Whether ’tis nobler in the mind to suffer
The slings and arrows of outrageous fortune,
Or to take arms against a sea of troubles
And by opposing end them. To die—to sleep,
No more; and by a sleep to say we end
The heart-ache and the thousand natural shocks
That flesh is heir to: ’tis a consummation
Devoutly to be wish’d. To die, to sleep;
To sleep, perchance to dream—ay, there’s the rub:
For in that sleep of death what dreams may come,
When we have shuffled off this mortal coil,
Must give us pause—there’s the respect
That makes calamity of so long life.
For who would bear the whips and scorns of time,
Th’oppressor’s wrong, the proud man’s contumely,
The pangs of dispriz’d love, the law’s delay,
The insolence of office, and the spurns
That patient merit of th’unworthy takes,
When he himself might his quietus make
With a bare bodkin? Who would fardels bear,
To grunt and sweat under a weary life,
But that the dread of something after death,
The undiscovere’d country, from whose bourn
No traveller returns, puzzles the will,
And makes us rather bear those ills we have
Than fly to others that we know not of?
Thus conscience doth make cowards of us all,
And thus the native hue of resolution
Is sicklied o’er with the pale cast of thought,
And enterprises of great pith and moment
With this regard their currents turn awry
And lose the name of action.

Hamlet, spoken by Hamlet

While Hamlet is speaking of a more permanent death, what I’m going to write on today is a smaller kind of death that is related to that of significant change that is not always something we choose to take on. The tiny deaths that happen every time a new part of us gives out and forces us to adapt our way of life.

For me, I’ve recently been facing this with my ability to create art. While I write in my spare time, I live for my art. It’s partly how I manage having EDS and its various complications. It’s how I grieve. It’s how I process some of my psychological trauma. It’s how I distract myself when pain is constant. It’s how I show love. It’s how I survive. I do so many different types of art that I can’t keep track anymore.

And now, EDS threatens again to take it from me. I faced this before just a few years ago. The connective tissue in my hands have always been extremely hypermobile, but my left hand had suddenly developed exceptional pain in my thumb. I couldn’t knead clay. I couldn’t bead. I couldn’t even wash my hair efficiently. Thankfully, I had just started seeing a rheumatologist that was a bulldog against my insurance and got me a full set of swan necks and a thumb plate for my MCP joint that started to fully dislocate with minimal effort.

I wrote a full other post about my ring splints to talk about it before, but it was done long after the period of adaptation. I was able to type without taking breaks to cry and scream how I hated the feeling. I had already started beading with regularity at that point, rather than throwing a piece only a few rows in because I couldn’t get the needle to do what I wanted. I was writing almost with the elegance I expected of myself, not the chicken scratch that I fought to get out with the new splints. I did this intentionally because I wanted to show that it’s totally worth it! It was worth all the screaming and crying over the difficulties and discomfort.

But, now I’m facing it again. My right hand…..my dominant hand….has betrayed me. The MCP freely dislocates and is constantly in significant pain. My hand that holds the paintbrushes. The hand that writes. The hand that holds the carving tools. That hand is now unable to hold anything without shooting pain.

The hurdle at hand (mind the pun) is whether I want to put in the effort to adapt yet again. It may seem to the reader, that this is an obvious thing. If art is life, then of course I would choose to adapt! While, yes, I’ve ordered a thumb splint…I assure you that it was not an easy decision or a simple and pleasant experience to decide this. It’s the turmoil of facing such a thing that I wanted to shine a light on.

The first aspect of this is the gut punch that is yet another failure of the body caused by Ehlers-Danlos. I’m angry. I’m short tempered. I’m heartbroken. I’m grieving. Whether or not I adapt, there’s a grieving process that comes with the small death of yet another thing I’ve gotten comfortable with having in my life that will cause significant change no matter what I choose to do. It happens with everybody and every body. Most experience it with old age, but some of us experience it younger than we ever should for various reasons. This only adds to the rage and grief. Angry at people who get to experience a full life of enjoying things they love without such challenges. Angry at people that have their mobility and abilities cut short from their own obvious poor choices because it feels like they’re reckless as a toddler. Angry at people with acute and curable hurdles because they can go back to normal. All of this is a lot of anger to process. I’m grateful to my sister, and I’ll take a moment to push her books by saying you should look up Kahaula on Amazon, for all of her help during this time to keep me grounded as well as helping with a safe space to scream without judgement.

As the grieving is processing, there’s the decision of whether to adapt or to let go. To be, or not to be, if we go back to the quote I used above. Because each option carries with it emotional baggage. Neither is the right answer or the wrong answer. They just are. And this is something of a problem in the chronic illness community as well. The overwhelming battle between saying “you’re not your productivity” while pushing “but don’t give up on things you like to do” to anyone facing such a problem. It’s a bit hypocritical and I don’t think we acknowledge that quite enough.

It’s not as simple as forcing yourself to be cheerful and pushing onward. It’s evaluating your own mental health and deciding what’s best for you in the long run. Because sometimes, giving up something you love, is actually healthier than trying to force yourself into adaptations because you are pressured into not giving up. Only you can make that decision for yourself, and that’s important. This is also a great point to mention that a therapist can be an important sounding board for such things.

For example, with my art, I know the new splint isn’t going to be comfortable, no matter how skilled the splint maker is and how perfect it fits. I know it took me several months to stop shaking my hands to stim from the discomfort last time. I know I’m going to absolutely hate the process of adapting because it’s learning skills all over again. It’s not a pretty process. It’s not like the inspirational video posts you see where someone disabled just tried hard, had a few frustrated moments, but pushed past it with joy and everyone was celebrating YAY! It’s trying.

The other side of this is knowing that it’s uncertain how long any adaptations I get used to will actually last. I **JUST** got back to painting my feathers even semi-regularly. Right after I fully adapt, I’ve got to go through the cycle **AGAIN**. It’s one more thing on top of the never ending accumulating mountain of things I’ve got to manage with this damned disorder and the constant malfunctions it causes.

It would be easier to just let it go. It would be less stress on me and my slowly falling apart hand. It would be less stress on those around me as I go through the emotional turmoil of the process to adapt. It would be more certain than trying only to possibly be let down as soon as I fine tune my adaptation. After all, I’m not my productivity. I’m not less if I come to the point that I can’t do my art. I’ll be sad, and I’ll grieve, and I’ll be angry…but I’ll eventually put my energy toward something else to keep my heart and soul occupied.

Is trying to keep doing art only speeding the decline of my hands? Am I creating a path to permanent disability that’s paved with dried paint and wood shavings? Could I extend the life of my hands by giving up most of my art? And could I live with myself if that’s what I chose to do?

There isn’t a right answer, only what’s right for me. Just as when you face your own hurdles, there’s only what’s right for you. I took a long time contemplating all the angles before deciding what was in my best interest. I can’t see surviving without my art at this stage in my life. As soon as I came to be aware of that, I made arrangements to order a splint to suit my failed MCP joint. And I’ll take all the rage and spite and fury and channel it into moving forward

I’ll cry and scream and probably throw a few innocent paint brushes. I’ll judge myself much too harshly for not adapting faster and producing the quality I expect from myself. I’ll get angry at the loved ones that remind me to be kind to myself on this journey. I’ll curse the gods and the body I’m in. I’ll do this all in spite of how much I’ll try not to. I’ll mask the myriad of emotions like the ocean hides Her rip currents until the wave becomes too much and crashes over. And I will do all of this not because I want to be an inspiration for everyone to push through and adapt no matter what. I will do this for one reason, and one reason only.

Because it’s what I’ve decided was important to ME.

Not to family that wants to see me continue. Not to people who want to commission me. Not to doctors that have their opinions on both sides. Not to people I work with that think I can adapt to almost anything. Not to my partners that both know what being able to create means to me. Not for other people to utilize for their own motivational needs.

For me.

That is what I want you to get out of this. Adapting is fucking difficult. It’s not a Hallmark movie with one sad day and a perpetual cheer squad. It’s horrible and I refuse to sugar coat it. It has potential for amazing things and I know that I’ll also cry tears of joy when I’m able to paint to my own standards again. I know the pieces I create out of all that anger and spite will be incredibly emotional statements that I can look back on. For now, it’s worth all of the struggles I’ll face in order to get there. While that makes it easier…it doesn’t make it EASY…and that is a significant differentiation.

It also brings me a great comfort that when I choose to let my artistic side rest for good, that it will be on my own terms and my own choice. I’ll know that I tried, and I know that I did everything I possibly could to extend the ability of my hands by doing things like wearing protective gear and mobility splints. So when that time does come? I don’t think I’ll ever be ready, but I’ll know it’s time. I won’t be a failure because I’m not somehow pushing through to find a way, just like I wouldn’t be failing if that was my choice right now. I’ll have the same comfort then, as I do now.

That the choice I made was mine, and for me alone.

I wish all of you the strength to make all of those hard choices that EDS, or any other hurdle, forces you to face for you, and you alone. Wishing it won’t hurt is futile, so instead, I’ll just wish you find peace in it and live your best life.

What’s The Core Issue?

A connective tissue disorder like hEDS means that our body has the wrong “recipe” for collagen. Collagen is the MAIN structural protein in your skin, ligaments, tendons, and many other areas in your body. Think of it this way; do you know those plastic rings on milk under the cap? Those are your ligaments. Strong, solid, and just flexible enough to allow for some movement. Now, do you know that thick, blue rubber band on vegetables like broccoli? Those are our ligaments. Thin, not as solid, allow for WAY too much movement, and a lot easier to damage with a lot less effort.

This is what causes our joints to easily hyperextend past the normal range of motion. While it doesn’t generally hurt to do it, it does still cause continual strain on those parts, just like continually stretching a rubber band to its limit causes strain. This also is what allows our joints to slip out of joint partially (sublaxation) or completely without needing a lot of force for it to happen as in a person with normal collagen. You can do a simple test of your own hypermobility by performing the tests on the Beighton Scale as the image shows below. Our joints allow for these movements easily, so please DO NOT try and force your joints to do these and if you CAN perform these easily…consider an appointment with your doctor to discuss your hypermobility.

How Does That Affect Your Exercise?

Syndromes like hEDS are a sliding scale and everyone has their problem areas, so it is a bit different for each of us. That being said, it affects our ability to exercise because we have to be as hyper-aware as we are hypermobile. Think back to the rubber band vs plastic ring analogy. Let’s take that and go further with it and use what seems to be everyone’s favorite suggestion…yoga. A lot of yoga deals with stretching, holding poses, and movement. It’s fantastic for so many reasons! It’s touted most for increasing range of motion and stretching out problem areas, but let’s look at that.

While there are many different types of yoga, remember back that our “rubber bands” already stretch and can tear easily. The “plastic rings” of the healthy connective tissue is what keeps the joints of a healthy person from slipping away from where they’re supposed to be and increasing mobility in a healthy and productive way while practicing yoga. This is REALLY IMPORTANT, the “rubber bands” we have don’t do this. Simple stretching can cause the muscles to move the joint away from where it is supposed to be, causing intense pain as well as damage to the surrounding tissue. This has less to do with “doing it properly” and more to do with “we’re not built right”. To give a more direct example, look at the simple stretch in the image below. Now, imagine that performing that stretch caused a rib to slip out of joint as well as the hip that is on the bent leg to slip and maybe even that knee when you try to straighten that bent leg again. And we’re just talking about easy stretching, not even into cardio yet!

So What CAN You Do?

It sounds pretty intimidating if you really think about it, but its not the end! First off, know your body and know your limitations. As stated above, syndromes such as these are a sliding scale so you have to be aware of your own problem areas. Second, discuss your options with a medical professional that is familiar with your situation if you can. I’ve had two incredible physical therapists over the years that have helped increase the strength of the tiny muscles surrounding the joint structures to compensate for my lax ligaments and eventually step up to increased ability to exercise! Third, try to learn to adjust and be excited for small victories and prepare for your progress to be slow going. Remember…you’re a zebra so you can’t expect to have progress the same way as a horse.

I know, that doesn’t go into any suggestions of what to do. That’s because there’s no one-size-fits-all suggestion. However, the important thing to take away is not WHAT you can do but to do what you CAN. Maybe this is only doing tiny little exercises that seem like you’re hardly doing anything for a year. Maybe this is swimming. Maybe this is walking. Maybe this is a stationary bike. Whatever you can do without causing further strain and risking injury to yourself? Be proud of yourself for putting forth the effort, no matter how small it seems or how slow the progress! Want to know what my “exercise” looks like for my legs? Something like this:

Sounds Like You’re Just Living in Fear and Making Excuses

Few things get me angrier than hearing a phrase like this. It an interaction where a phrase similar to this was uttered that motivated me to write this post if I’m honest with you. Constant suggestions of yoga and a myriad of other exercises that are considered “low impact” like bikes, ellipticals, light weights, and walking may seem like you’re encouraging. Most of the time, we even understand that you’re trying to help. But…sometimes we dislocate a hip by rolling out of bed or sublux a rib by taking a deep breath.

It also implies that all fear is negative an unproductive. You don’t have to be so afraid you’re trying to wrap yourself up in bubble wrap or never do anything enjoyable, but a healthy level of apprehension to your ability to perform physical activities will help you to stay more mobile for longer both in the short term and the long term of your condition.

Any physical activity comes with risk, even if you’re healthy. The difference with us zebras is that if we sprain an ankle on an elliptical…we’re not down for a week…we’re down for a lot longer and have a greater chance of surgery (which we shouldn’t be really subjecting ourselves to as I’ve said in an earlier post) than a person without a collagen disorder. Who in their right mind wouldn’t be a little bit more concerned about what they’re doing when it’s the difference between temporary pain and a high risk of permanent injury?

There is also a bit of victim blaming laced in statements such as that. Trust me when I say I would love nothing more than to go back to any of the activities I used to enjoy. However, I know even a low poundage compound bow poses incredible risk to the integrity of my shoulder joint. I could either be reckless and stubborn and try it anyway, or learn to accept the new limitations of my condition and make the effort to strengthen what I can as much as I can to ensure I have the use of my arm for as long as I can. Insinuating that not going and trying again is living in fear, is saying that I’m being weak-minded instead of working towards my best interest.

Statements such as these also insinuate that you are more aware about a person’s medical condition than they or their medical professionals are. I’m not saying that everyone is as hyper-aware as I am or that every doctor suggestion is to be taken without question. What I am saying is that if a medical team says “don’t do this thing for these reasons” and they’re known to be true or that if a person knows their hips easily dislocate…no amount of hopeful thinking is going to make it safe just because it’s low impact for a healthy person with normal collagen.

It is more important to try to live as best we can as healthy zebras instead of trying our hardest to pretend that we can be horses.

Going To A Surgeon That Knows EDS

I’ve always had weak ankles. They’d roll and give out walking on a flat sidewalk leading to constant sprains. My physical therapist said I had absolutely no planar stability as she held my ankles and easily shifted them away from the joint. My feet were flat and my body had built up thick calluses in the arch to compensate. Occasionally, I would sprain it severely enough I’d have to walk with a cane or take a break from walking for a day or too, but nothing too bad…until…October 2016. I had minor tears in my medial ligament.

After 9 months in a walking boot, I still hadn’t managed to have it heal completely and my rheumatologist was concerned about muscle atrophy so he sent me to a podiatrist that specialized in EDS. I researched everything I could before hand. I stalked the website of his office. I found a video on YouTube (that I’ll include below) that had him speaking at an EDS conference. I wanted to know everything I could before the appointment so I was prepared for bad news as well as able to discuss the procedures and why I didn’t want them. At the time, I wanted nothing to do with it and was convinced he’d say I wasn’t that bad and just needed some specialized therapy.

It only took a few minutes into the first appointment for him to say I needed surgery. “Have you looked up anything with your condition? Have you seen any of the lectures I’ve done?” I said that I did actually watch the lecture on the EDS Awareness page. “Good, so you know what procedures I do! We’re going to do a ligament replacement and an arthroereisis!” He explained that they’d only do local anesthesia and that his people were used to EDS so I would be in good hands and that the procedure had an amazing success rate. It was, of course, up to me, but at this rate I’m walking myself into a wheelchair if I continue. Despite my intense fear, the fear of not walking again was far greater so I agreed to the operations. Now, I’ve shortened my visit into a few sentences but he did take the time to explain everything to me and he wasn’t bullying as I suppose it could appear from how I’ve written it down. He really was kind and understanding and took the time to answer all the questions I had, I promise.

So far? Best decision I’ve ever made and I’ve since had the left one undergo the same procedures.

So What Exactly Was Done and How’s It Different?

The first procedure, the ligament replacement was done with a synthetic ligament and bone anchors from a company like Arthrex. This was important because this meant my body can’t replace the polyester with faulty collagen and had a very low rejection rate because of being artifical. It would go across the anterior talofibular and calcaneofibular ligaments to compensate for the fact they’re both more like rubber bands and can’t do their job to hold my ankle bones in place.

The second procudure, the subtalar arthroereisis, was a bullet shaped chunk of titanium that would be put the space between the talus and calcaneus bones called the sinus tarsi. This would further add stability to the ankle by preventing the bones sliding around and compressing the sinus tarsi.

Though the end result is a foot that isn’t as flat, it was the internal aspects of the ligaments not being allowed to stretch and end up constantly subluxed or dislocated that was the important outcome. And the x-rays of my foot show a noticeable difference. You can see where the one without the hardware is stretched farther than the one without. The first image can be noticed most where the ankle connects to the leg and the angles of where the joints are. The second one shows how far my foot was able to easily turn; it was so far you can see the bones of the outside of my foot in the hardware-less image.

Learning to Walk as the Bionic Woman

The healing, I’ve touched on in a few other early articles. It was slow, but not terrible. I’ve only had two teeny little incisions on either foot because it was done laparoscopic…so no horrible scars. Because I’m a zebra, I had a few complications with the first surgery. Despite that…I’m now walking short distances. So first surgery in September, second in December, walking in January.

The muscles in my legs feel a bit funny sometimes and I have trouble doing things like painting my toenails because I can’t just partially dislocate my ankle like it’s nothing anymore. I can’t put the soles of my feet together while my legs and hanging straight down anymore. I can’t stand on the side of my feet. All of which are fantastic, but it takes some getting used to having a normal range of movement.

I still have to take things like hiking and dancing slow because the newer surgery is still a bit on the sore side with a lot of work walking let alone using it on uneven surfaces or jumping. But I’ve yet to roll my ankle. The ligament pain that I was previously unable to escape from has been gone and not showing signs of returning. I can point my toes on the initial surgery foot. And all without the fear of my body slowly reversing the surgery because of the faulty hEDS programming it has.

It’s been a life saver for me and I feel blessed to have a surgeon close by that does this surgery as well as familiar with EDS to handle the more odd complications that we face.

Dr. Patrick Agnew and Ankle Surgery with EDS Patients

Patience… Is not my strong suit.

I had these procedures done in mid November. The short version is they replaced a ligament with a synthetic one and stuffed a bullet shaped implant into my ankle to keep it from falling out when I stood. I’ll elaborate later when I’m confined to a bed in recovery and need something to do.

It’s now mid October. The swelling is down, stitches have mostly dissolved. Last post op appointment the surgeon said I was “Healing like a teenager” and to start walking on it.

Okay

So… He didn’t exactly say start walking on it.

I was supposed to start walking on it GRADUALLY and SLOWLY increase the amount every week. If anyone reading this thinks I followed that order, my future posts are going to be a lot of fun for you.

To elaborate, I’ve not been properly walking since this time last year. I pulled a ligament in my right ankle and it never healed enough to reliably support my weight. Sometimes it would just randomly give up and I’d have to try not to fall over. Walking boots, constant ace bandage, walking stick, cane, support boots in the middle of summer. Can you blame me for wanting to be just that little bit independent again?

I did start out slow. Walking just to the bathroom once or twice a day. That quickly escalated to walking home from work and through the house at least once an evening. Granted, work is only 150 yards away. But I don’t think going from no weight on it to what adds up to a couple hundred yards a day… Is what he meant by gradual.

So today I’m paying for it. Massaging it is fine. No extra bruising or discolorations. But it feels like I’ve got a bruise on the inside on my ankle bones. Which…I should probably expect being there’s an implant in there that they need to adapt to.

But why do it? Why ignore the doctor’s orders?

Simple. Frustration. It sounds silly. I know. And it’s a terrible reason. Trust me… I am fully aware. But I’m a woman in her mid thirties that needs help carrying food from one room to another. I need help showering. My other half has to pick out clothes because I can’t get to my closets with the walker or knee scooter I have to currently rely on. I can’t even make, let alone get my own cup of morning coffee. I have to be walked home while I’m recovering in case I fall. I haven’t been on a proper walk in AGES.

Starting to see why I could want to break free ASAP?

But the pain today tells me that if I don’t regain my patience…I won’t be able to enjoy that freedom again. That I need to slow down and let those that care for me continue to help me. It’s either that… Or face that I’m risking needing a more permanent level of care.

That’s a difficult thing to face at times.