From time to time I like to interview people in our tribe, so that you can learn more about what it’s like for other people living with chronic illness or disability. Recently, I had the pleasure of interviewing Bella, a military wife and mother living with Ehlers-Danlos Syndrome.
Bella: My name is Bella and I’m 38 years old. I’m married with two boys who are 18 and 16 years old. My 18 year old is stretchy like me and my 16 year old is normie My husband has his hands full.
We are currently living in a small resort town in Florida. We will be moving in June and hope to go to a larger City in a place that has actual seasons!
The MM: When did you first notice that something was wrong with you?
Bella: From the time I was really young I knew there was something different about me. I wasn’t sure that there was something wrong though. I started dislocating when I was probably 10 or 11. I always just thought I was super flexible and that it was a good thing. I started taking dance and martial arts in my teen years and my flexibility was always a positive thing. I did notice that I could do things that other people couldn’t do. But still I didn’t know that it was something wrong.
As I continued to age my body got achy, but I always thought that it was because I was burning the candle at both ends. By the time I was 16 years old I was in college and working two full-time jobs as well as my extracurricular activities. I figured with hours like that and the sports I was doing it was normal to feel how I felt. I did notice that it seem to progress. I wasn’t sure why I was so loose everywhere but I knew that it was getting worse.
By 21 years old I had my little “parlor tricks” that I did. I used to make bets in bars for free drinks (ha ha). I would clasp my hands by my back and bet people that I could bring them to the front of my body without letting go – of course nobody thought that I could. I suppose I should have known at that point that there was something wrong. It was a couple of years into my twenties when the pain really started to set in. Up to that point I had just really been achy, but this was serious pain.
One day, when I was 23, I was working at a grocery store. I was standing at my checkout stand, and I was totally fine one second, but the next second my head started hurting so badly that I nearly fainted. My vision completely blacked out, and I felt like there was blood running down my face. I was carried up the stairs by some co-workers to the break area, and I eventually ended up in the emergency room. At that point I decided it was time to look into what might be wrong with me.
While in the hospital they ran a bunch of tests including a spinal tap. It was a terrifying experience. They eventually released me saying that they couldn’t find anything wrong with me. Over the next 8 years I was misdiagnosed with epilepsy, fibromyalgia, and a host of other random things. I had EKGs and blood tests, and I was sent to psychologist to make sure it wasn’t just in my head. Doctors treated me with anti-seizure medications and migraine medications for all of the head pain and with pain pills for my body aches. Still, no one could really pinpoint what was wrong with me.
The MM: WOW. That must have been really scary! How long did it take for you to get properly diagnosed?
Bella: When I was 32 I had gotten to the point where the pain that I experience everyday was becoming unbearable. I couldn’t function most of the time. I had days when I couldn’t get out of bed at all. My body acted like I was 90. I started to notice that there was more to it than just being stretchy and being in pain. Not only did I have headaches that crippled me, and loose joints that dislocated often, I also had some strange symptoms: I had a weird ringing in my ears that never really went away. When I close my eyes, there are flashing bright lights, as if I am standing in an empty field during a lightning storm at night. My balance was also really off. I just thought I was klutzy, but now, I was running into walls and tripping over my own two feet. I was convinced that the doctors were missing things. The pain finally got so bad that the neurologist decided to test me for multiple sclerosis. This would be the first time I’d ever had an MRI.
Thank God for MRIs! They didn’t find multiple sclerosis however they accidentally found that I have Arnold Chiari Malformation type 1. I have a 10 mm herniation with significant crowding. As it turned out that was the key to figuring out what was really wrong with me. Once I knew about the Chiari, my stretchiness made a lot of sense. The neurologist happened to know that Ehlers-Danlos Syndrome was found quite often in other Chiari patients. With some simple genetic testing it was confirmed. Something strange happens when we’re diagnosed with something that, depending on what Doctor you talk to, is anywhere between a chronic to terminal illness: it’s almost like the stages of grief. You almost mourn.
All of those things that I had dreamed about that I was going to accomplish seemed so far away. I was only in my early thirties, but with the way that my body already felt and being told that it would progressed even further problems, I saw all of my goals kind of melt away. It sounds stupid, but how am I going to climb Machu Picchu if I can’t keep my knees and hips in their sockets? At this point I can’t even walk around the zoo without paying for it for a week afterwards.
There was probably a good year to year and a half after my diagnosis that I laid around and felt sorry for myself and let my disorder take over. I was being treated by doctors who didn’t know a whole lot about any of it (Funny how they sent me to psychologists over and over again to make sure that it wasn’t all in my head! If it wasn’t in my head I wouldn’t have these problems ! Ba dump dum – chiari jokes are the best)
(The MM: GOOD ONE!)
…and try to make me feel better by pumping me full of pain meds, and telling me scary stories about what my future will be. I had a neurosurgeon pushing me to have surgery to decompress the Chiari. Had a primary care physician who told me that eventually I would start choking on my food or would fall down stairs, and that many Chiari and die from secondary causes. I had an ortho doctor telling me there was nothing they were going to be able to do to help the Ehlers-Danlos. They told me I would probably have to have multiple joint replacements as I aged, but that would be painful and wouldn’t really fix the issue. They really know how to scare the crap out of a person.
After a while of being a mopey person, I remembered that I’m actually a very strong person. So I put on my big-girl panties and start trying to live my life again. My husband joined the military, we moved to Florida, and I worked with my doctors here to get off all of the medications that I’d been put on. I was on very strong opioids that were starting to have a pretty negative effect on my body, and I was still in pain. I guess I kind of figured if I wasn’t going to get rid of the pain why was I poisoning my body?
I started trying to be more independent and trying to work through the pain that I experience everyday. I realized that the pain was worth it because at least I felt like a normal person. To this day my pain is unmanaged. I’ve just recently had to have my shoulder reconstructed (I guess those ortho doctors were right.) I’ve been told my other shoulder both my elbows, both my hips, and both my knees have a potential for needing replacement for reconstruction within the next 10 years.
The MM: What do you want people to know about your experience living with EDS?
Bella: For me, the thought of more reconstruction to come is really kind of daunting. With my shoulder surgery I was sent to someone who is considered a specialist with anything related to the arm. He found that I dislocated my shoulder so often, that he needed to shorten some of my ligaments just to keep my arm in its socket. During all of our preoperative appointments he tried to put me at ease and tell me he knew exactly what was needing to be done.
It turns out that you really have to be proactive about your care when you have Ehlers-Danlos (EDS). I guess I was naive. I should have asked more questions to make sure he knew what he was talking about. I guess because I knew the answers I didn’t think to ask him to verify that he did. It wasn’t until after major reconstruction and post-surgical complications that he admitted that he really was not very familiar with EDS at all. It’s very difficult for us stretchy folks, because it’s not like we look sick. People think we are normal and they can treat us the same way and with the same procedures as everybody else.
This doctor reconstructed my shoulder like he would for any normal person. The result has been disastrous. In fact, I’m now looking at having a revision to fix the issue. Unfortunately, most insurance companies really don’t like things that they don’t understand. My insurance is provided to us through the military and is generally very good. I have never had a problem getting treatment for any of my kids for any normal bumps, bangs, brakes and things that come from having two boys. But this rare stuff is just more than they can comprehend.
They don’t understand why I need to see special people. They don’t understand why Physical Therapy completely failed when most people’s shoulder problems would have benefited from it. When I go and see my primary care manager, sometimes I feel like I’m test rabbit or guinea pig. They want to try injections and pills and things, but the problem is none of that is going to ever make me not stretchy. There is a serious lack of education and information out there for a primary care managers that have anything to do with EDS or Chiari malformation.
Right now I’m stuck in the endless loop of trying to get a referral to somebody who knows anything about connective tissue disorders. I live in a small area where there are limited choices of health care providers, and needing something that is out of the ordinary is not what people want to hear. It makes things difficult and they don’t like difficult.
The MM: Bella, thank you so very much for telling us a little bit about your health and your EDS experience! We wish you and your family the best of luck, and hope you all have a wonderful holiday and New Year.