If I start looking like this. And I either can’t hold my head up or can only do so with difficulty. Or if I do hold my head up but by the moment I get it up, it’s practically already on its way to flopping its way down. Some of which is subjective, some of which is really frigging obvious…
Those are all bad signs.
Because it seems to go in sequence with me. The myasthenia-related weakness.
Eyeballs and related facial muscles are the first thing to have trouble. They contribute to my blindness, in fact, by pointing in different directions. But they point different directions different times of day, so there is no foolproof way to correct it at the eyeball level whether through surgery or prisms.
Neck usually comes next, if not first itself. Holding my head up is hard at the best of times, impossible at others. This has gotten blamed on poor posture most of my life. It’s not poor posture. Myasthenia is not poor posture. It sometimes causes poor posture, but not the kind you can correct for on purpose. It’s like when people congratulate me on my weight loss (I weigh less than I have since I was maybe 20 for the first time in my life since then) knowing I have gastroparesis, which is a disease very few people want.
Then come elbows and knees. I say elbows and knees because they either hit at the same time usually, or one right after the other. I’ve had other weird neuropathy-like stuff that went for my elbows and knees at the same time like that. I don’t know a whole lot about the neurology of this but I barely understand certain aspects of CMS (Congenital Myasthenic Syndrome) myself.
Sort of on equal footing with elbow and knees is some of the muscles involved in breathing. Sometimes it goes straight to breathing, sometimes it takes a detour through my elbows and knees along the way.
And obviously if it messes with my breathing muscles, I’m headed up Shit Creek without a paddle. My best bet at that point is my bipap. I normally use it as the usual nighttime vent for sleep apnea. But since I have both central and obstructive sleep apnea, there is a feature called AutoSV — Automatic Servo-Ventilation. It detects how much you are breathing and shoves air into your lungs to try to jumpstart your brain again. Which makes it so perfect as a noninvasive daytime vent that I have actually had ER breathing teams, ICU teams, and regular parts of the hospital teams, all would rather get someone to bring my bipap from home already set up, then set up a new one.
But that is not a place I want to be.
Because my mom was begging for a bipap just before she coded during a myasthenia crisis in a hospital with no pulmonologists on staff. They only bothered with the bipap after they had decided she needed chest compressions.
But at any rate my pulmonologists have always approved of this use of a bipap.
But I don’t want to get there. And my roomie is sick. And I am exhausted. And I am dealing with longer and longer periods on my own. And this is just how it is. At any rate, I get disoriented same as always, but nothing and nobody around necessarily to orient me. So i just do the only things I know how to do. Which are not always the best or the right things to do. But when your options are limited, you do whatever you can do, because you don’t have other choices.
So that’s what I’m doing.
BTW so-called “head drop” tends to be one of the early signs of agrin-type congenital myasthenic syndrome. One of the most terrifying things I did was find an obscure AGRN-related CMS paper with a picture of a young boy with an all-too-familiar posture. So this is why my neck goes floppy. This is why my eyes point different direction. This is why my eyelids drop asymmetrically a lot, etc.
In a minute I have to stand up and deal with meds. I am dreading standing up. Hell, I am dreading lifting my head up. But you have to do what you have to do.
I am falling or near-falling every day. People from howard still come in periodically. None of them are informed about the falls no matter how often I or they report them (since staff have witnessed some of these). I have to be really careful when i stand up, to use all four limbs, never just try two, or i will fall as often as not.