Living with Ehlers-Danlos Syndrome

So I’m sure many of you have wondered, what’s it like to live with EDS?

It’s a little difficult to describe, because I can never make up my mind whether I should include my secondary conditions. You see, the correllation between some of them and EDS seems to suggest there is some connection – so do I talke about EDS before I developed the secondary conditions, or after?

If I talk about it before, the main thing is that you’re constantly getting injured and the injuries don’t heal all that well. People think you’re terribly clumsy because you get hurt doing perfectly normal things like, oh, walking. At this stage, a lot of us don’t get diagnosed – I sure didn’t! You have to start getting careful about what you do, and listening to your body. In terms of preventing injuries, the best thing I ever did for myself was to take a yoga class. It strengthened my muscles (which helps prevent the kind of injuries we’re prone to) and improved my awareness of where my body was. By the time I had taken ballroom dance classes, if I was paying attention, I had a kind of precision that is quite rare. The only problem is that when I’m not paying attention, I don’t have it! So I still got injured walking around obstacles, bumped into tables and doorways, and the like. You get used to your body aching, because when your muscles have to hold your joints together, they get fatigued. And then there’s all the injuries. They linger, and ache, and create muscle aches because of the way the muscles get jerked around when your joints move out of place. But other than the low-level pain you have all the time, and the frequent injuries, you don’t seem that odd. I always had bruises, scrapes, and cuts from something, but everyone including me assumed it was just because I was a clutz. Yeah, I could do parlor tricks like touch my thumb to my forearm, or touch my foot to my opposite shoulder with ease. But that’s all, really, when you’re dealing with a comparatively mild case of EDS like mine.

When I got older, I started getting secondary conditions. At about 19, I had irritable bowel syndrome (IBS) show up, and suddenly I’d get diarrhea or constipation, or more often gas and cramps, for no apparent reason. I’ve dislocated about 3/4 of the joints in my body over the years. When I was 23, I developed fibromyalgia, leaving me with chronic pain everywhere. Fibro acts as a kind of amplifier of the nervous system. When something hurts, it REALLY hurts, at about three times the rate it did before. I have a hiatal hernia, which is to say that part of my stomach sits above my diaphram, which makes it easy for me to cough or laugh myself into vomiting. It also has given me acid reflux, so that I have to take pills to stop my stomach from producing so much acid and watch what I eat. My annoying bladder leaks all the time. My migraines are at least partially involved with my EDS, because the tension and instability in my neck and shoulders triggers most of them. I faint in the heat, or any time my hydration, salts, or blood sugars dip. I’m supposed to wear a whole pile of braces, but I have trouble with them – either they’re so hard to put on that I dislocate fingers doing it, or they cut into my easily torn skin, or they overheat me and make me more prone to fainting!

Now, I know that sounds like a pile of things gone wrong. And it is. But the thing is, I’m still happy. I can still go to law school, and I pursue my hobbies like spinning yarn, knitting and crochetting, painting, making jewelery, and sewing. Because of my disabilities, I have a wonderful service-dog partner. Because of my disabilities, I met some of my closest friends. I also have an awesome boyfriend, though meeting him wasn’t directly because of my disability (though I’ll tell you guys the story sometime, if you’re curious).

The big thing about living with EDS is that I could wish for a bit more undertanding from the world at large! I HATE being asked by strangers why I wear braces or have a service dog (though I explain the latter because having a service dog makes you kind of a de facto ambassador for service dog programs, not to mention the fundraising for your service dog program that you can do by educating). I’d really love if people wouldn’t assume that the girl with the dog must be blind. It would be great if people would ask me if I need help, or ask how they can help. I love when people open doors for me, even though I know many folks with disabilities don’t like it. I wish there were seats in more places, so I could sit down and take a rest when I’m out shopping. And for crying out loud, if you don’t know me, don’t tell me that you’ll pray I get healed! I was made this way, and I’ll stay this way thankyouverymuch. If you believe in a creator, then you ought to believe that their creations are intended to be the way they were made. I wish that doors everywhere had electronic openers, so that all I have to do is have the dog push a button and I no longer have to rely on the kindness of strangers to get in or risk hurting my joints. It would really be nice if the world was more willing to accomodate me without blatent signs of my need – no operators on our local public transportation would give me the disabled rate before I got my service dog, but nobody denies me that right now. And oh…oh do I hate being stared at. I’m not a show, I’m not a circus freak, I’m just somebody going about her day just like you. I’m tired of buildings that send me in a back or side entrance instead of having an accessible entrance right up front so I can walk in with everyone else. That, more than anything, is what I want – to be treated like everyone else. To have the same opportunities and the same access, to be viewed as just as valuable and just as welcome.

Phew, that got long.

In conclusion…if you want to know about EDS, about living with it, about being disabled…listen. Don’t disregard my voice. Don’t call me ‘too sensitive’. Don’t claim that no one could possible treat me the way I’ve been treated. Don’t deny my personhood, my existance, my life.

If you want to ask questions about anything I wrote here,or living with EDS, or being disabled, or specifics about how my body deals with things, or…well, anything at all, I’m officially opening the floor here. This is a get out of jail free, all questions accepted invitation.

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One in a Million

That’s the rate of Ehlers-Danlos Syndrome showing up in people. One in a million.

It makes it very hard to get diagnosed. When you are literally one in a million, odds are your doctor has never seen someone with your condition. To make matters more difficult, Ehlers-Danlos Syndrome has a fairly wide range of possible outcomes. The most recognizable symptoms – a particular set of facial features – are relatively rare. In order to diagnose EDS, a doctor has to look at your whole profile of symptoms. Otherwise, each individual symptom, each individual injury, looks like a singular event rather than a part of a vast web of symptoms.

There are some doctors who will tell you that if you aren’t in a wheelchair by the time you are 25, you don’t really have EDS, you have something like it but not as severe. Some doctors expect you to have the elastic skin that is common but not present in all of us. There is debate about whether hypermobility type EDS and benign joint hypermobility syndrome are the same thing.

Even if you do get your diagnosis, treatment is difficult. To be honest, the greatest difficulty comes again from the fact that there is such a wide range of symptoms. Most of us have fairly bad joint problems, but there are no set joints that are expected to be problematic. Some of us have gastrointestinal problems, bladder problems, central nervous system problems, heart problems, aneurysms, lack of muscle tone…the list is quite extensive. But because EDS is a syndrome – that is, a collection of symptoms that have been determined to be related – there is no guarantee that a person will have any given symptom. We also often present symptoms in atypical fashion. I have atypical trigger points: instead of having the typical point of tension, I tend to have ropes and bars of tension knotted into my muscles. I also have atypical bipolar, atypical skin sensitivity, and I’m sure there are other atypical things that I’m not thinking of off the top of my head.

Harder still is the fact that we are rather unpredictable in how we respond to treatment. A great many of us (myself included) do not respond normally to local anesthetics like lidocaine and novocaine. To have a dentist work on my teeth, I have to be injected with about twice the normal amount of novocaine, and it is a bit unpredictable how long it will take to kick in and how long the effects will last. I know a number of people who the same drugs have no affect whatsoever. My joints sublux, dislocate, and hyperextend doing exercises that are perfectly safe for most people. I’m highly sensitive to psychotropic drugs, but relatively insensitive to allergy medications.

The hard truth of having EDS is that you have to educate yourself. Why? Because we have to educate our doctors. Our doctors, especially general practitioners, rarely know much about EDS at all. It is not uncommon that they have a vauge memory of the involvement of stretchy skin and bad joints and nothing else.

We also have to be able to figure out when we’re getting adequate medical care. It is sadly not uncommon for us to be undermedicated for pain. There is a bizarre perception in some parts of the medical world that our condition does not cause pain. Especially when we are young patients, there is a tendancy to brush off our reports of pain, and as the doctors cannot actually measure pain…well, we suffer sometimes.

We have to know what the standard of care for our condition is, because again, our doctors often don’t. We have to know that we should be having yearly EKGs to check for deformities in our hearts and arteries that could kill us if untreated. We have to know about the kinds of braces used to treat our condition, because it’s rare for a doctor to treat the kind of joint injuries we develop. We need to know that our joints need protection and hyperextention is damaging, because unfortunately it is often written off by medical professionals.

We have to study not only the literature available on EDS, but also our bodies. We have to learn what our normal is, because it’s not the same as everyone else’s. We can’t take for granted what our bodies will and won’t do. We’re better judges of what assistive devices we need, and often we find ourselves in the position of convincing our doctors that the assistive device we choose is more help than harm.

EDS is a difficult condition to live with because of its complexity – it has an ability to reach into just about every part of your life. But that doesn’t mean that life can’t be good with EDS. You just have to be willing to walk, limp, or wheel a different path than most people take. What was it Robert Frost said about the road less travelled?

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Congratulations, boyfriend!

The boyfriend, who is an exceptionally wonderful guy, just finished his PhD and graduated yesterday.

I am so very, very proud of him.

He is a brilliant man, but he’d never admit it. It’s good to see someone else acknowledge just how smart he is! Maybe someday I’ll convince him to believe it.

We hope, someday, that he can teach collegiate level math, but for right now the market stinks and getting a job that matches his skills and degrees is hard. Keep your fingers crossed for us!

(on a somewhat different note – his college did a decent job of arranging for disability-friendly viewing of both his hooding ceremony and his graduation. They had both space for wheelchairs and rows that were only up or down a couple of steps for those of us who walk but not well. They also had designated people to help us get seats in said disability-friendly areas, which was a nice touch! We service dog users were treated with respect. Even the hotel we stayed in gave us no trouble. And late at night, when we went to get ice cream but the dog wasn’t in his harness, the ice cream shop didn’t hassle us either, once I said he was my service dog. It was a nice thing to be treated as we should be all weekend – like people, not an inconvenience.)

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The late night breaks are the hardest

One of the frustrating things about living with a lot of chronic health conditions is that you can’t escape watching them progress.

For me, one of the things progression means is more braces. A year ago now, I finally got insurance approval for a $1500 set of braces for my fingers.

I never picked them up.

They’re waiting for me now, in the same office as my physical therapist, who I need to go see in the near future. I desperately do not want them.

I don’t want my hands covered in silver braces that people will stare at and ask about. Bad enough that they feel no shame about asking me why I need a mobility aid – whether it’s my service dog or, in the past, crutches and canes. But to have them querying me about a set of braces that will ruin the slim, clean lines of my hands is particularly hard.

You see, I like my hands. I like them as they are – delicate, petite, long-fingered, slender hands. When I used to work Renaissance Faires, the men would all compliment me on having the hands of a lady.

But no more. My fingers will be bound in rings upon rings of silver. Piles of the damn things. Two to a finger, on bad days. On good days, I could probably get by wearing just 3 or 4 of them instead of all 8.

I’ll admit as braces go, Silver Ring Splints (http://www.silverringsplint.com/) are attractive and inobtrusive. But the fact exists that I will still have a pile of somewhat odd looking silver pieces wrapped around the joints of my fingers, and people will ask. It’s what they do, when it comes to disability – they ask without thought of whether they are prying or asking unwelcome questions. Whether they are prodding sore spots. What they really ask when they say, ‘Why do you have [disability-related aid]?’ what they are really saying is ‘What’s wrong with you?’

A bit rude, really, to pry into someone else’s health records like that. You wouldn’t go around asking random older males if they’re impotent, or asking every woman you passed if they might be pregnant. It’s no more polite to question people about assistive devices, because in all of those situations, you are asking for someone’s medical information, something we’ve deemed important enough to protect that we have laws in place like HIPPA that regulate when other people can talk about your health.

To get back to the topic, out of the blue, at 2 in the morning last night, this hit me. It’s been building for a few weeks, as they’ve tried to remind me to pick up the braces and I’ve selectively forgotten. I know it’s selective forgetting. That doesn’t help me with actually remembering it – I don’t want to.

But at 2:00 this morning, I had just finished my book, and I was far too awake. I turned off the light and tried to make myself start drifting, which will edge towards sleep a good amount of the time, but for some reason the splints rose up in my mind to torment me.

I started sobbing. I am lucky in that my boyfriend’s reaction to being bumped into and squeezed hard by a crying girlfriend some 3 hours after he went to sleep is to comfort, not to get annoyed at being woken. I cried, and I talked to him, and I cried some more. I tried to put into words why I found them so upsetting, and when it comes down to it, it’s a combination of vanity, fear/hatred of my disability advancing, and intense dislike of people staring and prying. There’s also some worry – I know that every new brace means getting your body accustomed to them, which is a very uncomfortable and sometimes painful process. I have a scar on the back of my calf from not being quite careful enough with a new ankle brace. The idea of having my hands be all painful again, when we finally got a combination of meds this time last year that gave me back my fingers, is pretty damn abhorrent.

The idea of people staring at my fingers and adults and children alike deciding to ask about the odd things I am wearing is very upsetting. It’s hard enough that about one in four people I have any contact with feel the need to comment about my service dog – that gets under my skin some days. Or, heaven help me, the sheer number of people who ask if I’m training Hudson, or why I seem to be able to see but have a service dog. On a good day, I can smile at them and explain and give them a card from my service dog organization that explains all the conditions the dogs can help with and includes a sample list of the tasks they do. On a bad day, I give them a much more frozen smile and tell them that I don’t walk well, and turn away so they’ll leave me alone.

I hate being this…visible. I hate the level of focus on my disability and the aids I need because of it. I hate the questions I get from people when they can see my knee or ankle braces. I don’t WANT everyone asking if I’m okay just because they see a new piece of equipment.

I’m sure a great deal of this sounds rather silly. Isn’t it supposed to be nice when people convey that they care about you by worrying about you?

Well, when it’s something that happens to you frequently, it’s not nice. It makes you feel like a freak. Some display of your disability/illness, hung up for the world to see, stared at and wondered over.

The hardest part is that there are few good ways to deflect the questioning. If I tell someone that their questioning is rude, oddly enough I am the one who comes off as being rude. If I’m abrupt in my answer or refuse to answer, much the same. I’ve taken to coming up with relatively short answers that don’t really answer anything. When asked about my knee and ankle braces, I just shrug and say, “It’s just new braces for the same old problems.” That tends to ease people’s worry without spilling my medical information to all and sundry.

But oh, if I could just get them to not stare, to not ask…if I could get them to look at me as no different than anyone else…how very much I would like it.

It would certainly knock out a big part of what keeps me from being happy about new braces that are supposed to improve my quality of life. Maybe then, my knee and ankle braces would come out of the closet and actually be worn.

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Ain’t I a woman?

In 1851, Sojourner Truth, a black abolitionist and former slave, delivered a speech at the Women’s Convention in Akron, Ohio. In her speech, she talked about the way women of color were not treated the way everyone seemed to be claiming women should be treated, and about the right to equality between women and men. (for anyone interested, you can read her short speech here – http://www.fordham.edu/halsall/mod/sojtruth-woman.html)

That is true, too, of women with disabilities. Without any further ado, my version of ‘Ain’t I a woman?’

They say that women should have the right to reproductive choice. But the same people tell me not to have children, and call me reckless, hateful, stupid, foolish, and mean because I might pass along my disgustingly flawed genetics, and even if I don’t, I’ll force a child to live with my so-terrible disability. They say I should not have the choice to reproduce. But ain’t I a woman?

They say that women are the equal of men in the workplace, and they have passed legislation that guarantees them equal pay for equal work. But they discriminate against me in hiring, and they cannot pass laws that guarantee me equal pay. My people work for a fraction of minimum wage through disability centers. Ain’t I a woman?

They say a woman has the right to the sanctity of her body. But when my people are sent to residential centers and they have severe disabilities, their caretakers are sometimes encouraged to have them rendered infertile so that when they are raped, at least they will not become pregnant. Aren’t we women?

They say a woman has the right to an education, the same right as a man, but when I sit there in my classes, they sneer at me and fight to avoid making it possible for me to take them. Ain’t I a woman?

They say a woman can choose who she has relationships with, and whether she has them at all. But the media and society say that no one would choose to love a grotesque figure like me. Ain’t I a woman?

They claim that we who are disabled have equality – laws that guard our employment, that call for accessibility, that regulate the way businesses and the government can treat us. And yet, the majority of my people live under the poverty line. We live in the streets. We can get neither work nor government assistance. We have no value, we are told, because we do not put out work. We should rely on the benevolent paternalism of charity instead of having the hard earned dollars of working people syphoned into programs that keep us in poverty but give us enough to keep from dying.

You may say that I am a burden. You may tell me that no one values me. You may patiently explain that I have no friends, no beloved, because I am not worthy of them. You may tell me that you will not hire me because my disability makes me less capable than the next person. You may tell me that I deserve to be in poverty and to not have money to do things that make me happy.

But there is where you are wrong. Why begrudge me my value as a human being? Why deny me the same achievements, the same desires, the same needs as you have? Why slash my value because of a disability that does not affect my ability to dream, to love, to be a human being?

We are one in five of the people around you. We slowly gain political clout, employment, and recognition. All we want is to be treated as human beings with the same value as you have, and one day we will achieve it. Better you stand with us, or out of the way if you cannot bear that, than that you stand against us. We will remember.

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My body REALLY doesn’t like exams this time

So, I mentioned earlier that I started my exam season with 2 ear infections and a sinus infection.

It got worse. On Saturday night, I ended up in the emergency room because the pain in my ear was so bad.

It turns out I additionally have an acute external ear infection. Great! Another infection, just what I needed!

I missed an exam, and then missed the day it was rescheduled to. Fortunately, I’ll be able to reschedule it (again), this time to Monday, so I should be well enough to take it.

The kicker here is that in addition to being sick as a dog, I’m having trouble concentrating. I thought it was just because I was sick, but now I’ve poked around online a bit and found that the antibiotic I’m on (augmentin) is occasionally reported to have a side effect that affects concentration negatively.

Well, this sure has been an adventure this go-round!

I have a pile of documentation I have to take in to school (perscriptions, ER release notes, a note from my doctor, the reciept from seeing the doctor).

The timing on this whole thing couldn’t be much worse. At least now I can walk around without holding on to things all the time!

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Getting it right – Blogging Against Disableism Day

One of the ways I think we can fight ableism is to talk about people who get it right. People who treat us as human beings, particularly people who are able to work with or around our disabilities and not treat us as our cultural status quo demands they should. We talk a great deal about the way the world gets this wrong, about the way we are shunted aside, about the way people turn our actual physical impediments into societal disability, but we do not often talk about when things actually go RIGHT.

(which is not to say that the discussion of how things go wrong is unimportant or finished – I think both halves of this issue MUST be publically, unceasingly discussed or we will never see change.)

So how do people get it right? The most basic thing is that they remember our humanity and respect our autonomy. To give a very basic example of how respect of autonomy works, the fact that I cannot cut up the potatoes I want for dinner does not mean that I cannot have them done the way I want. If a person who is aiding me to prepare my dinner asks me how I want the potatoes done, and does them the way they ask, then I still have autonomy because I was able to make decisions and direct outcomes. Autonomy is not about being able to do everything for yourself, it is about being able to direct your life and have things done to match your desires, whether or not you are personally doing them.

So who gets it right in my life?

The boyfriend.

The boyfriend and I have been together for over 2 years. He’s gotten used to the fact that if things need to be lifted, carried, or picked up, I generally can’t do it. That most of the responsibilities in the kitchen will fall to his shoulders, because my hands aren’t up to it. He even carries my purse when we’re out and about without getting goofy about it the way some guys do.

He’s also learned the boundary between things I can sometimes do and things I really shouldn’t do. So he’ll ask me how near to our destination I need the car parked, because some days I can walk 4 blocks and some days I need to be dropped off at the door. He doesn’t ask if I want him to take the bag after I’ve made a purchase, because we both know I shouldn’t be carrying things. It’s a delicate balance, between helping too little and helping too much, and it requires a great deal of attention to maintain.

He does not take advantage of my disability. A great many of people with disabilities, especially women, find themselves abused by their caretakers and/or partners. Any time you have a power imbalance, there is a risk that someone will take advantage of it without regard for the harm they do. I think the boyfriend would be nauseated at the idea of ever taking advantage of me in any way, and for that particular aspect of his character I am very grateful. I know, as a woman with a fairly severe physical disability and an emotional disorder, that I could easily be abused and harmed by a significant other – and some of that has befallen me in the past. But with the boyfriend, I am safe because I am never less than a person, never less than he, in his mind.

My doctor.

My doctor never talks down to me. He remembers that while I am the patient, and much less knowledgable about medicine (not to mention unable to look at places like the inside of my own ears), it is my body he is treating. When there are treatment options, he tells me, and explains why he prefers one direction over another. He does not tell me ‘You Must Do X’, he tells me ‘I think we should do X.’

It may not seem like much, but it’s a world of difference to me. Especially with complex, chronic patients (like yours truely), doctors forget sometimes that they are our advisors and employees, not gods. It’s a level of respect for my right to have input into the decisions about my bodies that is unfortunately rare.

Additionally, I never feel as if I am an illness to him. I am a person, a person he remembers is a law student who loves crafting and art and has a plethora of hobbies. It does not matter whether I am seeing him for a new issue (like my ear infection) or a chronic issues (like my back), he listens well and considers how my many medical conditions could be interacting with the issue we’re discussing. He does not grow frustrated with me because of the way my body reacts, nor am I ever abused for not being able enough or healed enough after his intervention. To my doctor, I am a patient he helps to be healthier, not a lump of flesh in need of his fixes and treatments.

My Personal Care Assistant

My personal care assistant, Nikki, has been with me for 15 months now. The relationship between a personal care assistant and a client can be very difficult to navigate. In most cases, a personal care assistant is responsible for a lot of taking care of the home and the body of a person with disabilities. There are a great many landmines that show up in the relationship between a PCA and a client.

My PCA does things my way, even if she’d prefer to do them a different way. Yeah, she thinks it’s a little funny how obsessive I am about cross-contamination, but she’s respectful about it and keeps my kitchen safe and clean. She remembers how insistant I am about having my books organized by author and series, so that I can quickly find what I’m looking for. She doesn’t complain about the messes I end up making and not being able to clean up because of my disability. When approaching things she doesn’t know the answer to, like how I want things cut up for dinner or where a new item should go, she doesn’t just decide for herself and hang me with the consequences if I don’t like it – she asks me, and she does what I’ve directed.

She also does not treat me as a paycheck. I know that some PCAs get into this industry because they just need a job and someone to pay them. Nikki actually cares about me as a human being, and we joke about our significant others, the state of the world, our neighbors, and so on. She does not lie to me. She does not steal from me. She contacts me if she is going to be late or if she cannot come so that I can get someone else to take care of me. She does not refuse to do things I request that are part of her job (and actually, is willing to go pretty far outside of her actual job requirements if I ask for help). She does what I request, when I request it. She does not make me feel like a burden. She does not use her power over me to harm me in any way.

All this may seem like things you would assume to be true about someone in the service industry, but I can assure you that I have known of PCAs who violate all of these trusts and more. Me, well, I’ve been lucky, as Nikki and the boyfriend are the only people who have ever worked for me as PCAs, and both of them have the basic respect and decency to remember that I am a human being worthy of dignity and respect. I consider Nikki a friend as well as an employee, and I value her greatly.

To sum this up, there are a great many ways that we as people with disability are vulnerable to abuse. That vulnerability springs in part from our impairments themselves, but mostly from the societal view of us as lesser beings, in much the same way that a woman’s vulnerability springs both from being (on average) less physically strong than a man and from societal ranking of women as less than men. Part of fighting that abuse is talking about it, but another part is talking about relationships that are safe, that do not involve abuse. Healthy relationships. Relationships in which we are equals, in which we are people, in which we can trust.

And so, people of the world, whether you are people with disabilities or people who are ablebodied, I challenge you to think not only on living with ableism, but to think also of times and places where we are do not.

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