I didn’t want to write another medical blog about my personal struggle of living with a rare, chronic medical condition. It seemed narcissistic and indulgent. But wherever I turn, there it is. I cannot write about any of my experiences without writing about EDS as well. I still feel that the reason for this blog is to answer the question ‘What is it like?’. It is an important question that requires an answer. Not just for me, but for everyone out there who has similar issues and the people who love them who live in a world where disability is often no more than an interesting topic of conversation. So much of what I experience is invisible and I doubt words will ever be able to describe it or I will be able to recall it all at once, but I will try. It’s important.
Ehlers-Danlos Syndrome is a motley group of heritable connective tissue disorders (HCTD) caused by a variety of gene mutations that impair the structure and function of collagen. Connective tissue gives tissue form and strength, impaired structure and function has a profound effect.
Joint Instability: The first and most obvious is joint instability, resulting in frequent and recurrent joint dislocations, subluxations, strains, sprains and a host of other injuries and conditions. Dislocations and partial dislocations range from pretty insignificant to very serious. I can put a finger joint back in from a simple dislocation/subluxation within a few seconds and immediately be able to use that finger again. A shoulder dislocation is unbearably painful and may take up to an hour to relocate and a trip to A&E is sometimes necessary if the weights don’t work. An elbow dislocation or a dislocation gone wrong is a definite and immediate trip to A&E. The upside is that the more a joint dislocates, the more the surrounding tissue stretch and the easier it is to get it back in. The downside of that is that the more a joint dislocates, the more it will dislocate until I can’t get it to stay in without external support or internal fixation/fusion.
Muscular Problems: I have moderate hypotonia and also suffer from muscle twitching and spasms. Practically speaking, this means that my reaction times are slower as my muscles take longer to contract, never contract fully and relax again before they’re fully contracted. In life, it means the natural mechanisms protecting against injury doesn’t work. If you grab my arm suddenly, I don’t immediately react and stiffen, my shoulder will dislocate from the force before I even notice that someone grabbed my arm. In gaming, it means that I am a little slower pushing buttons. Twitching and spasms means that I may push the wrong button or push the right button more twice instead of once or get timed intervals wrong. Muscle fatigue is a big deal as well. My muscles tire very easily and although I play from a very supportive position and rest every single muscle as often as I can, they still tire quickly and easily. The more tired muscles get, the less control I have and the slower my reaction times become. I am exhausted after using my hands for a few minutes, after that point, perseverance only keeps me going.
Peripheral neuropathy: I have peripheral neuropathy in both hands and feet causing tingling, buzzing, electrical shock-like sensations, partial numbness and sharp pain. It makes using my hands quite a challenge at times.
Proprioceptive dysfunction: I have impaired proprioception causing balance & coordination issues and poor spacial awareness. Even simulated movement is deceptive and misleading and I have great difficulty judging distance. I have difficulty discerning where I am in space in relation to other objects. This means that I walk into doors, bump into furniture, trip over my own feet and in Warcraft, have great difficulty flying around Malygos at a 30-yard radius.
Headaches – Headaches are the bane of my existence. They’re chronic, pretty much untreatable and I’ve had them since I was eleven. I get migraine headaches, including the weird ones like basilar type migraines. Headaches are generally worsened by light, sound and movement. The migraine aura that precedes a headache can be a little weird and include palinopsia, temporary loss of vision, double vision, flashy lights, distortion in the size and shape of things, jumpy vision, nystagmus, scintillating scotoma (worsening flickering light that grows into a zig-zag pattern), phantosmia, parosmia, dysarthria, visual snow, dizziness, loss of balance, reduced consciousness, loss of consciousness. Migaine aura’s usually last 30 – 60 minutes, followed by a severe headache phase that may last up to 72 hours. Sometimes I know hours ahead that an aura is about to hit, sometimes it’s two or three minutes. Once it starts, there’s nothing I can do but stop moving in a dark and very quiet room.
Dizziness/vertigo/fainting – I get dizzy often. Warning signs may often last for hours, so aren’t very helpful in predicting exactly when it is going to happen. Multiple factors coincide to produce my unique brand of EDS dizziness – vascular spasms or cervical instability affecting blood flow to my brain (thank you connective tissue in blood vessels) and autonomic dysfunction. Sometimes it seems as if the room is spinning, other times I am moving as if I’m on a merry-go-round or on a boat or in a car. Mostly, I’m just lightheaded and tend to faint when my blood pressure drops when I stand up. Dizzy spells may last seconds, minutes or occasionally for hours. Imagine trying to accomplish anything whilst you’re on a fast ride at a theme park and that’s pretty much where I am at when it happens. If I’m lucky, it’s more of a gentle rocking sensation, but most of the time, I’m not that lucky. Impaired proprioception also means that my brain has difficulty interpreting simulated movement. When I started playing WoW I couldn’t get my character to run in anything but a straight line without getting motion sickness. A year later, I can play for a few hours a day and although the nausea and spinning is still there, it has improved significantly. However, visual cues make it difficult to interpret where my character is and how to move it to get where I want it to go. This becomes a particular issue on flying vehicles in Warcraft.
Hearing: I rarely talk about hearing issues. It’s a sensitive subject for me. I have adapted to it well and dislike the way people treat someone with hearing issues. Yelling makes it worse, people. Stop with the yelling already. I have some hearing loss as a result of hypermobility of the bones within my inner ear. I also have tinnitus and hyperacusis. Some days are worse than others, but most days it makes hearing a tricky task. As not being able to hear certain sounds, not being able to tolerate others and having a continuous background noise effect distort sounds is not enough, I also have auditory processing disorder, probably caused by one of the more serious head injuries I have sustained. I don’t use phones or VOIP or Vent or Teamspeak unless I absolutely have to. Interpreting audio cues are difficult and I cannot listen and do anything else. I compensate as best I can, but being given verbal instructions have always been stress provoking and failing on my part hugely embarassing.
Vision: The muscles in my eyes don’t work very well and tire easily, my eye lenses can dislocate without much warning and takes minutes to hours to self correct. Migraine headaches have weird visual symptoms and having a neurological condition doesn’t help either. The result: blurred vision, variable vision acuity, (some days I can see the poison clouds other days it is a challenge), difficulty with tracking and relocation (looking from health bars to the boss and back), visual disturbances and extreme photophobia – light really hurts my eyes and seem a hundred times brighter than it does to anyone else. In-game, I can’t turn down screen brightness too much as I would not be able to see visual cues, so 25-man heroic combat scenes hurt and are disorienting.
Breathing problems: Lungs are made up partly of connective tissue, so is the rest of the respiratory tract. Mine don’t work well at all and are pretty unresponsive to standard treatment. I have severe and highly variably breathing problems. Before moving to the UK, I had something similar to brittle asthma type 2. I was mostly fine in between attacks and recurrent bouts of chronic bronchitis, but when I did have breathing problems, it was severe enough to require carrying an adrenaline shot with me at all times in case the ventolin was ineffective. Moving to the UK, my breathing did not respond well to the British climate and now I have something more similar to brittle asthma type 1. My peak flow readings vary greatly during the course of a day and I have 3-4 asthma attacks daily despite being on the highest dose of medication I can take for it. Breathing difficulties may respond reasonably well to ventolin and clear up within minutes or I can struggle to breathe at all for hours at a time or I can not be able to breathe within minutes of an attack starting. It’s unpredictable and volatile and needless to say, very debilitating.
Cardiovascular – Blood vessels contain connective tissue. Mine don’t contract and dilate in the way that they should. They either contract to much or too little or spasm for no obvious reason at all. I have a list of neurocardiovascular diagnoses because of it. The main effects are wide fluctuations in both blood pressure and pulse, very little tolerance for anything that places strain on maintaining a normal BP, such as sitting up after lying down or standing up after sitting down or temperature changes. I get chest pain, have an irregular heartbeat, palpitations which in turn can affect my breathing, cause dizziness and lead to fainting. When it gets a little too cold, blood vessels contract too much, extremities go numb, I start to turn blue and get hypothermia. When it gets a little too hot, blood vessels relax too much, BP plummets and I pass out. None of this is usually more than an inconvenience when sitting quietly at my computer in a temperate room, but standing up is dangerous.
Pain – Pain is distracting and somewhat debilitating. I don’t let pain levels govern what I do, it’s not a path I want to ever walk again, but sometimes pain is a legitimate warning to stop. My pain tolerance is pretty high – it’s not that I don’t feel it, it’s just that I am content to tolerate it – and as a result, it becomes a background hum that I can live with. Occasionally, something happens, usually an injury or a headache, that pushes it over the tolerance threshold. At which point, I can no longer function. Pain becomes the universe and I can’t do anything other than struggle to endure it. That’s when it affects me.
Cognitive Dysfunction: One of those things that are difficult to quantify and isn’t mentioned often enough. As a result of blood flow to my brain being less than stable, having had more than my share of head injuries and suffering from a neurological condition, my brain no longer works as intended. I have memory issues. Big blank spots sometimes where bad headaches take over. I retain information well for about 24 hours or so and then it gets a little fuzzy and fuzzier and fuzzier. I no longer have free recall, that is I can’t recall recently learned material at will whenever I want to. I no longer have the eidetic memory I was born with, but thankfully, I still learn new information with the same skill and speed as I used to. I just don’t retain it for very long and have to rely on associative triggers to recall it. Ask me what spells a certain boss casts right now and I couldn’t tell you. Ask me a complicated name of a character in a book and I don’t know. But place inside Naxx and I as soon as we enter combat I can recall exactly what I need to do and where I need to go. Same goes for stats and gear. Sometimes, information that I am intimately familiar with is just temporary unavailable. I also have issues with verbal fluency. I know that the chair over there is called a chair, but don’t ask me to explain a concept by using words. I can show you, but I just cannot tell you. Don’t ask me how I reached a complicated decision, I can’t tell you right there and then. Cognitive fatigue is a big problem – the longer I concentrate on a task the worse my performance becomes. It is a skill to learn how to not let performance be significantly be affected by it – just never perform at your peak so that you can still do the job you signed up for when at your worst. Impaired planning skills – I can plan very well, I just can’t do it quickly. Cognitive Dysfunction does not mean that I am less intelligent or cannot do something, it simply means that it takes longer for me to do and it can be an exhausted and highly frustrating process.
If I included everything on this list, it would be a much much longer list. However, I don’t feel the need to include the things that does not directly affect what I do. The above list is intimidating enough as it is. My life is shaped and moulded by EDS and what I do today solely depends on how I am today. My decisions are often governed by the potential snowball effects. I have allergies and hayfever. I open a dusty book or receive some flowers from someone who means well and it starts with hayfever which triggers an asthma attack, which makes me cough which sometimes means I start coughing up blood (lungs really not in the state they should be) and as a result of crappy muscle tone and lack of coordination, that leads to choking and then the choking and coughing triggers a bad headache or dislocates a rib or both and then… I end up in situations where abovementioned factors spiral into a dark nightmare and I have little or no control over stopping it or slowing it down. I can live with the isolated events and the potential risk factors doesn’t really bother me often. It’s the potential for snowballing that really scares me. It starts with a small, insignificant gesture and I can’t always stop it from happening or escalating. It doesn’t happen every day, but living with the possibility that it may happen today is as difficult as living with all the complications of EDS mentioned above.