To Queen's Park

   It was the spring of 2015 and I was still trucking my way through finishing high school. I busied myself with other things as well, such as seeking out others with Ehlers Danlos Syndrome. I got a tip from the neurologist who diagnosed me that every few months people gather at a Toronto library for a support group meeting. I was extremely excited to meet others going through the same thing as me and was hopeful that trading tips would help improve my situation. Interestingly enough, I was the only male attending these events. This is funny in a way because I faced a similar situation at summer camp, being the first boy to go for my age group. Whereas that was just happenstance there is a good explanation for the women with EDS. First of all, women are naturally more flexible and so it's more obvious when they have EDS. Then there's the fact that women are simply statistically more likely to seek out a diagnosis and treatment for their health issues. Lastly, the disease is just slightly more prevalent in females statistically for whatever reason. The plot thickened when I realized that everyone had some sort of trigger for their chronic pain in the same way Swine Flu triggered mine. The difference is that for most of them the trigger was childbirth, since that puts so much stress on your body. The rest suffered viral illnesses in the same way I did.

   Everyone at the meeting was pretty excited when a guy showed up and they immediately got to work on integrating me with their organization called EDS Canada. They asked if I'd be willing to create and administrate a Facebook group specifically for men with EDS. The idea was providing a safe space for men to express their difficulties and discuss gender specific questions and concerns. And just like that I became an advocate for both EDS and invisible illnesses in general. I built the group and it now stands at 20 members. That might not seem like a lot but that's almost every male within the EDS Canada community. Apparently we are a rare breed.

  I had helped in a small way but I certainly didn't stop there. I thought: what else could be done to advance the collective interests of all those with this debilitating disease? I realized I was in a somewhat unique position being the son of a Member of Provincial Parliament. The position of an MPP has inherent power that can be used for good or ill. Something all MPPs are able to do is read out statements within the legislature bringing aid and recognition to various causes. Working together with EDS Canada we wrote a speech that my mom would read out in the legislature. Virtually everyone on all sides of the political spectrum were happy we were there and applauded us for fighting the good fight. Some of my companions with EDS were moved to tears during the speech because they were finally witnessing the recognition of their struggles by the government. Little did we know that the best part was yet to come. Months later the provincial government announced plans to open an entire clinic for people with EDS! This is exactly what we needed as patients since the vast majority of doctors don't know or understand EDS in any substantial way. For most (as my doctor dad and brother can attest) they were given a single lesson on EDS in medical school and it was never followed up on again. What we need is specialists who can advise regular doctors on matters concerning EDS. That's exactly what we got. For those within the EDS community this has development has been viewed as a direct result of the statement in the legislature I planned. This means a lot of people are giving me much of the credit for the clinic opening, which is the biggest honour I can imagine. Later I would become one of the first patients to visit the new clinic. I couldn't have asked for a greater reward. Here's the link to the news story: http://www.ctvnews.ca/health/ontario-to-set-up-clinic-for-people-with-rare-disease-eds-1.2797357#_gus&_gucid=&_gup=Facebook&_gsc=kcXsPEK

Itty Bitty Cysts

    The neurologist who diagnosed me offered me the chance to participate in a case study to test the efficacy of what he called a vibro-acoustic chair. You put it on your chair and then lie your back on it which lets you feel the soothing vibrations. As a little side feature the chair also plays music in time with the vibrations. There was a more energetic mode for mornings and a more relaxing mode to be used before bed. The music didn't really accomplish anything and I didn't expect it to, but the vibrations helped undo the knots in my back and aids me in falling asleep. The chair had already been tested and deemed effective in the treatment of a similar condition known as fibromyalgia. This made them want to compare the results to those in people with Ehlers-Danlos Syndrome (AKA me.)

    The same neurologist refereed me to a physiotherapist who sort of specializes in managing EDS. He has 16 other patients with EDS all with varying types and severity. (He claims I'm on the severe side but he's seen worse.) He told me one hopeful story of a girl with EDS who was out of school for 4 years because she was plagued with horrible fatigue and pain. This is a similar situation to the one I'm in, and eventually her fatigue symptoms simply worked themselves out. We got to work right away on strengthening the muscles that surround my joints. My joints would never be able to stabilize themselves, so the most effective method is simply getting your other muscles to do the extra work. Trouble is, this causes the muscles to be very tight so he also pressed on many trigger points to help solve that even just a little bit. I have to say this is one of the most painful things I have had to go through. The muscles are always so tight that they're pressing on all kinds of nerves and so when he pushes on my shoulder the pain radiates all the way into my head like a splitting headache.

    We tested my walking ability to give us a point of reference for improvements in the future. I had a horrible stumbling gait and heavily leaned on one side, but it didn't stay that way for long. Week by week we documented my progress and now my gait has normalized except during localized pain spikes. It wasn't just during appointments that I did physiotherapy, it was something I worked on every day. Eventually I progressed to the point where my physiotherapist decided we should no longer focus on joint strengthening and should instead simply do our best to manage whatever symptoms pop up. This means entire appointments are now dedicated to pushing trigger points on my shoulder which still pops out (subluxates) constantly. Once my family got a pool in our backyard I was encouraged to swim as much as possible since this type of exercise is very easy on the joints. My legs were too bad to do effective kicks so I pretty much stuck exclusively to breast-stroke and back-stroke. I would never stop swimming because of muscle fatigue, it was always the increasing levels of pain that stopped me. Meanwhile I couldn't get the idea of my Tarlov cysts growing and causing future problems out of my head. It would be even worse if I forgot about this though because it might one day explain new symptoms that could pop up if it grew.

The Nose Knows

    I was very hopeful that the medical marijuana would work. You can't help but hear all these stories of those with chronic pain extolling the virtues of marijuana, and so I knew I had to try it for myself. The process didn't end with the prescription though. The doctor who is a canabanoid specialist insisted on raising the dosage very slowly. We started at 8% THC (street purity is around 23-27%) and at that dose I felt absolutely no benefit. At 16% I started feeling improvement and with that encouragement we raised once again to 22% THC because we'd rather I use more marijuana and less of other prescription drugs.

 


















     Shortly before I was prescribed weed it was time for me to work on improving my sleep through what is called sleep restriction therapy. The doctor said to imagine that you're putting peanut butter on bread. The longer you do it for the more it spreads out and weakens. To counter this I would have to set my alarm at 8 am every day and was not allowed to go to sleep regardless of how tired I was until late at night. Yes, this strategy made my sleep cycle more efficient, but it was not able to make up for the lost total hours of sleep. This dragged on for months and was truly one of the most torturous segments of my life. I didn't feel even anywhere close to well enough to hang out with friends. I was constantly missing family dinners. Even video games were denied to me again because I was simply too tired to play. For the most part I watched TV which of course I felt too crappy to enjoy. The doctor also dictated that I should partake in light therapy. This sounds nice but in reality I had to buy a fucking giant, expensive lamp and shine it full in my face for an hour every morning. The idea of this was to force my body to quit using Melatonin (the horomone that makes you sleepy) in the morning and to encourage it to release it in the evening when I'm going to bed. This helped move my sleep schedule to more normalcy, but the continuing poor quality of sleep left me unable to function. Then came the marijuana. It helped me fall asleep so we could orient the other medications to focus on staying asleep. I was finally well enough to go back to school!

    I was back at the new small private school (classes starting at 11:30 am instead of 2 pm) which felt great but that is not to say it was all smooth sailing from there. You see, while I'd been dealing with my renegade body parts I was neglecting attention to my nose. Yes, my nose and I have been through a lot together. About six years ago I was at summer camp and was playing a game called "Mosh Ball." To give you an idea of what we're talking about here, this game is a combination of football, kickball, and maybe basketball; I forget the rules. Anyway, I was chasing after the ball and someone on the opposing team was doing the same. The problem? The problem was that he was a councilor twice my height and weight. We both dived for the ball at the same time and my face collided with his shoulder, breaking my nose for the first time. Later that summer someone accidentally jabbed their soldier into my nose accidentally while dancing. It didn't end there either, at winter camp someone running through the hallways accidentally smashed a door into my face, breaking my nose again. That third time I simply reset the nose myself. So you see, my nose is a trooper. He was a little crooked, sure, but that just added character. Unfortunately I eventually realized my breathing at night was problematic due to the way the nose broke. Why is being able to breath through my nose so important? Well, at night I have to take a lot of sleeping medications which all have the side effects of drying me up. Opening my mouth to breath makes this worse and I would wake up at night with my tongue feeling like sandpaper. I was sent off to a nose doctor who then preformed septoplasty surgery to fix my deviated septum. Recovering from that surgery was another extremely uncomfortable period of my life, because my nose was constantly bleeding and stuffed with gauze. This meant my nose was now completely blocked which of course made the dryness issue fifty times worse. On the bright side my breathing slightly improved and my nose looks much better now.

Slang Saviour

   I remember one summer at camp well before I got my diagnosis I was sleeping in like I did everyday because my sleep was so shitty. While that was going on a couple of friends decided to wake me up by pouring shaving cream on me. It was more than just annoying because when I don't wake up naturally it essentially means the rest of my day will be awful. I was angry enough to chase after them but with my legs I of course couldn't catch them and then the pain made me regret running at all in the first place. If I remember right I got my revenge by putting shaving cream in their shoes, but it's just an example of something lighthearted that can be made worse by the situation you're in. I also remember being verbally jabbed at when I was lying down and listening to Eminem's Till I Collapse song but as that person's own health issues developed they understood why I did it. As my situation worsened though, I started listening less to Till I Collapse and more to Beautiful. The music I listened to depended on my mood and Beautiful is a depressing song. Speaking of my mood, at some point still before my diagnosis (we had no idea what was wrong) I reluctantly agreed to try another psychologist. She was probably the worst health care professional I've seen to this day. She literally gave me homework to do. They were little exercises where I answer questions and write about how I'm feeling. That's absolutely all she did. We never really talked. I don't think I saw her more than 3 times.

   I was finally 18, which means it was time to get my wisdom teeth out. The procedure went fine, though my jaw hurt as part of my joint issues since it's kept open for a long while while they are poking around. The interesting part is that the anesthesia for the operation got rid of my pain completely, temporarily of course. This was the second time since my pain started in 2009 that I was pain free even for just a few minutes, the first time being when I intentionally overdosed on painkillers and sleeping pills. It's not a nice memory to attach being pain free to, so I'm glad I've had more opportunities of respite. This occurrence would take place one more time, bringing the total tally to 3 occasions.

   Then it was summer, which means, you guessed it, camp again. This year was defined by one hectic, terrifying, stupefying, and nightmare inducing incident that we realized had occurred while I was literally stepping on the bus to leave for camp. I had been having severe stomach issues, a feverish feeling, strange nightmares, and irritability. It all felt too familiar, and so I eventually realized I was suffering from serotonin syndrome again! As you might remember, serotonin syndrome is a life-threatening drug interaction that occurs when you have too much serotonin in your system. We never realized that the medication I had started a few days earlier (the opioid Tramadol) had serotonin it as did the Mirtazapine medication I had been taking for months. Mix 'em together and you've got a real crisis, especially considering I was about to head to camp. Due to my familiar symptoms I looked up if Tramadol had serotonin in it and it did. We emailed my neurologist doctor who prescribed it asking what's going on and he confirmed it was serotonin syndrome. That meant I had to stop taking Tramadol immediately, which meant I would be going through opioid withdrawal for the beginning of camp. I was out of commission for a few days but thankfully my co workers were very understanding. This is a prime example of when you have "too many cooks in the kitchen," as the saying goes.

  At this point my sleeping problems were still completely out of control. We had managed to shift my sleep cycle to more normalcy (waking up at 11 am rather than 2 pm) but I was not waking up rested in the slightest. Sleep studies showed my sleep was very fragmented with multiple arousals (no, still not the sexual kind) and waking up earlier isn't helpful if you can't function while doing it, but then... it happened. The secret sauce, the holy grail, the blunt bomb, the dank dope, the hippie lettuce, the herbal refreshment the... the weed. Yes, since the Tramadol experiment was a fantastical failure we needed something new to treat my pain so I can do important stuff like maybe sleep and go to school. Since we had legitimately tried everything there is to try, he refereed me to a cannabanoid specialist. I had purposefully never used weed before because I thought it would help my case in getting a prescription to demonstrate I'm no stoner. The things I had heard made me pretty hopeful. Having something to deal with the pain at night so I could sleep would make all the difference in the world. Better yet, the side effects were almost non-existent, at least relatively so. This referral would be my first step in the newest journey of recovery.

Cysts and Sleep Cycles

   It was the summer of 2013 and that meant it was time for another year of camp. My sleeping situation was far less than optimal so this year I decided I would apply to be one of the Heads of Environmental Programming. Our camp had chickens, sheep and rabbits and we'd harvest the chicken eggs and vegetables as part of the job. The reason I chose this job despite my my love for having kids as a counselor is because the schedule is far more flexible and there's less legwork. I got a little stool that I would sit on while gardening and was less tempted to try to chase after kids. It still wasn't easy though, because sometimes I would sleep in all the way until 2 pm. To harness this we essentially said I was in charge of the gardens and animals in the afternoon and my co worker was in charge for the morning. With this system and my less stressful job I was able to get through the summer without too much difficulty. The only snag I ran into was getting stung by a wasp and then over the next hour realizing I was allergic with anaphylaxis. My tongue slowly started swelling in size and my throat started closing (according to the doctor, not just me.) The nurse used an EpiPen on me for the first and only time. Interestingly enough, the allergen tests I did afterwards all came out negative.

  Although camp went well, the following months did not. Not very far into the school year my sleep stopped becoming anything even close to resembling restful. No matter how long or how little I slept, I would wake up exhausted. I could either nap to feel better but make the problem worse or go completely insane from sleep deprivation. I tried both and neither worked. My doctor tried many things as well, alternating with every medication we could think of. Nothing even made a dent in my problems. It took hours to fall asleep, and when I did fall asleep I woke up throughout the night. If there's one situation where you're most likely to lose your mind it would be lying awake at night for hours for your sixth month in a row. Eventually my sleep specialist ran out of ideas and suggested I see a new doctor. Since I was seemingly out of other options, I did so and never saw that original specialist again.

   My new sleep specialist is considered a world class expert in the field. He took one look at me and decided we needed to do a total overhaul. I would put school on hold for many months while we worked on making my sleep restorative (this was one of several factors in how I fell so far behind my age group in school.) He said the way to do this is through sleep restriction. I was still waking up at 2 pm, so he told me to set my alarm for 8 am. Obviously this was very brutal for me and I spent many months exhausted as we worked on shifting my sleep schedule. I felt too crappy to spend my time hanging out with people. Even reading and playing games took too much energy. I pretty much alternated between watching movies and banging my head on my desk repeatedly. This was also a period of time where I drifted away from many friends. I learned that the phrase "use 'em or lose 'em" applied to friends as well. Eventually my sleep schedule did indeed shift. Instead of waking up at 2 pm without an alarm I woke up at 11 am. The problem was I couldn't function at 8 am but if I got up at 11 I wouldn't be able to fall asleep on time that night because the pain made it so I could only fall asleep when completely exhausted. My neurologist wanted to see if there's a fixable problem that is the source of my pain so I got another spinal MRI. The MRI showed some Tarlov Cysts which are associated with EDS. They were really small at the time and so unlikely to be symptomatic but they do often grow, which gave me yet another thing to worry about.



    Although the source of the pain wasn't a cyst, we were still determined to try to treat that pain. Unfortunately, all the pain pills we tried were either completely innefective or were slightly effective but had unbearable side effects. My neurologist wondered if medical marijuana might be a potential solution (relatively little side effects, good pain reduction) and so he referred me to another doctor who is a cannabinoid specialist. He would asses me and determine if I'm a good fit for a prescription of medical marijuana, something I purposefully have never partaken in before. I thought it would help my case if I didn't come off as someone who was already as stoner, which I wasn't anyway. It was something I wanted to try to help my pain for awhile and my patience had finally been rewarded.

The Truth

    Even though no one had as of yet diagnosed me with EDS I knew I had it. The puzzle pieces fitted together too perfectly. I knew it was only a matter of time until the diagnosis came. The first thing my new neurologist did after we told him our thoughts on me having EDS was refer me to a geneticist. There they preformed what is called the Beighton Hypermobility test. They had me stretch in many different ways to text my flexibility. 
    The results showed I had enough 'points' to be considered slightly hypermobile, but that is a bit misleading. One part of the test involved trying to touch your toes. This is something I am flexible enough to do but the pain from doing so is what limits me and so they took that into account. Based on my history, hypermobility, and symptoms, the geneticist clinically diagnosed me with EDS. This means based on what she saw it is her professional opinion that I have this disease.  To be more confident with this diagnosis I was sent get blood tests. Even if she thought I had EDS, we wanted to figure out what kind of type it was. There are many types of EDS but the vast majority of sufferers are afflicted with three main types. These are the vascular, classical, and hypermobility types. The hypermobility type was the most painful of these but the least dangerous. It affects the joints the most and is the most common type. People with this version of EDS are typically very flexible.

    The classical type mainly concerns one's skin, they have stretchy fragile skin that tears easily. The vascular type is the deadliest, for half of those who are afflicted by it are dead by their 40s. This is because it weakens your cardiovascular system including your heart and arteries. The tests I had done showed I had a gene that can be associated with the hypermobility type of EDS. While this was reassuring, (having the vascular type would mean I'd live a very short life) we still didn't have a definitive diagnosis. To remedy this, the geneticist referred me to a dermatologist. It was time for me to get a skin biopsy.

   The skin biopsy entailed me lying on my stomach while they cut a small piece of skin off my lower back. They used local anesthesia so it didn't hurt much. A few weeks later I went back to hear the results. The doctor showed me microscopic images of my skin and these images demonstrated major deformities of much of my skin fibres, which are important for maintaining the strength of your skin. As a consequence of this my skin gets cut really easily, making tasks like shaving difficult. My skin fibres were deemed to be in the shape of cauliflower, which is a classic sign of having the hypermobility type of EDS. I had so many abnormally shaped fibres that the dermatologist considered it to be a definitive diagnosis, which is a very uncommon occurrence for the hypermobility type. Upon hearing this news I immediately broke into a huge grin. I finally caught what I've been chasing all these years: the truth. The truth is that I had the Hypermobility Type of EDS, an extremely painful and incurable disease. So while I may live a long life, it will also be a very painful one. This wasn't easy to accept but still, I finally knew.

The Next Chapter: Closure and New Beginnings

    Although it was hard to see any progress on medical solutions being made, things hadn't come to a complete standstill. I was referred to another neurologist who was head of the pain clinic at Mount Sinai. He ran another electrocardiography test on my legs and I purposefully triggered an increase in pain beforehand by using my legs to stand up and do the slump test. I was so desperate I figured maybe increasing my pain level would make a difference on the test. It didn't. The test was negative like the one I had a couple years before. Then the doctor came in to share the results. He apologized that since I didn't seem to have an active neurological problem, he wouldn't be able to help. As I sat there despairing and gathering my things to leave I habitually cracked joints throughout my body. As anyone who spends time with me knows this is a common occurrence that I can't seem to help. The doctor noticed this and started asking me questions about my joints and flexibility. I demonstrated that I was hyper-mobile, meaning very flexible, but we were out of time and he needed to see his next patients. He told me to look up Ehlers-Danlos Syndrome when I got home and see if it fits with my story. I of course couldn't wait that long, the tantalizing glimmer of hope too great, so I looked it up in the car.

    As I googled what this strange sounding Ehlers-Danlos Syndrome (EDS) was several things caught my eye. First off was, "In some cases, the severity of the mutation can be life-threatening." I found that rather alarming, but who knows if there's a chance I even have this thing so there was no use worrying about it now. "There is no cure for EDS, and treatment is supportive, including close monitoring of the digestive, excretory and particularly the cardiovascular systems." If I had gulped at any point during that day, it would've been when I read that. I noted that this thing can be bad news but still hadn't reached any symptoms to compare with myself. "People with EDS are often misdiagnosed with hypochondriasis, depression, chronic fatigue syndrome, and other conditions because EDS is considered an invisible disability and there is generally poor knowledge about EDS among practitioners. Many sufferers of EDS have psychological difficulties as a result of frustration with the medical system and the socially inconvenient combination of appearing normal while being in severe pain." Now I was starting to see some connections. Throughout my years of illness I had been accused of having all 3 of those mental or exclusionary disorders. Suspicions of mine from that time have been confirmed many years later in that it was revealed to me that family and doctors had considered giving me placebos after the medical search turned up fruitless. The wikipedia post read: "Unstable hyper-flexible joints (it is possible to be very flexible or have "double joints" however, this is not the same as EDS) that are prone to sprain, dislocation, subluxation, hyperextension, myalgia (muscle pain) and arthralgia, (joint pain), which may be severe. Tearing of tendons or muscles and hernias are common."There we go. After reading that section I knew I had finally found my match. After over 4 years of searching, after years of being told the problem was in my head rather than my legs, I found my diagnosis in May of 2013. The description of symptoms didn't just accurately describe my current problems, they explained my entire medical history as well. I had bilateral hernias when I was nine which is not not altogether uncommon. The catch is that a year later I had a recurring hernia on my right side. To have multiple hernias of the same spot at that age is very very rare. They had to stitch up the muscle the adult way which leaves lots of scar tissue. Since I still hadn't gone through my growth spurt yet, the growing muscle laced with scar tissue caused a lot of pain throughout my childhood.

    I wasn't sure how I was supposed to react. On the one hand, I received the validation I so desperately craved after years of searching. I wasn't crazy after all. I learned that there are other people out there going through what I was. In addition, there were plenty of online tips on how to manage symptoms. Yet the other side of the coin could not be ignored. The disease was plainly stated as being incurable. It was autosomal-dominant, meaning if I had children they would have a 50% of inheriting it. I knew then that I would not, could not have biological children; at least not until a cure is found. Yes, although it is not curable now, progress is being made. They're still tracking down many of the culprit genes but gene therapy is on the horizon. Using an agent such as a safe virus or nanobots, someone's genes will be able to be rewritten much in the same way a virus spreads throughout the body. The correct overwriting genes are inserted into a medium, and they "infect" and change all the defective genes. I'd wager this cure is a few decades off, but there's no way I'd chance putting my potential kids through what I go through. I love them too much. As it turns out, by the time I reached home I was far happier than when I left. I learned I was suffering through something very real, that there are others going through the same I can start reaching out to. Most importantly, those I respect the most would stop questioning my sanity. Regardless of who was right in the age long argument of if I was ever depressed or simply miserable, I was neither by the time I walked through my house's front door.