can also occur due to a gene mutation in a family that has no prior history of EDS. HEDS can also cause digestive problems like GERD (or reflux), Irritable Bowel Syndrome, Delayed Gastric Emptying. It can also cause Autonomic dysfunction, Aortic Root Dilation, Mitral Valve Prolapse, and a variety of other issues. We usually have skin like velvet, which is nice. However, that velvet skin likes to scar funny so there’s that. There are many other ways that HEDS can affect us, but I really don’t want to lose you just yet.
The primary symptom of HEDS is pain. Yep, pain is the number one symptom of HEDS. Those with HEDS suffer from Chronic Pain as a result of constantly injuring our joints. This is from dislocations, subluxations, and we even injure our joints by just moving. You see our joints don’t move like they should, so simple day to day tasks can cause micro-tears in our tendons and ligaments. Most people with HEDS have had chronic pain since they were young. We also suffer from Chronic Acute pain, meaning that when we suffer a dislocation, we are causing an acute pain. Since we tend to dislocate or subluxate joints often, that acute pain occurs frequently, and is chronic. Many of us were not diagnosed until later in life. I didn’t get diagnosed until my early 30’s, after my mother who was in her mid-50’s was, my grandmother had it was well and she was in her mid-80’s by the time we pieced it all together. Most of us go through life seeing doctor after doctor, none of them really able to figure us out. They may see us for a dislocation, reduce the dislocation and send us on our way. We may see an orthopedic doctor for pain, but he doesn’t see anything on radiographs or other forms of imaging that indicate a reason for pain. An EDS patient has to be looked at as a whole person, examined from head to toe in the way we move. Even then the diagnoses is often missed. Most doctors are simply unfamiliar with EDS, though
they have probably seen EDS patients and did not know it. Many EDS patients go through life being told there isn’t anything wrong with them, it’s all in their head, they need physiological help, until EUREKA a doctor sees them that actually sees the hypermobility, and puts it all together. It’s very frustrating, and once you are diagnosed it can be overwhelming.
It’s My Friends That Keep Me Going
So, what is it like to live with HEDS? I can only tell you from my perspective, and everyone is different. Some people are effected less than I am, some more. The way HEDS affects a person varies as much as individuals vary from one another. That said, I will explain my life with HEDS.
I have no idea what it’s like to not be in pain, I really don’t remember. I know I had many injuries in elementary school, middle school, and high school that landed me in a variety of knee braces, foot braces, casts, and the like. I don’t remember if I was always in pain or not though. I really can’t recall when I was pain free, meh…thems the breaks. I’ve never really let it slow me down much. I have had periods in my life when the pain was severe, and through some of those periods it slowed me down a little. I know in my younger 20’s that caused some anger, and resentment. I literally resented my body for a few years. At that point I didn’t know what was wrong, and I’d had enough bad experiences with doctors that I quit going to them. What I did do, was live fast, and probably not the best way. I’m certain I did extra damage to my body by spending much of those years with a 65lb pack strapped on hiking, jumping out of air planes, learning to ski and surf the hard way. Doing anything and everything that gave a good adrenaline rush. I figured, what’s the harm? The worst I thought that could happen is I’m going to get hurt, and I do that walking down the road. As years passed by, more joints hurt, and I had more problems. I slowed down, because my body basically said “screw you lady”. I probably did more damage to my joints because I simply didn’t know there was a problem.
At this point I dislocate, and subluxate frequently. My shoulders, wrists, and elbows while sleeping are an issue. I’ve learned to sleep with wrist braces, should stabilizers were suggested, but we didn’t get along. I sometimes have to wear wrist braces during the day, sometimes knee braces as well. My right ankle pretty much lives in a moderate support wrap to keep it in line as I have nerve damage from all the times I’ve rolled it, and I often wear one on the left ankle as well. All of my joints hurt to some degree, every day. It’s ok, I don’t want pity from anyone. I can still smile, have a good time, and laugh my big obnoxious laugh. Fatigue catches up with me often; I’m bad about pushing through it. Some days I have to miss things I want to do because the pain and the fatigue are a little much. I’m lucky to have friends that understand when I don’t make it out when we have friends, scratch that – I am beyond lucky to have the friends I have. Sorry folks, but my friends are totally better than yours! That said I know I have a long way to go to get my pain, and EDS properly managed.
No More Party Tricks
So why haven’t I gotten this all under control? One of the most aggravating parts of EDS is that there are so few doctors that know enough about it to help their patients properly manage the condition. It’s not their fault, they get all of their connective tissue information over a short period of time, and most of that is spent on the conditions, and diseases they will see more often. Sadly, at this point I have not met a doctor that knows more about EDS than I do. This is why I feel it is extremely important for an EDS patient to educate themselves about their condition so they are well informed. It is the single most important thing and EDS patient can do for themselves. Many of us have to see several specialists until we find the right combination of doctors that either have a good understanding of EDS, or are willing to learn along the way. Either way, it is imperative for the EDS patient to stay informed, and involved in their treatment.
So what does a friend, or family member of someone with EDS need to know: Your friend is the same person they always were, they just have a name for the condition that enables them to do all the fun party tricks they can do. However, now that they know – they can’t do those fun party tricks anymore, no matter how much tequila is involved. You need to understand that your friend will sometimes be in pain, or have significant fatigue. Sometimes they may cancel plans at the last minute because their body just says no! They don’t want your pity, they don’t want to hear that “you know how they feel”, and they don’t want any pep talks about eating better or exercising more. These just are a fix for EDS. What they do want is your understanding. They want you to keep inviting them out, because they really want to go when they can’t, and sometimes they can. If your friend can’t, you can always offer to come over with some grub, and a bottle or wine, or a mason jar of moonshine. That way you guys get to enjoy each other’s fabulous company, and your friend doesn’t have to leave her sofa. Plus it’s always a good time to play 20 questions, I have never, truth or truth, and many other bits of shenanigans. We just want our friends to understand that we can’t always do everything, but we always want to do everything, and we feel left out when we we don’t get invited. And never be afraid to ask questions if you don’t understand something, we would rather our friends know what’s going on than assume. We are the same people we were before we were diagnosed, we just happen to have a funny name now for something that has always been with us.
Some days our bodies say “NOPE”
Please never hesitate to ask questions here on Livingbendy.com, or Atlanta EDS Support Groups page on Facebook. I will be happy to do so and answer as best I can. You can also send questions to firstname.lastname@example.org.