EDS From Head to Toe
EDS is a collection of genetic conditions that affect the collagen in a person's body. There are potentially more than thirteen different types. I have the most common of these, hEDS or Hypermobile Ehlers-Danlos Syndrome. At this time, about 1 person in 5,000 is diagnosed with this, making it a rare disease. Experts believe, however, that it is drastically under diagnosed and that the incidence is far more likely to be 1 in 500, making it as common as diabetes. So why isn't it diagnosed more? Simple. Doctor's don't know about it. In most medical schools, EDS is a paragraph in a chapter about rare diseases at best. Unless they are exposed to it for some reason, most medical professionals don't have EDS on their diagnostic radar.
So what is Ehlers-Danlos Syndromes? EDS is a condition where a person's body manufactures faulty collagen. Collagen is the glue that holds your body together and can be found in just about every part of your body. When your body makes glue that doesn't hold quite right, all kinds of things can go wrong, as you might imagine.
Beginning with the head, we learned that neuro diversity is significantly more prevalent in the EDS community. ASD and ADHD are diagnosed as much as 7-8x higher rates and depression and anxiety affect a larger percentage of the population as well. Eye conditions, like keratoconus, are more common. The nose session explored a frequent comorbidity, Mast Cell Activation Syndrome (MCAS). With this condition, you experience allergic reactions in multiple body systems, including allergic rhinitis. We learned strategies for coping with this challenge. Much of your mouth is composed of collagen containing structures and as such, people with EDS are prone to many oral problems. We were encouraged to be rigorous with our oral hygiene and counseled on options for decisions we might face with regards to surgery and other oral amendments.
Surgery became the contentious subject of the conference, with about 2/3 of the presenting experts cautioning us to avoid it at all costs and 1/3 of them telling us it was our only option. As we headed into learning about the neck, we discussed craniocervical instability and chiari malformations as well as other problems affecting the cervical spine. These, of course, are almost exclusively addressed by surgery. They did, however, offer some suggestions for physical therapy and bracing, though I can't imagine that would be a permanent solution. Having personally experienced a surgery, two actually, on my cervical spine at the base of my skull, I can attest to their extensive affects on the body and the extended time required for an EDS body to recover as well as our predilection towards complications. They are not too be entered into lightly.
Continuing down the body, we moved onto shoulders. These were a subject pretty much everyone in the room could identify with. Because the collagen in our connective tissues is weaker, people with EDS are prone to dislocations and subluxations. A subluxation is a partial dislocation that can usually be reset by yourself, meaning you don't have to go into the ER. You might be surprised to learn that it's not uncommon for people with EDS to sublux a joint multiple times a day, maybe even dozens. When it comes to shoulders, one of the most common issues is for us to sublux it, having it slide partly or even mostly out of its socket, at night while sleeping. As you read this you might think that because our connective tissues are looser it means this doesn't hurt. I wish this were true, but it's not. We still have all the same nerves, and in fact, sometimes they get even more sensitized. In addition to dislocations, people with EDS can experience labral tears in their shoulders or thoracic outlet syndrome where the nerves running through the shoulder become pinched. Again, opinions were mixed on treatment options between surgery and bracing + PT.
The hand and arm session was quite interesting. They actually had my hand surgeon present on shoulder surgery options, even though he doesn't perform them. They had two other experts discuss the hand, wrist, and elbow. I can't help but wonder if this was intentional. The way my surgeon presents, he says 100% of people with EDS have the issues he operates on and the only way to address them is surgically. I can't help but wonder if the conference coordinators wanted to present additional viewpoints. The other speakers shared occupational therapy suggestions. They also pointed out that many of the suggestions given to people with arthritis are helpful for us as well, so they pointed us to those resources. And, of course, they discussed bracing. Now this is my little confession time. From the time I learned that I had EDS and I learned that ring splints were a thing, I wanted them. Not because I needed them or I thought they would help ease some pain, I just thought they looked cool.
From here the speakers headed into topics involving the gastrointestinal system. Did you know your GI tract was made up almost exclusively of connective tissue? Let's just say there are A LOT of points at which it can break down. I was so encouraged by the first speaker on this subject. I even got to speak with her in the bar line at dinner that night for a while, and she was awesome. She had six rules for diet and nutrition that I LOVED and wrote down somewhere... But I remember the first two for sure
1. If it hurts when you eat it, don't
2. If it doesn't hurt you to eat it, don't eliminate it
Hearing this from an expert was so empowering! I've been on so many prescribed extreme elimination diets that the thought of having to cut something out of my eating is actually traumatizing. I don't mean to be dramatic, that's just the truth. I went through a year where I started from a plain chicken breast with nothing on it and boiled, pureed carrots and slowly added things in, one or two a week. Each thing (even lettuce) had to be peeled, deseeded, boiled and pureed before I could eat it. Essentially, my food had to be predigested.
People with EDS deal with all manner of GI complications. IBS, IBD, SIBO, MALS, you name it, there's probably an acronym for it! Hernias are also common. Some of these things have to be addressed with surgery. Some of them with equally argued over drug protocols. Some of them, like IBS, are often actually side effects of MCAS, and so are treated with antihistamines. One of the interesting things I learned two years ago at the conference virtually, which was confirmed at this one, was that there is no ideal diet for people with EDS. There is no food, or food type, that is inherently bad for us that we should avoid. Most of us are so limited already by food that triggers our MCAS, that they encouraged us to just eat and enjoy whatever we could.
Heading further south, the next two speakers were urogynecologists. I had no idea how common UTIs are for people with EDS, but it was ironic that I had one during the entire conference. Also of note is a likelihood of organ prolapse which they discussed at length along with various treatment modalities. To my dismay, I also learned that bladder leakage is a very common, though rarely discussed, problem. Not something I'm looking forward to 😕.
The next speaker posited that the pelvis is the center of the universe. It's the hinge point that links the upper and lower body together. At this joining point are many opportunities for things to go awry as well. The SI joint, where the spine and the pelvis meet, is a frequent point of instability as it is held together with connective tissue. The point at which the legs meet the pelvis is also at risk for labral and other connective tissue tears. We learned, though, that this joint is less prone to dislocation and what we experience is more likely subluxation and/or large scale shifting of tissue. I guess that's good news?
Heading down the leg, we didn't learn much about the knee beyond tape it, brace it, try to avoid surgery. The foot and ankle speakers were cool. Lots of gross pictures of cadavers and experiments. Did you know that one in four bones in your body can be found in your foot? That's a lot of bones! And do you know what you need when you have a lot of bones? A lot of connective tissue to hold it together. When that connective tissue is lax, then the foot... Well it melts. People with EDS tend to have a type of flat foot. It molds to surfaces and takes on shapes. It sounds cool in theory, but the practicality of it is less so. The challenge comes in that your foot is the foundation of your body. If your foot isn't positioned right, with the proper arch, then your knees, your hips, your spine, your shoulders, even your head ends up out of whack. This can lead to all sorts of trouble. Did you know your feet could cause migraines? True story! I even have trouble with sleeping at night occasionally where the weight of the blanket will misshape my foot, folding it if you will. I have to walk around funny and flatten it out when I get up. Yep, that hurts too. So these doctors talked about the importance of proper footwear a lot. No cute little heels or sandals for me! I need proper arch support and a good, strong heel cup. While walking barefoot is good for some to build muscle and proprioception, for those with lax connective tissue we require the extra support a good shoe brings, so please forgive me if I wear them in your house 🥺. Our ankles, also full of little bones and lots of lax ligaments, are equally unstable. It's not hard to get a sprain walking across a flat, open floor. And when paired with diminished healing ability, you meet me with two permanently sprained ankles. They aren't always active, but it doesn't take much to flare them and then it's days, or even weeks, before they settle again.
Having traversed the body from Head to Toe, there were still a few things they didn't want to leave unattended. They had a wonderful speaker who discussed the lymphatic system. This system works in your whole body to distribute fluid and remove waste. As you can imagine, this is an important system to keep healthy and functioning and the speaker encouraged us to stay hydrated, seek out a seasoned lymphatic massage practitioner, and learn how to practice self lymphatic massage daily. Oh yeah, also movement and vibration. Yep, she wanted us to grab onto our organs and give them a good shake 😂. As funny as that sounds, there's science behind it and I think I'm going to try to be more consistent about using the whole body vibration plate my husband bought me.
There were also sessions on fascia, but I confess, I got super tired and had to take a nap, so I slept through them 🥱. I look forward to watching their recordings when they come out in a month though!
You might think four days of hearing what could go wrong with you would be discouraging, or even downright depressing. That wasn't the case though! For one thing, it was often one aha! lightbulb moment after another. You're sitting there making sense out of the things that are going wrong with you. Over and over (and over and over) you are told by medical professionals, family members, and not so polite busy bodies that what you're experiencing is all in your head. You couldn't possibly have so many things wrong with you. And I hope you get better soon. For four days, I heard that what I'm experiencing is real. I'm not alone. There is a reason and it makes sense. There are things I can do to feel better and keep myself as healthy as possible.
Here's the thing: But here was the biggest thing, I wasn't the only one! I was in a room of 700 people like me. People in wheelchairs. People with canes. People with braces. People with crazy hair and piercings and a lot of matching scars. We were zebras. All of us had different stripes, but at our core, we were the same. I was in a room full of people, where I wasn't the weird one. Can you imagine that?
Awesome insight into EDS! So glad you got to attend :)
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