April Fool

I’ve always, always hated April Fool’s Day.

I’ve only ever been – at best – ambivalent towards the holiday. I don’t generally like pranks, because usually what I see aren’t so much as pranks as people being complete dicks to an innocent person. It’s a really mean-spirited holiday. I believe in open communication and trust, and this holiday celebrates being awful to people. The general rule is, “if it’s not at least as funny to the victim, it’s not a prank.”

Three years ago, April fools became something else to me. It became Diagnosis Day. Sadiversary. Three years ago, I sat in a doctors office, and was told I was going to die. Horribly. I had previously joked about having this appointment on this holiday, joking on Facebook that regardless of the results of this appointment, no one was going to believe me. I now tell people that my diagnosis was the un-funniest April fools prank ever.

Three years later, I’m taking stock of everything I thought since then, and everything that has become. I knew that no matter what I thought was going to be the problem, my real troubles were likely to be things that never occurred to me. I was mostly correct. I’m a pretty smart person, and observant, so I saw a lot of my troubles coming. I’ve surprised myself with how well I’ve handled some things I thought would destroy me. The loss of my hands. The death of my 23-year-old cat. And, predictably, some things surprised me by how intensely I reacted to them. Or, as has usually been the case, how little I reacted to them. My first fall. That was kind of a, “well that sucked.” Instead of a nuclear eruption of emotion. Often times a completely excusable meltdown has instead been met with, “yeah, okay, there’s that.”

Tuesday, I had my second semi serious fall. As is mostly usual I can’t even tell you exactly what happened to make me fall. I can tell you that’s a major contributor is that I OUGHT to have been holding onto something, and I wasn’t. That would’ve helped. Instead, I went down like a ton of bricks and somehow twisted my knee. It hurt badly enough that I was nauseous for a moment, and had to lie there a moment to catch my breath. I can tell you exactly how I managed to twist my knee, but I did so. Just so. And so for the last few days, I’ve been having a taste of what it’s like to be immobile. I’m used to being able to walk around my apartment, simply leaning on the walls for support. I couldn’t put any weight on my knee at all. And living alone meant that in order to get to the bathroom, I had to swivel myself onto my Walker and push myself around the apartment with my good leg. It was really…

… Lonely.

I wasn’t expecting that. I was expecting helpless, and frustrating, certainly. But it hasn’t really been the helplessness and being bedridden lately that got under my skin so bad. I’m a very independent person, and will fight to hold my own, on my own, until I am actually dead. It wasn’t really that I wanted help? But it was just as when I’m sick. I just wanted someone else around. Had I had a roommate at the time, I would have completely ignored them. As usual. But sometimes it’s pretty awesome just knowing someone else’s around. Especially when you’re hurt or ill.

Three years ago, I was completely able to stand up out of a chair on my own. Without using my hands, without even thinking about it. And now, when I try to get up off of the toilet I can’t even remember how my legs did that. How my body was able to just… Stand up. How I was able to run up a flight of stairs. It’s not even depressing so much as bizarre to me, that I have completely forgotten how to do simple things I used to do without thought. I expected frustration, anger at my ability to bend over, balanced on one leg, to pick something up off of the floor being taken away from me. But I find myself staring at whatever it is on the floor that is vexing me, baffled at how my body used to Do the Thing. Without will, without thought. Unthinkable.

It’s been three years since I was officially diagnosed. Self-inflicted injuries notwithstanding, I’m still on my feet. This is amazing. A lot of people with ALS would be dead by now. I’m losing the use of my hands, which is why I’ve been using voice dictation to create this post, but I can still do the basics. I can use the toilet by myself. I can go get myself a drink from the fridge, as long as I’m careful carrying it back. I can still pet my cats. For now. My progression is still very, very slow. And I am extraordinarily grateful.

I still hate April Fools’ Day. I can’t really blame the holiday for my diagnosis, or even the timing, because I was given the option to have this appointment on this day. I knew in my gut what this appointment was going to be about, by virtue of having been given the option to move the original appointment closer. I could’ve waited two days. But I already hate the holiday, so why taint any other perfectly good April day with an anniversary such as this?

Regardless of how you feel about the holiday, please treat your fellow humans with respect. Make sure your prank is funny, and not just you being a dick.

Life is enough of a dick as it is.

Spoiler Alert: #everyonedies

Today’s post comes with story time!

Story one:

I’m walking with the cane into the cafe. There’s a girl who works there that I smile and say hello to practically every day. She notices the cane, today. I don’t always bring it down when I get a drink, but today I have. I’m a little wobbly, anymore.

“OH no, did you hurt yourself?”

“No,” I shrug, “I have Lou Gehrig’s. I’ve just gotten weak enough finally that I need this a lot.”

“Oh, god, I’m sorry, I’ve heard of that. Is it painful?”

“Oh no, nothing hurts, really, but it’s just a loss of strength over time. I guess that’s a good thing, nothing hurts even if you ARE dying slowly,” I half-joke.

“Well aren’t we all,” she smiles back.

“Truth,” I tell her, and we part ways.

Story Two:

We’re walking to a restaurant, my coworkers and I. We parked kind of far away, and I’m struggling a little with my cane, to keep up with the crowd.

“Keep up,” he jokes, falling behind to walk next to me.

“I’m trying,” I tell him, grinning. “Y’all bastards walk too fast.”

“Well maybe you’re not trying hard enough to keep up,” he teases.

“Yeah sorry, everything’s slow with me. Neurons burning out, walking, you name it. I’m *sorry* I am slowly dying,” I joke.

“Well, everyone’s dying,” he shrugs.

“Some of us just take the fast track,” I tell him.

The Rant

Please, please stop saying “well technically everyone is dying”.

Please.

It’s like when the cashier is ringing up your stuff and something doesn’t scan and you snort, “well I guess it’s free.” It’s a dumb joke, everyone’s made it, she’s heard it a thousand times. And it’s already old and it wasn’t funny in the first place and you’re not that clever, just pay for your shit and leave. And you know it’s a dumb thing to say, but you said it anyway, and will say it again, but everyone politely laughs even though no one thinks it’s clever.

Only, …no. Okay. It’s not really so much like that. It’s..

It’s dismissive as FUCK is what it is. Yeah, okay I get it, everyone is dying slowly. We are all biding time until our own demise. Everyone, eventually dies. MEMENTO MORI.

When you tell me, “yeah well we’re all dying, right?” I know you’re trying to soften the blow. You’re trying to comfort me in a way, to include me with the rest of the human race, telling me that death is normal and it’s okay. To make light of the situation. And I will always, always joke back.

But I don’t want to.

What I WANT to say is “fuck you”. You’re completely dismissing my death. You’re diminishing the sadness of my struggle. You’re telling me that I’m nothing special, that my disease is no big deal. Everyone dies. So what? My disease will kill me but hey, everyone eventually dies anyway so what does it matter? What do YOU matter? What are you whining about? Everyone dies, so what.

So what? Yes, everyone dies. But YOUR book has a billion potential endings. Boating accident! Heart attack! Cancer! Pneumonia! Peacefully in your sleep with your loved one by your side! Gun accident~! You could die of ANYTHING! You could die during sex! You could die from mountain climbing and being exposed to the elements! You could join an international drug cartel and be gunned down on the private air strip in Boca Raton when Louie rats you out! You NEVER should have trusted Louie! You could fall on the sidewalk and hit your head JUST SO and become brain dead until your tearful mother signs the paperwork and they pull your plug. Choose Your Own Adventure Death! If you would like to die of accidental CO2 poisoning, turn to page 56!

My Choose Your Own Adventure book has three possible endings. A long, lingering loss of ability and strength, humiliation, frustration, and fear that ends in…..

OPTION ONE! Sudden accident. I mean, anyone can get hit by a car, randomly, or some freak accident, lightning strike, store robbery gone wrong. Anything could unexpectedly kill me. We’re even on that front.

OPTION TWO! Suffocation! I choke on my own spit, unable to breathe because my muscles have all atrophied and I can’t swallow or take a breath and eventually I choke to death. Drowned in my own spit.

OPTION THREE! Suicide! I decide somewhere along the story that I’ve had JUST ABOUT ENOUGH, thank you, and take some pills if I can still swallow, or push the meds into my guts via feeding tube.

THAT’S IT. Those are my options. Your roadmap to life has a lot of lingering little trails and you never know where they’re going to take you. You might decide to become a mountain climber at 60, you might die tonight, you might waste your life away at some meaningless job until you have a heart attack at your desk. Your maps are open and wide and the ends aren’t known but the possibilities are endless. My map branches three ways, and there are many many stops along the way. Loss of walking. That cuts off a thousand roads. Loss of hand/arm movement. Well there’s a ton of other destinations crossed off my map. Unable to eat. Well that’s a lot of stuff closed off to me, what with the wheelchair and the feeding tube and hell, you need a special van to travel now, you can’t just pick up and go. So my destinations are the trauma ward, a palliative care hospital bed, or a dose of pentobarbital in a place of my choice.

We’re all dying. Some of us have our stories written, and the endings are not happy. There is no happy ending for ALS. And when you compare your unwritten book to my Cliff Notes, it’s insulting.

Your story probably does not have chapters in it about falling for absolutely no reason and getting a really horrible looking scratch out of it but not allowing yourself to show pain because the people you’re with are freaking out that you fell and you have to assure them you’re okay. It probably does not feature you cleaning out a cat box and breaking out in a sweat over that small, stupid effort. It probably does not feature a feeding tube or respirator as a given course. It likely does not have six introspective chapters, each titled some variation of HOLY SHIT I AM GOING TO DIE IN A REALLY FUCKED UP NIGHTMARE WAY AND I KNOW IT’S COMING. Your story might have a little chapter about being embarrassed in front of someone when screwing up something you were trying to say, but I doubt it has six paragraphs afterwards wondering if that was a one time fluke or is it a sign your tongue is starting to atrophy too? Did I enunciate when I was on the call with my manager earlier? Is this guy saying ‘what’ because he didn’t quite catch what I said or because I have lost the ability to speak and he literally has no idea what I just said? Your book has going to work and going shopping, but does it have a pre-chapter about managing a ride that isn’t going to be too hard for you, or not purchasing #thing because you’re not sure you can lift it up in the cupboard where you’d like it to go? Your story’s ending is unwritten. Mine is written in stone, carved by hands that no longer have the power to pick up a chisel.

Telling me “everyone dies” is the same as co-opting #blacklivesmatter into #alllivesematter. You’re technically correct AND YOU ARE COMPLETELY MISSING THE POINT. And diluting the original message with your vapid need to be included. Of COURSE everyone dies. Of COURSE all lives matter. BUT THAT IS NOT WHAT WE ARE TALKING ABOUT RIGHT NOW. You are dismissing the message and selfishly, HORRIBLY, turning the story about you. In telling you that I am dying, I am not saying no one else dies and no one else has to mourn. I am not dismissing the value of your mortality. I’m not denying your story has an end. I’m telling you mine is brief. As someone put it, by saying “save the rainforests” I am not saying “fuck all the other kinds of forests, they’re deserving of destruction”. By saying black lives matter, it’s not to say others DON’T. To say that I’m dying is not to say that you aren’t.

It’s the same, also, as when you tell a friend your woes and s/he says, “That’s okay, I lost my job today.” IT IS NOT OKAY. YOUR PAIN DOES NOT DIMINISH MINE. You have a right to your suffering, and it does not trump or cancel out anyone else’s. People will often try to one-up your sadness, and I’m guilty of doing this too, sometimes, and it’s a horrible, horrible thing to do. I don’t understand what the point of it is. I see your suffering and raise you “my keys got locked in my car”. Your pain doesn’t matter, because I have a completely unrelated circumstance that I somehow have determined is more impactful than yours and therefor I am suffering worse and I WIN at the FML game! And LOSE at Friendship and Human Interactions! And I leave with a parting gift of making your situation worse by dumping all over you when you wanted comfort from me! I’m going to put that statement again in its own line, because it’s important.

YOUR PAIN DOES NOT DIMINISH MINE.

We are all dying. Some of us just know the way. And if you don’t, then I’m happy for you. Seriously. I rejoice with you in not knowing your end. It’s an amazing, free world of possibilities and I’m delighted you get to dance in that sunshine. I will read my own story, and dance as long as I can, while the rain comes, before I’m washed away. Both of our stories are fantastic pieces of literature, but because I got a sneak peak into the last chapter, it doesn’t make my book any less worth reading. Your book’s unknown end chapter doesn’t make your book better than mine, or different. And when I tell you the plot, you don’t have to tell me that EVERYONE’S story finishes. Because of course it does. I was just trying to tell you about mine for a second.

And I joke about it, because it’s a sad thing and I try to keep things light; but I want you to know that it’s crushing when you dismiss me like that. Everyone dies. Yes. This is an unfortunate fact. A fact that does not change that I have a terrible disease and I’d like to be able to talk about it without it being diminished to a non-problem by the words “everyone dies”. You don’t need to one-up this. You don’t WANT to one-up this. It’s okay. Just say ‘sorry’ or shrug and agree, or laugh with me about it, or tell me to man the fuck up, tell me anything but that I am insignificant because of course everyone dies. And none of this matters. Because I fucking matter. If I didn’t, you wouldn’t be wasting your breath to piss me off with those words.

Everyone dies.

Some of us have a story they’d like to tell, before that happens. Not because they think it’s the best book. Not because they don’t think you have one, too. But because they think it’s worth reading. So, thank you for reading mine, so far. I hope it’s been worth it.

Everyone dies, but I guess not everyone gets to blog about it, yeah?

I guess it does get easier.

Best, easiest breaking of the news so far; ran in to a guy in the cafe who used to be our network guru. He noted the cane I was leaning on.

“What happened, you hurt yourself?”

“Naw, man, Lou Gehrig’s.”

“Oh. …That sucks, I’m sorry.”

“Thanks. I’m alright. How’s the new job?”

“It’s going great! I’m really glad to not be in charge of things anymore.”

And then we dished about network infrastructures and technology and selling houses for ten minutes. And that was all. He asked as we parted if there was anything he could do, I told him I was okay, thanks, but I’d keep him posted. Sometimes the best reaction is just, “Well…shit. Sorry.” and then move on.

Genetic Go Ahead

As you may or may not remember, I was formerly denied genetic testing that would show if I had the genetic markers that would show if I’m a good candidate for trials. Since I’m on a different insurance plan, I asked if we could try and get approval again. Cigna said no, maybe Providence would say yes, since their own doctor fought so hard for it last year.

They said yes!

I’m not sure how much my portion of it will cost. I don’t really care, to be honest. I’ll afford it. There is a lot of exciting research being done right now, some promising results, and I’d love to be a data point among them.

I’m looking forward to having more information. To BEING information. I hope the tests allow me to participate. I hope I can be useful.

I hope this diagnosis is good for SOMETHING.

He, She, Me.

He:

A few weeks ago, a few very short weeks, a friend posted something in her facebook along the lines of “our routine doctor appointment turned into a little bit more. He’s being admitted right now, but please don’t worry!”

…and I worried.

She’s like me. Bubbly, happy, all about best possible outcomes, optimism, and smiles. She’s a joy to be around. He’s a sardonic, sarcastic, clever man who used to be my boss. You know he’s awesome if he used to be the boss of me and we STILL talk. He’s snarky and hilarious. They’re both a pair of my favorite people. Still can’t believe they hooked up, much less got married, but they’re fucking perfect for each other and I’m really glad they did. I love them to pieces.

So when she, bubbly, optimistic She, didn’t SAY what had gone awry, I knew it wasn’t good. And then I was invited to a support/information group created in facebook, to keep in touch with what was happening and how we could all help. And then, scary words eventually saw the light of day. Cancer. Stage 4. Scant months to live. Maybe more if chemo works.

And just like that, their lives were over as they knew it. And just like that, the floor dropped away from all of us who knew and loved them.

I can’t even pretend to say I know what it’s like to be told you have a short and definite lifespan. I know how it was for me, how it continues to be, but I can’t even fathom what he’s going through. His time is so much shorter than mine, his notice so much more sudden. He has a wife. And while they’re publicly taking it with grace, no one knows what’s going on inside. Some aspects I can guess at; the panic of Time suddenly a companion, yelling at you about all the things you have to do before you go. The complete bafflement of, how did this happen. Is there something I could have done? But then there’s also the chemo – unlike my timeline, there’s a chance for an extended cut, but only if you can withstand it. And now they have to decide quality of life vs. quantity. And I know that mental argument very well.

There’s absolutely nothing I can do but stand by and love them, and listen, and hold space. And when they make decisions, honor them. Be there as much as they will allow me to be. And then let him go.

It’s the only thing within my power.

She

I wrote about her awhile ago. She was suffering from bulbar onset ALS, and she gave me the chance to figure out and to talk about how I feel about assisted suicide. And she gave me the courage to tell all of you, and start that difficult conversation. It’s a really hard thing, to tell everyone that you are probably going to take your own life and they’re going to have to forgive and be okay with it. She did it with perfect grace.

She had been fighting the Boss Fight of ALS for awhile. Her decline was fast. I only knew her through facebook posts, and it seemed like daily there was another struggle, another development. But she faced it with so much fucking GRACE, and smiles, and gratitude. Her posts weren’t about how she’d never live to see her son grow up, they were about the daily joy she found in his company and the treasure trove of memories she was building for him. Her posts weren’t about her medical suffering, they were about the gratitude for the people who helped her through it all. Look for the rainbows, she says constantly.

April 4th, she had fought enough. She left a goodbye, and a video for her son, and the last words, “Enjoy. I have.”

And then she let go. And so I, too, let her go.

Me:

I’m losing strength in my hands.

I’ve been noticing maybe a month or two now, but I’ve been in complete and total denial. The mailbox lock has ALWAYS been hard, it’s just a bit more difficult to turn the key; must have frozen or something. The lid to the cup is way more difficult to pry off because it’s new. Cutting a piece of steak cramps up my hands, but hey, it’s just cramps. I had AGES before my foot strength was lost after the cramps started, right? My hands are shaking while holding my laptop because I’m just tired. The word of the day and things that I write up on my whiteboard every day just SEEM a little shakier. But I’m sure it’s nothing. Right?

Friday, April 4th, I fell. For no reason. It was the first time that happened; I wasn’t tripping on anything or trying to maneuver, I just…fell. And wrenched my ankle. And felt very sorry for myself and frustrated. And so I told Dr. Goslin this, on Thursday during my appointment. And she confirmed I’m losing strength in my hips.

…And I said I think I might be losing strength in my hands. She did the usual tests. And proved that I am.

I was absolutely right in that this? This is a trigger. This is panic and terror and the beginning of the end. And this makes everything so much worse. My timelines have shifted, and things I thought I had some time to do, I suddenly don’t. I have to write the letters while my handwriting is still stable. I have to do all the things I can’t, soon. And I’m freaking the fuck out. Because I don’t know what else to do but scream.

She asked if I’d like to borrow a motorized wheelchair to see how it works out. And internally I flipped the fuck out because I am NOT ready for that. No way no how. But outwardly I politely declined and said I’d like to wait awhile before going down that road. She agreed that I have a lot more time of mobility left, so there’s no rush. But it’s coming. Danielle suggested one of those old-people jar opening assist things. And I panicked a little but kept it in. I said maybe a walker, but not a wheelchair. Not yet. But my hands are going to have to be accommodated for.

After the appointment we went to the store to get some meds and some air fresheners for the empty rooms in my house that I’m clearing out for sale. And I couldn’t get the fucking tops of them off. I had to use my teeth. I still have dexterity, but my strength is going. And so, too, are all of the things I thought I could do to keep the loss of mobility tolerable. For now I can still type. I can still play video games. But I thought I had so much more time before I had to think about the end of those things. To a time when I can’t use chopsticks, to when I can’t pull myself out of bed, to when I can’t dress myself.

And it scares the motherfucking SHIT out of me.

I’ll get accustomed to the changes as they come. I’ll persevere. But I feel like this is kind of when I really start to die. No mobility? Whatever, that’s okay. Seriously. It sucks, but a wheelchair isn’t that bad. This is a hardship, but not the end. When I am no longer able to draw stupid pictures, no longer able to frost a cupcake, no longer able to chat, no longer able to launch Skyrim…that is the death of me. When I am no longer able to even fucking pet my cats. That begins the days of the useless shell that I become. I wonder if I’ll want to go get the prescription the day I drop something for no reason. I won’t use it yet, but I wonder if that’s going to be the preflight check. When I will start thinking seriously about the endgame.

And I don’t know if I’ll have the strength to let go, when all I feel like doing is trying to hold on.

And I’m really, really scared.

Anniversary

There’s a book called “Miss Peregrine’s Home for Peculiar Children”, which I love, and in the epilogue, it brilliantly describes how anything that changes you forever splits your life into two halves: Before and After.

Before, like anyone else, I had a lot of plans. I just bought a house. I had all the paint, and all the decorating ideas, and SUCH a garden planned in my head. My backyard is luxurious and I had many garden barbecue parties planned already. I had a spare room just for fostering kittens. My kitchen was a thing of beauty, I was planning amazing culinary ventures. This was going to be my forever home.

Before, my health was pretty good. I still had chronic headaches, but they didn’t really interfere with life much. I had lost a bunch of weight and was fitting into 32 inch jeans again – I felt healthy and cute, and was getting confident about my body. I wore size small shirts, and bought new clothes. I had energy, I was doing things and going out.

Before, work was reaching a comfortable zone. I had confidence in my ability to rise to whatever I was asked to do, and I saw a long career ahead. I was going to school to become an engineer and get promoted.

Before, I was comfortable in being single, I was self-reliant and independent. I could do anything by myself.

Before, I never really thought of myself as particularly important or special. I had people in my life I adored, but never felt worthy of their adoration in return.

Before, I never thought about death much. I knew academically that I agreed with assisted dying, I knew that getting paperwork done way in advance was important. I knew I should have an advance directive. I knew it happened to everyone, I knew on a high level what happens and that there’s a ton of complication and high emotion when it occurs.

A year ago today, I was in the middle of the Medical Folderol and had recently discovered I couldn’t stand on my toes anymore. A year ago today, I sat in Dr. Goslin’s office and stared at her hands while she told me that I have ALS.

After, I use leg braces, knee braces, and a cane to help me walk. When I walk down the hallways at work, I usually don’t bring the cane, but walk with one hand brushing against the wall the whole time. My social worker called it “wall surfing”. Walking a block exhausts me. I carried five empty boxes up the stairs last week, setting them on the steps, walk up a couple of steps, pick up the boxes and put them a few steps higher, repeat. I was sweating and out of breath by the time I was done. Walking the mile to the bus stop is out of the question. I carpool with an awesome coworker in his big red truck, and I know there’s going to be a time soon that I can no longer physically get in his truck. I can’t manage the one step up into my house, I have to brace my hands on the doorpost and pull myself in and up.

After, every crowded room is a minefield. Who is going to knock me over? I carefully watch my entire perimeter for unexpected people, or someone in front of me stopping suddenly. Every social interaction is a potential disaster, far and above my usual social awkwardness. There’s no more casually walking around, I have to be keenly aware of movement around me so that I don’t get tripped up or knocked down.

After, everything is a matter of energy budgeting. I wake up already exhausted, and everything is so much harder. My muscles have to work overtime to compensate for the ones that suck. There’s no more “just a quick trip down to the store room” at work. I have to plan that effort. Every little thing sends me in to a sweat. It’s super sexy. There’s no more getting a wild hair and deep cleaning the bathroom. Some weeks the bathroom doesn’t get cleaned at all.

After, my weight ballooned back up. Stress eating. Bleh. But the medical professionals encourage you to gain weight and keep it, with ALS. Heavier patients tend to have better prognoses. And you need that fat, for when you’re not able to eat anymore, like a whale living off its blubber. “Don’t go crazy, you don’t want to need a bariatric chair or anything, but..be nice to yourself and eat what you want.” Cause…fuck it, I’m dying.

After, I’m working hard to sell my house that I love and fought for because it’s becoming a physical impossibility to live there.

After, I am intimately aware of the legality and the complications of death. I’ve met lawyers and social workers and it’s more complicated the further you go. There’s nothing simple about the bureaucracy of death.

After, I know damn well how I feel about assisted dying. And I intend to exercise that right, if it comes to that, and it infuriates me that it’s not an option for Alzheimer’s patients, too. And an option everywhere. Brits should not have to take a permanent vacation to Switzerland to die in a strange hotel-like room. For a lot of money.

After, I am so, so, so blown away – daily! – by how much I seem to matter to people. By the sheer quantity of people who have stepped up to do something, even something small, to make my life a little brighter, simply because it was in their power to do so. And they love me. I thought I was insignificant, someone nice to be around, but certainly not someone who mattered much, and I’ve been told and shown how wrong I was. Constantly. In surprising ways.

After, I know how much I have impacted lives around me. I know how their lives impact mine. I know how important a seemingly insignificant gesture can become, years later. How memories define you, and can change your life without you realizing it. How important it is to reach out to people, all the time, because you never know who will show back up and be a key player when drama unfolds.

After, I know my strength. I know my calm and my pragmatism were not just theoreticals in my head, they are actual and they are real, and they will help me get through this. I know I have the grace and the quiet power that can see me through everything to come, because they have seen me through this far. I know my humor and my compassion will go far and help me survive for as long as I can.

After, I know that I’m seriously a morbid bitch. My dark sense of humor prevailed, and I’m finding things funny that would have appalled me had they been about anyone else. I am in love with a web series called Ask a Mortician, fascinated by the machinations of how we deal with death. I seriously believe we have done ourselves a terrible injury by trying so hard in the last hundred years to pretend that death doesn’t exist, it’s something that happens to other people. Because sometimes, it happens to you. And we, as a society, have forgotten how to deal with that.

After, I am intimate with the kindness of strangers. It never ceases to take my breath away, and it is so life-affirming when a total stranger gives me a kind word, encouragement. When total strangers sent me money to help. When a woman I’ve never seen before or will ever see again looks me sincerely in the eyes and says words of love and strength. And means them. It’s one thing to be told, “Good luck” or “have a nice day”. It’s another to feel someone reach out with their soul and tell you that they wish you all the best, and to keep up my optimism because it will see me through.

After, a year later, I reread my blog and see myself shift in little ways, and discover opinions I never realized I had. I see myself think about hard things, make difficult decisions, and become stronger than I ever thought I’d be. And I know that I’ll be okay.

Before, I didn’t know if I would ever have had strength and support to see me through After. After, I know love and support and strength and grace I would never have discovered Before.

After, I know that by the amazing and profound love of the people in my orbit, I’m going to be fucking FANTASTIC. And I can’t wait to see what the next year shows me.

“It’s a beastly, undignified business.”

Terry Pratchett died yesterday. He was 66 and suffering from early onset Alzheimer’s disease. He was a brilliant mind, and the world is so much poorer for his absence.

In 2011, three years after his diagnosis, he made a film called Choosing to Die. He met with an extremely British man, Peter Smedley, who had motor neurone disease – known here in the States as ALS. Peter was about the same stage as I am when he chose to die, weakness in his legs that made it difficult to walk and get up out of chairs. He had a very bright mind, and saw clearly the end of his path. He didn’t want his story to end that way, so he went to Switzerland and wrote his own exit. His wife was immaculate and also extremely British and very “keep calm and carry on”. They both kept a very strong face through it all.

I did not expect to actually see the man die.

I am glad they filmed it. It was a very good and honest look at the mechanics of the assisted death. And even though it was hard to watch, I am grateful that he shared his story. It was surreal to see someone at the same stage as I, with the same mindset, take the steps. Earlier than I would ever have. So much earlier. But he knew where he was going and did not want that undignified end, and so he took the poison and his wife stroked his hand and he fell asleep and died.

And he had to go to Switzerland to do it.

I am so, so grateful, again, to live in a state where it’s legal. How anyone can deny someone the right to die comfortably in their own homes on their own terms is quite beyond me.

It is, indeed, a beastly, undignified business.

Vanitas Veritas

Long before I was bestowed with the cosmic middle finger that is ALS, I was gifted with a genetic grab bag of fuckery called ectodermal dysplasia. (There’s GOT to be a cousin-marriage something or other back in my genealogy, because COME THE FUCK ON. My DNA is FUCKED. ) Anyway. I promise this is related to ALS, but I need to give you a bit of backstory.

Ectodermal Dysplasia, for those of you that can’t be bothered to google that shit, is a family of genetic disorders that causes defects in the hair, nails, sweat glands, and teeth. I have a VERY VERY mild case. Some people with these disorders are born with no sweat glands and have to wear cooling vests their whole lives, or have webbed fingers and toes, or no hair at all. I can sweat, I have some if not all of my teeth, I have hair at ALL, I’m ahead of the game. I am very fortunate that I was affected as little as I am.

But growing up with it as a kid?

Brutal.

My hair grew in transparent blonde and sparse, and only ever to about 2 inches long. Except on the sides of my head, that grew up to four in wispy little threads that flew away from my skull like feathery peachfuzz wings. When it got wet, it disappeared. My eyebrows were transparent blond, visible only when I got really angry and redfaced, so they stood out white on my face. My teeth grew in all kinds of crooked and brittle and some never grew in at all. My nails are these paper-thin shreds of nubbins. I was a very weird looking kid.

Fuck, man, you know how kids are little shitheads. I had the nickname Bald Eagle in my neighborhood. The older kids would see me coming and yell, “THE EAGLE HAS LANDED!” and run away. When I was in fourth grade, one of my girl classmates confided to me that one of the boys (that I happened to have a crush on) thought I *could* be cute – if I would just do SOMETHING to my hair, because it looked weird. I had a crooked, gappy smile that I hid behind my hand when I laughed. I had an expressionless face, because my eyebrows were THERE, they were just transparent. Even the adults joined in, unwittingly, mistaking me for a boy until puberty offered evidence to the contrary. I went to a flea market once, when I was about 8 or 9, and I was looking at this vendor’s pretty little necklaces and things, and the shopkeeper came over smiling, “Looking for something for your girlfriend, hmmm?” I was too embarrassed to correct him. When visiting my great grandmother, making the obligatory visit to her next door neighbor Mrs. Day (who always had Grandma Candy) I smiled and thanked her when she told me I was growing up to be such a big boy.

As you could imagine, I had a little bit of a gender issue growing up. It didn’t help that I have NEVER been good at “girl”; I really WANTED to be feminine and cute, but I felt like I was putting on an awkward costume that didn’t fit any time I tried. I was a social weirdo and never learned makeup or dressing girly, I felt awkward and weird, this androgynous thing that didn’t fit in anywhere. I was already Strange, my brain full of ideas that didn’t occur to most, a very intelligent and bored kid, standing out because I was a loner and never felt like I belonged to any of the little school cliques, not even the nerds. I was the weird kid in the back, and weird looking to boot. No seriously. Here’s me at 14, never having had a haircut in my life:

awkward14

My self confidence and ego never really had a chance.

Eventually I taught myself to embrace that weirdness and make it seem intentional – I dyed my hair strangely and scowled at everyone so they’d think that I MEANT to look that way. Androgyny was cool if you were a punk, man. Or something. I learned to hide it by being angry. I wore that anger as a shield, protecting the hurt and lonely little girl inside. The Bald Eagle is still a fucking RAPTOR man, and it will GOUGE YOUR EYES OUT AND FEAST ON YOUR LIVER. (Oh, poor, 16 year old me; I wish we could chat. How desperately you needed a hug.) But the anger just made me look weirder. My defiant, thrust out jaw just made my face square. My heavy lined eyes just made my invisible eyebrows more obvious. And I never smiled so no one would see my crooked teeth. And weird hair looks weird even if you try to make it look like you meant it.

Vasthi at 16

It took me a lot of years to work through that anger, slowly discovering and adding weapons to my arsenal in my Battle to Defeat Ectodermal Dysplaysia. Eventually I learned to draw on eyebrows to fix my expressionless face. To use false nails to hide and protect my little paperthin fingernails and have pretty, feminine hands at last (they were the one part of me I thought were pretty). I was introduced to hair extensions, after an unsuccessful flirting with wigs, and eventually I even came to revel in my ability to change my hair in a moment’s notice with them. Long hair today, short hair next week, long again the month after that. Through all of this, I let go of that angry teenager, who in turn stopped shielding the lonely and awkward little girl. I learned to allow myself to be a little bit feminine and dress like a girl sometimes, because I actually AM female, goddammit. And it looked cute on me.

And then I had good enough dental insurance to fix my crooked smile and have a beautiful smile for the first time in my life. That was a goddamned game changer. My brothers and I have all suffered the same over our crooked, missing, brittle teeth (I have the better teeth out of all three of us, but got totally ripped off in the hair department). All of us have dealt with being asked if we’ve ever used meth. By dentists. Having methmouth when you’ve never even so much as smoked pot or had an alcoholic drink makes you self-conscious as shit. And it’s cost all three of us countless opportunities. No one wants to hire a methhead. No one wants to date a weird looking girl with a wonky smile. So when I could afford to bridge the gap in my smile, to have straight teeth, I actually felt more normal and okay than I ever have in my life. I went from this:

to this:

And my world changed. And I felt like I finally won.

What does all of this have to do with ALS. I know. Relax, Sparky, I’m about to get there.

It was a slap in the face to be diagnosed with ALS RIGHT when I thought I had all my shit together. I had a really good job that I really like, I was financially stable. I had just bought a house like a Real Live Grownup. And at last? I was at a really good weight, my teeth were awesome, I knew how to do makeup sorta, my nails looked fantastic, and goddamnit I was PRETTY. FUCKING FINALLY. It took 38 motherfucking years, but I actually felt pretty, and smart, and stable. A Real Live Person Who Doesn’t Suck. I still had some shit to sort through, but I was doing pretty fucking good, all told.

…And then just when I think I have shit solid and good, ALS fucks it all up. I’m not going to be able to do my awesome job that I like, working with people I love, eventually. I have to sell the house I am in love with and didn’t even get to finish decorating because I can’t deal with stairs for much longer. I gained a fuckton of weight back because of my good friend Stress Eating. Hey, did you know there’s a German word for the weight you gain from emotional eating? Kummerspeck. It literally translates to “grief bacon”. Isn’t that the most AWESOME THING EVER. I mean, the weight gain sucked, but there’s a WORD FOR IT. And then being told by doctors DO NOT LOSE WEIGHT, you’re going to need it later, and people with extra pounds just tend to do better with ALS anyway. So here is your medical prescription to EAT WHATEVER YOU WANT. Don’t go all apeshit, I mean, we don’t want to have to fit you for a bariatric wheelchair, but you’re dying, fuck it, eat those nachos. Sucks about the not fitting into your clothes anymore though, yeah? Don’t worry, eventually you won’t be able to eat except through a tube and you’ll fit into all that again. So it all works out, yeah?

Where was I.

Right. Early on, it hit me, something stupid and vain – eventually I’m not going to be able to draw my fucking eyebrows on anymore. And I think I’ve just TOLD you why, that bothered the ever loving fuck out of me. I could rely on people to get me dressed, and probably put makeup on my face, but there were going to be days when none of us could be bothered to do that shit. And it really fucking bothered me to be reduced back to my 14 year old self. I had just CONQUERED that, I am not HER anymore. But I’m not going to be able to put on this Armor of Normal Seeming (+1 to appearance and +3 to charisma) forever. Towards the end of days, I’m going to be this emotionless husk, and I’m not even going to have any fucking eyebrows.

And it’s expensive, and vain, and fuck you I don’t care. I got permanent cosmetic tattooing done on Wednesday. I paid a stranger $395 to tattoo eyebrows on my face. And it looks fucking awesome.

And I can’t quite articulate the sense of..relief? Success? Booyah? Even though it was expensive and there are SO MANY better uses for the money, there’s a weight off of me with the knowledge that I can’t go back to 14 year old me anymore. I’m permanently done with her. My teeth are permanently okay, even if I DO still have a baby tooth on the bottom and not all of them ever grew in, they look like normal people teeth when I smile. And now my face is permanently okay, because I don’t have to draw on expression every day. What was already there has been highlighted, so when my hands no longer work, I can still quirk my eyebrow when you say something stupid. For awhile. And then I won’t be able to move my face at all, but my eyebrow game will still be fucking strong, yo. And I’ll never be that expressionless, angry little girl again. I’ve graduated, the tattoos on my face a diploma from Fuck That Shit University, signifying a degree in Being Just Fine, Thanks for Asking.

I am gonna go down, ALS is eventually going to kick my ass, but Ectodermal Dysplasia can fuck off forever. I beat it. I win.

The Eagle has fucking flown.

No Comment.

My local news did a story on a theoretical link between my employer and ALS. I declined to be a part of the story.

After seeing the report, I am certain I made the right decision to refuse to be a part of it.

They used my photo without permission, which is really annoying.

That’s all I have to say publicly about it.

Learning New Can’ts.

Every day is a voyage of discovery.

I have recently discovered that I can no longer stand up from a seated position without either swinging my arms wildly in front of me for counterbalance, or using my hands to lift my butt off the seat and pitch forward. I have also discovered that I can’t go in to my backyard when it’s muddy anymore, not even to close the shed door because it’s raining hard and the floor inside is getting soaked, because I WILL fall in the mud and bend my umbrella and muddy the hell out of my hands and knees AND lose the freaking key for the shed lock somewhere in the grass. I have also discovered that I can’t step over the threshold of my house without pulling myself up on the door frame or something. Stairs are becoming akin to mountain climbing.

I’ve had two proper falls since the last Amtrak one. I fell on a wet inclined driveway with mulch while getting out of a car. That didn’t hurt too badly except for very nearly ripping my middle fingernail off. That really sucked. And then I had a fall in my driveway while carrying things inside the house. It was my own fault, I was carrying things with both hands and I have recently discovered that well, I should not be doing that. The fall wasn’t horrible, I didn’t break anything, just skinned the hell out of my elbow and landed on my foot wrong enough that my big toe was a solid bruise for a few days.

Lessons learned.

On the plus side? My arms are fucking BUFF now.

I had my follow up appointment with Doctor Goslin last Wednesday. We mostly talked about meds, new insurance, and stupid administrative crap. She checked my strength in my thighs and hands and arms and was satisfied with the rate of decline – there wasn’t any. My calves, though, are basically devoid of useful muscle now and my feet are done. When I don’t wear shoes in the house, my feet just drop on the floor with each step – I call it froggy feet. I don’t walk down the stairs so much as clomp.

The last time I saw her, she recommended a sleep study to see if maybe my exhaustion was in part because I don’t sleep well. The sleep study found mild sleep apnea – no surprise, it runs heavily in my family – but nothing to explain the lack of energy. I’ve got a follow up study on Valentines Day, how romantic! And I’ve been referred to a pulmonologist to see if they have any recommendations about that, but I’ll probably be getting a CPAP machine. It will help with keeping my lungs strong, if nothing else, she said. I can see that. I have no idea how the cats are going to handle it. It doesn’t make so much noise once it’s on your face, but still.

Today, we start the voyage of discovery that is med changes. I was out of Nuvigil about a week before I had my appointment with her, and OH MY GOD the difference. I went straight back to sleeping 18 hours on the weekends and nearly falling asleep at my desk all the time. I went home from work and crawled in to bed with my laptop and passed out at like 9, those nights. Because this is a new year, new insurance, she tried to prescribe me adderall again, and gave me samples of Nuvigil just in case.

Insurance denied the adderall. But not a blanket denial! Just..she had prescribed one to two a day, and they only covered one. It’s the second to lowest dose of it, and I was only ever going to take one anyway, but it took a couple of days to sort it out. And by couple of days, I mean I just got it yesterday. Today’s the first day, we’ll see what happens.

It’s a world of flux and change, even if I have the answers. I know I’m going to lose my ability to walk, but it’s a question of when, and discovering daily the new can’ts. I discovered that I can’t function without some sort of energy med. I don’t have an answer why not, yet, but it’s a new can’t.

But sometimes can’ts are not a bad thing. I can’t do this on my own, because I have people who love me and won’t LET me. I can’t stop moving forward, even through all of the can’ts, because I have so many people carrying me.

I can’t stop believing things are okay, because I know they will be. They’re gonna SUCK and be full of more can’ts than I could ever imagine, but somehow, it’ll be alright. Things will work out.

It can’t happen any other way.

We Still Have a Way to Go.

The Ice Bucket Challenge was amazing in bringing awareness about ALS to the general public. It’s gotten to the point now where when I say ALS, there might be a reaction, and I don’t have to continue, “..Lou Gehrig’s?” People are starting to know what ALS is. And that’s WONDERFUL.

But we’ve still got a way to go.

I am looking forward to a time when someone asks what’s wrong, I say ALS, and there is complete understanding. Not just “oh that’s pretty bad, isn’t it?” but “Oh, this is terminal, I’m so sorry.” It would spare me so many awkward conversations about treatment prospects and recovery times. There’s no gentle way to say, “There is no treatment. This is a death sentence.” It’s hard to drop that on someone and tell them that you’re okay, honestly, in the next breath. “I’m going to die. But it’s okay.”

It would be so much easier if they understood the implications already so that I can be spared giving people tidings of death with every conversation about my disease. Not just the mortality part, but the whole gradually becoming stuck in a meat shell until I suffocate part. It would spare so much awkwardness. I can’t even imagine someone having one of these superficial conversations with me, learning I have ALS, and then Googling it later and HOLY SWEET MOTHER OF GOD THIS IS AWFUL IF I HAD KNOWN I WOULD HAVE BEEN SO MUCH MORE SYMPATHETIC OH GOD SHE PROBABLY THINKS I’M THE MOST UNFEELING PERSON EVER. (I don’t. I promise.) But the alternative is unlimited conversations like this:

“Hi, how are you?”

“I’m going to die horribly, thanks, but otherwise grand. How are you?”

Yeah. Awkward.

Okay, so: story time!

I ran into a coworker in the hall a little bit ago. He’s not with my group, but he works on my floor so I see him a lot. Really nice guy, though we got off to a rough start – we met in an argument over who had booked a conference room (I did! And I proved it!) and he was really bitter and snarky at us even though I GAVE him the room and we just found another one. But he had the good grace to make a point of finding me later to apologize and explain that he was really frustrated with getting kicked out of rooms a lot that day because I guess his admin sucked and didn’t actually reserve ANYTHING. But he was sorry he took it out on me. And we’ve been happy acquaintances since.

…Anyway. He stopped me in the hallway and asked me how I was doing. It was a genuine, “How are you?”, instead of the generic “How are you” that you pray to God the other person will just superficially say “Fine! You?” and you both can go about your day. He was actually concerned, and I was a little confused because we hadn’t talked about my disease before – had he seen the spot on the news?

“I’m good,” I answered him honestly. “Doing alright.”

He voiced that he had seen my walking kind of deteriorating and was wondering if I was okay.

“Ah, that. Well, I have ALS.”

There was a little bit of recognition there, and he sympathetically told me, “I’m sorry to hear that.”

“Thank you. But I’m doing okay.”

“So it’s a progressive thing?”

“Yep, someday I’ll be in a wheelchair.” I shrugged.

“Oh. Is it hereditary?”

“Sometimes. Not with me, but 10% of cases. Usually it just comes out of the blue.”

He was sympathetic, nodding.

“But nothing hurts,” I continued. “I’m doing okay. I’ll be working as long as I can.”

We’d reached the end of the hallway where our paths split. He gave me a warm smile and said, “Please let me know if I can help you in any way.” And he meant it.

I was touched. “..Thank you, I will.”

He turned to go, and said in farewell. “Well, I hope you feel better.”

…….

Yeah, awareness has a long way to go.

When I say Amazing, I mean Amazing.

I gush a lot about Dr. Goslin. BECAUSE SHE IS AMAZING. But, I’m also prone to hyperbole. I get it. You might think I’m exaggerating. But here’s this thing that happened.

Lately, I’ve been having a few more rougher days than usual. Some depression is absolutely expected with a terminal diagnosis. Duh. And I was on antidepressants before I was even diagnosed, because broken brains run in my family and I am no exception. But this last couple of weeks I’ve been more prone to let things get to me, like the Ice Bucket Challenge haters, and slight arguments turn into self-hate sessions, and I am just having a hard time with things right now. In addition to this, things are harder to do, physically. They’re taking a lot more energy than I would think. I’m tired all of the time. And I don’t know if I’m tired because I’m depressed, or if I’m depressed because I’m tired? But everything seems so much harder than it feels like it should be. Friends and family have noticed, and my little brother has mentioned several times joking-but-not that I should ask my doctor for some Adderall. Maybe I’d have the energy to get things done and cleaning won’t be a herculean task that wipes me out for the entire next day.

Monday was a holiday, and a classic Depression Day with lots of sleeping and moping. It carried over to the next morning, which is unusual. I’m typically over it the next day. So I got fed up with being a mopey, tired lump and that afternoon I sent Dr. Goslin an email:

We have an appointment to meet in a month, but I wanted to let you know that when we do meet, I’d like to talk about medication adjustments. I’m not sure the wellbutrin’s doing anything anymore, and I’ve been completely devoid of energy. I know some tiredness is to be expected of course, but for example, yesterday I slept from midnight to noon, ate some lunch, then slept from 2 to 7. And back to bed at 11. It’s to the point my brother told me I should talk to you about adderall or get a speed habit or something. hehe. So when we meet, can we talk about this?

I was expecting maybe an email in a couple of days to acknowledge the question, a quick “Yes, we can discuss your medications when we meet.”

Instead she called me after work. We talked for about about my symptoms, where I was at, and where I thought I should be. She asked what I’d like to do. Do I want to attack the depression, the fatigue, both? I told her I didn’t know, because, (as I said up in that second paragraph) I wasn’t sure if they were separate issues, or if the one was feeding into the other. She gave me many options, made sure I was seeing a therapist regularly, and told me about different drugs, what they did, what their side effects were; she usually prescribes another antidepressant that deals more on the anxiety side, that is a nice compliment to the Wellbutrin, would I like to try it? Additionally we COULD try some energy-producing meds, if I thought that was something I would like to try. She carefully explained all of my options, made her suggestions, and ultimately left it up to me to decide which route I wanted to take.

I didn’t even have an appointment. She won’t get paid for that time, probably. But she made the effort, she called me outside of her office hours, to talk to me and see that I was taken care of. Because she didn’t want me suffering for another month if we could start to do something about it NOW. And this is why I tell people she is amazing. And why I love her. She is one of the most powerful players in my support team and I really don’t know what I’d do without her.

So, without hyperbole and in all seriousness, Dr. Kim Goslin is the mutha-f**kin BOMB.

Having to Rethink

There was another way that yesterday was ALS-centric, and it deserves its own post.

I gave blood yesterday. The Red Cross comes to my work every two months or so, so I really have no excuse to not go down there and bleed in the parking lot for an hour. I’ve been deferred a few times for low iron count, but since I take the supplements daily now, it’s not been a problem. I made it past that screen, and picked a bed.

I had to wait awhile, because another coworker donor was having a bad time. Like, “could not stand up without nearly passing out” bad time with an emesis basin and everything. He was insisting he had to go to the bathroom, they were insisting he lie the fuck down until his color improved because they couldn’t have him passing out in the parking lot. While listening to this, I was thinking about how it’s strange that some people have that reaction. And wouldn’t it be weird if he was actually having a reaction to chemical fumes or something in the bus and not wobbly reaction to losing blood at all.

Cue a panic attack for NO reason at all.

I laid there for five minutes, dizzy and nauseous and not breathing very well, willing myself to CALM. THE FUCK. DOWN. It wasn’t really because I thought there were fumes on the bus, but I told myself IF THAT WERE TRUE THE NURSES WOULD ALL BE SICK, THEY’VE BEEN ON THIS BUS ALL MORNING anyway. Maybe because I was mortified at the thought of having a problem at work that might require ERT? I’m not sure. None of my thought trails led to further freaking out, except the FACT that I was freaking out very quietly. I CAN’T BREATHE. Of course you can, idiot, calm yo shit. I AM GONNA PASS OUT. No, you’re not, you’re fine, and even if you did, there’s nurses here, so so what. I AM GONNA THROW UP. No, you’re fine. Cheesy Christmas, woman, chill. I’M FREAKING OUT. Well, yeah, but you KNOW that, so what’s the actual problem? UH..NOTHING I GUess okay I think I’m okay.

The phlebotomist finally came back around to me, got the needle started, and as I usually do, I let my mind go wherever it wanted. And then a quiet voice spoke in my head.

Idiot, you have a terminal disease. Should you REALLY be donating blood?

Um.

While I bled into a bag, I looked things up on my phone. There are a couple of hospitals which have a policy against people with ALS donating blood. There’s a forum with someone asking about donating organs or blood, and some jackass going off on them about how they’re APPALLED YOU WOULD EVEN THINK THAT WAS OKAY FOR FUCK’S SAKE YOU HAVE A TERMINAL DISEASE AND NO IDEA WHY IT HAPPENS YOU ARE LITERALLY POISONING PEOPLE IF YOU DONATE YOUR DISEASED PARTS YOU EVIL SON OF A BITCH. Which is why I don’t go to forums usually. There was NOT, however, anything on the Red Cross site or anywhere on the web that said no. Blood donated to the Red Cross doesn’t always get transfused, it gets used for research and medicines, too, and I don’t know if that means even if my blood can’t go in to a person, could it be used for medicine?

I finished bleeding, they gave me the standard spiel, call this number if you experience any flu-like symptoms in the next 7 days, or think of anything that might mean your blood shouldn’t be used, go eat some cookies and have some apple juice.

I finished up in there, and called that number later. The nurse on the phone didn’t know if it was against the rules for ALS people to donate, she couldn’t find out anything that said no. She would do some more research, though, and call me back or send a letter.

We don’t know what causes ALS. It’s not contagious, as far as anyone knows. But we don’t know a lot about it, and I’m not sure it’s a good idea. The Red Cross will be the final authority, of course. I’m sad about not being able to help out this way anymore, it’s a simple, easy thing to do and it can be tremendously helpful and they ALWAYS need people and I’m O+ so 85% of the population can be given my blood.

Unless it’s tainted.

Denied

Dr. Goslin’s sidekick, the amazing and trusty Donna, emailed me this morning.

“Cigna denied the prior authorization request for Athena. Dr. Goslin did a peer to peer review, and they still denied it. It is unfortunate that this got dragged out so long, only to have them deny it in the end. I am sorry.”

Athena, of course, being the company that does the genetic testing. We were going to see if I had the markers to allow me to participate in the bulk of clinical trials going on. And Cigna said no. Even after Dr. Goslin explained to their faces why it was important. And it IS important.

I feel defeated. Like…this test? And the ability to participate in the best research going on? It gave me hope that maybe my fucking disease might be USEFUL to someone. We won’t see a cure for this in my lifetime, but goddammit I wanted to be a datapoint at least in GETTING there. I want to HELP.

I don’t know how much the test costs. The last one was nearly $12,000. So yeah, I won’t be able to just DO the test on my own. I am beyond frustrated. I am angry, defeated, disappointed, crushed, depressed, all of this and everything else.

I want this fucking disease to mean SOMETHING to SOMEONE. To get SOME good out of it. To be useful.

And now instead of having the chance to be a data point, I’m relegated to be a statistic.

Fuck You and Your “It’s going to get worse”.

Okay so wow.

https://www.facebook.com/fox12oregon/posts/10152550068903701?comment_id=10152550463373701

I KNOW BETTER THAN TO READ COMMENTS ON THE INTERNET. I KNOW.

But this was important. This is something I care a lot about. If someone out there wants more information about this, I’d like to be able to step in and help out. And Jack asked if I’d seen them, and linked me, so I clicked.

Most of them? Lovely and supportive. Hooray for those people. I love them. And my friends who spoke up in support. I love you.

But a hearty FUCK YOU to the shitshark who felt compelled to comment “Pretty lady if she didn’t put all that metal in her face.” Yeah, I got enough of that oh, at EVERY FAMILY GATHERING GROWING UP EVER. And I didn’t give a shit about how my FAMILY felt about it, why the fuck would you think YOUR opinion matters to me? Get fucked SIDEWAYS. I didn’t have to weigh in though. My posse stepped up and put him straight before I got there. <3 These other ones, though. HOLY SHIT, people. "My father died because of ALS. He was one of four in one family. And I tell you to have the voice record is the smallest problem you will have when you have ALS!!!" "Mom passed from ALS in 93, not bn able to talk was the least of our worries. Absolutely horrible disease" …I've actually heard of people approaching someone with ALS and say, "It's going to get so much worse" ..AT A MOTHERFUCKING SUPPORT GROUP. GOD DAMN IT, PEOPLE. Here's a clue you are so DESPERATELY NEEDING: Telling someone with a terminal, degenerative disease "it's going to get worse" HAS NEVER BEEN USEFUL TO ANY ONE IN THE HISTORY OF FOR FUCKING EVER. The only one who get ANYTHING out of that is YOU because you get to feel OH SO FUCKING KNOWLEDGEABLE. Your dad has ALS so OBVIOUSLY YOU KNOW ALLLLL ABOUT IT and someone who actually HAS this disease HAS NO IDEA WHAT THEY ARE IN FOR so you had BETTER TELL THEM. Fuck you. Keep your fucking mouth shut. NEWSFLASH: You are NOT helping. You are NOT helping me prepare for the harsh realities of the disease. You are NOT educating me. You are not even freaking me out. You are JUST PISSING ME OFF. Let me educate YOU. When you are going through the medical rat maze of trials and tests, and ALS is among the possible exits, THEY TELL YOU ABOUT THE DISEASE. If you didn't know about it already, THEY TELL YOU WHAT IT IS. When you narrow it down, THEY TELL YOU A LOT MORE. If you don't do the sensible fucking thing and research it yourself, there are medically trained professionals who will talk to you about it. THE PROCESS OF DIAGNOSIS COMES WITH AN EDUCATION. And here's something I did NOT know. When you are diagnosed? THEY GIVE YOU BOOKS ABOUT IT. Seriously. Like, six of them. My diagnosis came with an appointment with a social worker, and she had books for me, a book for the people who would be my caregivers, and pamphlets about estate laws and wills and power of attorney. People bend over BACKWARDS to tell you anything you could possibly want to know. And by "people" I mean TRAINED AND LICENCED MEDICAL PROFESSIONALS and not "some stupid opinionated bitch on the internet." So let me be the first and hopefully last to let you know, you're not helping. You're not wise. You're obnoxious, detrimental to my emotional well being, and a waste of my time. It alllllllllllllll goes back to the Silk circle, and the magic phrase: "I'm sorry this is happening." Comfort in. Otherwise shut the fuck up. And if you make the mistake of telling me "it's going to get worse" to my face, I will obligingly tell you all of this in person. And just when you think I'm done yelling? It's going to get worse.

The Interview

The interview I did aired last night; I sat in my living room with my brother and his wife and watched it streaming off his laptop to my television. I guess I didn’t look TOO stupid. She mispronounced my name, but it was otherwise a really good story and I’m happy I was a part of it.

Here it is!

http://www.kptv.com/story/25869456/als-patients-preserve-their-voice-with-voice-banking?autostart=true

So now the two of you who read this who don’t already know me personally know what I look and sound like. XD

I came in to work this morning to an email from a coworker:

I saw the story on KPDX regarding your diagnosis of ALS and the preparations for your future with this disease and wanted to reach out. I am the Employee Resource Group leader here in Oregon for the American Veterans ERG. If you were not aware, American Military members are twice as likely to contract ALS as the general population. The cause is yet unknown. Due to this connection between veterans and ALS, my ERG is using Q3 to promote ALS awareness here in Oregon.

I was reaching out to you to see if you would be interested in being involved in this event. I would love to be able to meet with you and talk to you about what we are planning to do in regards to raising awareness here at Intel. Feel free to call or email anytime so that we could arrange a meeting if you are interested in doing so.

I told him I’d be happy to be involved. I hope we figure out what the hell that correlation is someday, because yeah. Vets and ALS. Such a strange and scary statistic.

My facebook has been blowing up today, all kinds of people linking that video and tagging me, and SO MUCH SUPPORT and encouragement from those I love. Every day I learn how well I’m loved.

Hooray for getting more people aware of this stuff, and hooray for it being OVER so I can stop being nervous about it. Heh. “When she’s not baking, she’s banking.” I SEE WHAT YOU DID THERE, NEWS PERSON.

6-11-14 Followup, Checking In.

I had my followup with Dr. Goslin about the prednisone and whatnot. More shocks and stabs, more with the scrapey tool against the arch of my foot, more of the “hold your arms out” pushy/pully tests. She concluded that there’s no difference between this session and the last. So that either means the prednisone is keeping me stable, or my progression is just so slow that there wouldn’t have been a difference anyway. She’s taking me off the prednisone now, and we’ll check back in a couple months to see which is the case. If the prednisone was helping, then we’ll have to talk about alternative treatments, maybe IVIG.

You can’t, of course, just stop taking a steroid. It’s a recipe for bad times. So we are tapering me off; down 30 mg from the 40 I was taking, for 4 days, and then down 5mg every four days after that until I’m done. I’m happy to be done with it. I’m grateful that the bitchy side effect never happened; as side effects go, weight gain is not that terrible. It still SUCKS, do not get me wrong, but at least I wasn’t screaming at my loved ones while I bloated up like a whale.

Dr. Goslin is still a little stumped by me. I’m just…odd…enough that she’s not comfortable putting the ALS stamp on my forehead with assurance. She’s like, 99.9999999999999999999999999% sure that’s what’s going on, but how did she put it, something to the effect of, my symptoms are exceptional outliers. So basically, “I’m really pretty sure that this is ALS, but juuuuuuuuust in case some years down the road it turns out to be something different, remember she wasn’t completely certain.” She asked that I be open to further experimental treatments as ideas occur to her.

Let’s see. The only other thing going on with all of this is that I have a cough – which may just be a cough; sometimes? You just get a cough. It’s been about a month now, though. Eeeeeeverybody in my office got some kind of plague, but I don’t think it’s that, as I FEEL okay, it’s just that I cough and clear my throat a lot. My nephew was sick about a month ago and had a cough with it, but it also had a fever, which I never had. And my coworkers and nephew are all better now. For an unrelated reason, I was checking on the various side effects that my Fistful O Meds have, trying to track down the actual culprit for the heartburn I have a lot even though I’m taking ranitidine now. I found out that one of the possible side effects of riluzole is…coughing.

I’ve been on riluzole…about a month.

I really hope they’re not related. It would be a REALLY SHITTY CHOICE, do I live for a little longer? And have a cough for my entire life? Or no cough, but dead sooner. Or potentially take ANOTHER med to counter that.

Blehhhhhhhhhhhhhhhhh.

Fatty McFattersons

Rooouuuuuuuuuundabout four, five years ago, I hit what you might call “a rough spot”. End of my marriage, purchase of a house, the move, mom had a health crisis, things like that. A rough spot. And I grew up incredibly poor, so food has always been a comforting thing. Thus, I have always been…a little rounder than necessary, usually right around 160 when the charts all say I should be 140, but fuck those BMI charts anyway, I like a little curvy. But this little “rough spot” pushed me to be far heavier than I’ve ever been. I freaked out when the scales tipped past 200, did the paleo diet thing, and lost 40 pounds.

I weighed pretty close to my target weight, I had a shiny new house that needed some decorating, I looked cute, my job was going great!

And then this whole thing kicked in for real. Annnnnnd I began stress eating. And so I gained back ten pounds.

And then my little brother and his family moved in with me while they wait for their new home to become ready, and they have a little kid, and they eat a lot of processed food. I am surrounded by unhealthy food and people giving me permission and non-judgment to eat it. And it tastes good and is easy, which is why I got fat in the first place. So I eat fast food while we are out, I eat processed food smothered in ranch while I’m home, and drink sugared soda again instead of diet. And so I gained another ten pounds.

And theeeeeeennnnnnnnnnnnnnnnnnnnnnnnnnnnn prednisone.

Fucking prednisone.

With the water weight gain and the wanting to eat ALL. THE. TIME. And there’s nothing around me but junk food. And so I gained another twenty pounds.

So now I’m just about back to the weight I was when I lost my shit in the first place and started hardcore dieting. I had to buy new clothes because I’d either thrown away or turned my fat pants into painting pants. I feel gross, I feel unattractive, I feel like a loser. …As I shove another handful of candy in my mouth because holy SHIT does the prednisone make me want to eat, like, ALL THE TIME. And my life is incredibly stressful right now, so I console and reward myself with delicious food. And so I gain weight.

FUCK IT – YOU ARE DYING! EAT ALL THE CREAM PUFFS. ALL OF THEM. ALL THAT EVER WERE. WHAT’S IT GONNA DO, KILL YOU? HEY IS THAT SALTED CARAMEL SAUCE? I BET IT WOULD BE AMAZING TO DIP THOSE CREAM PUFFS IN. YOU’RE DYING – LIVE A LITTLE. YOU CAN’T PUSH CREAM PUFFS DOWN A FEEDING TUBE, BITCHES. ENJOY THIS WHILE YOU CAN.

I tell myself I’ll get back on the paleo diet just as soon as my little bro and his family are out – I’ll be cooking for myself only, and I can buy non-processed shit. I won’t have someone else to cook for me, and I won’t be going out to fast food every other night anymore. I will Eat Right and Lose Weight. And I won’t be on the prednisone for much longer, so that will help a lot, too.

Except eeeeeeeverything I’ve been reading about ALS has said in big bold print that if you have ALS, YOU SHOULD ABSOLUTELY NOT DIET TO LOSE WEIGHT. Because…well….you’re not going to be able to eat, eventually, and you’re going to NEED that extra weight to live off of. And statistically, my doctor tells me, people with extra weight tend to have better prognoses.

It’s every fat girl’s dream! Not only PERMISSION, but RECOMMENDATION to stay fat!

HEY CAN I GET A SIDE OF RANCH WITH THESE FRIES?

…But I don’t WANT to be fat. Yes, food is delicious, but I really liked being able to fit into 32s. I felt healthier, I looked cute. I want to be there again. I want to be rid of this emerging double chin. I want to fit into medium girls’ shirts – I’d say small, but I’ve always been too busty for that nonsense, and I am OKAY WITH THAT – because I have some really awesome ones. My Night Vale shirts are all mediums and I miss them. I want to not sweat so goddamned much. I want to not have my shirt flip up in the back when I sit down. I want to be not so self conscious about it when it does.

I don’t want to be fat.

I have the power to not be fat. I have the self control. I have proven this. In 5 months, I lost 40 pounds. I can do it again. I just don’t know if I SHOULD. I mean, yes, eventually I will not be able to eat at all. Probably. It doesn’t always happen. I don’t think this necessarily gives me permission to eat whatever I want though. And I don’t know that hauling around this extra weight is any healthier, in the long run. It’s such a slow progression, and I can’t be fat for years and years “just in case” I start to become unable to eat.

So do I trade healthier and happier NOW for just healthier later?

I will ponder this some more, while I eat my Taco Bell lunch.

Mmmmm Baja Blast.

Making Video about Audio

I’ma geek out for a minute about voice banking. Ready? Here we go.

Voice banking is one of the most amazing things to happen for people with ALS, or any kind of degenerative disease that robs someone of their voice. Model Talker allows you to record your own voice, from which they’ll make a synthetic version for use with a text-to-speech program. It effectively lets you “talk” with your own voice, after the disease takes away your ability to speak.

It’s amazing and important and I’m SO GRATEFUL that it exists and that I’m allowed to participate in it. It’s currently in beta, and anyone can apply to be a part of it. ModelTalker is a program you install on your computer, and then you record yourself saying prompted phrases by speaking into a USB microphone headset. It’s best to bank before the disease hits your voice, so that your computer generated voice is as true to your natural voice as possible; so the sooner people with ALS know about it, the sooner they can start banking. It’s a fairly sizable time commitment – I understand there’s about 1600 phrases to record before they have enough sounds to create your voice from. The sentences I’ve seen so far include lines from The Wizard of Oz, and the phrase “There’s more than one way to remove a tooth.” I’ve only just started; I recorded the calibrations and the first ten sentences last night. They’ll listen to the samples, and suggest changes, and then I’ll record the next set. I will be sitting at my desk and recording goofy sentences for hoooouuuuuuuuurs.

But it will be TOTALLY WORTH IT, when my computer generated version of me calls someone a fuckwit for the first time.

This technology is SO IMPORTANT. It’s completely dehumanizing, being unable to properly communicate with others, and that idea frankly scares me. The fact that speech synthesis exists at all is fantastic, don’t get me wrong, but we need to take it a step further. Just look at Dr. Hawking, his voice..it’s become a joke, how robotic his communication is. To have to rely on a robotic voice to tell someone you love them? To try to explain to your loved one why you’re crying with this…fake, cold, not-really-a-voice? That is the worst thing, and I can’t even imagine the stress that adds to an already horrible situation. ModelTalker gives you back some semblance of who you were, to continue to be who you are. It gives you back a little bit of what this stupid fucking disease takes away from you.

I was contacted some weeks ago by my local chapter of the ALS Association wondering if I would like to be part of a local news story about voice banking.

I said yes, please.

They’re going to come to my house next Thursday and film me doing some recording, and then interview me about it. I’m really happy to have the opportunity to evangelize about this technology, to let people know it exists, and it’s out there for free. Technology is solace for people with ALS. It helps us travel when we can no longer walk. It helps us communicate when we’ve lost the ability to speak.

It helps us continue to be human, for just a little bit longer.

Playing House with Godzilla

Before I had a name to my disease, I was calling it Godzilla Disorder.

…Okay, yeah, I need to explain that one.

I didn’t have a name for my problem, only symptoms. Only theories. And so, when someone asked why I was limping, I had no real answer. Just vague theories. “I have some kind of neurological thing going on.” Hand-wave. Again. How do you break that to someone who’s only a casual acquaintance, anyway? “Just losing my ability to walk, thanks. How are you?” While complaining about this dilemma to my very patient best friend and main babe Danielle, she suggested I just tell people “I got attacked by Godzilla in the legs.”

And this is why we love Danielle.

And so ever since, I refer to this as Godzilla Disorder. Even now that it has a proper name. A name scarier than Godzilla. Someone later asked if I called it Godzilla Disorder because it made me walk like I was in a rubber monster suit. Which also made me laugh. Thankfully this was pre-braces, so my walking is SO much better now. I’ll have to attack Tokyo another day.

So, to get to the REAL reason for this post. It’s about back when I was still calling it Godzilla Disorder because I had no name for it. When we didn’t know what was going on, and I’d been through a lot of doctors and tests and MRIs and a spinal tap, I noticed that everyone had their pet theories for what was wrong with me. I had many people playing Dr. House and suggesting obscure things that aren’t ALS or a neuropathy but have similar symptoms. Could it be a vitamin D deficiency? Lead poisoning? Shellfish poisoning? This other, obscure disorder that has maybe 3 documented cases in the US but it’s worth a shot to look in to because it just causes weakness in the feet and stops there?

I’ve noticed this is a pretty common way for people to react to a scary medical unknown. It’s curious as a coping mechanism – you feel powerless to help, so you look for answers and try to be helpful, just in case the doctor ‘forgot’ something. And I’m sure there are doctors that have overlooked everything. It’s really tempting to think that maybe it’s not this complicated, maybe I just need to take some supplements and be cured! And there’s the tiny, egotistical desire to find the problem and be the fixer! 

For example, my aunt asked me to tell my neurologist about the time I got really really sick from shellfish (on my BIRTHDAY. We rented a cabin on a really awesome bed and breakfast THAT WAS A BOAT ON THE OCEAN -fucking awesome and I was looking forward to it for WEEKS – and we checked in and then went out to a really nice lobster dinner that I didn’t even get to eat because we started with steamed clams and I won the shellfish lottery and got CRAZY SICK from one while everyone else was fine and I spent the rest of my whole amazing romantic birthday trip throwing up. FUN TIMES.). She’d read that shellfish poisoning can actually stay in your body for years and cause problems that mimic neurological disorders.

First of all, good memory, Aunt Phyllis. Second of all, way to do your research! 

All of this isn’t a rant – I’m not complaining about this at all. Far from it, actually! I am totally fascinated with this, and I’ve learned some really interesting things. There are an AMAZING amount of things that mimic symptoms of ALS. And it’s important for those I love to cope with this in their own way, and sometimes that way includes researching for that one thing that I might have that is totally curable. Something, anything that isn’t ALS. Anything to remove Godzilla’s teeth. Here, just take these vitamin D pills and it will all just…go away.

A lot of people in my life have done this, with a few really ardent researchers. Like Lance. Research is what he does, in all things. When things first started coming to light, every day he had a new wiki entry about some new disorder – and they were all about obscure disorders that get better with treatment, or some weird variant of a scary disease that is a not so scary version of it. Always something optimistic. 

I’ve told my friends and family that I am happy to forward these thoughts along to Dr. Goslin; she’s never been insulted or offended with my questions. In fact, she responded to Phyllis’ food poisoning theory with, “I don’t think you have this, but I appreciate that people are continuing to think about this.” She’s amazing.

Sometimes, it has actually been useful – my brother suggested I tell her about the genetic defect that I share with my brothers, because apparently it has been shown to have a possible link with neurological disorders. I had no idea. My neurologist is very interested in that research, and has asked me to find out which particular variety of that defect I have, because there’s like 200 of them. If I find out, and there’s a link, then I can help that research by becoming a data point. So, by all means continue. I know that it’s important for you to feel like you have some control over what’s going on, to be helpful.

And who knows, maybe you WILL find the answer and it will be simple. 

Just, please don’t be offended when I shoot down your theory with, “I’ve been through that line of reasoning, here’s what came of it.” I’ve been tested for heavy metals. At this point I’ve been tested for every damned thing missing or present in my blood. And my spine. Yes, the doctor has heard of that disorder. And that one, too. She’s a neurologist specializing in motor neuron diseases, she’s very smart.

And she has access to Wikipedia and WebMD, too.