Update on my progression

 by Kevin 

It's been a while since I've posted. Thought I'd do an update on my progression since people are probably curious, and there really is no casual way to ask, "So! What can't you do today?" I generally don't like to dwell (privately) on my physical disability too much because it draws my mental state down. Self pity is a deep dark hole, and a mercilessly difficult one to climb out. [That's not to say that I don't like sharing triumphs or empathy with others fighting beside me.] I do gain strength trying to help others out of their well. Whether they have ALS or are collateral victims, we're all climbing out of something. Just best to keep an eye on my own footing. I had tried to do this awhile ago and even had a longish post typed up where I tried to articulate my physical and mental struggle unfiltered. Kind of a raw, uncensored, director's cut, if you will, of what I feel like I'm dealing with every second of every day. ... That turned very dark very quickly. And ultimately, I feel is only helpful when shared with others that experience it too, as a means of gaining that genuine trust of a comrade in arms. So I deleted it. Anyway, that was last September after Jane posted an update on my health. Much of my "update" here will likely repeat what she said, just, with many more words, less tact, and subliminal self pity. After I deleted the Wes Craven "Nightmare" version, it took a while for me to pick the pen up again. 

But I got an email from a friend recently that reminded me that I haven't shared outside my little inner circle for quite some time. As regrets go, I do regret not keeping in touch. I never was good at keeping in touch with old friends. Time and life just have a way of frog-marching us forward. I kind of fell off the end of the world that December. Then the world turned upside-down with the pandemic. It's really been a surreal couple of years. I haven't been writing much lately. It's frustratingly slow with my eyegaze device and I can't easily use my head mouse any more. I haven't written any code for a year now. It's terribly hard to tinker with things when all I can move is my eyes and head a little. 

I'll start with what I can do. It's easier. I can still move my head around a fair amount, although if it falls back too far, I can't pick it up. And my neck muscles are tiring more quickly if I need to rely on them for any length of time. I can still chew and swallow soft food and thicker liquids. It's just very slow going and tiresome. So I probably get half to three quarters of my calories by tube. I take all water via tube. I'll still sip on pop, milk, or a beer carefully through a straw. I can still vocalize somewhat. I say "vocalize" and not "talk" because I do sound more like Frankenstein's monster from Young Frankenstein. Even Jane can't understand me much of the time without context cues. I can make some movements with my arms and legs, but gravity wins all the time now, so useful movement no longer exists. A couple fingers on my left hand still sometimes allow me to work the joystick on my chair if someone places my hand in a particular way for me. I can still think, see, hear, taste, smell and feel touch. And! Because I can still move my eyeballs around and see at the same time, I can use eyegaze technology to type. It's like walking and chewing gum for you ... both of which, I can't do, but you still likely can  ... So it's like that, but harder. It's cumbersome because it doesn't work well for me for some reason that escapes those who told me this technology was the bee's knees. But what I'm most most happy about and grateful for, is that I can still breathe unassisted by a ventilator. 

Breath. Breathing. One of those privileged abilities that we take so much for granted most of the time. My neurologist, with the help of a respiratory therapist on his team, keep a close eye on this. And now, I do too. There's a ton of acronyms and fancy medical terms, for what seems like a simple process. There's also a bunch of different breathing tests all wrapped up in what they call a pulmonary function test (PFT). Regardless of what I can or can't move, the PFTs are one of the main predictors used for survivability over say, the next year. This is because, when compared over time, they give an objective rate of progression in the weakening of the muscles involved in breathing. One metric in particular is my Vital Capacity (VC). Since it contains the word "vital", it must be... errr... important. In the most simple terms, what they're keeping their eye on, is how well I can breathe out the air I breathe in. This is not a direct measure of the strength of my diaphragm (that would be my Maximum Inspiratory Pressure, or MIP), though they measure that too. The problem with ALS is not a lack of oxygen. Rather, as the diaphragm and chest wall muscles decline, it's harder to get rid of the carbon dioxide built up in the lungs. Especially at night when trying to get that elusive deep sleep. This is why I perform the PFT both sitting upright, like normal, and laying on my back, or supine (not a species of Christmas tree). Like I said, there's numerous little technical details, numbers and metric units of garbledygook, but let's just say VC is a percentage. Normal would be anything above 80%. We watch the progression as it approaches the 50% range. This will trigger things like getting set up with cough assist and suction machines, having PEG tube surgery, and starting respiratory therapy with a positive airway pressure (PAP) device. The reason for the PEG placement, especially if not quite needed yet for nutrition, is that the procedure requires anesthesia and possible intubation. They want to reduce the risk of requiring a tracheostomy and becoming dependent on a ventilator. I'm not at 50% yet. But I'm close enough that I've triggered the above interventions. 

I sleep at night with a vent now. I can still sleep without it, but the idea is to get used to it before I'm totally dependent on it. It's a non-invasive ventilation (NIV) system, which just means I wear a mask as opposed to being hooked up permanently via trach. There are a bunch of kinds of these, and a continued alphabet soup full of acronyms and manufacturer names and modes they can operate in. You've likely heard of CPAP and BiPAP and the like. I have a vent with Volume Assured Pressure Support (VAPS). There are two main ones backed by different manufacturers and commonly referred to as AVAPS and IVAPS. They have completely different model names as well, but that's irrelevant here. The extra "A" is for "average" and the "I" is for "intelligent". So I technically have an IVAPS, but since the AVAPS units have been out longer we'll tend to just call it my "avap". The big whoop about this gizmo, is that it senses my breathing patterns and adapts its pressure support (PS) accordingly while still "assuring" a certain "volume" of air in a given amount of time. The PS is different for inhale versus exhale (a la "Bi"PAP) and is programmable to a max setting based on my size and preference. Since it adapts to my breathing "intelligently", it's easier to drop into that deeper REM sleep without "fighting" it as it tries to inflate me like an air mattress. 

Right now my days and nights are pretty well split in half. I spend 12 hours in bed, and 12 hours in my wheelchair. I'm obviously rounding, since Jane does swing me around a bit during transfers in the hoyer (patient lift), and my showers are spent in a much less comfortable, but far more moisture resistant, shower chair. While in bed, I'm either sleeping or wishing I were. I always have some part of me that itches. Not being able to scratch it myself leads to a thrilling game of "chicken" in my head. Kind of Mexican standoff style. I can try to ignore the itch and hope it goes away. I can try to "talk" someone through scratching it for me. Or I can join forces with it and just pretend we're friends and it's doing me a favor (I tried disdainful antagonization, but that just makes it mad). The only other option is screaming inside my head until I pass out. Let's just say, you can learn to tolerate A LOT more than you think you can. Let's face it. Sometimes you just get a killer itch in places you don't want to try and describe or even admit to. Let alone trust someone else to find the exact spot. 

Well that about wraps up this update. If you've made it this far, you're a true friend, or my mother. Thank you for all your thoughts, prayers, meals and wheels! Every single one is felt and appreciated. As Red Green would say, "Remember, I'm pulling for you. We're all in this together." and "Keep your stick on the ice."