A Punch in the Gut

First, a quick check in about the research study in San Francisco. That’s still going on, still going well, and now that it has shifted to once a month rather than every two weeks, things are calming down and much easier. My brother Gecko is the kind of advocate a girl dreams of. He’s been amazing throughout all of this.

So, clinic. Well.

It wasn’t an easy one. My breathing is only a little weaker than it was before, so that’s good. My overall limb strength is about the same. My diet is great, my weight is stable, although I am the heaviest I have been in my entire life and I hate it, I’m doing what I should. Overall, each of my many appointments went as per usual except for two.

For my one-on-one with Dr. Goslin, I had to finally admit that over the past few months I have been losing bowel control. Admit to someone besides J who I tell every-fucking-thing to and my mother, who’s had to help me with the um. Fallout. There have been a few major accidents, and many minor ones. It’s exactly as my bladder incontinence happened, I don’t have an urge to go at all until I suddenly do, and that when I stand up to go I’m just… going. It was a fucking humiliating thing to talk about, never mind in such clinical detail and with two other people in the room with us. But those two other people were J and Gecko, people who need to know exactly what’s going on with me. She listened carefully, asked a lot of questions, and then told me that she’s never had another ALS patient completely lose bowel control. Accidents, sure. But you have two sphincters and one of them is involuntary, so there’s no reason I should lose complete control.

So…

So that means I’m either a medical anomaly and an ALS first, at least for her, or I have another severe, separate medical issue happening.

I did not take this news very well. I’d already worked out before Clinic that the next step was going to be to speak to a gastroenterologist, and I did as much research as I dare and realize that it’s there is…really not a lot to be done about losing bowel control. I dared not delve into the humiliating tests that were likely to be performed; this has already given me too many panic attacks these last months. There are artificial sphincters that can be installed, but they only work some of the time and are not considered worth the surgery risk. Or there’s the colostomy route. Punch yet another hole in my abdomen, another medical accessory to be maintained. A very large piece of my dignity destroyed, at any rate.

…ALS sucks, don’t ever get it.

So I came away from that with a referral to a gastroenterologist, and a referral for an MRI of my lumbar spine and pelvis, just in case there is something obvious going on. Maybe the lumbar punctures I’m getting for the medical study screwed something up, though I’m pretty sure it started well before I was in the study. Maybe it exacerbated it, I don’t know. In the meantime, I was told to stop my magnesium supplement, and I’ve started taking Imodium every day. We have a plan of attack.

The last appointment for the day was with my pulmonologist. He looked over my chart notes so far for the day, and then asked me if I had thought about survival measures when the time comes and I’ve progressed too far. Do I want to be on a ventilator, that sort of thing. I told him I thought very long and very hard about these things, and so I have a POLST form (Physician’s Orders for Life Sustaining Treatment – basically an Advance Directive, but with a lot more legal weight).

“Well, if you’re considering a feeding tube,” he told me, “you had better consider that sooner than later.”

I… was not prepared to hear that. I blinked at him.

“It’s your breathing,” he explained. “Ideally we want to do the feeding tube when your breathing is somewhere between 40% and 20%. After that it becomes much too dangerous to put you under anesthesia. Right now you’re at 48%, so we’re looking at somewhere between the next six months and the next two years.”

Which, I knew this surgery was coming. Even back in the early days of diagnosis, when I looked at the roadmap ALS typically leads you down, I knew I would be getting a feeding tube before actually needed it. And I knew it was because my breathing would make the anesthesia too dangerous to wait until there was actual need. I just…

… I just wasn’t expecting it so soon.

I FULLY realize that soon is relative. I’ve had ALS for five and half years now, way longer than I have any right to expect. My progression is very slow, and I am very very very lucky. And I am very very grateful. And I expected this talk, eventually. It was just a really harsh reality check. A metaphorical punch in the gut. This is really happening, it’s really progressing, and the end stages are a lot closer than I’d like to think.

So depending on how the gastroenterologist appointments go, I may have three holes LITERALLY punched in my guts. I joked with my therapist that I’m going to need a little sidecar for my wheelchair for all of the medical gear coming out of me. I’m not really fooling anyone with the catheter bag at this point, I think. If anyone gives me more than a casual glance they can totally tell that’s what’s going on. Add to that a colostomy bag and a feeding tube, and it’s going to be nothing but baggy sweaters for me ever again.

A little bit of my old life chipped away. A new normal to adjust to. New change, new life, less a bit more dignity and control.

For now? On with the thing. This hurts, but it’s hardly the end of me. I spent last week feeling very sorry for myself, and now I will do what comes next. I will follow up with the GI and hopefully learn and control my obnoxious new symptom. I will continue with the study until it is completed or until I can’t. I will adjust to my new life of being completely on government financial support. I will embrace my new normal because it is my life and it is still possible. I’m still here dammit. And whether or not my dignity is intact, I will keep breathing until I can’t. And that’s not for some time yet.

I still have so much to do.

Like, FRS Sure

It’s been a long time since I’ve done a general symptom check-in to let you know where my functionality is at. I also don’t think I’ve ever talked in-depth about what the ALS-FRS scale is, at least a quick peek through the tags comes up with nothing, so I think this is as good an excuse as any to go a little deeper into something that is pretty dang integral to my life now.

In the simplest terms, the ALS-FRS is a way to numerically represent my decline. It stands for ALS Functional Rating Scale, and it’s a series of 12 categories that cover the basic symptoms of ALS. You rate your functionality on a scale of 0 to 4, with 4 being completely unaffected, 0 equals no functionality at all. The FRS very helpfully provides examples of each category’s numbering. At the end, you add up your score and that gives you a numerical score that tells you how badly ALS is impacting your life. By tracking this number over a period of time, you can also determine your progression rate.

The last time I took this test was for the clinical study I’m involved in, about 3 weeks ago. I gave my answers verbally so I don’t know what the tally was, but my last Clinic Day’s score was 29 out of the possible 48.

Let me show you around the ALS-FRS while I tell you about my current state!

1. Speech
Normal speech processes
Detectable speech disturbance
Intelligible with repeating
Speech combined with nonvocal communication
Loss of useful speech

For a lot of people, speech is the first thing they notice going wrong. A little bit of a slur, being slightly mush mouth, nothing too dramatic. That’s called bulbar onset, as opposed to limb onset like I’ve got. Fortunately I haven’t really noticed anything amiss in this department yet so my score here is four.

2. Salivation
Normal
Slight but definite excess of saliva in mouth; may have nighttime drooling
Moderately excessive saliva; may have minimal drooling
Marked excess of saliva with some drooling
Marked drooling; requires constant tissue or handkerchief

Again, this is a pretty common bulbar onset symptom. A friend of mine who had ALS had to keep a handkerchief in her hands at all time so as to blot up the drool. The same mechanism that doesn’t allow you to speak properly means you can’t swallow very well either, so there’s nowhere for your saliva to go. Another common thing this symptom causes is choking on your own spit a lot. Fortunately I don’t do that anymore often than I ever did. So for now, my score is a four.

3. Swallowing
Normal eating habits
Early eating problems-occasional choking
Dietary consistency changes
Needs supplemental tube feeding
NPO (exclusively parenteral or enteral feeding)

Slightly different than choking on your own spit is food going down the wrong pipe. That will eventually start to happen a lot to people with ALS. Eventually you move to a thick liquid diet (thin liquids are too easy to aspirate) and then eventually you can’t swallow at all so you have to make a choice about supplemental tube feeding or starving to death. Fortunately that decision is some distance away for me. My score here is a four.

4. Handwriting
Normal
Slow or sloppy; all words are legible
Not all words are legible
Able to grip pen but unable to write
Unable to grip pen

The key word here is functional. This scale doesn’t measure in terms of strength but rather in terms of what activities your symptoms are preventing you from doing. It doesn’t matter that my hands curl up when they’re at rest, it only matters that I can or cannot hold a pen and sign my name. It’s a very practical scale. More medicine should be based on practicality in my opinion. Unfortunately for me I do not have a perfect four in this category. I have to sign my name by writing from the shoulder rather than from the wrist in order for it to be legible at all. I can very painstakingly write a short simple note, but it’s gotten to the point where not all the words say what I meant when I wrote them. It’s hard to tell what I was going for sometimes. So right now my score here is a two.

5. Cutting food  
gastrostomy
Normal
Somewhat slow and clumsy, but no help needed
Can cut most foods, although clumsy and slow; some help needed
Food must be cut by someone, but can still feed slowly
Needs to be fed

Here the scale diverges. There is a 5A and a 5B. It depends whether or not you have a feeding tube. My hands fail me at this point to where I can’t cut up a steak or serve myself from a common dish. I can stab at food with a fork, or spoon something into my mouth, but if food needs cutting, someone else has to do that for me. I could probably butter my own piece of toast, but butter knives are pretty heavy actually, and so that’s becoming more and more difficult. If the butter is cold, forget it. My score here is a one.

6. Dressing and hygiene
Normal function
Independent and complete self-care with effort or decreased efficiency
Intermittent assistance or substitute methods
Needs attendant for self-care
Total dependence

This category should probably be two, in my opinion. Dressing and hygiene are completely separate, particularly if you are a woman. As far as self dressing is concerned, men would probably score higher in this category than a woman of the same progression, simply because that dude never has to deal with bras. I could pull a shirt over my head and sweatpants on, but dressing more feminine requires so much more effort than that. We won’t even talk about shapewear. Anyway, I digress. Gender and/or biology disparities aside, hygiene is also a completely different ballpark. And yet it is one category. At this point for me, taking a shower by myself is impossible because I cannot operate the mechanism to get the showerhead to work. I have to have my mom come in and activate the shower. Once the water’s on I’m okay, but it’s extremely difficult and exhausting and I don’t feel fully clean at the end of it. I never get that freshly scrubbed feeling anymore. Toileting – and that is such a great word – is going okay in the wiping up and taking care of things department, but it’s becoming very difficult. I have to use a wand that holds toilet paper. I resisted it for a very long time simply because those devices are the punchline of jokes about morbidly obese people. I’m fat by medical directive, but I’m not morbidly obese and I am stupid self-conscious about it, and so I resisted buying a device that actually made my life easier for far too long. Yay society. So while I am able to take care of myself in this department, sometimes some help is greatly appreciated such as when zipping up boots and putting on socks. I usually rate myself as a three here, but if I’m being fully honest I’m a two.

7. Turning in bed
Normal
Somewhat slow and clumsy, but no help needed
Can turn alone or adjust sheets, but with great difficulty
Can initiate, but not turn or adjust sheets alone
Helpless

See? Practicality. Pure and simple. This category represents something you don’t really think about until you start to have problems with it, and then it becomes dire. Turning in bed is no big deal until you spend literally all of your time in bed. I am so, so grateful I have an adjustable bed. My life would be so much harder without it by now. I’m not to the point yet where I’m completely helpless, I can still adjust my own blankets and adjust my body, but sleeping on my side is no longer possible. That’s more to do with breathing than physically turning in bed, and that comes later, but for now I don’t have to struggle so much to sit up in the morning because I push a button and I set up automatically. So for all intents and purposes, my score here is currently three. It’s teetering on two, but we’re not there just yet.

8. Walking
Normal
Early ambulation difficulties
Walks with assistance
Non-ambulatory functional movement only
No purposeful leg movement

This category is where my symptoms began. It is the most noticeable change in me. I can currently focus really hard and move my toes the slightest little bit, but with my heel on the ground I cannot lift my toes. I cannot turn my feet to left and right. I used to be able to wiggle my left little toe independently of the rest of my toes but that stupid party trick is now gone. With weight on them, my feet are now completely useless for balance. Even the weight of a blanket pressing down on my toes is too much, so I have a device on my bed that keeps the covers off of my feet. I described my legs as useless meat stilts, and that’s pretty much what they are. I can prop my body up on them and clumsily move one forward at a time to perpetrate some semblance of walking, so long as I have both hands on some sort of other assistive device. I can kick my legs up while sitting, but I can’t lift them parallel anymore. I can lift my knee just the slightest bit, but once the doctor puts any pressure on them they go right down. I’m currently able to use the walker to get to the bathroom, but any trip longer than that and I become winded and seriously worried that I’m going to fall. I’m technically able to walk still, but for all practical purposes I am in a wheelchair. My score here is a two, but in real life is much closer to a one.

9. Climbing stairs
Normal
Slow
Mild unsteadiness or fatigue
Needs assistance
Cannot do

HAHAHAHAAHHAHAHAHAHAHAHAHAHAHAHAHAAHHAAAAAAaaazero.

10. Dyspnea
None
Occurs when walking
Occurs with one or more of the following: eating, bathing, dressing (ADL)
Occurs at rest, difficulty breathing when either sitting or lying
Significant difficulty, considering using mechanical respiratory support

This is a very fancy word that means shortness of breath, specifically when exerting yourself. What makes you winded. Luckily my breathing is not so terrible yet that I struggle for air just sitting around, but I know that day is coming. Just not yet. I do get winded at the stupidest little things, and it is frustrating. Getting dressed for example. I break out in a sweat and breathe a little harder when I have to get dressed in people clothes. A walker trip to the bathroom leaves me very sweaty and breathless. Taking a shower involves frequent breaks to catch my breath. Talking for long stretches of time can leave me breathless. I claimed three here probably longer than I should have if I was being honest. For now I’m holding steady at a two.

11. Orthopnea
None
Some difficulty sleeping at night due to shortness of breath. Does not routinely use more than two pillows
Needs extra pillow in order to sleep (more than two)
Can only sleep sitting up
Unable to sleep

Another fancy word for shortness of breath, but this one pertains to breathing while lying down. I don’t use more than two pillows, but my bed tilts so that’s cheating. I can no longer sleep lying completely flat, because it’s hard to breathe. Not impossible, but difficult. My score here is a two.

12. Respiratory
insufficiency
None
Intermittent use of BiPAP
Continuous use of BiPAP
Continuous use of BiPAP during the night and day
Invasive mechanical ventilation by intubation or tracheostomy

When I was first assigned a BiPAP machine, I didn’t really need it. I had no breathing problems at all, and just enough sleep apnea to barely register. I was prescribed this machine more because eventually I was going to need it and I should get used to it sooner rather than later. That machine died, so now I’m using an AVAP machine – which is the strongest noninvasive ventilator there is. Not because I need that strength yet, but because I will. I can definitely sleep without it, but it’s beginning to get to a point where I like to have it. My breathing is still above 50%, but only just. My score here is a three. I fear this category the most, because this is what’s going to kill me.

So unless I’ve done the math wrong, my score is still 29. It’s a good thing, when the score stays steady.

So that ALS-FRS scale. It’s not perfect obviously. There are symptoms besides these that come up; my incontinence for example. There’s not a damn thing about that on the scale. There is nothing about muscle cramps or twitching. It only covers the major symptoms that happen to the most people with ALS. It is not a complete picture of living with ALS, but it is a very good numerical representation of how well someone is surviving – not necessarily living – with ALS. It is a standard against which to measure, a way to measure decline, a common language. It is a very useful start.

And hopefully this post was useful to you.

Rise and Shine

If you call me when I’m sleeping, I’m gonna miss your call. That’s how it is. It’s a physical comedy of errors when the phone rings while I’m sleeping, and it goes like this:

  1. Realize the phone is even ringing. This has always been a problem, specially if I’m having a weird dream. All my dreams are weird.
  2. Untangle limbs from the blankets by flailing like a flipped turtle. I sleep with blankets free-floating on the bed, to be scrunched up as body pillows and bolsters as needed. When I fall asleep, I usually have the comforter and sheet across my belly, one blanket scrunched up under each elbow like arm rests, and one bundled up across one shoulder to cradle my head in place. God knows how that looks when I wake up. Some days, all of the blankets are still on the bed. Some days.
  3. Find the phone. Where is that noise coming from. Unearth it from the stratum of blankets.
  4. Pick up the phone. This needs both hands.
  5. Determine who is calling. If it’s someone I need to contact, proceed to step 6. Otherwise, go the hell back to sleep.
  6. Find the bed remote, to raise the head and sit up.
  7. No seriously, where is the remote. I left it on my stomach when I fell asleep. Did it fall alongside me? Is this it? No, that’s the AVAP hose. Is this it? No, that’s the catheter tube.
  8. Seriously where is the fucking remote.
  9. Give up and try to sit up from laying nearly flat. This involves flailing my arms like a contorted back stroke, realizing I can’t sit up because I’m tangled in a Gordian knot of throw blankets, unearth myself, perform the bed-ridden backstroke again to get myself up on my elbows, and heave myself upright.
  10. Pull off the AVAP mask.
  11. Lean over to turn the AVAP machine off.
  12. It’s too far. Scootch my butt closer so I can reach the button.
  13. Paw ineffectively at the machine because my fingers are garbage meat noodles, finally manage to turn it off.
  14. The phone has stopped ringing.
  15. Find the fucking bed remote lying one inch out of my previous reach.

So yeah, if I’m not anticipating your call, I’m missing it. Leave me a voice mail. I’ll call you once I catch my damned breath.

Friday Night Panic

(It’s Friday night. J is over, we’re playing Horizon Zero Dawn. It’s late. We’ve been playing for hours and my back is a little cramped from sitting weird on the bed. Things quickly went sideways.)

we need to move

Ok, Body, we’ll shift a bit.

no, like, we need to move right now. right now. move. need.

Move where?

the chair i don’t give a fuck need to move now it’s kinda hard to breathe

Ok, I’ve asked J to let us out. We’ll sit in the wheelchair for a little bit. He’s gotten up to go to the bathroom. Let’s take our time.

HEY WHY CAN’T WE BREATHE RIGHT NOW

We can, Brain, but it’s always a little hard to catch our breath when trying to scoot across the bed. It’s an awesome bed, holy crap so comfortable, but damn is it hard to move around in. We’re just short of breath from trying to haul ourselves to the edge. It’s ok.

it’s absolutely not ok

OK YAH SERIOUSLY? WE CAN’T GET AIR IN. LIKE AT ALL.

I can reach the breath stack exerciser. Let’s use that to get a proper breath and then we’ll move some more.

it’s not working can’t breathe

WHY IS IT NOT WORKING WHAT’S GOING ON WHY CAN’T WE BREATHE HOLY SHIT

Calm. We’re just tired, let’s get to the edge of the bed and sit up straight. Expand the lungs.

can’t breathe can’t breathe can’t breathe can’t breathe can’t breathe can’t breathe can’t breathe can’t breathe can’t breathe

Ok, how about the AVAPS machine? It’ll help. It’s designed to breathe when we can’t.

IT WON’T POWER ON WHY WON’T IT POWER ON??? HOLY FUCK!! ARE WE GOING TO DIE RIGHT NOW?! WHY WON’T IT POWER ON? WE ARE DYING!!

No, just..

can’t breathe can’t breathe can’t breathe can’t breathe can’t breathe can’t breathe can’t breathe can’t breathe can’t breathe

J IS GOING TO COME BACK AND FIND US DEAD ON THE FLOOR IF WE DON’T START BREATHING!! RIGHT NOW!!

Maybe we can call him for help.

not enough breath to yell can’t breathe can’t breathe can’t breathe

WE ARE GOING TO DIE RIGHT NOW

Oh god. We really can’t breathe. What do I do?

You Calm Down. You’re Not Dying.

Um…you’re new. Hello? You are?

Hello. I Am Your Higher Brain.

can’t breathe can’t breathe can’t breathe can’t breathe can’t breathe can’t breathe can’t breathe

YOU KNOW WHAT IF WE DIE RIGHT NOW IT WOULD TOTALLY BE OKAY IF THIS IS WHAT IT’S GOING TO BE LIKE ALL THE TIME BECAUSE WE CAN’T BREATHE

I have a Higher Brain?

Sure. You Know How When You’re In A Crisis, You Get Really Pragmatic And Don’t Panic But Just Do What Needs Doing? That’s Me. I’m The Opposite of Lizard Brain, Your Primal Instincts.

sssssUP, hOW’SSSS iT gOING?

can’t breathe can’t breathe can’t breathe can’t breathe can’t breathe can’t breathe can’t breathe can’t breathe

HEY WE SHOULD TOTALLY CALL 911 RIGHT NOW. TRY TO STAND UP, GET SOME AIR IN HERE?? WE ARE GOING TO DIE. LITERALLY DIE.

I’m standing but it isn’t helping!

can’t breathe can’t breathe can’t breathe can’t breathe can’t breathe

Listen. Calm The Fuck Down. If You Can’t Breathe, You’ll Pass Out. That’s Not Dying. You’ll Have More Than Enough Time For An Ambulance To Arrive. This Is OK.

BUT EVEN IF THEY COME IN TIME THIS IS JUST GOING TO HAPPEN AGAIN AND AGAIN. MAYBE WE SHOULD DIE AND BE DONE WITH IT. THIS IS THE WORST THING THAT’S EVER HAPPENED.

J is here, let’s have him call an ambulance. I’m standing here gasping like a fish and I can’t tell him what’s wrong. Oh no, he’s ..kinda panicking.

Then Don’t You Think We Should Stop?

can’t breathe can’t breathe can’t breathe can’t breathe can’t breathe

STOP WHAT? DYING?

Panicking. If We Calm Down, We Will Be Able To Breathe.

Wait, is that what’s going on? …Is that why we can’t breathe?

ALS Isn’t A Switch. It’s Gradual. You Don’t Suddenly Lose Use Of Your Hands, Why Would You Suddenly Stop Being Able To Breathe?

Oh. Ohhhhhhhhhhh. Shit. OK. Yeah. J even just asked us if that’s what this is. That’s exactly what’s happening. This is a panic attack. This is the worst panic attack I’ve ever had in my entire life, but it is just a panic attack.

can’t breathe can’t breathe can’t oh…ok..i think i remember how to. ok. gonna try to pull in breaths nice and slow. ok. i think we are ok.

NOT DYING! WOOOOOO!! HEY, OUR BAG JUST CAME UNDONE AND WE ARE SECONDHAND PEEING ALLLLLLLL OVER THE CARPET!

My Job Is Done. Later, Guys.

Cool, only now what do I do about calming J down and dealing with the pee going everywhere?

Not My Department.

HEY IT PROBABLY CAME UNSCREWED A BIT WHILE WE WERE SCOOCHING ACROSS THE BED, YEAH? THAT WAS LUCKY.

Wait, what?

yeah what, how is this helpful?

WE’RE NOT PANICKING ANYMORE. NOW WE’RE WORRIED ABOUT THIS. THAT WAS A USEFUL DISTRACTION. J’S WORRY AND THIS MINOR CRISIS HELPED PULL US OUT OF THE WORST PANIC ATTACK OF OUR LIVES. OH ANDI GUESS HIGHER BRAIN HELPED A BIT. BUT NOW WE CAN BREATHE. HOLY SHIT THAT WAS SCARY.

You make several fair points.

COURSE. THAT’S WHY I’M THE SMART ONE.

…Whatever You Say.

Aftermaths

Well hello. I survived the surgery, it went really well and I am recovering nicely.

…Except for the part where the nurse used chlorhexidine on my hand when starting my IV. Despite the bright red wristband stating I am allergic to chlorhexidine and latex. So that was a weeks worth of itchy fun.

…Except for the part where the IV came out of my hand during surgery so they had to start a new one on my other hand.

…Except for the super nasty UTI that happened and went undiagnosed for a couple of days because I figured it was just irritation from the catheter during surgery. That sucked, really really bad. PROTIP: urine should not be dark and cloudy with red bits floating in it. Get that checked out.

…Except for the doctors really, really not fucking around when they say OxyContin causes constipation. Do not ignore this advice when they tell you to get lots of fiber.

…Except for my hands being unable to fasten and unfasten the elastic bands that keep the bag strapped to my leg. So the bag just kind of kicks it with me in bed.

…Except for the night before last when somehow the bag became unscrewed? And I secondhand peed the bed?

…Except for my body apparently deciding overnight that since I don’t HAVE to use the toilet to pee anymore, getting up off of the toilet is not a thing we get to do. Not easily. Helluva thing to discover at 1 AM due to getting stuck sitting on the toilet for 45 minutes until I finally managed.

All sarcasm and bitterness aside, this thing is fucking amazing. I wore proper underwear for the first time in almost a year. I did not have to put on a pee pad for the first time since October 2017. It’s taking some getting used to, of course – being able to feel balloon inside my bladder has taken some serious adjusting. I also wasn’t really expecting it to be literally a slit cut in my belly with a tube shoved in. There is no connector, no futzy plastic anything, literally a slit with a rubber tube sticking out of it. Thank your lucky stars I’m not about to show that to you. I now have the most hardcore body piercing EVER.

There’s a couple of things I have to do to fully adjust, including hitting up my seamstress friends to help me build some kind of a cover for this thing – so that it doesn’t accidentally become unscrewed again, I don’t get super sweaty having this plastic thing that doesn’t breathe sitting against my skin, and I don’t have to look at a literal bag of urine sitting next to me all the time. I want to get some fat quarters of spooky fabrics and make cute little bags to stick the bag in and disguise it somewhat. Anything to make it look even a little less than exactly what it is.

For now though, I am obscenely grateful for having only to get up once or twice to empty this thing a day, which only involves me getting in the wheelchair and rolling up to the toilet to dump it. I don’t have to transfer all if I really didn’t want to, I have a receptacle to empty the bag into while still sitting in bed. But so far it hasn’t been a problem. I’m not sitting in my own pee right now. I can’t tell you how amazing it feels to be DRY. How awesome it is to be able to wear my cute underwear again. It was worth it. Absolutely no question about it. I’m so glad I did this.

And I’m sure you’re super glad too, because this is hopefully the end of my urine stories. I’m not even gonna make the title a pee pun. You’re welcome.

Pee Brain

When will I stop writing about urine, I hear you ask.

NOT FUCKING TODAY, FRIENDS.

Strap on your diapers, because I am not done yet. BUT MAYBE SOON!! For today I’ma tell you how my urology appointment went.

The short answer is: disasterously, and then really well. The longer answer is: I fucked up and thought my appointment was at 10:30, not 10, and so I missed it like an idiot. I made another appointment for their next free spot, June 4th. Meh. Luckily, someone ELSE fucked up THEIR appointment and missed it, so they called me as I was wheeling myself back to my van and they were able to see me after all.

Two wrongs do, in FACT, make a right.

Sooooo I wheeled myself back to their clinic, we filled in my new patient paperwork even though I am not a new patient, and after all the stress of the morning, J and I got a bit testy with it. “Please list all of your medications” it said. “Go fuck yourselves, you already have this information like eleven times and I am NOT WRITING OUT ALL THIRTEEN MEDICATIONS AND VITAMINS I TAKE,” I thought, and J angrily wrote in “see your records”. I felt guilty for like…a microsecond, because they were being nice and seeing me after I fucked up but HOLY SHIT SERIOUSLY YOU GUYS ALREADY HAVE THIS INFO AND MY HANDS DO NOT WORK SO I WILL NOT BE WRITING YOU A LIST. AGAIN.

We were called back to the room. I was cheerfully asked if I could provide a urine sample. I thought, but did NOT say, that they were welcome to wring out my diaper for some if they want it that badly. Out loud I said that would be very hard, can we skip it. She cheerfully said it was no problem, ushered us to our room, and then came back with an ultrasound machine to see how much urine I had in my bladder. Luckily I was able to lean back my wheelchair so she could turn down the waistband of my skirt to get goop all over me, swipe a wand across my belly, and announce I have 208.

208 what, was never explained.

She disappeared and the urologist came in. I am getting of an age, finally, when sometimes my doctors are younger than I am, but my first thought was “what is she, TWELVE??” so I had to quiet my internal grandma. In order to address my outer grandma problems. She recapped my problems in a nutshell, from my records, to see if she understood properly. She pulled a list of my meds from her chart folder to confirm them with me.

I FUCKING TOLD YOU SO.

She asked how often I get up to go to the bathroom. 3 or 4 times a day, I told her, but it’s closer to say anymore that I go to the bathroom, then get up. Or get up as I am going. Depends on the day. Depends on my body. Depends, literally, are on my body. I would like that no longer to be necessary. She told me that 4 times a day isn’t enough, I really should be going every 3 or 4 hours.

My heart sank. PLEASE PLEASE PLEASE DO NOT SUGGEST KEGELS AND REGULAR BATHROOM BREAKS. PLEASE. PLEASE KNOW WHAT ALS IS. I CAME TO YOU BECAUSE THE OTHER ONE DIDN’T.

“That said,” she told me, her manner becoming instantly less dismissive, “that isn’t very useful to you. You can’t spend half your day and all of your energy just peeing. Not to mention the dramatic increase in fall risk, from transferring in and out of your chair all day.”

Friends.

Friends?

FRIENDS.

The heavens split asunder and choirs of angels with pom poms appeared in the sky and the stars spelled out SHE FUCKING GETS IT. I remembered to breathe.

“Let’s talk options,” she said, and she told me everything I’d already researched myself. We agreed the superpubic catheter was a great option for me, she told me the risks and rewards. It’s a procedure done under sedation, she said, but she’s done emergency ones at bedside with just a local; it’s really simple. I did not tell her I had watched a video of one being done bedside. She said her people would call me to schedule the procedure, and to expect to have it done within the month.

Just like that, five minutes. All agreed and going. They called me the next day. My procedure is on the 4th, the day my rescheduled appointment was supposed to be. Within 2 weeks, I will be on my way to diaper independence. I have so many pairs of cute underwear I can not wait to be able to wear again. I can wear my awesome leggings again without having to worry about peeing them if I can’t peel them off fast enough. I will be done sitting in my own urine because I could not get out of bed fast enough and wondering if I have the energy to get up and do something about it or not.

Most importantly?

I will be done telling complete strangers on the internet all about my incontinence.

And now you’re as excited about this as I am.

Scars

ALS is whittling my body, and marking it as it goes.  Most of the changes to my body are slow, gradual shifts that are only noticed once the damage has gone pretty far.  Holy shit, I’ll realize one day, the palm of my hand is concave at the base of my thumb.  Huh.  My calf just tapers from my knee to ankle, instead of the graceful curve it used to have.  A slow, glacial injury without drama, but still with much import.

Some of the marks ALS has left on me were more sudden; sharp, violent signs of change.  I’m no stranger to scars – I used to self-harm as a teen, into my twenties, and what is a tattoo after all but a pattern of scars filled with ink?  Each of these little marks my disease has left me tells a little piece of my story – a concession, a loss, a search for an answer.

The first scar is the first nail in my coffin.  One and a half inches, on the outside of my left thigh.  A thin, straight line of white against pale skin, flesh tone once described as “ghost-ass white”.  I’m regaining sensation there, but for a long time it was a patch of numb skin.  I got this scar from the biopsy that sealed my diagnosis.  A little chunk of flesh taken to examine for nerve degeneration, degeneration that was confirmed and my fate thus sealed.

The second scar was First Blood. An L shaped mark behind my right side, under my rib cage. I took a fall getting out of a car, catching my flesh on the corner of the door as I went down. My first disease related injury, and sadly not the last – but so far the only one to leave a mark on the map of my body.

The third scar is two-part. A dash and a dot. The scars of my port surgery. A dot over the right artery in my neck, where a line was fished through, snaked into my system of valves and tubes and blood, and connected to a bubble of plastic that rests under the second scar. A one inch line cut and pulled apart for the port to be shoved in and connected to the plumbing. This was a violent scar but a relief to get; it’s made infusions of medicine indescribably easier. My only visible to the public scar, a surgical badge of Legitimately Sick.

The fourth scar is one of persistence. A year of puncturing the port for infusion has left a pink dot under the incision line. Scar tissue building up with each stab, eventually making the stabs less painful. A welcome scar.

I have an appointment on the 20th of May to discuss acquiring my fifth scar, the scar that will hopefully make my life oh-my-GOD so much easier. I’ll speak with a urologist actually familiar with ALS and therefore not liable to suggest that I do some motherfucking Kegels to keep from peeing myself all the time. I’ll ask for a superpubic catheter to be inserted, and hopefully get approval and a surgery date. And hopefully then I can go back to wearing clothes that I don’t have to strategize how to get out of in 30 seconds or less otherwise they get peed on. I wanna wear my shark onesie again.

I’m willing to get a scar over it.

Clinic Day

Okay kids, it’s that time again. Let’s have the update on the status of things.

My first appointment of the day was with the social worker from the ALS Association. I was introduced to a new employee, and I had been asked if this new employee could sit in with me for the whole day to get an idea of what clinic is about. I’ve had a couple people sitting with my sessions; I think I’m a good candidate for kind of easing someone into the job. I’m not so far down the road that I’m a bitter, depressing train wreck, I’ve got a pretty good attitude about things, and – as evidenced by this blog – I am not opposed to sharing. He was a really nice guy and understood a lot of the gaming and geek references that J, Gecko, and I shot back and forth with each other throughout the appointments as usual. There was nothing new to report on my part for this segment, the house search is still ongoing, the appeals for help are coming along, I’m doing okay here.

Second appointment was with Kelly the dietitian. Because she is also my friend, she had brought me birthday presents of two graphic novels from a series I adore called Lackadaisy. Happy birthday to meeeee! The official part of my appointment went smooth as usual, I received my typical good-natured lecture about drinking water instead of soda, otherwise everything checked out. I remarked to Tony, the observer, that presents aren’t usually part of appointments, but I would never be upset if they were. ALS shuld come with fabulous prizes. Or at least an administrative assistant. Kelly is incredibly thoughtful and it’s a pleasure to be her friend – not just for the comic books.

Third appointment was nursing. I had a few things to talk about here, things that would be addressed by the corresponding specialists as the appointments happened that day, but it’s always good to give the nurse a heads up so she knows what to follow up on.

Fourth was respiratory. My absolute least favorite appointment of all time. We started with the standard cough check, which is still great. Second thing we always do is check my inhale capacity, a test where I put a mask over my nose and mouth and inhale as sharply and strongly as I can. I’ve always done really well on this test. The respiratory specialist agreed with me and told me my sucking was great, upon which J, Gecko, and I did our level best to not lose our shit laughing. We are 12. The last test is the worst one, where I lean into a mask again with my nose plugged and after a couple normal breaths I forcibly exhale as much as I can and keep exhaling and keep exhaling until I feel like I’m breathing myself inside out, and then sharp inhale. I do this three times. During this appointment, the test machine said “great effort!” on all three tries, which it doesn’t always do, so I felt really encouraged. Until she told me my breathing had actually still gone down a bit. Not as dramatically as before, though. Still not dangerous levels yet, but still very much declining. I tried not to get discouraged and mostly succeeded.

Appointment number five was neurology with Dr. Olney, the new partner for Dr. Goslin. I mentioned to him the biting my tongue in my sleep thing, and how I’ve been using a night guard to get around it, and he asked me a very obvious question. “When did you start your new medication, again?” I replied it was… Right around when I started biting my tongue. Derp. It had not occurred to me at all that it might’ve been the side effect of the new medication, but when he asked that question it seemed very obvious that it was the problem. It’s still not a dealbreaker, just like the weird sleep pattern is not dealbreaker. That is the price I pay for not spiraling into a black mood at 4 AM every day, and it is worth the price of admission. I also talked to him about bladder control, or in my case the absolute lack of, as a follow-up to my previous conversation with Dr. Goslin. She had told me what my options were, and I went home and researched the mall because that’s what I do, and I had pretty much made up my mind how I would like to address this. I’m getting really sick of wearing adult diapers, they’re expensive, and they don’t always work properly and I wind up wetting the dang bed anyway, and I’m getting preeeeeeetty tired of sitting in my own pee. I have two options for a catheter, one is the traditional Foley catheter which is just a tube snaking into your urethra and a bag attached to the other end. It’s functional, but prone to infection, and for women it’s not something that is fun to have to sit on at all times. The other option, the one I’m now pursuing, is called a superpubic catheter. It’s where they punch a hole into your abdominal wall, connecting a tube through there, which drains into a bag, same as the other. It’s reversible, it’s not a horrible procedure to have to endure, and it would eliminate the need for a very uncomfortable tube in a very sensitive place. We talked about my previous experience with the urologist, how I stopped going to her because it was obvious she had no idea what ALS was. (Here’s a clue: we are not going to fix my problem by doing Kegel exercises.) He gave me the names of two urologists that have worked with their patients before and were trusted. I’ll keep you posted on how this goes.

Sixth appointment was pulmonology. The last clinic had me meeting with the pulmonologist also,and so this is a new part of the Clinic routine. My standard physician was on vacation in New Zealand (jealous!) so I had seen a practice partner. Who I actually really liked, though my regular doctor isn’t a bad guy at all; his partner has a better sense of humor is all. He had grabbed my breathing machine records from the net, because my AVAPS automatically uploads my sleep reports to the cloud and that’s not even creepy at all what are you talking about. He was overall pretty happy with my results, but still chided me about needing to use my machine for more than four hours a night to get the best results, and more than 60% of the time. I was properly chastised and promised to be better about using the machine. Which I stayed true to by the way. So far. J came over and helped me this last weekend with cleaning and organizing, and now my AVAPS doesn’t live on the arm of my wheelchair or the corner of my bed anymore, it has a proper home now, on a shelf just out of reach when I’m lying in bed. Since I have to wake up properly and sit up to turn the machine off, I’m not pulling the mask off after only four hours anymore. I also was encouraged to keep up with breath stacking. Because of course I was.

Seventh and final appointment was with PT/OT. I didn’t get to see Deb unfortunately, but I like all the specialists. We breezed through the appointments since I didn’t really have anything to report. We tried the grip meter on my left hand just for funsies, and it registered nothing at all. “…You got it to move,” she told me encouragingly. But not enough to register as a grip. Meh. I promised to hit them up when I start needing help with other things, like toileting and whatnot. I’m still doing pretty okay on my own with those things, for now. But I will be very happy when I don’t have to get up to pee anymore. I can still do the necessary things, but only having to do them once a day instead of four or five will be a very welcome change.

So that’s what we did. And that’s how it went. Steady as she goes, with nothing new to report except a 6% decrease in breathing capacity. They didn’t even have me bother talking to speech therapy this time; I never have anything to report. Oh, there is one weird thing that happened. We did the FERS scale and my self-evaluation actually had me at 32, higher than the 26 I was last time. When I got the letter afterwards, they hadofficially put me at a 30. Which is still higher than it was last time? I have another appointment with Dr. Goslin in a few weeks and I will ask her about that then.

And now you are up-to-date.

Body, Mind, Self

So as usual, it’s 3 AM and instead of sleep I am staring at the ceiling. It’s been kind of a rough day. I took a shower, which anymore is a Herculean task and wears me out pretty well. J came over and shaved my head for me, and the shower afterwards was both marvelous relief from all the little itchy bits and frustration at my newfound inability to scrub my skin properly. I am out of breath, tired, and not completely clean when I get out. I feel a little defeated, if I’m being honest. I’m still doing infusions, and so there is two weeks out of the month when I cannot take a shower. I scrub the best as I can with washcloth and baby wipes, and after the last infusion of the cycle is done I take a very long warm soak in the shower. It feels like a second skin sloughs off and it feels wonderful. Anymore though, my attempts to scrub are coming up short.

After J goes home, I’m sitting in bed and screwing around on the Internet as always and someone posts a meme on Facebook gets under my skin. It wasn’t directed at me personally, but it was something I am six shades of defensive about and it bothers me a lot. I left a sarcastic comment reply, closed my laptop, and declare that’s enough Internet for one day. As usual it takes me a very long time to fall asleep. As usual it’s because of unwanted conversation.

I feel a little queasy.

hey uh sorry about that i think it’s the new meds

Oh, hey, Body. How’s it going.

not so great today. i couldn’t get hands to work proper, so now i’m not actually as clean as i want to be, but i really don’t think there’s anything i can do about that? i mean i’m trying really hard?

Yeah, that sucks. It’s almost time to admit defeat,huh?

HEY LOSERS WHAT’S HAPPENING

What’s up, Brain. Kind of expected you earlier to be honest. Body and I were just talking and it’s probably time to start thinking about getting some help for real.

YEAH THAT SUCKS AND I DON’T WANT TO THINK ABOUT THAT.

Well, we’re going to have to. Eventually. And you know this.

WELL LIZ IS COMING OVER TUESDAY TO START TALKING ABOUT SHARE THE CARE NONSENSE. LET’S DEAL WITH THAT THEN.

We kind of need to think about that beforehand, though. And Liz can’t really help us come to terms with needing bathing assistance. She didn’t sign on for that. That’s something we have to do.

HEY REMEMBER THE SONGS WE USED TO SING IN SUNDAY SCHOOL?

… are you serious right now

For real though, Brain. I need you to focus for a second.

FAAAAAAAAAAAAATHEEEEEERRRRRRRR ABRAHAM! HAD MANY SONS!

Are you fucking kidding me right now. Brain.

MANY SONS HAD FAAAAAAAAAAAATHER ABRAHAM!

Dude. Get your shit together. This is serious. Body is not really able to help us as well as she used to. I’m not asking you to pick up the slack or anything, we all know that you can’t. You know Body is going to fail us completely, and you and I need a plan for when that happens.

i don’t want to let you down

I AM ONE OF THEM

I know that. It’s not your fault.

whose fault is it then

I really wish I knew. I’d kick their teeth in.

well we couldn’t really. not anymore

AND SO ARE YOU

Yeah I know that too . But we have an entire posse full of people willing to do it for us.

we are really lucky aren’t we. all things considered

HEY HEY HEY

YES, Brain, what.

REMEMBER IN SUNDAY SCHOOL? THE SONG WITH THE STOP AND GO SIGNS? AND HOW IT WOULD COMPLETELY MAKE OUR WEEK IF WE WERE THE ONES PICKED ON TO HOLD UP THE SIGNS DURING THE SONGS? WITH THE LYRICS ON THE BACK? “STOP!! AND LET ME TELL YOU”? REMEMBER HOW PROUD WE WERE WHEN SISTER MENG CALLED ON US TO LEAD THE SONGS?

….Yeah. What about it?

I MISS THAT. LIFE WAS SO MUCH SIMPLER.

i could do anything

Yeah. I miss that too.

DEEP! AND WIDE! DEEP! AND WIDE!

Yep. So much simpler. Go to sleep, asshole.

Clinic Day 1-14-19

Hello my lovelies!

Monday the 14th was Clinic Day. I had originally wanted to do a video update for this, but I just haven’t felt up to it? And if I wait until I feel up to it this update is going to take for freaking ever and then you all will start to worry because you think it’s all bad news. Spoiler: it is not. I am just lazy.

When we first checked in, I was given a sheet to fill out. I am pretty sure I did this last time as well, but I do not remember. It was a self assessment sheet for the ALS functional rating scale (ALSFRS-R). This is a standard way to track the progression of the disease. Usually though, the care team fills this out for me. During the nursing portion of the appointment, it was explained that they are heading towards having patients fill this out for themselves, as a sort of experiment to see how the self assessments differs from the professional assessment. I actually assumed that the self assessments would be worse, since I tend to understate my difficulties when talking to someone, but am more honest with myself filling out a form. Apparently not everyone is like that, since the nurse would tell me that for the most part the scores are consistent. Huh. There are 12 categories, and each category is rated from 0 to 4, with four being completely normal and zero being nonfunctional. For instance the walking category is rated as 4 being normal, three being early ambulation difficulties, to his walks with assistance, one is nonambulatory functional movement, and zero is no purposeful leg movement at all. I am currently at a two. So then you take all of the numbers and add them up, which gives you a number from 0 to 48 and 48 is a normal human being with no difficulties at all. It’s a nice numeric way of tracking progression, and encompasses a lot. It isn’t perfect of course, but it’s a nice shorthanded overview of everything at a glance. You can check it out for yourself here, if you wish.

Last Clinic Day, I was at a 34.

My first appointment of the day, after getting to our assigned room and being handed a schedule, was nursing. In addition to explaining a couple of substitutions in scheduling (I’ll get to those) she went over what I just talked about with the self assessments. Nursing checks are always an overview of the big picture in my life, if I need any special appointments made, if there are any concerns I have outside of the specialists I’ll be talking to that day. We talk about any changes since last visit, and in general she is my master coordinator of all things. Nurse Nancy is amazing and I am lucky to have her on my team. She also explained she would be playing the part of my dietitian for the day, as Kelly was sick. Poor thing. I always look forward to talking to Kelly, even though I never have anything to report. My eating is fine, we usually wind up just chatting for most of the appointment anyway. Today, however, it was noted that I had lost a whole 11 pounds since last clinic. Normally, this would be a good thing for someone, but not so good when you have ALS. I weigh 211 pounds currently, up from 160 when I was diagnosed, all of that on purpose. I am under strict orders to not diet to lose weight, as extra weight statistically helps with prognosis, and when I am no longer able to eat I will need that extra wiggle room. So to speak. So we talked a little bit about my dietary habits and I mostly attribute the weight loss to no longer being at work and on any kind of schedule, so I kind of eat whenever I feel like it, which is usually only once a day. Anymore I also need help with food prep, so if I want to eat something besides the frankly embarrassing amount of snacks I have stashed in my room, I have to bother my mom. Not that she minds, at all, but I am terrible at inconveniencing others for my own sake. Nurse Nancy made me promise to stop that. I am making an effort to stock more snacks and not just drinks in my fridge that I can get to whenever I want.

My second appointment was with Dr. Goslin. It had actually been a few appointments since I’d seen her specifically, since the previous appointment was my introduction to the new doctor, Dr. Olney, so it’d been half a year. We spoke mostly about the medications for this appointment, my increasing depression specifically and overwhelming anxiety. Unfortunately no longer reporting to work means I no longer have a distraction to keep me from spiraling into bad moods when I think too much. It’s been a rough couple of months because of that. I still don’t have a permanent place to live and I am continuing to freak out about that. We doubled my dose of Ativan in the meantime, and she gave me a couple of options to think about for ongoing anxiety and depression. Otherwise, as usual, she is very pleased with the rate of my progression.

My third appointment was with the power duo team for occupational and physical therapy. Physical therapy pointed out that my calves are getting tight and I need to be better with my stretches, and since I can’t really stand on my feet and touch my toes anymore to stretch them out, I was given bands to put around my toes and use my forearms to pull up on them while I’m laying in bed. The whole point of that is to keep my muscles limber so that I can continue to use the walker to visit the bathroom while I am at home for as long as possible. Occupational therapy was entirely centered around keeping my hands functional as long as possible, and preventing my fingers from curling up and cramping while I sleep at night. We also measured my hand strength as usual, and of course they are still garbage meat noodles of uselessness, no big surprises there. Deb the Amazing OT had previously suggested a new kind of brace for me to buy, and I had, but they need adjusting and some modifications to make them actually usable on my own as they are primarily built for bicyclists and didn’t have crippled people like me in mind so they’re not exactly easy to get on and off. We made plans to have another appointment outside of clinic to go over all of those things. I’ve since had that appointment, and like a total genius I forgot the braces in question at home, so she wasn’t able to adjust them for me and now I have a second appointment this coming Monday to take care of that. I was told if I forget them next time I’ll be sent right back home. Hehe.

The fourth appointment was speech. This appointment also includes swallowing, and all of those muscles in general. Luckily, this is the one area that I have yet to experience any problems, so these appointments always go very fast. She just verifies that I am not choking on my own spit anymore than a normal human being does, watches me swallow liquid, and eat a dry graham cracker, and make some funny faces including blowing my cheeks out and trying to touch my nose with my tongue etc. to prove that all of those muscles are still in tune in good shape. So far so good.

The ALS Association was fifth, for social work. I remain eternally grateful for their help. Unfortunately the problem I most need help with is housing, which is not their specialty by any stretch. They do however have resources and connections to other services that are useful, and most importantly they have always been willing to do research on my behalf to do what they can to help. During this appointment, we talked a lot about senior services to get my mom some assistance in whatever way we can. It’s all very useful information, and I truly appreciate the help. They have connections I would never even dream of and that alone is extremely helpful.

My sixth appointment was respiratory. This is the one I hate the most. It’s exhausting, and for the last few Clinic Days, it’s also been somewhat disheartening. We spoke a little bit about new policy changes, specifically about the clinic no longer being able to keep equipment for their patients and so I have to take my respirometer home with me every time now. No big deal, it’s not that big and it fits in my purse just fine. The breathing test came and went as usual and I was surprised to find that my breathing has actually remained perfectly stable since last time. I don’t even need to tell you how happy I was to hear that. I think sleeping with the new AVAPS machine has been helping, and I still need to recommit to breath stacking of course, even though I hate it. A lot. I still owe you guys a demonstration video of exactly why that’s so miserable. But still. No change. Great news. I will take it.

Usually, that would be the end of it. I typically have six appointments. However, since I have graduated to the new sleepy time breath machine, we have added a pulmonologist to the mix. I met with him last. My standard doctor was apparently on vacation in Ireland or something, so I met with a substitute from his practice. It’s a shame he was a substitute, because I really like him actually. He had some suggestions about the mask I’m using at night, some accessory suggestions for the machine, and sheepish apologies that a lot of these commonsense accessories aren’t covered by insurance at all. I came out of that appointment with another doctor appointments to make for a new mask fitting. That will happen on Tuesday.

After that, I headed downstairs to the lab for some overdue bloodwork – I was supposed to have done so last time apparently, but we missed it. Most of my levels came back normal, but I am once again experiencing a vitamin D deficiency. Probably because now that I’m not going to work I don’t really get out into the sun at all. Ever. I take a 1000 IU supplement, but after seeing my levels Dr. Goslin told me to bump it to 4000. Apparently my deficiency is not screwing around.

After all of this, and my summary letter came in the mail, I discovered that my ALS FRS rating has gone down to 26. Down eight points out of 48 total in three months worries me a bit, I admit, but I don’t know if that’s me shifting from doctor to self evaluation or what. I certainly don’t feel like I’ve declined that far in so short a time. But my breathing is the same, and that’s what I care the most about right now. So I’m happy.

And that, my loves, is how Clinic Day went.

Surreal

Man, what even IS my life anymore.

I got a voice mail the day after that last post from my awesome nurse. “Hi, I read your blog, and I have some ideas about the AVAPS.”

Uhhhhh..hi there. I forget that people read this, sometimes. I have the best freaking care team ever, you guys. I got contacted from three different folks to ABSOLUTELY ASSURE ME that the machine would get paid for, the rules are more bendy for folks with ALS so please don’t stress about it. And then thanks to Nurse Awesome, I was contacted yesterday by Providence Respiratory to adjust my settings remotely to ramp that sucker down.

OMG SO MUCH BETTER GUYS.

It had previously been forcing air down my throat so hard my ears were popping. Seriously. I can handle it now. I might even get compliance on my own without my awesome peeps having to argue for leeway. It’s still not exactly comfortable, but it’s so much more manageable.

Thursday was noooooooot a good day, there were a million things going wrong, including hurting myself by trying to lean over in the chair to pull something out from under my wheels. I felt and heard something go POP, and owowowowowow. Not a crack, though, and breathing was still ok, so I just rode it out and after a few days’ soreness I’m alright. Then Thursday night someone said something a bit unkind and I kinda completely lost my shit for awhile. Occasionally there’s a trigger and I mourn the person I don’t get to become. Five years ago, I had my very own house, I had just lost a lot of weight and was fitting in all my cute clothes again, I was going to school to further my career, I had all my shit sorted out and my life was pretty fucking great, actually. Thursday night I let myself grieve for her, because that future is dead, and I let myself be angry at people who say really stupid shit.

Friday I had the distinct displeasure of taking the sodding van in for repair. It cost me $919. I’m extremely grateful to Intel for their short term disability benefit easing me out of the workforce, because fortunately I HAVE that money. For now. Although now the brake light’s coming on. So that’ll be fun.

Saturday I did nothing but sleep and fuck around on the internet.

Sunday I did something SUPER COOL but is not my story to tell, so just believe me when I say it was amazing.

This morning my cat woke me up because I could hear him chewing on plastic. When I located him, he had a huge long piece of cellophane coming out of his mouth, and was choking on it a little. I could not get him to come to me for ANYTHING, and when I stood up and tried to get to him, of course he backed away. I took another step towards him and fell right on my ass. It didn’t hurt, even though I’ve kinda got no muscle in my ass to cushion the fall anymore, but it knocked a bit of breath away.

And then since I was on the floor, my fucking cat decided to come check me out and I was able to get the plastic out of his mouth.

Motherfucker.

The fall didn’t hurt, but getting off the floor did. I wound up sitting on my foot rests and using that wheelchair lift feature to get myself up off the ground, but even with the grab bar on my bed, the chair to push off of, and my walker on standby, I wasn’t able to get over up on the bed. I had to lower myself back to the floor and let myself catch my breath before I tried again, and was very, very grateful to the Depends company for their fine product and my forethought to start wearing them to bed otherwise I’d have exacerbated my situation by having to catch my breath sitting in a puddle.

Welcome to the TMI Show.

Now, I have my awesome watch, so I was never stranded. I always had options. I’m just stubborn as fuck. I texted J but he was not immediately responding, so I tried the wheelchair lift again, and used it to ALSO steer myself right up against the bed this time, and was able to get in bed. And then after catching my breath, getting up to go to the bathroom to change my disposable shorts. I cried for like half a second, but realistically I know this is definitely going to happen again. Probably not the cat part, but there will definitely be another fall, and next time I very probably will not be able to get myself up. It’s twelve hours later and even though I’ve been in bed all day I’m still exhausted from the ordeal. So that sucks. If I hadn’t had such a huge meltdown Thursday, it would have happened today.

So that’s my life, this last week. Kinda a shit show, but there were definitely some good bits in there.

Also I wanna restate that my care team is amazing, especially my nurse. <3










Catching My Breath

Jeez, peeps, it’s been a month since I’ve posted. Good thing this blog isn’t monetized or anything. A kind commenter left me a little prod about being so quiet, and I realized I should get off my ass and say something. Or…get ON my ass, since writing involves that position. It’s been awhile that I’ve been in a place to be able to pace and dictate or whatever.

I digress.

The first couple weeks of October, I don’t even have an excuse for not posting anything except perhaps laziness. Well, obviously laziness. No ‘perhaps’ about it. The last two weeks were kind of rough if I’m being honest. Politics caused me a lot of sleepless nights, and I wish I had it in me not to care about any of it, but I don’t. I often say the one silver lining to this disease is that I get to check out early, whereas the rest of you people have to stay and deal with this. I won’t be alive to see California slide into the ocean, or Handmaid’s Tale cease being fiction, or Auschwitz’s rebirth in my own goddamned country. There is a small grace in that. It still doesn’t prevent me having sleepless nights just absolutely freaking out over everything I cannot change and crying a lot. I wish the only thing I had to worry about was my actual disease.

The main thing going on in the last couple of weeks has been transportation problems, and breathing problems. I had an appointment to get my new AVAPS sleep machine, which I was very nervous about because it’s a whole new goddamned concession to this bitch of a disease, and I’d asked J to go along with me. Unfortunately, when we got there I was not able to get out of the fucking van because the automated door mechanism decided not to work. At all. We were used to it being a flaky bitch and sometimes not latching closed properly, but this was new. The door would not open all the way even with J pulling it open manually, so the ramp could not extend. And so I sat in the parking lot missing my fucking appointment while Jay feverishly tried to figure out how to free me. The door mechanism has been kind of flaky pretty much since I got it, and she’s been in for repairs twice already. J managed to get the door to open all the way, but he missed work the whole day instead of just a couple of hours while we took her in for an emergency appointment. The mechanics there were able to temporarily Band-Aid the problem.

By pretty much breaking my door.

Their fix to my door not automatically opening was to simply cut the cable that opened it and turn it into a manual door. Which is not, of course, a fucking fix. It was a preventative measure to keep the cable from snapping of its own accord and shearing apart the motor. We got a proper appointment later that afternoon to see what could actually be done, and after having my van for over an hour they explained that the entire mechanism would need to be replaced. Oh and now also the cable. Which, after the last time the van was in for service, we already knew replacing the assembly was going to be the next step which is why I had previously asked them to replace the entire assembly in the first place, and they never called me back. A month ago. So yes please can we actually REPAIR THE FUCKING VEHICLE.

This was all after getting pissed off that the mechanic would only talk to J and ignore me – because I’m just stupid dumb woman who doesn’t know anything about vans LOL. J very politely asserted that it was only MY name on the paperwork and registration and I would be the one paying for the repairs, so really he should be addressing me, not him (J is a champ). Thankfully, the mechanic did speak directly to me after that. But seriously. Dude wasn’t even old, he really ought to know better. They always do that; they will look at the paperwork read my name, and then turned to Jason and address him as Mr. Ross (THAT IS NOT HIS NAME EVEN) and explain what needs doing.

BITCH I AM SITTING RIGHT HERE AND WILL BE PAYING FOR THIS HI HELLO HOW ARE YOU. FUCKER.

Moral of the story is that we agreed the door assembly and cable need replacing. I kind of had to insist that we do that – the mechanic was seemingly trying to talk me out of it like, the door is working? (HI NO IT IS NOT BECAUSE IT IS A MECHANICAL DOOR THAT IS NOT FUNCTIONAL AT THIS TIME) The repairs will be expensive? (I HAVE A FUCKING WARRANTY) We got our van back, with the door still needing to be manually opened and closed before the rant will work, and the mechanic sets to ordering the parts that were needed and getting warranty authorization for repairs.

…A week later he calls to inform me that the warranty is declined for the repairs, because my contract does not cover those parts. He thought it sounded fishy and asked to see my actual contract. I have agreed the hell it doesn’t cover the freaking door, I paid $2000 for that fucking warranty it had better, and agreed to send him a copy. Turns out my fucking contract does not cover anything but the ramp itself, and the drive train. Literally nothing else. I paid $2k for them to not fix anything but a catastrophic failure THANK YOU ARIZONA MOBILITY FOR YOUR USELESS GODDAMNED SERVICE CONTRACT. So I am on the hook for $1000 repair. I have an appointment to go ahead with the repair on Friday, and then yesterday the ramp itself decided it doesn’t want to work either and refused to work for a moment. And then did work. Much like the door itself would sometimes decide to close and sometimes not. So I probably get to look forward to getting that repaired soon as well. Theoretically at least that will be covered. IN THEORY.

After missing the breathing appointment, I came to find out that the respiratory folks would actually have been able to come to my apartment in the first place to set up the machine. THIS IS USEFUL INFORMATION I WOULD HAVE LIKED TO HAVE. YOU KNOW, BEFORE. So we set up an appointment for them to come to me, and last Tuesday they delivered my new machine. It is, as promised, the most powerful noninvasive ventilator you can get. It costs $6400. If I can prove compliance – by which I have to use this machine at least four hours a day for 21 days out of 30 – then my insurance will pay for it outright. Otherwise I’m going to have to rent this thing for $700 a fucking months Covered by insurance, sure, but my co-pay is about to reset in January and I will be goddamned if I am paying for this fucking machine on top of everything else. I’ll save my precious out of pocket money for the $17,000 a month infusion meds that aren’t helping. I guess. So I just need to prove to Providence I am going to use it when I sleep. Pinky promise.

That’s turning out to be much easier said than done.

This machine is indeed much more powerful than my old CPAP was. Too powerful. It’s kind of like breathing from a turbine engine. It has a ramp function, where it will slowly turn the air up, but even that is not exactly comfortable. By which I mean it is genuinely hard to breathe with that machine on. I can’t take a little bit of air, it’s like drinking from a fire hose. If I try to breathe shallow to prevent the machine inflating my lungs like balloons, it freaks out and tries even harder. Eventually I have to pull in a huge lungful of air, which the machine helpfully cuts off the airflow after a second and I have to breathe through my mouth to catch my breath entirely. I have fallen into a routine of putting the mask over my face and then just checking out the Internet or watching TV or something for an hour or so before bed to try to get used to it before I am actually attempting to sleep. Like, distract myself and trick my body into being cool with my new overlord breathing on my behalf. It’s not really working. The only way I’ve really been able to sleep with it is if I medicate myself either with Ativan or Ambien first. Even then, I wake up fairly often. I find myself constantly checking the machine to see if I’ve hit four hours of use yet so I can pull the mask off and sleep comfortably.

This machine has taught me what my actual limit for life is.

As I lie in bed struggling to breathe, I know that when this becomes my existence, and not just an artificial effect produced by a machine, I am going to be done. I cannot live that way. It is abjectly miserable, and it is impossible to do anything else when you are struggling so hard just to get air into your body. That is not going to be a life worth living. When the machine is doing my breathing for me, and there is nothing in my brain except an animalistic fight for control over the oxygen coming in and out of my lungs, that will be the time to say goodbye. Because that is not a life.

For now though, I have the ability to pull the mask off. I counts down the time until four hours has passed, when I can pull the mask off and sleep properly. For now life is still great. I have ideas and plans. Including a very important plan to call the respiratory specialists and see if there is some adjustment that can be made to make me a little less abjectly miserable when I’m trying to sleep. To make sleep possible at all without taking potentially addictive medications. Friday I will take the van in and try to get it limping along for a little while. I literally just needed to last a couple of years. Sunday, I get to do something really really awesome I am super excited about. I have things to do. People to see. Life to live.

One breath at a time.










Clinical Anxiety

Clinic was Monday! Let’s break down how it went, shall we?

PT/OT: My hands now no longer register ANYTHING on the strength test. Fuck. My arms are still plenty strong, though. My biceps are a force to be reckoned with from essentially doing push-ups on my walker every day. I have an appointment to follow-up with Deb the Awesome to reimagine my spider hand braces, since my wrists droop badly enough now they’re not helping much. It doesn’t do a lot of good to keep my fingers propped up if my hands as a whole are curling under. My finger joints are doing great though, still a lot of flexibility in them so I’m not going to be clawhands any time soon. Yay!

Dietician: (Hi, Kelly!) My weight remains stable, so I’m to keep doing what I am doing. I need to keep mindful of feeding myself while I’m at home, now, since I don’t have the routine of work to set that schedule for me. My mom doesn’t know to bring me food unless I ask her because she’s old as hell and eats like, a tic-tac a day and calls it a meal. (Hi, Mom, love you!)

Nurse: I forgot to ask her what my chair weighs. Dangit. It’s written down somewhere in my chart and I’m curious what that thing weighs without me in it. Combined, we are 627 pounds of geddafuggoutmaway. She arranged my appointment with Deb, and I didn’t otherwise have much for her. I rarely do. That’s a good thing.

Social Worker: Have I waxed poetic lately about how amazing the ALS Association is? Because damn. Single-handedly saving my sanity more than once, and saving my ass multiple times. We arranged for them to pick up equipment that I’ve borrowed (FOR FREE) that I no longer need because my disease has progressed beyond their use. We then spoke about some other situations that are stressing me out, like the lack of social services for my elderly disabled mother, and she promised to dig up what resources she could for my mom in our area. She sent me an email not even a day later with a bunch of places to check out. THAT is how amazing ALSA is. My mom’s not even on their roster, but because helping her would help ME, they were totally on it. I LOVE THE ALS ASSOCIATION.

Neurologist: Usually I’d be seeing Dr. Goslin, but today I met with her new partner. I’d seen him talk at the ALS Research Symposium, and I’d been given his bio before when I was asked to write something up for him explaining why the ALS Multidisciplinary Clinic was such an awesome thing. It was nice to meet him, and the dude has one of those old-school doctor bags that J wanted to steal. Plus for geek. It was a general get-to-know-you kinda appointment.

Speech: These appointments always go fast because I’ve got no symptoms at all yet. Puff up cheeks, move your tongue, eat this dry-ass graham cracker so I can watch you swallow. NBD, nothing to report.

Respiratory: Yeeeeeah this is always my absolute least favorite, not least of all is because it’s actually HARD. I’ve actually been noticing decline here, and since this is the part of ALS that actually IS going to kill me, I don’t like having a concrete measurement of how shitty my disease is. And yet. I want that measurement, so that I know, so that I can plan, and manage expectations. I came to this appointment knowing my breathing has gotten a bit worse lately; it’s taking a bit longer to recover when I exert myself, and there’s been a few times I wake up in the night because my breath is a little short. I also had to report that my CPAP machine (which I am now supposed to use every night) is busted, doesn’t power on at ALL. We are going to get me a new machine, called an AVAPS and I have no idea what the difference is because I keep forgetting to look it up.

Hang on.

“Noninvasive mechanical ventilation with average volume assured pressure support”

That tells me nothing. 2 secs.

…Oh. It’s…basically a non-invasive respirator. So it’s hardcore. OK then. That’s…intimidating. But I had the choice between getting my CPAP replaced or getting this new hotness, and since I still have Cadillac Intel Insurance for another year, I really want to get the expensive stuff now.

With that out of the way, we did all the usual tests. First they stick a rigid plastic thing in my mouth and I exhale as hard as I can to make these little indicators move; it measures cough strength. Cough strength is still normal; it was down ten points from last visit but she wasn’t worried about that at all. The next test involves a soft plastic mask over my nose and mouth and inhaling sharply; I always ace that one by going beyond what it measures; I guess I’m really good at..sucking…? Monday was no exception. The last test is the worst. Both in what it portends, and the work it takes to perform. My dudes, it is HARD. It blew goats even when my lungs were as strong as ten oxen. It involves inhaling deeply, plugging your nose, and then blowing out as hard as you can, for as long as you can, while getting encouragement shouted at you to GO GO GO MORE MORE MORE MORE and then when you can’t possibly exhale anything else and you feel like you’re going to pass out, another sharp, fast inhale.

Do that three times.

It actually makes J a little uncomfortable to watch, because it’s so obviously hard. It’s intense, it feels like hell, and at the end you have a number that represents your average lung capacity. When I started going to clinic, my scores were over a hundred percent – a very strong set of lungs. Over the last year, I’ve watched that number go down. She wasn’t concerned, really, even 80% was still really good! and she had no recommendations for me except to continue with the breath stacking exercises, which is where you inhale as much as you can and then use a balloon and tube to squuuuueeeeeeeeeeeeeze more air in. and hold. and release. And when you’re no longer light-headed, do it again. And again. I often describe it as reverse drowning, because that’s what it feels like. I do that, but not as often as I should. Six months ago at Clinic I hit 70% and she was a little less cavalier about me not doing them every day. 3 months ago on Clinic day, the machine was busted so I was spared. She wasn’t worried about it though, as my other tests were about the same as last time and she expected the same for this test, too.

I knew it wasn’t going to be the same. I feel a difference. When I eat too much food, I can feel that it’s harder to breathe – not that I’m short of breath, exactly, but I feel that when my lungs don’t have proper room to expand, there’s less strength in my diaphragm to bully the rest of my guts out of the way, maybe. It’s not harder to breathe, exactly, but I notice that I am breathing. And I was keenly aware that the breathing test this time was the hardest it’s ever been. I could feel veins on my forehead. She told me the result.

60%.

I’ve gone down 10% in six months.

I am now to do breath stacking twice a day, and sleep with the AVAPS every night, once it arrives. Next Clinic maybe we’ll do the respiratory early; having it be the very last thing in the day might have fudged my numbers a bit since I’d be tired. But somehow, I didn’t think that will matter. I didn’t take it well at all, and was in a shitty mood the rest of the night, and spent pretty much all day Tuesday crying or sleeping. I feel better now, hence why I have it in me to post tonight, but it kiiiiinda cemented something I’ve been thinking the last few months, something that I haven’t said out loud or posted or anything because I don’t want panic, either from myself or from any of you.

I am pretty goddamned sure I don’t have another 4 years.

I mean, it would be nice? But I’m not going to live to 50. I know that. I’ve been really fucking lucky to make it 4 years, and still be able to be on my feet awhile and wipe my own ass and everything. Some people with ALS don’t make it through ONE, and I’ve already had four, officially diagnosed, and probably closer to six since symptoms first appeared. I’m so, so fucking lucky. I get to see my death coming and plan for it. It was just rude as fuck to see that imaginary timeline become somewhat ..truncated, from what I was telling myself. But now, the part of ALS that will kill me has officially begun to kill me and I don’t have as much time as I thought.

You know what though?

That’s okay.

It really is. This is how ALS goes. This is normal. It’s okay. I’m alright.

Tonight, I am sanguine. There will be more freaking out; count on it. (See you at 3am, stupid brain) At this exact moment though? I have a clarity most people will never, ever experience. I see a world in 5 years without me in it, and it’s a good world and those I love are doing fine, in that long-term place. There’s a delicious release that comes with knowing so far in the future is officially Not My Fucking Problem. Today though, I am making many short-term plans. Hangouts with friends. An art show opening. The Walk to Defeat ALS on Sunday. A zoo trip with family. Neil DeGrasse Tyson – TWICE – in November. I still have a future to plan. It may be abbreviated, but goddammit I have SOME time. I get to make plans. It’s a fucking privilege to tell someone I’ll come to an event in April and know I can. After that. Who knows. My timeline is finite, truncated, and not guaranteed, but I have one. I can see what’s coming and make peace with it before it happens. I get the rare and amazing privilege to become friends with my own death.

And that is fucking awesome.










A is for ALS

A is for Almost.

Two more days. TWO MORE DAYS. And then I’m done with my working career. Three weeks of vacation as a formality. The rest of my life is a blank book, with ALS having already written in all the margins.

A is for Atrophy.

My muscles continue to waste away as ALS kills the neurons transmitting signals to them. My legs are meat stilts, capable of minor movement only; walking on them is a matter of mechanics and getting my knees to lock properly so I can balance ON them rather than WITH them. My hands are curling up into claws of uselessness. My mouth still works, to the detriment of some, and my brain always will. My body is wasting away into the meat shell it will eventually become.

A is for Avoidance.

Most days I don’t really think about it all, except as an abstract idea. Sure, I’m going to die. I have that roadmap. In my day-to-day life, though, the Big-M-Mortality idea makes way for the general practices of getting through life. ALS intrudes in all things, of course; drinking a soda is now a two-hand operation and I can never even pretend that my life is normal again. All of that, though, is background radiation anymore. It’s amazing what can become normal, given time.

A is for Abbreviated.

My life has a shortened length. For some ALS folks, this throws them into a fervor of living as much life as possible in the time they had left. I didn’t go that route. I’m far too pragmatic to have abandoned my job and traveled the world while I still could. I focused my efforts on making my future life more comfortable, and that meant working as long as I could. If we had universal healthcare I wouldn’t have had to worry about it so much.

A is for Adjustments.

The disease progresses, and whatever I could do a month ago, I can’t necessarily do today. Life is a constant series of micro-adjustments and new behaviors, new rules and limitations. I learn of these new limitations, often the hard way, and another compromise with life is created. The new normal evolves.

A is for Afraid.

Just cause I’ve accepted death, doesn’t mean I’m ready. I’m terrified of what this disease will continue to do to me, and what it’s going to cost my loved ones. What it’s already cost them. I hate that I’m so reliant on everyone around me, and it’s going to get so much worse.

A is for Advance Directive.

Seriously, you have to have one. Fill it out today. If I have one positive impact on your life, let it be that I inspired/coerced you to do this one thing. It’s a hard thing to think about, I know, but your family needs to know what you want. They can’t know unless you tell them.

A is for Assisted Suicide.

I don’t know for sure that I’m going to go out this way. But I’m grateful every fucking day that I have this option.

A is for Anger.

I don’t think I’ve ever questioned “why me” so much as outright stated, “It is pretty fucked up that this is happening to me.” No one deserves ALS. (There are a few people I would like to have it temporarily though. It’s a short, sharp lesson in humility and reliance on others.) I’m angry that this disease exists at all. That we know next to nothing about it. It’s brutal and unfair.

A is for Allies.

It’s absolutely true that you don’t know who your friends really are until disaster strikes. I’m grateful in a perverse way for this disease, for showing me what grace actually looks like. I knew my friends were awesome before. I didn’t quite understand the enormity of that power they have. I do now; I am witness to it every day.

A is for Alive.

For now. I continue to breathe, and so I will continue to write and think and feel and rant and swear. And as long as I am alive, I can bear witness to the ravages and the comedy and the love and the struggle and the disaster my life has become. Al of it, often at once. And so long as I have the best medical care team on my side (I do!), the support and love of friends (check!), and a sense of humor about it all (absofuckinglutely), I’ll be okay. Even when I’m really not okay. And when I die, you will know that it was all okay, too. Somehow. Someday. You’re going to be okay.

A is for Acceptance.










Bent Out of Shape

Holy SHIT people are angry about straws right now.

If you’re lucky enough to be completely ignorant of what I mean, now is a GREAT time to stop reading this entry and move on with other happier aspects of your life. No one would blame you. It would probably even save you some heartburn, because damn, there are a Lot of Opinions on the Internet right now.

Quick backstory: a picture of a turtle which had ingested some plastic straws (do not Google it, it’s super sad) has gotten a lot of people up in arms and clamoring for a worldwide ban on plastic straws. Whole and complete, no exceptions. PLASTIC IS EVIL AND MUST BE ERADICATED. This is a great and noble idea, and I fully support nature conservancy and saving the planet and all of that other awesome stuff. Go Mother Earth.

The problem, of course, is that some people actually fucking NEED plastic straws.

I have read more disability erasure bullshit in the last couple of days than I have read probably in the last year. Actual sample quote: “Why is this even a question? You just pick up the glass and drink from it, how hard is it? No one actually needs a straw ever.”

…Well, Susan, you ableist piece of shit, it actually is NOT that fucking easy. Friends who have been out with me to restaurants recently can attest to this, as they have uncomfortably watched me attempt to drink from a water glass without one. With my current rate of disability, picking up a drinking vessel means clasping it between my two fists (because MY FINGERS ARE USELESS GARBAGE MEAT NOODLES) and taking a sip before placing it back on the table, hopefully without spilling or running into anything. If I can pull it off and get the glass back to the table with a simple clink of glass on ceramic plate, I’ve done well. But that’s becoming impossible. FUN SCIENCE FACT: WATER IS HEAVY.

…I need a goddamn straw.

I currently carry around paper straws, for these instances. They’re still pretty wasteful, but it’s a compromise. Carrying around a reusable one is not practical, because I can’t operate fiddly little brushes or squeegees to clean the thing when I’m done with it, necessitating me to carry a sticky dirty straw in my purse until I can get home and ask someone to run it through a dishwasher for me. In a life already fraught with humiliating reliance on other people’s kindness for the simplest dumb stuff, and existing as an increasing imposition on others, a reusable straw is just one more fucking thing to have to ask people to take care of on my behalf. Paper straws are a concession to my disability and a temporary compromise for conservation.

I actually use a lot of disposable things, and feel ashamed for every fucking one. My liberal snowflake heart cringes every time I do, but using paper plates means I can actually lift the thing without spilling food all over my lap because ceramic is too heavy. Using a paper cup means a condensation-slick glass is not going to fall out of my hands and soak my bed when I try to quench my thirst. My hands don’t work well enough to clean out the cat food tin, so it goes in the trash. Every item disposed of makes me feel incredibly guilty, but these are things I have to do now. I don’t have the privilege of washing dishes anymore, or making my life more difficult in the name of reduce, recycle, reuse. It is an inconvenience to you, and a Major Fucking Undertaking to me. And I know in my heart that this is completely understandable, these are sacrifices ALS has demanded of me, and in the grand scheme of things, the amount of trash I accumulate is really not that big a deal.

Not to hear Susan tell it though. I am single-handedly raping the planet because I need a plastic bendy straw.

There’s an awesome chart going around on the Internet right now, and I’ve had the occasion to share it many, many times over the last few days. I’ll share it here, too, because it’s goddamn useful and I am tired of explaining why Product X is not a universally viable alternative to a disposable bendy plastic drinking straw. Observe:

Useful Chart of Usefulness

Currently, I have the luxury of getting away with paper straws. Keyword here: LUXURY. Soon though, soon that will not be an option. The day is coming when I’m only able to slightly turn my head to the side to get a sip of water. Eventually, not even that. I will not be able to lift my body and position myself above a cup with a straw sticking straight up out of it in order to hydrate myself. I need that stupid little bendy thing, that corrugation that makes it almost impossible to make out of any material but plastic and makes cleaning a major undertaking instead of a quick rinse in soapy water. I need the straw to be positionable, and I don’t have a devoted full-time staff who are able to hold a cup to my lips in order to hydrate myself, or constantly wash my dishes, and all of the other things that you don’t even really think about. Because you’re not disabled and you don’t have to.

But I think about them. Because I have to.

I’m learning new things all the time, myself. Before the above chart, straws as a choking hazard didn’t really even occur to me, but now that I think about it, of course they are. Of course putting a rigid thing between your teeth is an injury hazard when your jaw suffers spasticity and clamps down for no reason. Of course temperature tolerance is going to be a concern, when you are relegated to an all liquid diet and not just sipping cool drinks or refreshments. These seemingly no-brainer ideas are new to me, even.

So I’m simply asking that maybe you pause and think about these things too, before you go off on me and people like me who actually need the fucking things. Understand that the ability to do without straws completely is a luxury. Understand there is no simple answer to the horrible problem of plastic waste. Understand that consumer waste is a tiny fucking fraction of this problem, and huge corporations need to be held much more accountable for their part. As the chart says, the burden of a solution should not rest upon this shoulders of the disabled. We are the victims of this problem, not the fucking perpetrators.

Someone who thought they were being clever asked what people did before the invention of straws then, if they are so necessary? Medical professionals answered bluntly: people aspirated liquids, got pneumonia, and died. Plastic straws are LITERAL FUCKING LIFESAVING MEDICAL DEVICES.

So Susan, I’m extremely happy for you that your reusable plastic cup and rigid ass plastic straw is a viable option. For you. Captain Planet would be really fucking proud of you. Go ahead and wear that gold star. Just please recognize that other people on this planet exist, and that your solutions are not perfect ones. Recognize them for what they are. A good idea. A place to start. The beginning of the necessary conversation.

And understand you’re not taking my plastic bendy straws away from me until I’m dead. You can quite literally have them over my dead body.










Saddiversary Part the Fourth

Four years ago, I was told I was going to die.

Everyone dies. To know the mechanism of your demise, though, is a terrible and powerful thing. Oh, certainly, something else might kill me before ALS squeezes the breath from my body, but there is now a subtitle to my timeline, a definite path. The future is a language tainted with exceptions and qualifications.

I took the news and buried it deep in my chest that day, taking the bus home alone. I don’t remember what I was thinking. I remember tripping over a curb walking home from the bus stop. I remember wincing internally, absolutely certain that was going to be the catalyst for the meltdown to come. It wasn’t. I picked myself up, and thought to myself, “There will surely be much more of that.” I got home, looked around the house I had just bought, the house I would no longer get to keep, and wondered how the ever loving fuck I was going to break it to everyone.

My life is a timeline of things lost, now, a perverse sort of baby book in reverse. Vashti’s last unaided steps. Vashti’s last time putting on makeup one-handed. Vashti’s last time dressing up all by herself. Vashti’s last time feeding herself. Vashti’s last words. Vashti’s last breath, someday.

For now, I can still speak, and breathe, and feed myself mostly. I need help cutting food these days, a job my friends do graciously. It’s very sweet, even. Walking with a walker is still possible, but exhausting, and it feels more precarious than ever. I stay in the wheelchair when I can. I have the motorized one now, but no way to transport it (but I’m working on that!). My hands are just about useless; I type with two fingers that have very little strength left in them. I need two hands to lift a soda can to my lips. I bought a hand strap yesterday to put eating utensils in because I’m almost unable to grip them. Bladder control is almost completely a thing of the past.

But you know what? Fuck this disease. It doesn’t own me. I have to make allowances for its dumb ass, but it’s not who I am. I am still going to eat at all the fancy places. I am still hanging out with my friends. I am still working. In one week, I will have another birthday. I am still planning for a future, even if that future has heavy caveats.

Because fuck that shit.

Even four years later. Even knowing what it’s going to take from me. Even though it would seriously be so much easier to end it now, before it gets REALLY hard. Fuck that shit.

My saddiversary has come around once again, and it’s one more year I can give this disease the middle finger. It doesn’t fucking own me. Even after I’m a non-speaking, drooly, pees-my-pants useless lump of meat, it won’t own me. Even if I decide to take my own life before it gets that far, it doesn’t win.

One more year down. One more point for me.

Fuck yeah.










Brutal Honesty

Spoiler alert: I don’t really like children. I’ve never wanted them, I don’t generally like being around them, they are messy and loud and completely irrational and they trigger my social anxiety like woah.

Disclaimer aside: I fucking love how brutally honest and open children are.

They can, as Fred Rogers said, spot a phony a mile away, and they will call it exactly as they see it. And a quality I’ve come to adore: they will ask questions. Adults will stare and make a point of NOT staring, and talk about anything BUT what they want to ask, and dodge the subject so thoroughly you’d think it was a game everyone is playing but you. Don’t Mention the Wheelchair, the worst party game ever.

But kids? Kids will come out and ask and feel no shame, and it’s refreshing as hell.

We went to dinner tonight, at a place with a LOOOOOOT of stairs. There’s a secret elevator entrance way in the back, but you have to have a host/hostess escort you because it’s seriously a maze and you have to go through a business building’s security desk. So tonight, when J wheeled me to the front counter to await seating, it was understandably baffling to a little girl how someone in a wheelchair was going to get up all the stairs she’d had to navigate to get in.

She couldn’t have been older than four. Adorable little thing in sparkly shoes and pigtails, and she turned to her mother when she saw me rolling up. Asking in that louder-than-normal-voice-whisper that kids have, she asked, “How’s she gonna get up here?”

To her credit, her mom was unembarrassed and handled the question honestly. “She’s not.”

“There’s a back way,” I told her.

The girl asked her mom, “Why’s she in the chair?”

“I don’t know,” her mom answered. To my delight, she did NOT try to hush the child up or make a big deal about it. When parents try to silence their kids’ questions, it feels like I’m some sort of shameful thing that has to be swept under the carpet. And hey guess what, when you skirt the issue? You pique the kid’s interest. Oh I’m NOT supposed to talk about this? GUESS WHAT WILL BE OUR TOPIC OF CHOICE TONIGHT. I *can’t* say those words? Well then BUTTS BUTTS BUTTS FART DOODY oh hi Grandma! Today I learned FARTS!

The child then, sensibly, turned to me. “Why are you in that chair?”

“My legs don’t work,” I told her honestly.

“How come your legs don’t work?”

“I have a disease. It makes them very weak. I’m not very strong anymore.”

“Oh.” She considered this new information, and then very logically continued, “well *I* am.”

“I can see that! You look very, very strong.”

And she flexed her little arms for me, beyond proud.

And that was the end of that. We shifted the topic to her shoes, which were very sparkly and lit up when she stomped, and she danced her own little disco until our table was ready and I was wheeled away. Hopefully, she will retain that honesty and people in wheelchairs will remain something normal, to have frank discussions about, and hopefully her parents continue to raise her well and when the answer is “I don’t want to tell you why I’m in this chair” or “It’s private why I only have one eye”, both parties deal with it with grace.

I see it as a continuation of all the conversations I’ve had with children, “Why is there earrings in your nose” or “how come you got purple hair” or “why did you draw all over your skin forever”. I enjoy those conversations because of their complete lack of judgement, their total curiosity. Not, “ewww you are weird and that’s bad” just “why are you different?” It’s an honest, open conversation and the world needs more of that.

So that’s the story of a completely charming child I spoke to last Sunday.










Cyborg Status: Confirmed

So uhhh I have my port installed. It was …an adventure. Lemme tell you all about it!

When last we left our intrepid heroine, she had a really nasty reaction to the adhesives on her PICC line dressing. We moved to a different type of dressing that didn’t adhere for shit. The day I was done with the 14 day initial run, my awesome home infusion nurse came by to change the bandaging. She recommended we get the PICC pulled ASAP so I could get the dang adhesives off my skin.

It took a little convincing to get the nurse to refer me for a port; she was concerned that if I had a reaction to the adhesive as it was, having a port would be just as bad. I was concerned about not having tubes sticking out of me at all times; at least the port would give me a 2 week break every month. We eventually secured the referral, and my surgery was scheduled for 12:30 on December 27th, with a separate appointment that morning at 8:30 to get the PICC removed. When I told my home infusion nurse, she arranged to come by that evening (after her company holiday party, even!) to pull the PICC so we could get the wrappings off me and let my skin recover a bit. We love Deena, she’s rad. It was surreal to see her pull the line…it kind just kept coming out, like a magician’s scarf of surgery and horror. It was MUCH longer than I’d thought. And it was friggin’ DELIGHTFUL to have all that out/off. And the shower I was finally able to take was goddamned MAGICAL.

I received a call from St. Vincent’s hospital the next day – the surgeon I’d been scheduled with wasn’t actually going to work that week, could I go to Providence Portland instead? At 9:30am? Sure, no problem. The next day, they called again – sorry, could we make it same time, but back at St. Vincent’s after all? No problem. J worked that day, so I asked my sister-in-law-of awesomeness Jeanette to be my ride.

And then I made a terrible mistake.

OK so I’m a smart person, and with intelligence comes curiosity, and I wanted to know exactly what I was in for with this port. I watched a video of a woman demonstrrating how to access a port; no big deal, kind of persnickety with regards to keeping everything absolutely sterile, but whatevs. And thennnnnnnnnnn I watched a video of a port install surgery. OK, I am not really squicked out by surgery, and I found the whole thing fascinating until the phrase “BLUNT DISSECTION” came up, and the surgeon LITERALLY TORE A HOLE IN THE PERSON BY JAMMING HIS PINKY IN THE INCISION AND WIGGLING IT AROUND. HOLY FUCKBALLS. My brain went NOPE, FUCK THAT, FUCK THIS, AND FUCK YOU FOR SCHEDULING THIS MOTHEFUCKING MEDIEVAL-ASS SHIT. Intellectually I knew it was fine – MORE than fine, because I was gonna be hopped up on Versed and remember NONE of it and get some good drugs afterwards – but the actual crux of the mistake had been watching this video righe before bedtime, so my brain just did donuts in the parking lot for awhile.

The morning of the surgery, I got a call from the hospital – would I mind coming in early? It was 8:30 and I was scheduled to check-in at 9:30, so I was a bit confused, but I told them I’d be there as soon as I could. We parked too far away and I was a sweaty mess when we arrived at check-in, where I was told…I was scheduled at the OTHER hospital, still. I explained the conversations, they luckily sorted it out. I went upstairs, put the goofy little gown on, and waited. The nurses came in, partly to take vitals and start an IV and make sure I wasn’t pregnant, and partly to play peek-a-boo with my adorable niece. The doctor came in to introduce himself and ask if I had questions. he was delighted that I referred to the Huber needle I’d be using to access the port as “the crooked needle”. I was delighted by his use of the phrase “twilight sleep” to describe the conscious sedation I’d be under. They’d give me benadryl in my line along with the sedatives and a dose of antibiotic, he said, hopefully I’d just sleep through the whole thing.

I remember them wheeling me into the surgery, and seeing the GINORMOUS fucking monitor he’d be using to make sure he was placing the port properly, with x-ray. It was weird to see my name there. The several nurses had their A-Game banter going, and we were having a great time explaining names and tattoos to each other. One nurse thought I had an accent, and asked how long I’d been a Yankee. “My whole life?” A male nurse/technician/whathaveya scrubbed me down with a blue solution, making me the world’s least convincing Smurf. I was freaking cold, despite the warmed blanket they’d put over me. The IV nurse told me he was putting the antibiotics in my line now, and I felt the cold sting. he told me he was starting the sedative now, and then I…

I do not remember a thing after that.

I woke up with a very large image of my x-ray on the monitor, showing the line running into my artery. I was shivering. They wheeled me back to the recovery room, and I slept a bit. Jeanette had taken my niece Jewel to get some lunch or something. They brought me a turkey sandwich, which I devoured. They had put the dressing on a bit shitty; I couldn’t raise my head all the way, or turn my head much because of the way my skin was taped up. It itched a bit. I was given discharge instructions and no pain killers (“Just take some Tylenol”). My sister came back, I got dressed, she took me home, and I slept some more.

The next morning I was aware something was up. I was naughty and pulled the dressing off a bit to allow myself the ability to raise my fucking head, and where the dressing had been tugging was now an angry red wound. I was very itchy. All over my chest. Angry red hives, all over my chest, up my neck, and across my chin. Tylenol, it turned out, did not fucking suffice, so I took some of the hydrocodone I had leftover from breaking my ankle that the doctor hadn’t wanted to give me either because ibuprofin should be fine. YOU JUST TORE A HOLE IN ME BIG ENOUGH FOR A 1 INCH SQUARE MEDICAL DEVICE AND ALSO CUT MY NECK TO SHOVE WIRES INTO MY VEINS – NEWSFUCKINGFLASH TYLENOL AND IBUPROFIN ARE NOT SUFFICIENT FOR THIS. BLUNT. DISSECTION. BITCHES. I slept some more. I woke up to even angrier hives, annnnnnnnnd my eye was a swollen bag of fluid.

IS THIS NOT LOVELY?

So uh…yeah. I was having an allergic reaction to something. It couldn’t be the dressing itself, since the hives were everywhere. It got worse and itchier over the night. I had a different IV home nurse visit that day, to change the bandage since they’d thoughtfully left a needle in the port for me to start the infusions, but I’d bled a bit so there was gauze under the dressing which needed to go. She was very concerned about the reaction, namely that it might be a reaction to the port itself. My delightful friend Lizzie was coming over that day with delicious food for me, and she kindly picked up some Benadryl on her way in. Luckily I had a doctor’s appointment already scheduled for the next day, for an unrelated issue and holy FUCK will I rant about that in a bit. I took Benadryl, ate delicious home made food, and slept a lot.

My PCP is a cool guy, and didn’t mind dealing with the reaction as well as the initial problem we were scheduled to deal with. He suggested two more antihistamines in addition to the Benadryl, and to contact the hospital if it didn’t clear up by Tuesday (Monday was New Year’s). Luckily it did, mostly. I think the problem was the smurf shit they scrubbed me down with, since the hives pretty much appeared everywhere they used it. I have no explanation for my eye becoming a blister, tho.

We’ve now completed the second round of infusions, the port works beautifully, and we pulled the needle out last night. I am once again dressing-free for two weeks. The scar is already nearly healed up, and the itching has almost entirely subsided now that I’ve got no more adhesives on me. I can feel the port under my skin, and it’s weird.

Now I am become cyborg. beep bloop bleep.










Welllp

Good thing we’re gonna go ahead and do the port; when the dressing was swapped out Thursday, my skin was all KINDS of pissed off. I was pretty sure I was having at least a mild reaction to the adhesive, because surely it shouldn’t be THAT itchy? Apparently no. It should not.

Soo we are trying a different adhesive that doesn’t adhere as well, I’m finding, and we will hopefully get the port installed soon.