Your Questions 101: My Crutches part A

I’ve decided to start a series (even though I’m behind in others, sorry about that, but I’ll catch up eventually) on the most questions people ask me. This way, as more and more people ask the same ones, I can send them this way to give them the most comprehensive answers possible… with pictures!! So feel free to take this opportunity to ask as many as you like and I’ll answer them the best I can 🙂

Q: Why do you say you can’t walk without your crutches, yet now & then I see you without them?

This (or a version of this) is one of the questions I get asked most. In fact, often, it isn’t a question, it’s more a You don’t have your crutch!! I’m so glad you’re better!!! And when I try to explain that I’m not, most people don’t listen to the answer, they just tell me I must be better. So here is the answer….

With EDS, every single collagen cell in my body (over 70% of the tissue in all our bodies) is faulty. Each and every one of those cells has a tiny piece missing. It’s genetic, it’s like missing a leg, an arm, or anything else. Therefore there is no cure.

Tendons (the usually strong fibrous “strings” at either end of every single muscle in our bodies, that connect each on one end to the bones and the other end to the muscle) and ligaments (made from the same stuff but are much shorter and wider, less stretchy but much tighter and connect each and every bone to the next bone in every single joint of our bodies) are made out of collagen. Therefore, mine are not as strong as yours.

This means that:

  • My body uses up a LOT more energy than most people to keep it stable.
  • I still wobble a lot, even if I may not look like I do.
  • I fall over and bump into things a lot.
  • And when I do, my body receives an injury that another person would need a much greater trauma to produce.

Here are just a few of the many injuries that you may have known about in a time that you have known me (all pre-diagnosis):

  • I slipped and fell on my right wrist in Fiji. No bones were broken, yet I spent 11 months in a cast, had surgery on both sides of my wrist, wore a brace for 5 years, and still suffer from deep pain. The ligaments and tendons were damaged in a way that is not consistent with a simple fall, and have never healed fully, even though that was 17 years ago.
  • My right hip never recovered from three pregnancies and over time and without knowing what was happening, it is my most severe tendon/ligament injury. It has been excruciatingly painful for 29 years, and I did not realize that I was compensating for it by carrying the vast majority of my weight on my left hip/leg.
  • I tripped on a suitcase I’d left on the floor, on my way to the bathroom just over 3 years ago now. I landed on my left foot which twisted it. I couldn’t weight bear at all for months, and it took 5 months and an amazing specialist to eventually diagnose the Lisfranc Injury. The dislocation is extremely subtle, not unlike the Xray photo below, but it is none-the-less out of alignment because the Lisfranc ligaments have been damaged so badly and cannot repair themselves due to the weakness of my tissues. Lisfranc injuries are rare, with only about a 50% full recovery rate for healthy people. Those which don’t recover, require surgery to fuse the bones together, and even then, many of those patients are left with lifelong pain. Because of the fragility of EDS tissues, this surgery is categorically not an option for me. Therefore, my Lisfranc Injury (Frank as we call him) is permanent and will never heal.

The pain of tendon or ligament damage is excruciating. What makes any damaged tendons or ligaments in the leg/foot even worse is that weight bearing of any kind compounds them. For hips, even lying down, rolling over etc, contribute as well.

Basically, I can’t fully weight bear with either leg (hip on one, Frank on the other). Standing is VERY bad for me, walking less of an issue because I’m moving and not putting weight in any one spot constantly. But I HAVE to walk a little every day to keep my muscles strong and compensating. When Frank happened, and I was in a cast for 3 months, I couldn’t walk at all, the muscles wasted and my knees, feet, everything else, would dislocate just rolling over in bed. Don’t get me wrong, this still happens, but at least when I am strong they happen way less.

Our hands are not designed to take the weight of our bodies, and neither are our shoulders. So when they put me on crutches, the first things that went (and by “went” I mean excruciating pain as the tendons and ligaments stretched and broke) were my hands and wrists. So I cannot use “normal” crutches which are SOOO bad for everyone anyway.

Therefore I walk with a SmartCrutch. Invented, designed, and made here in South Africa about 7 years ago, which are my lifeline. They use my elbows to carry my weight. They provide a handful of lifesaving jobs for me:

  • They transfer my weight meaning that when I walk, Frank, my hip, and the crutch, each carry about a third of the load. That’s enough to make a HUGE difference, and that difference is the gap between being able to walk and not!
  • They keep me stable. I wobble less with them which protects ALL my joints, and I have gone from falling multiple times a week to having not fallen at all with my crutch. So they constantly save me from major injury.
  • They are a visible sign that I’m struggling. We don’t notice how much we jostle in life and bump and knock other people. It’s fine for most people, but for those of us who are a little more fragile, it is those bumps and knocks that hurt me. When I have my crutch people give me a little bit of extra space, possibly without even noticing. When I need to sit, it makes sense to strangers if I have a crutch in my hand. If I don’t have it, people think (and tell me) I’m lazy.

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But can I walk without the crutch? Sure.  But only on absolutely flat ground in the safety of my own home, the homes of my children and friends, … all places where I know, am not at risk of being bumped or pushed, and only for 20 minutes or so, and in fact, these bursts of walking are what keep me strong. They are what keep me moving. They are a necessity. But if I walk for too long, with or without my crutch, then I suffer for it terribly later.

And, walking is not the same as standing. I do stand, in fact, I often stand, and I ALWAYS pay for it later. I am also very bad, and when people tell me to sit, I often brush it off, which I shouldn’t do.

Where is the damage?

THIS is the best article on Lisfranc Injuries that I have been able to find, and it includes an Xray very similar to mine. You can barely tell the difference in the gaps on the left and right foot, but when our bones aren’t aligned correctly they pull hard on all the ligaments and tendons and cause other injuries.

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I’ve no idea of who Julio Jones is but THIS article has a good picture of the same Ligament that is damaged in my foot (though this is a right foot, mine is my left):

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Where does it hurt?

In THIS article on Lisfranc Sprains (another word for a mild Lisfranc Injury, but the position of the pain is the same) has a good diagram of where the pain is and is a good article on some of the issues:

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For the first time since I got my Smart Crutches, I am without them. In the rush to get out the door they were left behind. And suddenly I am extremely vulnerable. A walk 3 blocks up the road to the restaurant last night (leaning heavily on my husband’s arm for support) was not the same and I was in agony by the time we got there. Other outings are now not even an option. I often push myself too hard and too far, and I pay for it, but now I can’t do some of the very basic things I am able to.

Thankfully my crutch is on its way thanks to a number of people, but it has been a MASSIVE reminder to me of how much I rely on it every day for what other people see as very basic needs. It has also sparked a whole lot of questions which is great, and I hope this has answered all of them. If not, just ask!

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A Despicable Excuse to Touch!

I didn’t get very much sleep last night, and what I did get was wracked with nightmares. You see, the last thing I did before turning off the light was skim this article on the EDS Society Facebook page. I didn’t do that intentionally, it’s just what happened to pop up on my News Feed as I was closing down.

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In an instant, it brought back one of my own nightmares, and that was the end of any illusion I had that I could get some rest. Nights are difficult for me anyway. Every night is filled with deep, excruciating pain and discomfort so I have to manage my fragile environment in all kinds of minuscule ways; are there no cracks in the curtains? Are the right meds within reach? Do I have the pile of pillows on hand ready to prop joints in the dark when they strain or pop? Are the extra blankets within reach if the temperature drops more than a couple of degrees? is there water by the bed? And I need to decide which level of pain and sleep medications will I take before turning off the light.

Despite taking the strong meds I tossed and turned, flung pillows, and moved them constantly. I overheated and shivered in turn, and did something I never do, I took a second set of medications in the wee hours of the morning, knowing that that could well have consequences to the day ahead. But I desperately needed an escape that didn’t come, from my nightmares, ….and even more desperately I needed sleep.

Over twenty years ago, as an extremely naive and fragile young wife and mother with a family history of skin cancer, we had just moved to a new city and I needed to find a new doctor for a skin test. I’d been having them every second year for about a decade, and while in theory I was supposed to have them annually, I already had so many as yet undiagnosed health issues that finding new doctors filled me with dread and fear. So I was behind schedule already, and I had the extremely rare opportunity of having my mother-in-law in town, so I convinced her to look after the babies while I headed to the local GP for the first time.

I was used to skin tests, and with a variety of doctors and specialist. I was used to stripping to my underwear and lying on the examining table with a sheet to cover the bits he or she wasn’t currently checking. I had never been asked to remove ALL my clothes, as most underwear allows movements to the left, right, up or down, to cover every inch of skin. I’d I’d never been left without any kind of covering.

So I was left a little disorientated and uncomfortable when the young doctor insisted I remove EVERYTHING. There was nothing to hide behind, nothing to protect my dignity in any way shape or form, and I felt extremely uncomfortable and unable to protest without feeling like an idiot. I felt humiliated and vulnerable and exposed. I didn’t know what to do with my hands and I desperately wanted to cover as many bits as possible.

But there was nothing I could do so I gave myself over to him and closed my eyes, trying in vain to disappear to somewhere else in my head. I wasn’t all that good at doing that back then, and it wasn’t working, but I concentrated with all the strength I could muster. So when he suddenly spread my legs and proceeded to do an “internal examination” I got the fright of my life. I didn’t even stop to think, I instantly pulled away and protested, but he was insistent and told me he needs to check for suspicious moles on ALL skin, internal as well as external.

I could barely breathe, the level of violation became extreme and I felt totally powerless. I don’t remember getting dressed, paying for the privilege, and getting myself out of there, but I arrived home a mess.

My mother-in-law (a prominent Psychologist) saw immediately something was wrong, but I first needed to phone my husband and ask him to come home, then phone the local sexual abuse hotline. I spoke immediately to a social worker who assured me that on so many levels what the doctor had done was wrong, even if some internal skin needed checking (though in the two decades since I have never experienced it again). She gave me instructions on going to the police station to make a formal complaint on the day it had happened and talked me through the process.

I then told my mother-in-law what had happened, whereby she proceeded to scold me severely. She told me that bringing this onto my husband would bring him so much unwanted stress and added responsibility. That I needed to spare him the journey ahead and that if it led to court charges and court case, it would be an awful journey for my husband. She made it very clear that she would not be supporting me if I proceeded and that she would not encourage my husband to either.

I decided anyway that I would like to speak to a policewoman and so my husband drove me to the police station. I told her my story and she responded by telling me not to waste police resources, and that they only dealt with “real rape cases”.

I went home feeling more humiliated than ever.

And so it was decided by the authorities and those that I trusted and looked up to, that I would say nothing, do nothing, and that I would spend the rest of my life in silence… until last night!

I was 24 years ago in another city and another country, a lifetime ago… But it has simmered deep in the background of my soul till today. The violation, the humiliation, the control, the betrayal, … My heart breaks for those women in the article and their bravery at standing up to tell their stories for all the world to hear and see brings me to tears of thankfulness! Could I have been that brave 24 years ago? And if I had been, would anyone have listened??

PS: I’m so proud of myself, looking back, after the trauma of the incident as well as the compounded trauma of being forced to squash it, that nonetheless, I phoned and made a complaint to the AMA (Australian Medical Association) which I followed up with a written letter. In a small way, I spoke up.

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How the Dentist Went!

img_4162Well, I cannot tell you how petrified I was going to the dentist this morning!! It’s hard to tell in the photo (ever tried to take a pic of teeth with a phone???), but that crack goes all the way to the gum and it’s right through to the back. I’ve already lost a tooth because it cracked below the gum line, so I wasn’t holding much hope for this one.

I had also stopped going to the dentist because I was feeling I was getting nowhere. Mr. S goes to the same dentist I used to go to, and he’s happy but I’ve had two (yes TWO) fillings done twice over in the last decade or so, so I wasn’t feeling confident in him anymore. After my EDS diagnosis, I was sent to a “gentle dentist” to replace my old metal fillings. He did one-quarter of my mouth last January and I spent the next 3 months in agony and didn’t want anyone to touch my mouth again EVER.

That was till this happened over this Christmas!

Here is a quick sketch of my mouth, and that crack is through my right Lateral Incisor.

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I had no idea where to go, or who to trust, I am soooo burnt by doctors who are stumped and their pride gets in the way of real help. But Mr. S convinced me to go back to my old dentist and amazingly his wonderful PA welcomed me back with open arms and found me an emergency appointment.

Dentist pics 03I don’t want to bore you with basic Biology/Dentistry, but I knew the basics of teeth as this: Dentine (or Dentin in the US) makes up the bulk of our thinner front teeth, with the pulp & root at the very centre, and a thin layer of Enamel covering the part of the tooth that’s not hidden by the gums.

Enamel has NO collagen in it. Dentine, on the other hand, is mostly collagen…. and I was born with stuffed collagen. It’s too soft. I knew this, but most tooth pictures show the big fat back teeth which have much thicker layers of enamel.

So, anyway, between my natural fear of dentists, my fear of him not being able to save the tooth and what the outcomes of that could be, my fear that they will forget and there’ll be adrenaline in the anaesthetic (which is there is in all standard locals), and my worse fear that it will wear off too quickly without it (as usually happens, and they stitch me up without any), I couldn’t see this was going to go any way but badly.

I started by explaining that since seeing him last I’ve been diagnosed with EDS. I explained that more importantly, it’s a connective tissue disorder, and that my collagen is not as strong as it should be. He responded by pulling out his book of conditions and of course, it’s too rare so it wasn’t there.

But then he did what I have rarely seen a medical specialist do and he put the info he had together and went with it! He used his brain and on the spot worked out what was going on! I was so impressed I wanted to cry!

He said that the “crack” is right on the line of the filling that he did there 3 years ago, and that while yes, it goes below the gum line, it can be redone. He said that it isn’t a crack at all (even though he could wiggle the piece!!), but that the filling had  “debonded” (rejected, come unstuck) from the tooth! The bond of the filling was mostly with Dentine, which should be rock hard, but if it isn’t, then it can’t bond.

It might not be great news, but at least it makes sense now, and also explains why the other filling near the front of my mouth took 2 goes as well (and may need doing again). He said the cheapest option would be to replace the filling, which may not hold long-term of course, but both crowns and caps require filing back the enamel and bonding with the dentine, which would be a disaster in my case. An implant requires surgery and strong bone structure which of course relies on strong collagen in both the bone and the gum. So basically, we need to avoid implants, crowns and caps at all cost. Things which may be great for other people, but basically we have no option but to go with the simple filling.

The bad news is that this is going to be a problem long term, and I know EDSers who’ve lost all their teeth as early as 39. I’m passed that already but still, I have a mouth full of pain and it’s not likely to ever get much better. But at least now it MAKES SENSE which it never has before. There is a level of comfort in that.

Dentist Pic 04The good news is that he said, “well, we just have to make it stick more” and as he cut away the old filling to expose the raw edge of my tooth again, one normally leaves a sharp “flat” edge. But he decided to file away the edge of the enamel so that it has a bevel edge! While still being a very small surface, it’s twice the surface it would otherwise be! How clever, talk about thinking on your feet and making a plan!

The other thing he did is smooth some of the filling material so that it is overlapping some of the rest of the tooth. The idea being that the filling is now using way more of the enamel that it would have, and we have a chance of it lasting much longer this time. So the new tooth is slightly bigger than it was before and we’ll also have to keep an eye on that, but hopefully, it’ll be fine.

For how long? Who knows, and I think I’m going to be a bit paranoid about using that tooth, but I’m paranoid about so many of my teeth now that I’m running out of options 😦

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I have hope for the first time in a long time. And a few more answers!

So I’m going back on Tuesday and he’s going to check out the rest of my teeth and see if there is anything urgent and hopefully fix whatever it was that the stupid guy a year ago did.

I am pretty sure it’s the first time EVER I’m excited about going to the dentist!

 

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Which draws blood?

This is a follow-on from THIS story, where I tried to explain that it is not the obvious that breaks me. This is another really tangible way I can explain what it likes to have my particular Connective Tissue Disorder.

An Orange, or pair of scissors … which one draws blood?

I love scissors. I don’t know what it is, but cutting paper, fabric, anything really, brings out the creative best in me. A bad pair of scissors is a nightmare for anyone if they don’t cut well, dig into the skin etc, but with a good pair, one that fits my thumb and forefinger perfectly, I can spend hours cutting and I’m fine.

But I have never been able to peel an orange. There is so much about a connective tissue disorder that is very hard to measure, but orange peel is a great example of something that is measurable for me. When a healthy person digs their fingernail under the skin of an orange, even the most resistant peel will always give way before the fingernail gives way from the flesh of their finger right?

But for me, it is the other way round. Even the softest peel sticks stronger to the flesh of the orange than my fingernail sticks to my finger. It is always, and by always I truly mean always, the peel that wins and my fingernail that loses. It is extremely sore, especially as the acidic juice gets into the bloody wound…

So all my life I have either avoided oranges altogether (which I have become allergic to anyway, but that’s a story for another day), or used a knife and spoon to peel them.

You know how you physically cringe when you think of stubbing your toe badly or slamming your fingers in the car door? That’s how I feel at the mere thought of peeling an orange!

Invisible illnesses and conditions are very hard to measure; Pain impossible, along with his ugly cousin Fatigue.

 

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What does EDS FEEL Like?

Sometimes things are just easier said than written. I’m so uncomfortable in front of a camera but I am getting used to it with Facebook Live because I get to say things that would take forever to write (or read). As part of EDS awareness month, on Monday I did a video on the new criteria for EDS and why they are still a good thing for me, how they empower me, even though I technically now “fail” the test.

Today I got to speak about what EDS feels like. This was particularly meaningful for me because I could bring my actual voice into the whole thing and people could hear me as I speak instead of just in their own version of my voice in their heads. But I also talk with my hands a lot and so I got to explain things like the ways in which our bodies move and why that hurts, in a way that words on a page could never do… using my fists and my arms.

I’m not sure I’m getting any better at it yet but I am learning to feel more at home now that I have stopped trying to do all the things that people say you “should” do on Facebook Live streams (like introduce yourself and brag about who you are and what you do etc). I pretty much just get into it and I am enjoying that.

So HERE are this week’s episodes and what EDS feels like. Please watch and share for EDS awareness month!

EDS Facebook Live Front RoE

PS: If you want to win a signed copy of my book “The Lion and the Peacock”, and a pair of my Zebra earrings, then watch the video or sign on for details in this month’s “no spam” newsletter by signing on HERE 🙂

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To book a talk with me and discuss my schedule you can contact me on JPeaSmith@KingsRoadChronicles.com

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Unpredictability!

This morning my back went. Anyone with Eeds know what it means when something goes. It’s not so much about falling or banging into things, tho those are major issues too. It is about the smallest things sometimes, and the tendons twist and twang and tear and it is gut wrenchingly painful. I have passed out from the pain in the past.
Today was supposed to be my art day. This is a crucial part in my normalcy and something that I am forced to do less and less of. But this morning my back went. It doesn’t help that a misplaced hug a fortnight ago did my left arm in either, so I can’t hang on so well, or even roll over without help.

So in the blink of an eye I have gone from relative independence and a day out planned, driving myself, to needing assistance for everything from rolling over to sitting on the loo.

Thankfully my husband is not traveling with work and he was able to drop everything and come home. But it’s a huge scare and reminder of the unpredictability of our lives.

I am writing this one finger at a time, on my phone, feeling a mixture of thankfulness for this technology and frustration that it is so slow…

At least a have a bunch of books that I need to catch up on!! 

Vulnerability Exhaustion

img_8724This last week has been a hard one. I haven’t broken any of the rules; I have managed my rest, medication, self care, calendar and I have even been very good at asking for help when needed. In many ways, this past week has been the poster child of what a good chronic illness week should look like. …..Except for one thing: I have been plagued with an unusually high level of lethargy, brain fog and general (both physical an emotional) fragility. My body is far from it’s best despite the care, and my soul feels more than a little battered and bruised.

But worse than all of that is that I have been struggling to write, struggled to be with people without being completely wiped out, struggling with motivation during the day and sleeping like a log at night (which is unusual for me). For much of the past few days I have felt somewhat unsafe on all kinds of levels, and basically I just want to curl up in a ball and sleep for a week. To hide under a rock and never come out…

It isn’t depression exactly. I know what that feels like and how it tries to creep into my life and why. This is close to that but somehow a little different and I couldn’t quite put my finger on it.

But then today as I look back I realize a few things. The new specialist appointment this week was a new one. It required quite a long drive to a town that I have never been to before. That meant a lot more pre planning and leaving much earlier than I would need to if I knew where I was going. But that is OK, I planned for all of that in the schedule and my self care plan. (“pat myself on the back here”).

A new doctor also meant a much longer consultation, filling in loads of forms, answering nearly an hour of vital and valuable questions. But that too is OK, because I planned for this as well. I had my file, my list of medications, my medical history, everything that would be needed so that it was easier on them, and easier on me. (“well done Jenn!”)

Then it involved a physical examination as well. This was the “least bad” one that I have ever had. She could not have been more kind, more gentle, more helpful or more respectful. It was also both thorough and quick, she didn’t waste time checking anything that she wasn’t specifically looking for. I came home form this appointment feeling more positive than I have for a long time. It was a good one.

So why do I feel like this? Why do I want to hide from the world and why do small things like writing exhaust me? Only last week I wrote nearly fifty thousand words for my book and it empowered and energized me, but this week I felt exhausted by the end of the second paragraph here. I feel thoroughly depleted on so many levels.

So what is different? What is the new ingredient this week? What has wiped out all my emotional and physical resources? I realized this morning that I am suffering from what I call vulnerability exhaustion. Many people think of medical forms as purely factual and inanimate. But they aren’t at all. Every tick, every answer, every label, every diagnosis, …is a story, a journey. Each one was a failure, a triumph, a win, a loss, a fight or a battle.

Each and every small tick on the paper could represent a myriad of tests, a fortune that I didn’t have, ….weeks, months or often years of agony, denial, fear, outrage or even a journey to acceptance. Each single tick could include a number of life threatening experiences that we could easily have lost.

Over the years my body has been exposed, touched, poked, prodded, tested, analyzed, traumatized and worse… by hundreds and hundreds of individuals, most of whom would not even remember my name, ..or worse, never even knew it in the first place. And amazingly, each new doctor or specialist has the power to override every tick, to belittle every story, to brush aside every diagnosis, and with the strike of a pen to turn everything on it’s head. Which would be great if that was a good thing … but it rarely is.

It takes a tremendous amount of vulnerability to open up my body and soul, to expose myself to a complete stranger, to invite them in, to ask for their opinion, to let them have a say …yet again. For the potential gain of perhaps the smallest amount of life saving or life changing help, I need to take the risk, to give them my all, to expose the deepest, most painful parts of me, to condense them to a few paper forms and a handful of sentences, is mindblowingly scary.

I had never really thought that through before but this week I guess it was all amplified by a number of other vulnerable experiences like a particularly eye opening Psychologist session in the week and a couple of quite intense conversations with people who I don’t really know very well.

Everything about the week was carefully planned, but I think in the future I need to allow more for the effect of vulnerability exhaustion in all kinds of obvious situations.