Tag Archives: difference slot

Almost Alike: A Medical Cautionary Tale

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Blue medical bracelet with a medical symbol in white and the words "Adrenal Insufficency" on a metal plate.

Medical bracelet that says “Adrenal Insufficiency”.

I’ve been thinking about medical stuff a lot lately, so apologies if my posts tend towards the medical for a little while.  It’s what happens when you suddenly realize how lucky you are to be alive, and how close you came to death.  My father’s cancer has me thinking about life and death and medical care a lot, too.

In my dealings with doctors, I have found that they like the solutions to their problems to be neat and tidy.  In particular, they want there to be one diagnosis that explains all the symptoms they’re observing.  They want their patient to have that one diagnosis, and if their patient shows signs of more than one thing, it fouls up everything the doctor wants.

Case in point:  I had this neurologist at the headache clinic.  I told him that they strongly suspected my mother of having myasthenia gravis, or hereditary myasthenia.  Both are neuromuscular junction diseases that cause specific muscles to wear out quickly as you use them.  So for instance my eyes start out tracking the same object fairly well, but as time goes on, they drift outwards leaving me seeing double.  I had told my neurologist all about this, and about other muscular problems I’d been having.

I don’t remember why myasthenia came up, but I told him I was going to start on Mestinon, a medication that treats myasthenia.  His response was swift and a little annoyed:  “It’s not going to do anything.  I don’t think you have myasthenia.” 

“Why not?”

“Because people with myasthenia have trouble with specific muscle weakness. You have generalized weakness.  It’s not the same thing.”

He explained it as if I didn’t know this.  But he also explained it as if I hadn’t told him time and time again about the specific weakness, that was separate from the generalized weakness.  As if I hadn’t told him things were more complicated than he was expecting.

He offered to run an EMG but told me the results would be negative because “You just don’t have myasthenia gravis.”  I declined the testing.  I don’t like to be tested under circumstances where the doctor has already determined what the results are going to be.  Plus, I’d just been through an invasive procedure that left me in horrible pain for weeks, and I didn’t feel like being poked and prodded again.

But I did try the Mestinon, and it did make a difference.  It was subtle at first.  I could walk around my apartment without falling.  My eyes tracked things better, and for longer, before the double vision kicked in.  It was things like that.  The more Mestinon we added, the better those things got.  So it seemed my headache doctor was wrong, and there was something real about the effects of the Mestinon.

But in other areas, I was getting weaker.  In fact, as far as I could tell, I was dying.  I was hesitant to tell anyone this fact, because it felt like a fairly dramatic thing to announce.  But I’d known terminally ill people who had more energy than I had at times.  And I have instincts that tell me when something is going badly wrong.  Something was going badly wrong, and it went along with that more generalized muscle weakness.

I’ve already told the story of how I got diagnosed with severe secondary adrenal insufficiency.  And that’s what happened.  They found no measurable evidence of cortisol or ACTH in my blood.  When they flooded me with ACTH, I made cortisol, but not as much as expected.  Meaning my pituitary gland is not making enough ACTH to tell my adrenal glands to make cortisol.  And this was the reason for, among many, many other symptoms, my severe muscle weakness that affected my entire body.

I went into treatment for adrenal insufficiency and everything seemed to be looking up.  No longer bedridden.  No longer required to use a wheelchair for anything.  Not that I minded these things so much when they were happening, but it’s nice to be able to get up and walk up and down a flight of stairs when you want to.  It feels good to be able to exercise, after six years of bedrest.  Dexamethasone makes me feel alive again, instead of waiting for the next infection to kill me.   I feel strong, and sturdy, and robust, in a way I haven’t in years, and my friends sense the same thing about me.

The only problem?  Not everything went away.  I still had weakness in specific muscles.  I’d been referred to a new neurologist at the same time they were testing my cortisol.  This neurologist never pretended he had any answers.  He was simple and methodical in the way he worked.  He would come up with a list of every possibility, no matter how remote, and then he would run tests for every possibility.  This made me trust him in a way that I didn’t trust my migraine neurologist.  So I let him do any test he wanted to do.

Many of the tests, he came in and did them himself, which is unusual for a doctor.  Usually they delegate that stuff.  He did a regular EMG that turned up nothing, and I thought “See, my mother didn’t have an abnormal EMG either, so whatever we have isn’t going to show up on tests.”  Neither of us showed up as having the antibodies, either.  I began to think this was going to be one of those things that we never solved.

Then he called me in for something he called a single fiber EMG.  He was going to stick a wire into my forehead and measure something about the muscles.  I remember that on that day I had a lot of trouble even holding my head up on one side, and that I was seeing double.  He stuck the wires in, made me raise my eyebrows and move my eyes around.  There were a lot of electrical noises.

At the end of the test, he told me he wanted to see me as soon as possible because the result was abnormal.  The muscles were firing asynchronously. 

I didn’t know what that meant, but a week later I was in his office being told that I probably did have a neuromuscular junction disease after all.  Probably myasthenia gravis, possibly a much rarer hereditary form of myasthenia.

And to think that literally a couple weeks before I got the single-fiber EMG, my regular doctor and I had been discussing whether I really needed to be on Mestinon anymore.  We thought maybe my only real problem had been the adrenal insufficiency all along, and that my response to Mestinon might have been some kind of placebo effect (even though I don’t seem very prone to that effect even when I want to be).  Even I was starting to fall prey to that idea that a diagnosis is just one thing.

Right now, we don’t really know what exactly my diagnosis is.  We know for certain that I have secondary adrenal insufficiency.  And we are pretty certain that I have a neuromuscular junction disorder, and the most common one of those is myasthenia gravis.  (I’m just going to refer to it as myasthenia gravis for the rest of this.  Because it’s shorter than saying “the thing we think is myasthenia gravis maybe”.)

But the important thing — the thing a lot of doctors miss — is that there is not one diagnosis here.  There are at least two diagnoses, possibly more.  This is not the first time, and it won’t be the last time, that I’ve had doctors miss something fairly obvious because they thought that the simplest explanation is always a single diagnosis. 

I still remember back when I was dealing with three different diagnoses that affected movement in different ways:  Adrenal insufficiency, myasthenia gravis, and autistic catatonia.  And any time we’d try to bring up a symptom of one of them with a doctor, they’d bring up a “contradictory” symptom from a different one of them, and that would mean that… it couldn’t be myasthenia gravis, because sometimes I froze stiff instead of limp, because I also had autistic catatonia.   And it went on like that for years, where every condition I had was ‘contradicted’ by some other condition, so many of the doctors refused to see the complexity of the situation.

Sometimes that resulted in situations that were almost funny, but other times it could turn deadly.  There was a time I was hospitalized for aspiration pneumonia connected to gastroparesis, and my doctor refused to treat me for anything other than the pneumonia.  So I had collapsed in my bed after vomiting so much that all the muscles involved had gone limp and I was starting to have trouble breathing.  In retrospect we think it was the start of an adrenal or myasthenia crisis, and that I belonged in the ICU.  But at the time, the hospitalist simply refused to treat anything that wasn’t pneumonia.  So I had to lie there totally immobilized, delirious, and hallucinating, wondering whether I was going to survive, for days on end.  All because a doctor was only willing to think about one condition at a time.

Over the years, I’ve picked up an impressive collection of diagnoses.  Many of them are based on symptoms and my response to treatments.  But some of them are based on hard-core medical tests like high-resolution CT scans — things you can’t confuse for anything other than what they are.  I’m going to list the ones that  were diagnosed by those hard-core medical tests, and understand I’m listing them here for a reason:

  • Bronchiectasis (high-resolution CT scan)
  • Frequent bowel obstructions (x-ray)
  • Central sleep apnea (sleep study)
  • Obstructive sleep apnea (sleep study)
  • Early-onset gallbladder disease (ultrasound)
  • Exotropia (eye exam)
  • Gastroparesis (gastric emptying scan)
  • GERD – reflux (barium swallow)
  • Esophageal motility problems (barium swallow)
  • Dysphagia (barium swallow)
  • High cholesterol (blood test)
  • Hypermobility syndrome (Brighton criteria)
  • Myasthenia gravis or related condition (single fiber EMG)
  • Secondary adrenal insufficiency (cortisol test, ACTH test, ACTH stimulation test)
  • Urinary retention with spastic urethra (urodynamic testing)

So this is fifteen different conditions right here, that there is no possible way that I don’t have them.  They’ve been tested for, the tests are valid, there’s nothing unusual about the tests I was given, they exist.  I’m diagnosed with a lot of other conditions, but even if we pretended that those conditions turned out to be misdiagnosed because some of the diagnosis was subjective… I’m still left with fifteen conditions here that are very much real.  Some of them are more serious than others.  But many of them are difficult and complex both on their own and in combination with each other.  (Also, many of them went years misdiagnosed because doctors refused to even test me for them, believing that a person with a developmental disability or a psych history couldn’t possibly be telling the truth about their own symptoms.)

Now imagine you’re a doctor, and I’ve walked in your door, off the street, with no medical history.  And I’ve got the symptoms of all of these fifteen conditions.  Some of the symptoms are severe enough to be life-threatening.  And your very first instinct is to try to find one condition that accounts for all of these symptoms.  You’re going to be looking for a very long time, and you’re going to be lucky if I don’t die before you figure it out.

Of course, it’s still possible that there really is one condition that explains all this.  Or at least, a small handful of conditions.  There are many genetic conditions that can cause problems all over your body, and they can be notoriously difficult to pin down.  But for the moment, we’ve had to diagnose all of these things separately in order to get a handle on how to treat them. 

It may be there’s some genetic condition that causes neuropathy (my mother and I both have symptoms of autonomic and sensory neuropathy), which could in turn cause the gastroparesis and esophageal motility problems (and dysphagia, and other things that aren’t listed above), just as one example.  But right now we don’t have that information.  Right now we just know I have gastroparesis, and that it doesn’t play well with reflux and bronchiectasis, and that if I hadn’t gotten a feeding tube in time it probably would’ve killed me.  There could also be something behind the adrenal insufficiency, but that damn near did kill me a number of times before we even knew enough about it to put me on dexamethasone. 

And that’s why it’s important that medical professionals not restrict themselves to a single diagnosis when they’re looking at what’s going wrong with someone.  If you see symptoms that look contradictory, then you ought to be wondering if you’re looking at more than one condition at once.

If there’s one thing I have noticed, having been in and out of hospitals for a long, long time… it’s that my roommates are usually people like me.  They’re people with multiple medical conditions all at once.  They’re not textbook illustrations of a single condition in all its pristine glory.  They’re a mess, just like me.  Like my roommate who had both Lesch-Nyhan and myasthenia gravis (and was a woman, which is rare for someone with Lesch-Nyhan in the first place).  They really treated her like crap, too — they wouldn’t believe a word she said about herself, unless they could verify it from some outside source, which they always did, but still never trusted her.  Sometimes I heard her crying after they left.  At any rate, I can’t remember a single hospital roommate who had only one condition, unless they were in there for a routine surgery.

Which tells me that those of us who end up in hospitals on a regular basis, at least, are people with complicated medical histories.  Not people who just have one simple thing that can be figured out.  Which means that no hospitalist should ever do what one of mine did and say “I’m only treating the pneumonia, nothing else matters, no matter how bad things get.”  I’m really passionate about this issue because I’ve seen how close to death I’ve come, how many times, just because everyone wanted my body to be simpler than it was.

Maybe the problem is that we train doctors too much on textbooks, and on the people who most resemble textbooks.  We don’t want to confuse them with too much, all at once.  So they grow to look for the one explanation that will explain it all, instead of the fifteen or more explanations that will explain it all.  And in the meantime, their patient could die while they’re waiting to get properly diagnosed.

And that’s the part that worries me.  I’m very lucky to be alive.  My doctors know I’m very lucky to be alive.  And I have a pretty amazing team of doctors.  I have a great GP, a great pulmonologist, a great neurologist, and a great endocrinologist.  These are doctors who are willing to listen to me when I know more than they do, but also willing to argue with me when they know more than I do, it’s the perfect combination. 

My GP has been here since I moved to Vermont, and he is known in the area as one of the best doctors around.  We have our disagreements, but he always explains his decisions to me, and I always explain my decisions to him.  We respect each other and that makes everything work.  He has done his best to stand up for me in situations where my social skills have caused problems with other doctors.

My pulmonologist is amazing.  She always anticipates situations where I’m going to face discrimination, and she’s always ready.  When she knew I was heading for a really bad pneumonia, she had my lungs CAT scanned to prove the pneumonia was there, because she knew nothing less than that would get me admitted to the hospital.  And even then it took all she and my GP could do to get me into the hospital and keep me there long enough to get me a feeding tube.

I’m new to my endocrinologist, but he’s clearly really good too.  He’s been helping me through the first stages of being diagnosed with adrenal insufficiency, including things as difficult as when to stress-dose and how much.  He’s given me the confidence to figure out on my own the amount of steroids I need to give myself in physically or emotionally stressful situations.  That’s a key skill you have to have to avoid adrenal crisis, and I think I’ve finally got the hang of it.

My neurologist is also new, but he’s clearly highly competent.  There’s nothing flashy about him or anything.  It’s not like he has some kind of flashy swagger like you see on TV shows.  He’s very quiet.  What he has is the ability to be mind-bogglingly thorough.  He listens to everything you have to say, he asks very careful questions, and he takes very careful notes.  Then he thinks up every possible condition that could result in the symptoms you have, no matter how rare or improbable it seems.  Then he figures out which ones are the most important to test for first.  And then he pretty much tests you for everything.  If there were two words for him, it would be methodical and thorough.  And it’s paid off — we now know I have something similar to myasthenia gravis, even though all the signs were pointing away from it for awhile.  Like my GP, he’s one of those doctors that other doctors hold in very high regard.  I can tell by the way they talk about him.

I wanted to make a point of talking about these doctors, because the point of this post is not to bash the medical profession.  These are people who have saved my life.  These are people I have built a relationship with over the years, or am in the course of building a relationship with now.  I’ve had plenty of truly awful doctors, but I’ve had a surprising number of truly great ones as well.  Most are somewhere in the middle.  But the great ones are the ones I owe my life to, many times over.  They have done things for me that, I am sure, they have never even told me about, and probably never will.

But all doctors, no matter how great, need a reminder that medical conditions don’t come in neat, orderly packages the way the textbooks make them sound.  Most disabled people and people with chronic illnesses have multiple conditions, not just one.  Often, these conditions have symptoms that can seem to contradict each other.  And even when there’s one overarching condition that causes all of them, there’s a good chance you’re going to need to find all the smaller conditions before you can put the puzzle together.  Many times, finding all the smaller conditions is a matter of life and death.  People simply can’t wait around to find the perfect most elegant answer when we’re going into adrenal crisis or myasthenia crisis on a regular basis.  Maybe there’s a reason I have adrenal insufficiency, and maybe one day they’ll find it, but for now I need to be on dexamethasone so I don’t die in the meantime.

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Please violate only one stereotype at a time.

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What seems like a really long time ago, elmindreda wrote about The difference slot. The idea being that:

The basic idea is that each and every person has their difference, and that it should be respected. Note the singular form, however. When they learn of my autism, which is usually the first major difference to come up in conversation, they seem to think “oh, so that’s her difference”. They then proceed to fill in my difference slot in their mental table, and everything is as it should be.

Or, so they think.

Then, a little while later, I happen to mention some other thing that makes me very different from most other people, and their belief system collides head-on with reality. Usually, it’s another one of my disabilities that triggers it. This is when they almost invariably go “…” for a while, only to finish with “you have that too?” In other words, “your difference slot is already filled, and you can’t have another one”.

What I’m writing about is similar, but perhaps from a different angle. A phenomenon I’ve seen over and over again runs something more like, “Please violate only one stereotype at a time.”

This can apply even if you only have one “difference” (be that autism, physical disability, whatever).

If you have several differences, of course, the problem becomes exponentially harder to deal with.

And there are a number of different ways to deal with the prejudice you encounter where people might be able to handle you violating one stereotype, but leap all over you if you happen to violate more than one.

Some (false) stereotypes I happen to violate, just by way of example:

  • People who use mobility aids such as wheelchairs, canes, crutches, walkers, etc. must use them full-time.
  • Autistic people who are non-speaking or nearly so, must always have had no speech.
  • Autistic people who are non-speaking or nearly so, must be non-speaking entirely because of autism, not because of something else.
  • Autistic people can never pass to be normal or ‘just eccentric’.
  • Autistic people can only pass for normal or ‘just eccentric’, never for anything else.
  • Autistic people who pass can never stop passing, or if they do it’s always by choice.
  • Autistic people are only allowed to lose certain skills within a short window in the first few years of life.
  • Autistic people only lose certain skills right after a vaccination.
  • Autistic people, when they lose skills, only lose skills because they are autistic, never because of anything else.
  • Autistic people, when they lose skills, never gain skills at the exact same time.
  • When autistic people lose skills, it’s always immediately obvious to everyone around them that this is what’s going on.
  • The time an autistic person is diagnosed reflects the time that they became (or appeared) autistic, rather than the time anyone else noticed.
  • Everything unusual that an autistic person does is because they’re autistic, they never have additional conditions (i.e. the “difference slot“).
  • Autistic people are completely unaware of other people and their surroundings.
  • Autistic people can’t communicate at all.
  • Autistic people live in their own little world.
  • Autistic people have one pattern of mannerisms all the time and never vary them and never lack those mannerisms altogether.
  • Autistic people are incapable of love.
  • Autistic people can only be interested in one thing.
  • Autistic people who have an interest in people always look (to people who think that standard gestures of interest are the only way of showing interest) like they have an interest in people.
  • Autistic people can either speak to communicate or not speak to communicate, never alternating between both, and certainly never some odd in-between state.
  • Autistic people who chatter on and on about their interests are a ‘kind’ of autistic person, and that ‘kind’ of autistic person never has trouble communicating in speech and/or language.
  • When an autistic person needs everything the same, you can really tell.
  • Autistic people who have meltdowns do it for no good reason.
  • Autistic shutdowns always take the form of falling asleep.
  • Autistic people are never classified as gifted.
  • People classified as gifted never lose that classification as they get older.
  • When autistic people violate stereotypes (such as, in my case, doing things like failing to hide my facial hair), it’s because they don’t know any better, never because they have made a reasoned choice to do this.
  • Autistic people who do advocacy work don’t really care about other autistic people, they just want to make trouble and/or go on an ego trip.
  • Autistic people who do advocacy work or other work that pertains to autism can really only speak from their own experience, they never have expertise from other sources than their own experience.
  • People who can’t take care of themselves, can’t take care of anyone else either.
  • People with movement disorders always have the exact same degree of difficulty with movement in all situations.
  • People with movement disorders have the same degree of difficulty with all forms of movement.
  • Disabled people have no sexuality.
  • Disabled people never have more than one thing going on at once (that difference slot again).
  • Women who are romantically interested in women have never dated men.
  • Autistic people have never dated anybody.
  • There is no difference between the act of producing speech or typing, and the act of using speech or typing for communicative purposes.
  • There is no difference between the act of producing speech or typing that sounds right (or approximately right) for the situation, and producing speech or typing that actually communicates what the person is thinking (unless the person is being deliberately misleading).
  • Disabled people always have the exact same type and degree of difficulty with something, it never changes or fluctuates or anything.
  • Lesbians can’t also be Christians.
  • Two people with the same disability label are going to have the exact same difficulties with everything, or else one or the other of them should not have this label.
  • Whatever the majority of the current society a person is in considers “a disability”, is the same thing every society a person could be in considers “a disability”, there is no such thing as a set of strengths and difficulties that in one place and time is considered within the realm of normal and in another place and time isn’t.
  • Because of the last stereotype, if a person is not noticed as “disabled” by the society they live in at one time, then they must not have had the same condition that another society (or even another part of society) considers “disabling”.

That’s a whole lot of stereotypes, and that’s just off the top of my head. I’d venture a guess that most people violate at least some stereotypes of some kind. But some stereotypes have more consequences to violate than others.

And what I’ve found, is that people prefer people to violate as few stereotypes as possible at once. If you can violate no stereotypes or only one stereotype, that is great, that is expected and mostly acceptable. The more stereotypes you violate, the more trouble you get in.

And there are a number of ways to react to this, as a person who violates many stereotypes. I’ll just list some of them, not an exhaustive list either.

  1. You can take the attitude of basically, “Yeah I violate a lot of stereotypes, screw ’em if they can’t handle it.”
  2. You can be open about violating stereotypes, but ashamed at the same time.
  3. You can be open about violating stereotypes, but claim that everyone else fits the stereotype.
  4. You can be open about violating stereotypes, but claim that none of the stereotypes ever apply to anyone.
  5. You can actively try to hide some or all of the stereotypes that you violate.
  6. You can just fail to mention some or all of the stereotypes that you violate. (I’m talking about on purpose here. I’ve certainly failed to mention some that I violate by accident, only to find that people really thought I was doing it to hide the fact that I violated them, when that was the furthest thing from my mind.)
  7. You can try to hide some or all of the stereotypes that you violate, while at the same time condemning people who violate the exact same stereotypes openly.
  8. And you can even take a step beyond that. You can go to people that you know cause trouble for people who violate those stereotypes. And you can, while hiding a lot of the stereotypes that you do violate, say, “Hey, look at me. I’m okay. I don’t violate all those stereotypes. All those people who violate those stereotypes are really bad people. I’m not a bad person though, and I’ll say whatever you want me to say, including condemning people just like me, as long as you accept me.”

Like most people who violate these stereotypes, I’ve done most of them before. And I’d never entirely condemn anyone for doing any of the ones that involve hiding, even the last one I mentioned, because sometimes it’s what people need to do to survive in any number of ways.

But obviously, some of them can be hurtful, either to the person doing them or to a lot of other people, and this can be both directly and indirectly, and intentional or unintentional.

Doing the ones that involve just hiding those traits in some way, while in some ways innocuous, do make it somewhat harder for people who do violate them to be open about them. It’s easier to be open about something like that when you know that other people are as well. (And yep, autistic people can find things easier just because other people do them, we’re not immune to that whole thing.)

Doing the ones that involve actively condemning other people who violate stereotypes, and the ones that involve actually aiding people with more power who condemn (or do worse things to) other people who violate stereotypes, can not only really twist up the conscience of the person who is doing them, but actively do harm to people who violate the stereotypes. In these cases you’re basically actively adding to the prejudice that already exists against people who violate the stereotypes, and in the last case you’re aiding people who have the power to act on that prejudice in ways that can shut people out of powerful positions, shut people out of receiving services, or other things like that.

And there can also be a kind of harm in saying that nobody fits a stereotype. A person saying that should take great care to see whether it’s actually true. Otherwise, you can end up inadvertently creating an opposite stereotype. And if you say that you don’t fit the stereotype but everyone else does, you’re obviously reinforcing the stereotype.

But, in the end, I have to say that the idea that people must violate only one stereotype at a time is just as nonsensical as the difference slot. (And I’m still not feeling great, although I’m feeling way better than I was, so I’ll end here and hope that any dots I have not connected in my writing above, can be connected in the heads of people who read this.)