I actually drafted this blog post over a month ago, but hadn't had a chance to proofread and post it. And in the meantime, a couple weeks ago after calling the hospital directly to try and figure out what the hold up was, I got The Call to come in Monday (of last week) and start my loading dose of IVIG. I did the standard high dose loading dose over 5 days last week (plus 3 ER trips - it's been one hell of a week and a half and I'm still feeling REALLY rough). I got a severe reaction which I wasn't at all surprised about - aseptic meningitis just like I've had from other medications in the past - but it was much worse this time. I'd read it was a possibility, and knowing I was prone to it I tried to get more preventative meds, but they didn't feel it was necessary - until I ended up in the ER all night with 10/10 exruciating pain in my head and neck, and extreme nausea and light sensitivity. I was delerious in the ER, I've never been in that much pain in my life. Anyway, after that they gave me adequate pre-meds to avoid it spiking again, and while it came back a couple days after my last dose, I'm now taking extra meds to manage pain and keep the brain inflammation down until it settles down all the way. In a couple weeks I'll start on my regular infusions which will continue every two weeks through the summer - with adequate premeds to hopefully prevent this happening again, my neurologist assures me that going forward it should be easier on me. I sure hope so, as this has been absolute hell and I'm extremely worn out. I hope to write about loading dose week in more detail when I'm feeling better, but wanted to get this posted for posterity. It's probably still full of typos but it's good enough! All fingers crossed I respond well to treatment and can look back on this in a year or even six months and see the improvements happening.
A month and a half ago, I posted about my recent diagnosis with Autoimmune Autonomic Neuropathy (AAN) and Sjogren's. Since then, I've basically been stuck in a whole new purgatory waiting for treatment. Because things were progressing so quickly, my doctor put in the request for my IVIG treatment early, and the admin assistant said I should hear from the hospital within a week. That was well over a month ago now (maybe 6 weeks?) and nada. I called her to ask what's going on, and she gave me a bunch of reasons why it might be taking longer, and essentially not to call her again and just wait...
Anyway, so that's where we're at. My (and Bruno's) life is for all intents and purposes on hold until I at least get the dates of my treatment. And in the meantime, my bodily functions are shutting down more every week:
- My GI motility and swallowing have gotten way worse - food doesn't go down my esophagus properly, sometimes gets hung up in my larynx, and then once it does go down, it sits in my stomach for hours longer than it should and even overnight. It makes me feel really sick, have trouble sleeping, and has various other unpleasant consequences.
- My larynx weakness has gotten a lot worse, meaning my voice has become very weak even when I haven't been talking much or at all.
- My reflux has gotten even more severe, and has been bothering me during the day, which has only happened occasionally during flare ups. It's Laryngopharyngeal reflux and shoots right up into my throat and sinuses. This is worst when I'm asleep, and the reflux seems to be damaging my lungs even though I'm on PPIs, and doing everything possible to minimize it.
- I've developed a lot of issues with muscle weakness in my arms and legs. Basically if I do anything requiring significant muscle strength (crouching/squatting down repeatedly, holding up my arms a bunch), my whole body gets jello-y and weak for hours afterwards.
- The one positive - I've been on a new medication, Midodrine, which helps constrict your blood vessels and raise your blood pressure. It's got some very annoying but tolerable side effects, but otherwise is like a freaking miracle - I can actually stand for several minutes now without getting tachycardia and dizziness, and starting to pass out.
- On top of all that, the GI testing I had to do a couple weeks ago sent me into a major hypokalmic episode, that in hindsight I really should have gone to the ER for (it happened over the weekend, so I just rode it out at home, somewhat foolishly, but I didn't realize my potassium was probably at a dangerously low level). It caused severe cramps in my legs, and mild paralysis/spasms in my left quad that I'm still trying to shake. I've been double dosing my potassium supplements (4 times what I was taking just a couple months ago), and finally starting to get the last bits out. But between that and the muscle weakness, I've been really unable to do anything and only just left the house for the first time in two weeks yesterday.
- My pupils have been getting even more sluggish, and I'm having tons of eye pain whenever it's bright, having to keep blinds closed, wear hats and sunglasses all the time outside, etc.
You get the picture - the autonomic failure is getting worse at a still alarming rate, and I have no freaking idea when I'll get to start treatment. It's frustrating as hell, and just plain cruel. (I'm trying not to go as far as thinking the goverment is refusing to approve it, after my internist planted that lovely seed in my head...but apparently IVIG is horrifically expensive, and has to go through an approval panel if you have anything outside of a few pre-approved conditions. My condition is so rare that it's not on that list.)
Apparently all this muscle weakness stuff is not that uncommon in people with AAN, the weakness is considered "motor" nerve problems, whereas most of my issues are "autonomic" nerve problems, (there are also "sensory" nerve problems, which cause a lot of pain, burning, tingling, itching, etc. but so far knock on wood I haven't had that). They're all subtypes of small fibre neuropathy, which is damage in the small nerves that go to your skin, muscles, etc. But between the weakness and my GI/swallowing motility worsening so quickly, it's very obvious that my condition is still progressing faster than I'm comfortable with not knowing when it will end.
So, I figured while I wait, I want to document a baseline - my "month zero" status before I start on treatment. I'm going to write this up as it my symptoms would be without the Midodrine, and I'll update it if anything changes substantially before treatment begins.
- GI motility: sluggish the whole way through, and I mean the whole way through. None of the muscles contract properly anymore and I have to use physio tricks to do everything from swallow to poop. I can't take pills without putting them in puree (and can't swallow larger pills at all), and can no longer swallow dry meats at all. I have to drink water after most mouthfulls so it doesn't all get stuck in my esophagus, eventually causing pain and aggravating my respiratory symptoms.
- I still have significant malabsorption/maldigestion, even with being on the pancreatic enzymes. My diet is extremely restricted, mainly consisting of rice cereal, rice crackers, pea protein, lean chicken and ham, and vegan low fat/fibre protein bars. Fat and fibre make me extremely sick, as do many other foods like dairy and gluten which I've been off for a long time already.
- Reflux is very bad at night, and also bothering me sometimes during the day. Even with all measure - PPI, dietary changes, sleep routines (sleeping on incline, no food/water 3 hrs before sleeping) - it's still really bad and affecting my throat/breathing.
- My breathing is often restricted, I think it's related to the reflux, as it seems to flare with it. It also happens when my swallowing is really sluggish, so I suspect I'm aspirating a bit.
- My eyes are extremely light sensitive, I have to keep blinds drawn inside even on cloudy/glarey days, and wear a hat and sunglasses outside.
- My mucus/tear dryness is still manageable during the day, but severe at night when it all essentially shuts off. I wake up with very dry eyes even with gel + drops, nose (with gel), and my mouth and throat are also dry and irritated from the reflux that doesn't get washed down from saliva.
- Without Midodrine, I can't stand for more than a minute or so before getting tachycardia, sweating, and becoming lightheaded - if I can't move around or sit or lie down, sooner or later I'll start blacking out. Sitting upright with my legs down makes me very fatigued. With Midodrine, I can stand comfortably for a few minutes or even longer sometimes, with no major symptoms. I sometimes still get tachycardia, but sometimes not. And I can sit upright with my feet down for much longer before getting fatigued. My blood pressure is still rock bottom most of the time, but sometimes on Midodrine it'll get into the mid 90's, and then I feel a lot more energetic.
- I can't really leave the house on my own anymore, not having a power mobility aid. I can only walk about a third of the block before having to sit down due to fatigue, lightheadedness, and currently also my semi-paralyzed/spasmed left leg.
- From the Sjogren's, my joint pain and stiffness is also still a real bother, especially my hands and neck. My ear cartilage is also still super painful at night when I'm sleeping on my ears.
- I still have the achilles tendonitis that started at the end of last summer as well.
- The vestibular dysfunction that I've had for a decade has gotten quite bad - I get tinnitus (sometimes pulsatile), sound sensitivity, frequent dizziness, and true vertigo (more rarely), and have been getting motion sick very easily. It's much worse in my right ear, and is somehow linked to my neck pain/stiffness, as when my neck is sore or I sleep wrong, it really acts up and I get the severe tinnitus and full blown vertigo.
- I still have the dermatographism and delayed pressure urticaria (hives) that started during the big flare I had about a year ago. I have to take antihistamines and dress carefully or else I break out in tons of super itchy hives wherever there's any rubbing or pressure on my skin.
- We still don't know why I have osteoporosis or hypokalemia by the way, so not sure whether to expect any improvement of those from treatment.
- My fatigue and muscle weakness are severe (though when the Midodrine is active, my fatigue is better than it was before going on it), and prevent me from doing any of my hobbies or being able to do many of the chores I was able to even 6 months ago. I can't do sewing or gardening, or do much of anything that requries me to hold up and move my arms, or squat up and down. I use a stool to do any cooking, and try to batch cook for the freezer so that I only have to do it every couple weeks. It's very difficult for me to get up our stairs right now, so I almost never go to the office/sewing area, and some days have had a hard time even getting up to the bedroom. I have to minimize trips up and down as much as possible.
- I've lost a ton of weight over the years - about a fifth of my body mass, though it's currently stable I'm about 20 lbs underweight. I've lost a ton of muscle mass, and can tell even my feet and hands are smaller as rings, watches, shoes no longer fit.
Maybe I'll add some actual stats/numbers from tests and what not here later if it makes sense to, but I'll leave it there for now and hopefully this will be something to look back on if the treatment helps.
That's it for now. I really hope that this is close to rock bottom, as I don't want to see what much worse than this looks like.
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