5:41am March 19, 2015

I’ve been more tired than I can even explain.

Fair warning: This is gonna be loooooong, and I don’t even care because it’s been so hard to write lately and writing means I’m getting back to my normal long-winded self. I can work on conciseness later, the fact that I am typing means something important about communication, although I’ve still got that weird speech thing going on triggered by the presence of other people. This starts out about not getting any sleep and getting tired, and ends up with all the breathing-related conditions I have. This makes sense in context.

Also this is my first night turning the lights out. I was afraid if I turned them off it could disorient me and throw me further into delirium. And aside from the occasional transcendent moment (the real kind, not the “brain is having a seizure or something that fakes it kind” – for instance Saint Julian of Norwich had what seem to me clearly real religious visions while delirious… like her ) delirium just plain sucks.

I represented one of those transcendent moments with the lioness in my comic The Scarf about my experience of delirium during a roughly 5-week hospital stay for pneumonia and gastroparesis. Delirium is usually something that scrambles reality. But it can also lower your defenses against the ultimate parts of reality, the parts some people would call God(s) or spirituality. I represented that in my comic with a lioness who has a specific and meaningful image on her forehead (no I’m not going to explain – to explain to people who don’t understand would confuse them, to explain to people who do understand isn’t necessary, all I can say is that whatever it looks like, and whatever some of us may call it, it is not a symbol, it just looks like it ought to be one… it shows up in a lot of my paintings when I want to depict some kind of transcendence, but I usually pass it off as the moon).

In that case, as in this one, the delirium did not go away when I left the hospital, but leaving the hospital was essential to getting rid of the delirium. Delirium is a form of brain damage, among other things. It gets your brain into the habit of responding to various things – illness, sensory deprivation, sensory overload, unnatural environments like hospital wards and especially ICUs – by creating more delirium. The more delirium you have, the more likely you are to have more of it in the future. It can leave you with permanent cognitive deficits (I suspect my IQ has dropped – again – last count, age 22, it was 85). If you still have major cognitive deficits six months out from the start of your delirium, your mortality rate goes up by a lot.

I’ve had so much I’ve wanted to write about, but every single time I tried to type on my computer I’d fall asleep before I could get anything of substance out.

Part of it is, it’s just hard to get quality sleep in a hospital.

And then this hospital, they don’t pay much attention to your sleep apnea status. I mentioned having central sleep apnea towards the end of my stay, and a respiratory therapist flipped out on my behalf. She refused to help anyone else until she had gone through all my records to find out how severe it was, and then got me a ventilator that could function like a bipap.

When I brought my own bipap in, the other RT was like “wow that thing’s so old it’s like the Starship Enterprise, but I guess if you’re still using it that’s a good sign, it means it works.” And it did work, all my oxygen readings stabilized at night. We just hadn’t had the time to go and grab the bipap while I was busy with the Dreaded Both Ends virus.

If my bipap is outdated I’d like to see about getting a newer one, a better one, though. I see my pulmonologist soon enough I can ask her about it. They also have this bunch of masks they give to patients, so I have a backup mask now. Basically if you find one that fits, and you use it, then either they’ll throw it out or you can take it home. I opted to take mine home. I don’t like the idea of it going to waste and I’ve learned with any machine you can never have too many backups.

They also told me that my sleep apnea is entered into the computer in a messed-up manner. Basically, she tried to look up the settings for my sleep apnea. First off, it just said something like “severe sleep apnea”, it didn’t mention what kind I have, which is horrible. You have to know whether someone has central, obstructive, or both. I have both. So does my mother. And my mother and I have both had to use bipaps as ventilators during the daytime during myasthenia flares (and in my case adrenal insufficiency flares) too.

She finally found mention of central sleep apnea. But it was buried in notes taken by my pulmonologist, it wasn’t anywhere obvious. It was like she had to do detective work just to find it.

But she did find it, and even though I’m unaccustomed to the kind of vent she used on me, it worked really well (even though she had to guess what setting to use(!!!)) and I woke up feeling rested for the first time.

At home of course I always use my bipap. It has all the settings familiar to people with obstructive sleep apnea. Obstructive sleep apnea is where something about your airway blocks your breathing when you sleep. Central sleep apnea is when your brain just up and forgets to tell you when to breathe. There’s no struggle for air, you just… don’t… breathe. Which is obviously a serious danger.

My BiPAP comes with something called AutoSV. I don’t fully understand how it works, other than it basically inhales for you. It blows air into your lungs as hard as it can. This is not for the purpose of opening your airway (if you don’t have OSA, you don’t even need that kind of help). This is for the purpose of jump-starting the breathing cycle. It keeps doing this until you are breathing on your own. And it starts off doing this by detecting that you’re not breathing. If, after a time, you’re still not breathing on your own, it wakes you with a blaring alarm.

Here is a FAQ on AutoSV (Automatic Servo Ventilation):

http://www.healthcare.philips.com/main/homehealth/respiratory_care/bipapautosv/faq.wpd

This is my ‘dinosaur’ of a BiPAP with AutoSV:

http://www.cpapsupplyusa.com/Respironics-BiPAP-AutoSV-with-Humidifier-Core-Pack-1040716.aspx

Seeing all the new BiPAPs out there, I want to see if I can get a better one. This is the first one I ever got, I’ve had it since forever, it’d be nice to see if insurance would cover a replacement, especially since this one has a habit of leaking and crap. It doesn’t leak enough to be dangerous, but it does make it hard to sleep with all the hissing noises it makes unless you position all the parts exactly right.

I’ve decided to start writing some stuff about central sleep apnea. Not a lot of people have it. And the last time I tried to connect with another blogger about it, she got kind of pissed at me for missing the point of her blogging and for writing some of my long, hypergraphic text that she is unable to read (actually I’m unable to read it too, but people who get irritated by it never seem to understand that I can’t read my own writing). She did give me some tips for writing more concisely (I found it weird that someone who’s written several books – far more than I’ve ever written, I haven’t even written one and not for lack of trying – would get annoyed at the length of my writing, but oh well, maybe she can’t read her own writing either). Maybe one day I’ll even be able to follow them.

I think my writing was also confusing to her because I didn’t just write about central apnea. I wrote about breathing problems. I have lots of breathing problems, and not all of them are or resemble, even in name, central apnea. But I can’t easily separate out what’s what because I’ve only got one set of lungs. Here are the main ones:

Central apnea (sometimes also called CSA, Central Sleep Apnea): Where basically your brain forgets to breathe. It’s most well-known as a type of sleep apnea, but I’ve also had apnea events when I’m awake. They feel really weird. It’s like someone let the air out of a balloon (my lungs) and the balloon stays deflated, with no felt desire to inhale again or do anything about it at all. There’s no panic, no fear, nothing. Just an absence of breathing. (1) CSA can also be a form of Cheyne-Stokes Respiration that happens in your sleep. (When I mentioned that, the other blogger tried to convince me it was a panic attack. I know what panic attacks are like, and there was no panic at the time when I worked out that Cheyne-Stokes might fit the kind of CSA I have).
Sleep-Related Breathing Disorder (SRBD): This was my childhood diagnosis, before they diagnosed any kind of sleep apnea. I went into Stanford’s sleep clinic and they weren’t able to get conclusive results because… and yes I’m kind of ashamed of this now… every time they tried to stick a probe up my nose and down my throat, I threw the technician across the room. It didn’t help that I had an undiagnosed deviated septum on the right-hand side, it probably hurt really bad. Apparently those probes aren’t often used anymore, but at the time their sleep clinic prided itself on being state-of-the-art. Anyhoo, sleep-related breathing disorder is actually a category of sleep disorders that includes: central sleep apnea (CSA); obstructive sleep apnea (OSA); snoring; upper airway resistance syndrome (UARS) – like OSA, only without oxygen desaturation, but does cause daytime sleepiness due to waking up too much during the night; all kinds of things. So SRBD was a very vague diagnosis probably due to the fact they didn’t have enough data to know what was happening to me. But later I got diagnosed with mixed OSA and CSA.
Obstructive Sleep Apnea (OSA): When most people think of sleep apnea, they think of OSA. People are very unaware of CSA. OSA runs in my family the same way CSA seems to, only more so. I have been told that the entire shape of my airway – small mouth, small throat, huge tongue in comparison, huge tonsils too – practically guarantees OSA. My throat relaxes in my sleep and blocks off my ability to breathe. Without treatment, my oxygen level drops drastically, I wake up every few minutes, and I am exhausted day and night due to unrefreshing sleep and not getting all the sleep stages.(2) OSA is one of the most treatable conditions out there, but without treatment it can kill you due to strain on the heart, lungs, and other parts of the body, as well as lack of oxygen. When my mother was diagnosed with CSA and OSA, they said that her oxygen was so low they were shocked she was still alive.
Bronchiectasis: This is not bronchitis. It is a little-known form of COPD which is extremely rare in the USA and other ‘developed’ countries. This is because the biggest risk factors are vaccine-preventable diseases. As someone with bronchiectasis, I take anti-vax views very personally. In the USA, the most common causes are cystic fibrosis (to the point some respiratory therapists have assumed I have CF until told otherwise), and immune diseases like AIDS that can result in opportunistic lung infections. Bronchiectasis is damage to the air sacs in the lungs. They become loose and floppy. Then they produce more phlegm than usual, but they have a hard time clearing the phlegm. Left untreated, this results in a vicious cycle of getting infections, getting more severe damage and more severe bronchiectasis, getting more infections, etc. Eventually your heart can’t pump blood through your lungs and you die, unless you get a lung transplant. Being severely disabled and autistic, I don’t expect to ever make a transplant list because of discrimination. (Yes, I know there’s a limited number of transplant organs. The way to handle this is to make selection more random, not to say “This person will get more out of having a transplant than that one will,” or any of the other excuses they use to pick and choose who lives and dies.) So I am diligent about my treatment: 7% hypertonic saline twice a day in a nebulizer, after using levalbuterol to keep from having severe bronchospasms. I’m also on oxygen some of the time, which cuts down on the labor of breathing. Bronchiectasis is the disease where I kept telling my pulmonologist “It’s so hard to breathe, breathing takes so much out of me, it’s exhausting to breathe” and being told it was just my asthma (didn’t feel like asthma) until I got a new pulmonologist who ran every test in the book and found bronchiectasis on a high-res CT scan.
Asthma: My asthma is actually pretty mild except during acute exacerbations. It makes me wheeze and have trouble breathing out.
Aspiration, aspiration pneumonia, aspiration pneumonitis: My gastroparesis (paralyzed stomach) means that bile doesn’t drain into my intestines fully. Some of it builds up in my stomach. If the drainage bag on my G-tube malfunctions somehow, I aspirate. Aspirating means the bile comes up into my lungs, I wake up unable to breathe, and either call 911, or if it’s less severe, I spend hours coughing out the bile. Then because of the bronchiectasis, I always end up with either aspiration pneumonia (a kind of pneumonia caused by aspiration) or pneumonitis (same symptoms as pneumonia, but caused by irritation rather than infection).
Myasthenia gravis (MG): An autoimmune disorder (sometimes hereditary instead of autoimmune, sometimes both) that both my mother and me have, possibly my grandmother too. It results in severe muscle weakness after using a muscle, and can impair breathing. Before I got treatment, I used to frequently use my bipap as a daytime ventilator if I got too weak to breathe, and my pulmonologist said this was the right thing to do.
Adrenal insufficiency (AI): First off, this is not “adrenal fatigue”.(4). With that footnote out of the way… I have severe secondary adrenal insufficiency. When tested (blood test, the real kind), my cortisol was too low to measure and so was my ACTH. ACTH is what the pituitary glands use to tell the adrenal glands to make cortisol. They tested my cortisol several times with always the same result: Too low to measure. Then they flooded my system with ACTH to see how my adrenal glands would respond, and I did make more cortisol, but not as much as expected. They think this is because after 6+ years of this, my adrenal glands are out of practice. Anyway, AI can result in breathing trouble if you experience stress. Stress here can be physical or emotional. Both deplete your cortisol, and AI makes you unable to make more cortisol. Again, I used to use my bipap as a ventilator if my lungs got too weak during the day and my oxygen level dropped below a certain point. AI can make you so weak you can’t even lift your hand. It can also make you much sicker than you should be with any given illness. My doctor says it’s a miracle I’m alive when I belonged in the ICU several times and was ignored because of discrimination against people with developmental disabilities. I guess I’m tougher than I look. But it can definitely affect breathing muscles.

So when I talk about breathing problems, I’m talking about all of the above and probably more. Those are just the ones that come to mind. I can’t separate them out clinically and discuss them that way very easily. They all blend together because they all affect my lungs.

Oh and by the way – the hospital has for unknown reasons changed its name from Fletcher Allen Health Care to UVM Medical Center. UVM Medical Center is now the name also used for the main hospital (UVM Medical Center, formerly Fletcher Allen) what used to be Fletcher Allen’s University Health Center (UHC) and Fanny Allen (which mostly has a walk-in medical clinic, outpatient rehab and physical/occupational therapy, minor outpatient surgeries, some radiology equipment, etc).

My favorite is generally Fanny Allen but it’s also the furthest from home. Fanny Allen’s walk-in clinic is much more friendly and relaxed than the ER, though they will send you to the ER if your problem is too severe for them to take care of. Their radiology equipment isn’t as fancy as the radiology equipment at the main hospital, but their radiology techs are far more creative as a result. One time when I was too weak to get out of bed for an x-ray, or even to sit up or stand, they actually MacGyvered up this solution so I wouldn’t have to get out of bed. At the main hospital they’d never have done that, at least not so thoughtfully and patiently. I would’ve become a problem, instead of a person with a problem. That’s why I prefer the walk-in clinic where possible. Not being in a wheelchair means people can drive me there now, which I am thrilled at because I hate the ER with a passion. Although this last ER stay wouldn’t have been bad if I hadn’t been so sick that my pain kept hitting nine.(3)

FOOTNOTES UNDER CUT (cut for length, believe it or not)

(1) I think the very first thing that confused the other blogger was that I was talking about situations in general where I had pulmonologists not believing me about things. Central apnea was only one of them. So I was talking about the struggle to breathe that comes with myasthenia gravis, adrenal insufficiency, and bronchiectasis, and how I kept talking to pulmonologists about how hard it was to breathe, and how they kept blowing me off until one of them finally did a CT scan and found bronchiectasis, as well as doing some other tests that showed I was aspirating on a regular basis. The blogger said something like “But with central apnea there is no struggle to breathe.” Of course there isn’t. It’s just that I wasn’t ever only talking about central apnea.

(2) The same problems can happen with CSA because it causes a drop in oxygen saturation as well, the difference is that with CSA there is no urge to breathe at all (like in Cheyne-Stokes Respiration, which happens in your sleep some forms of CSA but is more famous for happening to people as they die – people with CSR are scary to watch because of the length of time they don’t breathe, but they report no discomfort, just as in central apneas which can occur while waking or sleeping).

(3) They always ask, “On a scale of 1 to 10, 1 being no pain and 10 being the worst pain possible, what is your pain?” It has taken me years to be able to adapt a pain scale to my needs and it can still be difficult to use: I tend to move things towards the middle, so low numbers may be pushed up to 5 and high numbers may be pushed down to 6. My version of the pain scale is logarithmic, probably because I grew up on the Richter scale in California, where each number is twice as bad as the last. (So a 7.0 earthquake is twice as bad as a 6.0 earthquake.) That’s opposed to a linear scale, where each number is one more as bad as the last. On a linear scale, going from 5 to 6 is the same amount as going from 6 to 7, each number is 1 more than the last. Some people use linear pain scales, I don’t.

Here is the pain scale that is closest to what I use. It is not perfect, but it is as close as it gets. It’s called the Comparative Pain Scale, I’m linking to it on a blog called The Daily Headache.

Anyway, for me, if I can say I have a 10 then I don’t have a 10, because 10 means totally incapacitated or passed out. I can say in retrospect that something was a 10, but I can’t say it at the time.

A 9 is hard to communicate during but it can be possible. It can also result in severe delirium, disorientation, vomiting, and other really unpleasant stuff.

In the hospital this time, my pain was usually a 7-8 but then would spike up to 9. Once it hit 9 I’d start screaming or crying. I don’t generally scream until I’m at 8.5 or 9. I always have a hard time convincing people I’m at a 9 though because, as my doctor says, I have a very stoic appearance that most nonautistic people can’t read. I was really upset when I saw that my father has the same body language for pain that I do, because it meant as he was nearing death I could tell when he was in severe pain and I sympathized too much. I have posted selfies of myself at different levels of pain, and many other autistic people have said they’re able to read me, even when doctors can’t. So that’s sort of comforting – it isn’t invisible, it’s just some people don’t know what to look for.

(4) Adrenal fatigue is a fake diagnosis given to people with real diseases (including sometimes real adrenal insufficiency) by quacks. This quackery results in people’s death so I take it very seriously. The quacks have gotten wise to the fact that people are starting to realize adrenal fatigue is a fake diagnosis, so they are starting to call it things like “borderline adrenal insufficiency” or just “adrenal insufficiency”. I have the real thing, though, and this really pisses me off that people are using the name of a real condition to cover their asses when they know they’re making up conditions. It means that people like me, with severe adrenal insufficiency that could kill me if I went off of steroids, get treated as if we have “adrenal fatigue”, and it means that those who are diagnosed with adrenal fatigue are not getting treated for whatever they really have. Everyone loses except the quacks.

Notes: