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Drama- Episode Three!
DRAMA ITEM #2- Continued!

After this, things slowly started gathering momentum- at first, things felt glacially slow as I went through months of sporadic 15 minute appointments where it felt like nothing really got accomplished, but while things continued to get worse, I got a little better at expressing them, and while house crap ate October, medical crap ate November- my rheumatologist put me through what he termed the "full court press"- a series of tests, all of which had several different associated appointments, so that from November 6th to 9th- which just happened to also be the week of all my midterms- I had a doctor's appointment every single day, then only one the next week, then three the week after that... to, well, now, I've had at least one appointment per week, but things seem to be tapering off.

The doctors I saw were a cardiologist, a neurologist, a medical geneticist, and a sleep specialist. (There's also an orthopedic surgeon I've had a referral for for months now, but he's in the process of getting approved by Cigna, and has been supposedly about to be done for *months.*)

I saw the cardiologist for a tilt-table test, which confirmed my problems with orthostatic hypotension and neurocardiogenic syncopy- or, as I like to call it, "wuss faint." I crashed in a little under 14 minutes, without supplementary adrenaline. Kind of interesting to actually have one when I was waiting for it- a lot of things that I thought of as just "feeling tired" or "fuzzy" turned out to be part of the "prodrome" or early warning signs.

In people with HEDS in particular, this is commonly caused by venous pooling in the legs- my veins are so stretchy that the blood just kind of stays down there, instead of circulating back up to my brain. It's actually visible, too- as is easily tested by my going out in a pair of ankle-strap high heels- even though the strap is too loose when I first put them on, within an hour or so- even if I'm just sitting- I have to let the strap out a couple notches, or else the strap starts biting into my leg. All the ankle straps on my shoes are stretched out like crazy (>_<) This connection was discovered, interestingly enough, not in a study of patients that are hypermobile, or in a study of patients with NCS or POTS, but in a study of patients with CFS. Turns out that people with HEDS/HMS are frequently misdiagnosed as having CFS or Fibromyalgia, because of the high symptom overlap. Now that certain physical differences in the nervous system of people with CFS and Fibro have been identified, this might start changing.

This turns out to be related to what I saw the neurologist for. The first time it happened, I thought I'd had a partial-complex seizure as a bad reaction to the Diclofenac I was taking at the time for pain, (since Cigna denied coverage for Celebrex, grr....) I figured it was an isolated event, and that as long as I stopped taking the Dicolfenac, I'd be fine. After all, on the long list of documented side-effects I found online, seizures *were* listed. But, if I couldn't take Celebrex or Dicolfenac, and the whopping amounts of Naproxen I was taking a) were dangerous to my stomach and liver, and b) no longer effective, anyway, that left opiate painkillers as the only other option- and if I had some sort of borderline seizure disorder, those could make it worse, so that had to be ruled out. (Not to mention that I wasn't all that keen on the idea of switching up to that level- the complications, stigma, expense, side-effects, dependencies... Though when I protested vehemently to the doctor on my first visit that I didn't want opiates, and was definitely *NOT* med-seeking, the rheumatologist said that having symptoms like mine is already on a level comparable to opiate withdrawal, so considering myself 'not dependent' was a fallacy to begin with. That still cracks me up.)

The neurologist took tests to check for epilepsy, aneurysm, or stroke- all of which bear a strong resemblance to what happened. Aneurysm in particular was a concern because, although I have no MVP "click" or aortic dilation "murmur," the symptom overlap with Marfan's was strong enough that they decided to be extra cautious. (I'm guessing that my apparently "marfanoid habitus" is also the reason why they ran me through all those weird tests at Swedish when I went in for my back spasm, instead of just giving me a muscle relaxant to stop the damn thing- which seemed like the obvious solution to me at the time.) Anyway, all my tests came back totally normal- which would have confirmed to me that it had been a total fluke, if it hadn't happened again *twice* in the interim. (Yes, I will explain what *it* is momentarily.) But before I could even mention those, or defend myself as Not Being Crazypants, Really, I Swear, the neurologist started telling me about something called an Acute Confusional Migraine. This is normally something that only happens with kids, or so most of the literature says, but since it seems to have a relation to cerebral hypoperfusion, it makes sense that it would happen to me. (Plus, as I only just found out on the EDNF FAQ, migraines have been added to the list of comorbid complications.)

This again warrants a bit of background information (sorry!): I have long since developed the habit of, whenever I would feel myself fading from the NCS, propping myself up against something to avoid falling over, and would trying to ride it out. After all, I had no idea that what was going on was any different from ordinary fatigue or a "headrush," or that it wasn't normal. When I passed out as a kid, I got accused of faking it for attention, or being melodramatic, so I learned to cover for it- I didn't want to cause trouble, or get *in* trouble, and I was ashamed that I was such a wuss that I couldn't handle what was obviously no big deal for other people. So I got into the habit of covering it up, all the while assuming that it was either normal, or just really, REALLY uncool. Turns out to be the latter ^_^;; (Interestingly enough, though, I have never once blacked out from pain. I've come close- but never actually gone under. It's fatigue that nails me- I can't seem to resist it.)

So the theory supported by both the neurologist and the cardiologist, (as well as this thread on a migraine message board, from a woman who's daughter seems to have the same thing,) is that when I lose blood flow to my brain, but stymie my body's attempt to recover said blood flow by lying me out flat, sometimes my heart manages to pump *just* enough blood to my head to keep me from losing consciousness *entirely.* But not enough blood/oxygen to function *well.*

Instead, I start fading in and out of consciousness, while my body just keeps doing whatever it was doing. If I'm standing, I keep standing. If I'm walking, I keep walking. I get confused about where I am, or what I'm doing, and have trouble remembering anything from moment to moment. I move clumsily, have trouble writing or holding an object in my hand. I can't hear or see very well, sometimes things just get faint and blurry, or dark, sometimes my field of vision gets blocked out by orangey starbursts (that was one clue that it was a migraine, rather than a TIA,) and things seem very far away and very slow, including thoughts. Sometimes my heart will pound *very hard*, so hard that it hurts, and sometimes my pulse and blood pressure rise a little- but not enough to fix the problem. I'll feel nauseous and headachey, as well as very, very tired and sleepy (and if I would just learn to listen to that feeling to begin with, I wouldn't get into this kind of mess...) I have trouble speaking- either making my mouth form the words, or remembering the word I want, or what I say out loud won't match what I meant to say in my head.

Needless to say, that shit is SCARY. Not just for me, but for anyone watching. Although the effects aren't permanent, (though that's one limit I don't want to push,) even now that I *know* what's happening, not only does the confusion cloud my common sense, (it's not that bad... just let me wait until I'm done doing x, and I'll go find a quiet place to lie down then....) but it also interferes with my ability to explain what's happening. I seriously need to get one of those MedicAlert bracelets, and maybe pin a note to my jacket or something (>_<)

A favor I ask of you guys, though, who are reading this: if I seem spaced out, or start making grammatical errors, or mixing words around, or forget something you *just told me*- MAKE ME GO LIE DOWN. Even if I try to bluff it off, or get annoyed. I seriously suck at catching the early warning signs, and if I go lie down while I'm still at the stage I like to call "Teh Brain Fog," I can head it all off at the pass. (I really need to document this for my profs, too. Nothing worse than getting called on and having a Brain Fog Blankout. I might as well dress up like one of the lobotomy patients from Monty Python.)

The suspiciously ironic coup de grace to this, though, is that- well, remember how I was always too busy to bother getting my driver's license? Now the same condition that is rapidly fucking up my ability to get around on foot has effectively barred me from ever getting one- NCS and ACM both fit the criteria for medical suspension.

But I swear, if some Narrator is trying to make a parable out of this crap, they're in for a serious crotch-punting.

The sleep specialist I saw was to rule out sleep apnea as a cause of fatigue, while confirming the diagnosis of RLS, (one of many comorbid complications of dysautonomia and the HCTD set, particularly EDS and Marfan's,) and involved my staying overnight at their clinic while they smeared more of that toothpaste gunk on my head, stuck wires all over me, and tubes up my nose. (I seriously wish I had brought a camera- I looked HILARIOUS!) Unfortunately- the morning after that test just happened to be the morning of my final presentation in Japanese class- which was a TOTAL fiasco, more about THAT later...) and the facility had no showers, so I had to bus home at 6am with that crap STILL all over my head. I consider this stare gauntlet training for being the lone gaijin on a Tokyo train. Then shower, then assemble project, then present it while all KINDS of sleep-deprived... Ugh.

Insult to injury, while it did confirm RLS, it did NOT rule out sleep apnea- apparently I snore like a timber mill if I'm on my back. On my side, though- as I prefer- I'm just fine, so it's ruled 'borderline'- the doc offered to help me get a refurbished CPAP cheap, but said that while it might help, I'm not in any danger without one- though I should get it checked up on every now and then to make sure it isn't getting worse. (And thank deity-of-choice for that, as my evening routine has been de-sexy-ed enough by this crap already, thank you.) This seems to be related to more autonomic nervous system fuckery.

Anyway, it's one more thing to add to my list of arguments for getting my Tonsil Nemises removed once and for all.

The visit to the medical geneticist was not very informative, and pretty frustrating. On one hand, the guy I went to see is *the* guy for Vascular EDS- anywhere in the world, if they take a test for it, it's HIS lab they send it to. Unfortunately, (and I *was* warned that this might be the case,) although he *did* see me, his focus is almost entirely on the Vascular type- in fact, it seems dubious as to whether he considers the Hypermobility type to be a kind of EDS at all. Either way, he said that while I was definitely hypermobile, and probably had NCS, (I saw him before my tilt had confirmed that diagnosis,) and had some tissue involvement, that since he didn't hear any signs of cardiac involvement, he didn't want to confirm a diagnosis of HEDS, because "the only type of EDS anyone has heard of is Vascular, and we don't want them to overreact." While I can see his point- I don't much care for the prospect of paramedics cracking open my ribcage and diving into open-heart surgery just because I have a back spasm- when it comes down to overreaction and UNDERreaction, at this point, I'd take the former any day. Given the questions he asked about our family history, and the measurements he took of my bone structure, I suspect that the only reason he saw me at all was that during my phone screening with one of the other geneticists, I must have set off their Marfan's alarm. (Which, I'll admit, not having had any lab tests done, is still technically in the running. But since my major problem right now is *low* blood-pressure, not high, we're letting that dog lie.) Anyway, given that only about 22% of people with HEDS have any cardiac involvement, his emphasizing it so heavily as a diagnostic criteria threw me off a bit.

One problem is that, (as is rather well represented by that sample of references,) there is a huge amount of disagreement as to whether what I have is a kind of Ehlers-Danlos or not, if there are separate hypermobility disorders or not, what to call them, etc. And if there are two separate kinds, the distinctions seem to boil down to whether systems outside the joints are affected, and whether it gets worse with adolescence or better. When they aren't being considered interchangeable, the HMS type seems to be considered to be limited to the joints and childhood, non-progressive, and usually outgrown by adulthood, whereas HEDS takes into account connective tissue affects on systems other than joints- particularly, skin, muscles, the vascular system, (though I'm talking about pooling and MVP here, not the Vascular EDS,) autonomic nervous system, gastrointestinal system, etc, and gets worse with adolescence, and continues to degenerate throughout adulthood, eventually stabilizing with old age.

The initial reaction of my rheumatologist, when I asked about EDS, was that a) he'd only heard of the Vascular kind, really, and b) since he was treating the symptoms, regardless of whether it was or wasn't, his treatment wouldn't change. This was similar to what I heard from the medical geneticist- that he couldn't say one way or another, and it wouldn't change anything. But it seems to me like the presentation of the prognosis, and the general attitude towards the problem, is pretty different between the two- and even if they are the same, they get treated pretty differently depending on which name you attach. Either way, there's one thing that's been established pretty clearly: My collagen is Fucked. Up.

I would also like to point out that, in retrospect, the odds of having any of those other false-alarm illnesses are a hell of a lot higher than this one. RA affects 1:108, Lupus 1:200, and the SpAs are 1:1000. The *highest* estimate I have ever heard for HEDS/HMS/BBQ is 1:5000- and that estimate tries to factor in under-diagnoses. The more commonly cited figure is 1:10,000. So while I often joke about being a "Career Outlier," I think I must have just gotten a promotion or something.

The most frustrating thing about the whole experience, one that is pretty well shared, is that I just wanted to get diagnosed so I could fix whatever the hell was going on and get on with my life. Naturally, I would prefer something physical over something mental, because the physical is a hell of a lot easier to fix. But in this case, even after all this crap, the crazy-pants looks endured and the expense and the time spent on figuring it out, there isn't any fix. There are a few ways to make the symptoms a little easier to put up with, but no way to get rid of them for good, or even stop them from getting worse. The most I can do for now is wait for them to identify the gene, and maybe even find a way to fix it with stem-cell therapies- something likely to take a long fucking time with this regime. Though if they do find a cure, even though it's a stupid rare disease, the cure is in everybody's interest- unlock a stem cell cure to fix connective tissue, and you've got yourself the Fountain of Fucking Youth.