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.
The latest word on the street in ALS Land is Radicava.
It’s a new, FDA approved treatment, and we’re all pretty dang excited about it because there’s been nothing new AT ALL in 30 years. All we have is riluzole (brand name Rilutek) which you take twice a day and maaaaaaaaaybe gives you another six months or so? Or maybe destroys your liver? Other than that, the only treatment is really an attempt to mitigate some of the symptoms, like the muscle twitches (‘fasciculations’) and cramps, your muscles locking in place (spasticity), the complete fatigue because everything is a lot harder when half the muscles in your legs are gone, depression and anxiety – because it’s not really the five stages of grief so much as the five constantly cycling whirlwind moods of grief. Sometimes I hit them all on the same day.
Radicava (generic name edaravone) isn’t a miracle drug, by any means. It’s completely ineffective for a lot of people, and at best gives just a 33% reduction in the rate of decline. It has a lot of issues, of course, it can cause problems in people with asthma, and well..it’s expensive as all hell. I’ll get to that in a moment. For now, here’s the basics, if you want to read some more, and here are some of the naysayings about it.
Me, though, I wanted to try it from the get-go. I’ve been abundantly clear (or so I thought) that I am willing to try any new treatment, just to be doing something, to provide a little more data for future research, if nothing else. Every Clinic Day, we asked about the latest updates, the most recent news, when is it available. And so I was frankly…crushed, and a bit angry when I found out at last Clinic that people have already been trying it and I wasn’t contacted. That anger increased when they seemed surprised that I was interested in trying it. Like..how could I have been more clear?
Anger turned to rage when I was told that insurance would not pay for it. Because..once again..I have had ALS for too long. Because statistically, I should be dying any minute now. The average life expectancy is three to five years, and I hit three years in April. Never mind in actuality I’m still VERY MUCH alive and not going anywhere soon, thenkyouverymuch. And it wasn’t the pharmaceutical company denying me, it was the insurance company. I wasn’t worth the cost. If I’m statistically almost dead already, it’s not cost effective for them to pay for this. You’ll recall I said it was expensive – let me show you how expensive. Radicava is administered by infusion. Each infusion lasts for about one hour and administers a 60mg dose. The cost for each infusion is as follows:
The medication itself: $1238.80
The medication to flush the IV/PICC/port line into your vein: $180
If you do in-home infusion, the nurse’s time costs $251.20 for the first 2 hrs, $86.10 each hour after if needed
If you go to an infusion clinic, the cost for nursing is $107.10 for the first 2 hours, $50 after.
Now, since it’s not easy to get anywhere and do anything with ALS, we’ll go with in-home infusions, so that is $1,670. Per dose. How many doses do you need? Well, you start treatment with 14 consecutive days of infusions. That’s $23,380. Then you take 14 days off. Then you do 10 consecutive doses over the next 14 days. Then 2 weeks off. Then 10 over 14. For the rest of your life. It’s over twice what I make a year, to use this drug. As much as I hate it, I get why insurance would consider this a dumb investment. I hate that that is even a consideration in whether or not I have access to this.
Knowing that I was going to get denied, I asked to try anyway. I filled out a form giving the infusion clinic access to my medical records, on the off chance they could use this to leverage my insurance company to pony up some dollars to help a dying girl out. Monday I got a call from Searchlight, the company coordinating the treatment, and they said that typically my insurance covered this treatment after a 5% copay. Luckily, she said, once I hit my out of pocket max, it’s all covered. Did I have any questions?
“Yes,” I said. “…Does this mean that I actually am going to be covered for this?”
“Well, we’ll be sending your information along to your doctor provider, but it looks like we’re good to go.”
I dared to let myself hope. Just for a moment. I went out with J and had celebratory sushi, but said nothing to anyone else (except my mom, who was here when I got the call) just in case it was a misunderstanding.
Wednesday I got a call from the nurse to discuss the actual cost, how much I was responsible for, and to start making appointments. She gave me the above price list, and we discussed would I like a PICC line or a port? Since this was pretty much going to be a regular thing, infusions by regular IV aren’t an option – imagine having to get your vein stabbed every day for an hour long IV drip. Folks on dialysis, infusions, or chemotherapy usually opt for either a PICC line or an implantable port. A PICC (peripherally inserted central catheter) line goes in your upper arm, and then into a central vein in your chest. An implantable port, or port-a-cath, gets implanted under the skin in your chest and also snakes into a central vein. PICC lines are technically good for a month, though if you treat it carefully, it can last much longer. A port-a-cath is considered a permanent implant. PICC lines are much simpler to install, it’s just a complicated IV insert with a chest x-ray to make sure it’s done right, but a port requires at least conscious sedation. A PICC line needs upkeep once installed; it’s basically two little ‘pigtails’ as she called it, sticking out of your skin and held in place by tape. You can’t get it wet, and have to wear some kind of sleeve to keep it from getting caught on things. A port is just a little bump under your skin, no maintenance required. PICCs are easy to remove if you change your mind. You’re kind of stuck with a port for the rest of your days.
So yesterday I had a PICC line installed.
If I tolerate the meds, we’ll install a port.
This is happening. Even if it does me no good at all, even if it actually makes things worse, we have tried. I am a data point. I did something. I did everything I could. I should start infusions next week.
Ok, so this isn’t one of those “little things have big impacts” kind of stories, though it sort of is. It’s a “help from unexpected sources” story more than that. In a really stupid goofy way. Some background:
1) My friend Nathan bought me a subscription to LootCrate. I’ve raved about that before, but let me do it again. We weren’t ever really the best of friends or anything, just work friends, and we lost contact for a few years. Like ya do. When he found out about my diagnosis, he bought me this subscription so I could have something fun to look forward to every month. It was an unexpected surprise and I can’t even remotely convey how much joy this brings me, for a lot of reasons.
2) LootCrate is a collection of VERY geeky things, from all kinds of fandoms. I’ve gotten t-shirts from Teenage Mutant Ninja Turtles to Overwatch to James Bond and everything in between. It’s current pop culture and retro childhood stuff, and I’ve gotten a lot of really awesome swag, including stuff you literally can not get anywhere else. Tetris fridge magnets. A Tron pencil bag that glows in the dark. SO MANY TOYS. And awesome aforementioned t-shirts. Like, half of the t-shirts I wear are now LootCrate shirts.
So this month’s crate theme was “animation”. It included swag from a couple of things I’m not that into (it happens, but I ALWAYS find someone who really loves said fandom and is happy to take things off my hands), and drink koozies from the show Futurama. Full disclosure? I’ve always kinda hated drink koozies. They strike me as a bit white trashy and that’s not helped by them USUALLY being branded with some stupid or plain offensive not-really-a-joke. But I loved Futurama, and this was a fun thing, and I’m ALWAYS drinking soda (Sorry Kelly, I know I need to be drinking water but CHERRY COKE ZERO IS DELICIOUS), so I slid my can into one.
Oh my god guys.
THE CAN IS SO MUCH EASIER TO PICK UP.
I typically have to use two hands to pick up a full can of soda, and as I drink it, I press a dent into the can to help me grip it. Hang on..lemme take a picture.
Every can I drink from has that little divot for my thumb. heh. But with the drink koozie, I don’t need it! It’s squishy so I can get a good grip on the thing without leaving a little dent in. I bet Nathan never knew he was signing me on for handicap aids. But that’s what I got this month, and I never would have figured this out on my own.
I have things to report and ruminate on and whatnot, but it’s a lot, and I’m still processing. It was a very complicated weekend after a very emotional week (by which I mean panic attacks and crying and fighting and more ativan taken in three days than in the last three months). Things are okay. But they’re hitting hard and fast and yeah. I’ll tell you all about it, in a bit.
But for now? One of my fellow ALS peeps posted this to her Facebook page today and I love it, so I’m sharing it with you.
A vast number of things have prevented me from completing my voice banking. Changing acoustics in my office due to the move being the primary, but various other things have factored in, like the time my hard drive died and I lost the existing in progress file. I finally had it occur to me that work has phone booths, private little sound-muffled rooms. So I have brought my headset in to work, and finally – FINALLY! – started the process over.
Maybe you don’t know Tom Waits, and don’t care. That’s okay! You should go YouTube something of his and then imagine a digital voice like that. I said that I should make a tumblr for Tom Waits poetry inspired by the ModelTalker software prompts. It would be awesome. And then read that poetry with the Tom Waits digital voice, and the world would cease to be, because it’s just too cool for words. And voices.
I might continue to bug you guys with examples of the ModelTalker reading prompts though. They’re delightfully bizarre.
“The wolves surged to meet him.”
“He had rides in the wheelbarrow.”
“The grizzled old fellow could only see on one side.”
Oh, and the original line was “There’s another way you can get a tooth out.”
I imagine a lot of people out there share my weekly regime of tipping pills from many bottles into little plastic containers that mark the days by day and night. Times were, I took nothing (though my recurrent anemia said I really ought to be taking iron, and my living in Oregon says some vitamin D would be good). Occasionally I’d get a wild hair and buy some supplements and taking them maybe a week or two before I tired of it. I don’t have the luxury of tiring of it and setting the pills aside anymore, so once a week, I pull many bottles off of the apothecary shelves, and count them out into little daily pods.
9 in the morning. Gabapentin (twitches/cramps), riluzole (the only ALS drug), buproprion (for depression), armodafinil (for energy), citalopram (for anxiety), ranitidine (for heartburn caused by these pills), vitamin D (for missed sunshine), coconut oil (because maybe it helps, studies are out). Usually magnesium (for muscles and nerves), but I’m out of it just now.
At 2PM, another gabapentin.
When I get home, another riluzole and buproprion. Also vitamin C, iron, and a multivitamin (because you know why). Yes that is a children’s chewable. Deal with it.
At 10PM, another gabapentin.
5 of these are to deal with effects of ALS. One is to counter the effects of the drugs I take to deal with the effects of ALS. And then supplements, because my body needs all the help it can get. So many pills, and I have never calculated how much this costs me per day. Maybe I ought to. I’d probably be afraid. And then there’s the three optionals I have; cyclobenzaprine (for really bad headaches and stress tightness), lorazepam (for when I start to freak out), and zolpidem tartrate (for when I can’t sleep). I don’t take those very often. The cyclobenzaprine (flexeril) is an emergency maneuver – I’m prone to headaches and this is for when they last for days and for fucks’ sake I just want to relax and sleep. The lorazepam (ativan) is usually taken as a preventative when I am going in to a stressful situation (why hello, legal paperwork regarding my death) or when I have panic attacks. And I’ve had the zolpidem tartrate (ambien) on prescription for ages because I sleep for SHIT. But I rarely ever take it, one bottle of 30 of them lasted me nearly 6 months. They’re also an emergency maneuver (hello, trying to fall asleep with CPAP for the first time), reactionary rather than preventative.
And there is another one out there. A possible addition to my chemical combination.
It’s called GM604. There has been a very limited trial, it’s still crazy early, but they’ve shown it to slow the progression of the disease, and even one specific trial showed a minor return of ability. As you might expect, there are a lot of people trying to get it fast-tracked through FDA approval. There’s a petition here, and a Google Group here. The company producing it is called Genervon, which sounds like something Transformers use to make new Transformers. They’ve been keeping the world aware of their progress through press releases.
At the moment, Genervon is awaiting a decision from the FDA. If they’re approved, GM604 will be available and covered by insurance. If they’re not, they must continue through Phase 3 trials, which even at an accelerated rate that the FDA has promised will still take 3 years. Which means most of the people alive with ALS today will not live to see it. There really hasn’t been enough evidence, though, that it works. There’s enough evidence to prove that they should keep studying it, and have further trials, definitely. But not enough to prove it works.
“But we QUITE LITERALLY DO NOT HAVE THE TIME TO WAIT,” says just about everyone with ALS or caretaking someone or in the Silk Circle somehow. “GIVE US THE DRUG.”
“We don’t have enough tests to prove it’s safe,” says the FDA.
“What’s it gonna do,” ALS peeps say, “kill us!?”
And so the world waits. Maybe this is a miracle drug. It’s certainly not a cure, but it may be a substantial step.
Next stop: someone stealing the formula from Genervon and producing it in Mexico. Cue many, many ALS peeps taking vacations in Mexico. Because they just want to live awhile longer and will risk anything to get it. Their life is literally on the line.
I don’t know how I feel about it either way, to be honest. My progression might be slow enough that I’ll live to see the results of that trial. But I also fully identify with wanting to take a chance, if it means more time. More ability. More quality of shortened life. I’ve already said I would participate in trials, and I meant it. If I can create clinical data for this drug by taking it and checking in with doctors, sign me up. If it kills me faster, well, now you have a data point. And if it doesn’t, you also have a data point. From a medical trial standpoint, you win either way. From my standpoint, I might come out better than I went in. Or I might die, which I was going to do anyway. I definitely want to see more testing. Either controlled by the FDA or released into the wild and see what happens.
I’m excited that there is SOMETHING happening, in any case. Even if it’s potentially one more pill in my cases.
I’ve gone on and on before about how grateful I am for the support I’ve gotten, how much I appreciate the support I’ve been given, how blown away at the love I’ve been shown. It’s probably become a little bit tiresome.
Well, suck it. There’s a lot more coming.
I admit I totally got press-ganged into doing the Walk in the first place. The Veterans Resource Group had a table in the cafe at work. I stopped by to chat, and met another person who ALSO had ALS for the first time. (I’ve met a fair few since then. We’re a small crew, but we run – or hobble or ride – in the same circles.) Part of the table’s purpose, besides awareness, was to recruit people for the Walk to Defeat ALS. “You should form a team,” I was told. “I bet you’d get a lot of support.”
I was of two opinions on that. On the one hand, it’s asking for something. I’m not good at that. On the other hand, a tiny irrational fear, ‘what if I form a team and no one shows up?’ While I was debating this in my head, a coworker walked up to the table to see what I was up to.
“Vashti’s making a walk team, do you want to join her?”
He looked at me, “You are?”
And that’s how it started. I put up a poster outside my cube, I wore the red wristband, I talked openly and honestly about the diagnosis when I was asked, but I felt really weird about asking my friends to come over in support of me. I caved and asked my friends to help me name the team at least. We had a lot of really good suggestions, but in the end, The Godzilla Squad won out. On the 16th, I posted my team link.
On the 17th of August – the next DAY, for those of you playing at home – I was at 17 members and over $1000 raised.
To say I was overwhelmed is a gross understatement. So, fun fact! I’d never cried for joy before. I always thought it would be kind of cool if something like that happened to me, but I am not sentimental in the right ways, I guess, so it never happened. Until then.
The Ice Bucket Challenge gained serious momentum, and so did my team. On the 26th, I was at $3k and 26 people. A dear friend of mine in Sacramento also started a team in my name, Team Dinsdale. We met online waaaaaaaaaayyyyyyyy back in the day, before the Internet was a thing, when you had to dial directly in to someone’s computer and leave messages on a digital bulletin board. In the BBS days, my first handle was Dinsdale.
Life continued its usual frantic pace, there was a lot happening, and before I knew it, it was the final weekend. I had four people staying at my house to attend, and one flew in from Sacramento to be here for me. I was spoiled absolutely ROTTEN that weekend, with homemade Ethiopian food of amazingness, fancyface ice cream and donuts for dessert, and the best company a girl could ever ask for.
And then, Walk Day. This is my team:
Amazing people, every one.
We gathered in a spot that was strategic and awesome until the live band started playing. Right. Bloody. There. But we were VERY easily distinguishable in the crowd with the hoodies (OMG SO AMAZING LEENDAH I LOVE YOU) and Danielle, my main babe, had printed out the kitten-vs-Godzilla picture I’d been using for my Walk page, and attached them to an umbrella. And Matt. Oh my golly Matt. He had commissioned a mighty cape of DOOM and a head cover for his staff:
IS THAT NOT AWESOME.
Yes of course it is, don’t even bother answering.
There were a LOT of people there. Oh my god so many. I’m really glad I had my team around me so I was constantly distracted by OH MY GOD HI I HAVEN’T SEEN YOU IN FOREVER instead of ..holy crap I am in the biggest of big crowds and this sucks. We borrowed wheelchairs,Danielle and I, because I can walk a mile, but it sucks, and I think three is out of the question. Danielle had to borrow one because her foot is borked and it hurts her a lot to be on her feet at ALL and walking three miles is similarly out of the question.
It was a FANTASTIC walk. Well. Roll. I got pushed. The chair was surprisingly easy to wheel myself around in, but I had a lot of people willing to help me out. There’d been cold and rain suddenly, but it cleared up in time to be LOVELY for the walk day. Even a little too warm to wear the hoodies all day, for they were made of fleece and are SO COMFY AND WARM but maybe not the best when standing for a while in direct sunlight. Megan was the smart one, she held the umbrella. Some surprise faces showed up – I didn’t expect my older brother there, he told me he had to work but then didn’t have to! – and met a couple new friend-of-friend faces and did not at ALL have time to introduce everybody to everybody. We walked a really pleasant stroll along the waterfront, and groups connected and drifted as we walked.
We finished, exultant, and some of us stayed for a picnic, and some of us had to get back on the road.
I am so. so. so incredibly grateful. I am grateful to everyone who came. Everyone who couldn’t come but donated. Everyone who couldn’t come OR donate, but thought about me.
In the end, my team was 49 members strong, more than 35 of whom showed up to walk, and $5460 raised.
I’ve always strived to be the kind of person someone would care deeply about, and like having around. I …I guess I managed that, if the support and love I’ve been shown is ANY kind of indicator.
I love you all. You’re amazing and the world is lucky to have you in it.
She and her fantastic husband Colin actually made me the cards. They are a physical thing. They are sparkly embossed and amazing. They ALSO gave me the Jack Skellington and Oogie Boogie figurines you see (and I heart them SO HARD) and the black heart decoration which does not at ALL show up in this picture. But it is soft and awesome.
Megan is one of the most thoughtful people I know. She once made me a little box of lip cutouts that she’d kissed with lipstick on, for when I need smooches and she is not there to give them. I can’t tell you how amazing she is. Her husband Colin, who I’ve known just as long, is also amazing and full of love. He is the perfect partner in crime for her and I love them more than I can ever possibly tell you.
And THIS, THIS is how I survive with a smile. I am orbited by planets of awesome, and the pull of their gravity keeps me from collapsing in on myself.
I love these cards and I am looking forward to the looks on people’s faces when I actually use them. I love the people who made them. I love the people who gave suggestions for them. I love that I have such amazing people in my life. I love that my diagnosis has shown me exactly how loved I am, and how completely I am surrounded by the brightest and best people in the universe.
Longer post to come. But I am so, so grateful to everyone that came out to show me love and support today. So grateful to those that could not but wanted to. So grateful to everyone everywhere who ever gave a shit about another human being. I am so glad to be alive and in such excellent company.
When I get up in the morning, once I’ve managed to drag my ass out of bed (NOoooooo sleep is my favorite hobby), this is what I have for breakfast. 8 pills and enough juice to wash ’em down.
And then once I get to work, I have Second Breakfast! Like some demented hobbit on a day pass.
George Carlin wrote in one of his books, “The sicker you get, the harder it is to remember if you took your medicine.” I’m finding it’s truer (more true? Truthier?) than I figured when I first read it. With the sixteen pills I take a day come rituals and reminders. You can’t just take some meds and be done with it. They all have their own rules and regulations and side effects. I have to take Riluzole on an empty stomach, it is very specific: “One hour before or two hours after eating”. And then prednisone has to be taken with food. I have to take them both in the morning, so I have to create a behavior that takes those rules into consideration. I take the Riluzole along with everything else in the morning (so it’s not technically an empty stomach, sure, but pills aren’t food, even if the dinosaur multi and the vitamin C ARE chewable). It takes me about an hour to get to work, so by the time I get in, I can take the prednisone. So I have something to eat and then swallow a couple pills. I have a reminder set at 2PM to take my second dose of gabapentin. And then another at 4:30 to take the other Riluzole, so that an hour’s gone by when I get home and start thinking about dinner. When I get home, another three pills wait for me – the second daily dose of buproprion and ranitidine, and the magnesium supplement. At ten PM, another alarm goes off on my phone and I take the day’s final gabapentin.
Six appointments a day. For medicine.
Thank the merciful LORD ABOVE for technology. I don’t know how people did this before phone alerts. I’ve forgotten to brush my teeth some mornings, how the hell am I supposed to be able to remember at what times to take what? So I rely on my phone’s alarm function and one of those stupid little day-of-the-week pill boxes with night and day dose rows. Once a week, I tip the right pills in the right boxes, and then just empty the right one into my hand when I head downstairs to work and swallow them before I leave. And then there’s three bottles of pills in my drawer at work, to be accessed at the right time. I have pills EVERYWHERE. And I should have a small stash of those three pills in my bag, in case I go somewhere on a weekend. Again, a George Carlin quote. “Now you’ve got shit all over the WORRRRLD!”
The sicker I get, the more pills I’m going to have to take.
The sicker I get, the harder it is going to be to remember if I took my medicine.
It’s an obstacle I’ll overcome when I get to it. I’ll have to. At least my phone isn’t affected by all of this – the sicker I get, my phone don’t GIVE a shit – so as long as that works, I’m set. …As long as I remember to take it with me. These rituals and reminders are part of my life now, and that’s okay. It’s not bad, just different. Just one more habit I have to form, one more shift in my lifestyle. And someday I might have a feeding tube, so I won’t even have to swallow the little fuckers. Just bloop bloop bloop, in they go.
As long as I remember to have my phone, to remind me take them.
The top shelf is either decorative bottles or perfume. Because fuck yeah, lovely smells. Kristy, you were EXACTLY right; Cost Plus had precisely what I had in mind. Thank you! It’s going to be AWESOME when all of the bottles are labeled and the cabinet painted/stained.
I got my braces today! These AFOs (ankle/foot orthotics) make walking SO SO SO much easier. All the fashionable Wicked Witches of the East are wearing them this year. I’m going to make leather straps for them, though, because the crunchy crunch of the Velcro is I walk is driving me nuts.