Unless you’re new to this blog, you know I’m an optimist. If you are new to this blog, welcome! Pull up a chair, have a look around. I hope you find something useful here. And oh, by the way, I’m an optimist. I can’t even tell you why that’s so, but I’ve always been. Even when things are absolutely shit, I still believe to the core of me in some way, somehow, things are going to be okay. Even if it’s a new definition of okay. I don’t think it really serves in purpose being pessimistic, because when you’re a pessimist and things go wrong, not only are things bad for you, but you’ve been miserable for a long time up to that point – because you’ve just been waiting for it turned to shit. “I told you so” is a cold comfort. Seeing it coming doesn’t necessarily make anything better. It makes you right, I guess, and if that makes you happy good on you. But the anticipation of misery just makes the lead-up miserable also. Besides, it really will be okay. I know it.
There are those of you out there who call yourself a realist. You’re not, really. You’re just a pessimist without imagination. If you are going to expect things to be bad, at least have some imagination on how you get there.
So, yeah, optimist. Even though the end of my path is set and dark, there’s still a lot of light here. More than I ever would’ve thought possible. I’ve waxed poetic elsewhere and I will again, and again, and again. Because it’s accurate. There is so much good in my life, more than ever thought I deserved, or possible, even. It’s here. I see it. It’sbright and glorious and why I continue to wake up every day.
Some days it’s really fucking hard to see that light. Occasionally the darkness and the unfairness and the all-around bullshit and fuckery that is ALS creeps in the edges and obscures everything until it’s really hard to see anything good. Everything looks like a shit sammich and the world feels awful and hard. And when that happens, I take a spa day. Or rather, a S-P/A Day. A day to sit and think and allow myself to be sad. To dwell in self-pity and anger.
Because I mean, it’s really fucked up. I’m not such an optimist I can’t see how fucked my situation is. It sucks that this disease exists at all. It sucks that I have it. It sucks that I got it so young. I wasn’t even 40 years old yet. You’re not supposed to get this disease until your mid 60s. It sucks that it’s taking my hands, and my joys in life are all to do with using those hands to create. Create delicious things, create drawings, create these words that you’re reading right now. I’ll never make another wedding cake, or draw a pretty girl in a corset, or teach myself to knit, or pick back up calligraphy. No evenings whiled away on video games. No more dancing. I loved to dance. Eventually I won’t even have a voice with which to dictate these words. And alllllllll of that …sucks ass.
It sucks that I was diagnosed less than a year after I bought my house. My life was falling into place. I had a job I really loved, I was going back to school to further that career that I loved. I had signed up for driving lessons to easily get myself from my new house to the job that I loved. I had successfully dropped weight I didn’t want and was fitting in my cute clothes again. My plaid miniskirt was a wardrobe option again. I was wearing medium T-shirts and looking good in them. I was cooking healthy food for myself. I had my very own living room to dance in. I was dancing. I was mayyyyyyyyyyyyybe open for a new romantic possibility; my divorce was amicable and well in the rearview and there had been a few crushes. I was decorating my new home to be exactly the living space I had always wanted. I had a huge, gorgeous backyard just begging for a garden, and I had such plans for that garden.
It’s not. Fucking. Fair.
So yeah. Usually I can take it on the chin and keep smiling and find the good. Because there really is a lot of good. And it almost always outweighs the bad. But some days it doesn’t. It can’t. And on those days I sleep a lot, I take Ativan, I cry, and just generally wallow. I allow myself self-pity. I allow myself to get angry. And when the anger comes, I let it fill me and I feel it to the core and I rage. And I hate. And I keep crying. And then I sleep some more.
And then when it is over, when I’ve given it a whole day, I can put it aside again. I allow it one day of my life, and then the rage and sadness get shunted aside in favor of the day-to-day living that must happen. It gets overshadowed by the joy that still here. My anger serves its purpose and then it’s done. Until the next time. I try not to let mourning for who I could have been – and who I was becoming – overrule the happiness I could still grasp if only I allow myself to look for it. It’s not all doom and gloom, but sometimes it has to be. Just for a little while. So it can fill me, and pass through me, and keep me in touch with my own grief.
Every now and again it’s important to give those emotions their own time, so that I can put them away and get on with the day-to-day living that’s necessary, and to fully appreciate all of the fucking amazing things that are still very much a part of my life.
Okay kids, it’s that time again. Let’s have the update on the status of things.
My first appointment of the day was with the social worker from the ALS Association. I was introduced to a new employee, and I had been asked if this new employee could sit in with me for the whole day to get an idea of what clinic is about. I’ve had a couple people sitting with my sessions; I think I’m a good candidate for kind of easing someone into the job. I’m not so far down the road that I’m a bitter, depressing train wreck, I’ve got a pretty good attitude about things, and – as evidenced by this blog – I am not opposed to sharing. He was a really nice guy and understood a lot of the gaming and geek references that J, Gecko, and I shot back and forth with each other throughout the appointments as usual. There was nothing new to report on my part for this segment, the house search is still ongoing, the appeals for help are coming along, I’m doing okay here.
Second appointment was with Kelly the dietitian. Because she is also my friend, she had brought me birthday presents of two graphic novels from a series I adore called Lackadaisy. Happy birthday to meeeee! The official part of my appointment went smooth as usual, I received my typical good-natured lecture about drinking water instead of soda, otherwise everything checked out. I remarked to Tony, the observer, that presents aren’t usually part of appointments, but I would never be upset if they were. ALS shuld come with fabulous prizes. Or at least an administrative assistant. Kelly is incredibly thoughtful and it’s a pleasure to be her friend – not just for the comic books.
Third appointment was nursing. I had a few things to talk about here, things that would be addressed by the corresponding specialists as the appointments happened that day, but it’s always good to give the nurse a heads up so she knows what to follow up on.
Fourth was respiratory. My absolute least favorite appointment of all time. We started with the standard cough check, which is still great. Second thing we always do is check my inhale capacity, a test where I put a mask over my nose and mouth and inhale as sharply and strongly as I can. I’ve always done really well on this test. The respiratory specialist agreed with me and told me my sucking was great, upon which J, Gecko, and I did our level best to not lose our shit laughing. We are 12. The last test is the worst one, where I lean into a mask again with my nose plugged and after a couple normal breaths I forcibly exhale as much as I can and keep exhaling and keep exhaling until I feel like I’m breathing myself inside out, and then sharp inhale. I do this three times. During this appointment, the test machine said “great effort!” on all three tries, which it doesn’t always do, so I felt really encouraged. Until she told me my breathing had actually still gone down a bit. Not as dramatically as before, though. Still not dangerous levels yet, but still very much declining. I tried not to get discouraged and mostly succeeded.
Appointment number five was neurology with Dr. Olney, the new partner for Dr. Goslin. I mentioned to him the biting my tongue in my sleep thing, and how I’ve been using a night guard to get around it, and he asked me a very obvious question. “When did you start your new medication, again?” I replied it was… Right around when I started biting my tongue. Derp. It had not occurred to me at all that it might’ve been the side effect of the new medication, but when he asked that question it seemed very obvious that it was the problem. It’s still not a dealbreaker, just like the weird sleep pattern is not dealbreaker. That is the price I pay for not spiraling into a black mood at 4 AM every day, and it is worth the price of admission. I also talked to him about bladder control, or in my case the absolute lack of, as a follow-up to my previous conversation with Dr. Goslin. She had told me what my options were, and I went home and researched the mall because that’s what I do, and I had pretty much made up my mind how I would like to address this. I’m getting really sick of wearing adult diapers, they’re expensive, and they don’t always work properly and I wind up wetting the dang bed anyway, and I’m getting preeeeeeetty tired of sitting in my own pee. I have two options for a catheter, one is the traditional Foley catheter which is just a tube snaking into your urethra and a bag attached to the other end. It’s functional, but prone to infection, and for women it’s not something that is fun to have to sit on at all times. The other option, the one I’m now pursuing, is called a superpubic catheter. It’s where they punch a hole into your abdominal wall, connecting a tube through there, which drains into a bag, same as the other. It’s reversible, it’s not a horrible procedure to have to endure, and it would eliminate the need for a very uncomfortable tube in a very sensitive place. We talked about my previous experience with the urologist, how I stopped going to her because it was obvious she had no idea what ALS was. (Here’s a clue: we are not going to fix my problem by doing Kegel exercises.) He gave me the names of two urologists that have worked with their patients before and were trusted. I’ll keep you posted on how this goes.
Sixth appointment was pulmonology. The last clinic had me meeting with the pulmonologist also,and so this is a new part of the Clinic routine. My standard physician was on vacation in New Zealand (jealous!) so I had seen a practice partner. Who I actually really liked, though my regular doctor isn’t a bad guy at all; his partner has a better sense of humor is all. He had grabbed my breathing machine records from the net, because my AVAPS automatically uploads my sleep reports to the cloud and that’s not even creepy at all what are you talking about. He was overall pretty happy with my results, but still chided me about needing to use my machine for more than four hours a night to get the best results, and more than 60% of the time. I was properly chastised and promised to be better about using the machine. Which I stayed true to by the way. So far. J came over and helped me this last weekend with cleaning and organizing, and now my AVAPS doesn’t live on the arm of my wheelchair or the corner of my bed anymore, it has a proper home now, on a shelf just out of reach when I’m lying in bed. Since I have to wake up properly and sit up to turn the machine off, I’m not pulling the mask off after only four hours anymore. I also was encouraged to keep up with breath stacking. Because of course I was.
Seventh and final appointment was with PT/OT. I didn’t get to see Deb unfortunately, but I like all the specialists. We breezed through the appointments since I didn’t really have anything to report. We tried the grip meter on my left hand just for funsies, and it registered nothing at all. “…You got it to move,” she told me encouragingly. But not enough to register as a grip. Meh. I promised to hit them up when I start needing help with other things, like toileting and whatnot. I’m still doing pretty okay on my own with those things, for now. But I will be very happy when I don’t have to get up to pee anymore. I can still do the necessary things, but only having to do them once a day instead of four or five will be a very welcome change.
So that’s what we did. And that’s how it went. Steady as she goes, with nothing new to report except a 6% decrease in breathing capacity. They didn’t even have me bother talking to speech therapy this time; I never have anything to report. Oh, there is one weird thing that happened. We did the FERS scale and my self-evaluation actually had me at 32, higher than the 26 I was last time. When I got the letter afterwards, they hadofficially put me at a 30. Which is still higher than it was last time? I have another appointment with Dr. Goslin in a few weeks and I will ask her about that then.
And now you are up-to-date.
Hello my lovelies!
Monday the 14th was Clinic Day. I had originally wanted to do a video update for this, but I just haven’t felt up to it? And if I wait until I feel up to it this update is going to take for freaking ever and then you all will start to worry because you think it’s all bad news. Spoiler: it is not. I am just lazy.
When we first checked in, I was given a sheet to fill out. I am pretty sure I did this last time as well, but I do not remember. It was a self assessment sheet for the ALS functional rating scale (ALSFRS-R). This is a standard way to track the progression of the disease. Usually though, the care team fills this out for me. During the nursing portion of the appointment, it was explained that they are heading towards having patients fill this out for themselves, as a sort of experiment to see how the self assessments differs from the professional assessment. I actually assumed that the self assessments would be worse, since I tend to understate my difficulties when talking to someone, but am more honest with myself filling out a form. Apparently not everyone is like that, since the nurse would tell me that for the most part the scores are consistent. Huh. There are 12 categories, and each category is rated from 0 to 4, with four being completely normal and zero being nonfunctional. For instance the walking category is rated as 4 being normal, three being early ambulation difficulties, to his walks with assistance, one is nonambulatory functional movement, and zero is no purposeful leg movement at all. I am currently at a two. So then you take all of the numbers and add them up, which gives you a number from 0 to 48 and 48 is a normal human being with no difficulties at all. It’s a nice numeric way of tracking progression, and encompasses a lot. It isn’t perfect of course, but it’s a nice shorthanded overview of everything at a glance. You can check it out for yourself here, if you wish.
Last Clinic Day, I was at a 34.
My first appointment of the day, after getting to our assigned room and being handed a schedule, was nursing. In addition to explaining a couple of substitutions in scheduling (I’ll get to those) she went over what I just talked about with the self assessments. Nursing checks are always an overview of the big picture in my life, if I need any special appointments made, if there are any concerns I have outside of the specialists I’ll be talking to that day. We talk about any changes since last visit, and in general she is my master coordinator of all things. Nurse Nancy is amazing and I am lucky to have her on my team. She also explained she would be playing the part of my dietitian for the day, as Kelly was sick. Poor thing. I always look forward to talking to Kelly, even though I never have anything to report. My eating is fine, we usually wind up just chatting for most of the appointment anyway. Today, however, it was noted that I had lost a whole 11 pounds since last clinic. Normally, this would be a good thing for someone, but not so good when you have ALS. I weigh 211 pounds currently, up from 160 when I was diagnosed, all of that on purpose. I am under strict orders to not diet to lose weight, as extra weight statistically helps with prognosis, and when I am no longer able to eat I will need that extra wiggle room. So to speak. So we talked a little bit about my dietary habits and I mostly attribute the weight loss to no longer being at work and on any kind of schedule, so I kind of eat whenever I feel like it, which is usually only once a day. Anymore I also need help with food prep, so if I want to eat something besides the frankly embarrassing amount of snacks I have stashed in my room, I have to bother my mom. Not that she minds, at all, but I am terrible at inconveniencing others for my own sake. Nurse Nancy made me promise to stop that. I am making an effort to stock more snacks and not just drinks in my fridge that I can get to whenever I want.
My second appointment was with Dr. Goslin. It had actually been a few appointments since I’d seen her specifically, since the previous appointment was my introduction to the new doctor, Dr. Olney, so it’d been half a year. We spoke mostly about the medications for this appointment, my increasing depression specifically and overwhelming anxiety. Unfortunately no longer reporting to work means I no longer have a distraction to keep me from spiraling into bad moods when I think too much. It’s been a rough couple of months because of that. I still don’t have a permanent place to live and I am continuing to freak out about that. We doubled my dose of Ativan in the meantime, and she gave me a couple of options to think about for ongoing anxiety and depression. Otherwise, as usual, she is very pleased with the rate of my progression.
My third appointment was with the power duo team for occupational and physical therapy. Physical therapy pointed out that my calves are getting tight and I need to be better with my stretches, and since I can’t really stand on my feet and touch my toes anymore to stretch them out, I was given bands to put around my toes and use my forearms to pull up on them while I’m laying in bed. The whole point of that is to keep my muscles limber so that I can continue to use the walker to visit the bathroom while I am at home for as long as possible. Occupational therapy was entirely centered around keeping my hands functional as long as possible, and preventing my fingers from curling up and cramping while I sleep at night. We also measured my hand strength as usual, and of course they are still garbage meat noodles of uselessness, no big surprises there. Deb the Amazing OT had previously suggested a new kind of brace for me to buy, and I had, but they need adjusting and some modifications to make them actually usable on my own as they are primarily built for bicyclists and didn’t have crippled people like me in mind so they’re not exactly easy to get on and off. We made plans to have another appointment outside of clinic to go over all of those things. I’ve since had that appointment, and like a total genius I forgot the braces in question at home, so she wasn’t able to adjust them for me and now I have a second appointment this coming Monday to take care of that. I was told if I forget them next time I’ll be sent right back home. Hehe.
The fourth appointment was speech. This appointment also includes swallowing, and all of those muscles in general. Luckily, this is the one area that I have yet to experience any problems, so these appointments always go very fast. She just verifies that I am not choking on my own spit anymore than a normal human being does, watches me swallow liquid, and eat a dry graham cracker, and make some funny faces including blowing my cheeks out and trying to touch my nose with my tongue etc. to prove that all of those muscles are still in tune in good shape. So far so good.
The ALS Association was fifth, for social work. I remain eternally grateful for their help. Unfortunately the problem I most need help with is housing, which is not their specialty by any stretch. They do however have resources and connections to other services that are useful, and most importantly they have always been willing to do research on my behalf to do what they can to help. During this appointment, we talked a lot about senior services to get my mom some assistance in whatever way we can. It’s all very useful information, and I truly appreciate the help. They have connections I would never even dream of and that alone is extremely helpful.
My sixth appointment was respiratory. This is the one I hate the most. It’s exhausting, and for the last few Clinic Days, it’s also been somewhat disheartening. We spoke a little bit about new policy changes, specifically about the clinic no longer being able to keep equipment for their patients and so I have to take my respirometer home with me every time now. No big deal, it’s not that big and it fits in my purse just fine. The breathing test came and went as usual and I was surprised to find that my breathing has actually remained perfectly stable since last time. I don’t even need to tell you how happy I was to hear that. I think sleeping with the new AVAPS machine has been helping, and I still need to recommit to breath stacking of course, even though I hate it. A lot. I still owe you guys a demonstration video of exactly why that’s so miserable. But still. No change. Great news. I will take it.
Usually, that would be the end of it. I typically have six appointments. However, since I have graduated to the new sleepy time breath machine, we have added a pulmonologist to the mix. I met with him last. My standard doctor was apparently on vacation in Ireland or something, so I met with a substitute from his practice. It’s a shame he was a substitute, because I really like him actually. He had some suggestions about the mask I’m using at night, some accessory suggestions for the machine, and sheepish apologies that a lot of these commonsense accessories aren’t covered by insurance at all. I came out of that appointment with another doctor appointments to make for a new mask fitting. That will happen on Tuesday.
After that, I headed downstairs to the lab for some overdue bloodwork – I was supposed to have done so last time apparently, but we missed it. Most of my levels came back normal, but I am once again experiencing a vitamin D deficiency. Probably because now that I’m not going to work I don’t really get out into the sun at all. Ever. I take a 1000 IU supplement, but after seeing my levels Dr. Goslin told me to bump it to 4000. Apparently my deficiency is not screwing around.
After all of this, and my summary letter came in the mail, I discovered that my ALS FRS rating has gone down to 26. Down eight points out of 48 total in three months worries me a bit, I admit, but I don’t know if that’s me shifting from doctor to self evaluation or what. I certainly don’t feel like I’ve declined that far in so short a time. But my breathing is the same, and that’s what I care the most about right now. So I’m happy.
And that, my loves, is how Clinic Day went.
Jeez, peeps, it’s been a month since I’ve posted. Good thing this blog isn’t monetized or anything. A kind commenter left me a little prod about being so quiet, and I realized I should get off my ass and say something. Or…get ON my ass, since writing involves that position. It’s been awhile that I’ve been in a place to be able to pace and dictate or whatever.
The first couple weeks of October, I don’t even have an excuse for not posting anything except perhaps laziness. Well, obviously laziness. No ‘perhaps’ about it. The last two weeks were kind of rough if I’m being honest. Politics caused me a lot of sleepless nights, and I wish I had it in me not to care about any of it, but I don’t. I often say the one silver lining to this disease is that I get to check out early, whereas the rest of you people have to stay and deal with this. I won’t be alive to see California slide into the ocean, or Handmaid’s Tale cease being fiction, or Auschwitz’s rebirth in my own goddamned country. There is a small grace in that. It still doesn’t prevent me having sleepless nights just absolutely freaking out over everything I cannot change and crying a lot. I wish the only thing I had to worry about was my actual disease.
The main thing going on in the last couple of weeks has been transportation problems, and breathing problems. I had an appointment to get my new AVAPS sleep machine, which I was very nervous about because it’s a whole new goddamned concession to this bitch of a disease, and I’d asked J to go along with me. Unfortunately, when we got there I was not able to get out of the fucking van because the automated door mechanism decided not to work. At all. We were used to it being a flaky bitch and sometimes not latching closed properly, but this was new. The door would not open all the way even with J pulling it open manually, so the ramp could not extend. And so I sat in the parking lot missing my fucking appointment while Jay feverishly tried to figure out how to free me. The door mechanism has been kind of flaky pretty much since I got it, and she’s been in for repairs twice already. J managed to get the door to open all the way, but he missed work the whole day instead of just a couple of hours while we took her in for an emergency appointment. The mechanics there were able to temporarily Band-Aid the problem.
By pretty much breaking my door.
Their fix to my door not automatically opening was to simply cut the cable that opened it and turn it into a manual door. Which is not, of course, a fucking fix. It was a preventative measure to keep the cable from snapping of its own accord and shearing apart the motor. We got a proper appointment later that afternoon to see what could actually be done, and after having my van for over an hour they explained that the entire mechanism would need to be replaced. Oh and now also the cable. Which, after the last time the van was in for service, we already knew replacing the assembly was going to be the next step which is why I had previously asked them to replace the entire assembly in the first place, and they never called me back. A month ago. So yes please can we actually REPAIR THE FUCKING VEHICLE.
This was all after getting pissed off that the mechanic would only talk to J and ignore me – because I’m just stupid dumb woman who doesn’t know anything about vans LOL. J very politely asserted that it was only MY name on the paperwork and registration and I would be the one paying for the repairs, so really he should be addressing me, not him (J is a champ). Thankfully, the mechanic did speak directly to me after that. But seriously. Dude wasn’t even old, he really ought to know better. They always do that; they will look at the paperwork read my name, and then turned to Jason and address him as Mr. Ross (THAT IS NOT HIS NAME EVEN) and explain what needs doing.
BITCH I AM SITTING RIGHT HERE AND WILL BE PAYING FOR THIS HI HELLO HOW ARE YOU. FUCKER.
Moral of the story is that we agreed the door assembly and cable need replacing. I kind of had to insist that we do that – the mechanic was seemingly trying to talk me out of it like, the door is working? (HI NO IT IS NOT BECAUSE IT IS A MECHANICAL DOOR THAT IS NOT FUNCTIONAL AT THIS TIME) The repairs will be expensive? (I HAVE A FUCKING WARRANTY) We got our van back, with the door still needing to be manually opened and closed before the rant will work, and the mechanic sets to ordering the parts that were needed and getting warranty authorization for repairs.
…A week later he calls to inform me that the warranty is declined for the repairs, because my contract does not cover those parts. He thought it sounded fishy and asked to see my actual contract. I have agreed the hell it doesn’t cover the freaking door, I paid $2000 for that fucking warranty it had better, and agreed to send him a copy. Turns out my fucking contract does not cover anything but the ramp itself, and the drive train. Literally nothing else. I paid $2k for them to not fix anything but a catastrophic failure THANK YOU ARIZONA MOBILITY FOR YOUR USELESS GODDAMNED SERVICE CONTRACT. So I am on the hook for $1000 repair. I have an appointment to go ahead with the repair on Friday, and then yesterday the ramp itself decided it doesn’t want to work either and refused to work for a moment. And then did work. Much like the door itself would sometimes decide to close and sometimes not. So I probably get to look forward to getting that repaired soon as well. Theoretically at least that will be covered. IN THEORY.
After missing the breathing appointment, I came to find out that the respiratory folks would actually have been able to come to my apartment in the first place to set up the machine. THIS IS USEFUL INFORMATION I WOULD HAVE LIKED TO HAVE. YOU KNOW, BEFORE. So we set up an appointment for them to come to me, and last Tuesday they delivered my new machine. It is, as promised, the most powerful noninvasive ventilator you can get. It costs $6400. If I can prove compliance – by which I have to use this machine at least four hours a day for 21 days out of 30 – then my insurance will pay for it outright. Otherwise I’m going to have to rent this thing for $700 a fucking months Covered by insurance, sure, but my co-pay is about to reset in January and I will be goddamned if I am paying for this fucking machine on top of everything else. I’ll save my precious out of pocket money for the $17,000 a month infusion meds that aren’t helping. I guess. So I just need to prove to Providence I am going to use it when I sleep. Pinky promise.
That’s turning out to be much easier said than done.
This machine is indeed much more powerful than my old CPAP was. Too powerful. It’s kind of like breathing from a turbine engine. It has a ramp function, where it will slowly turn the air up, but even that is not exactly comfortable. By which I mean it is genuinely hard to breathe with that machine on. I can’t take a little bit of air, it’s like drinking from a fire hose. If I try to breathe shallow to prevent the machine inflating my lungs like balloons, it freaks out and tries even harder. Eventually I have to pull in a huge lungful of air, which the machine helpfully cuts off the airflow after a second and I have to breathe through my mouth to catch my breath entirely. I have fallen into a routine of putting the mask over my face and then just checking out the Internet or watching TV or something for an hour or so before bed to try to get used to it before I am actually attempting to sleep. Like, distract myself and trick my body into being cool with my new overlord breathing on my behalf. It’s not really working. The only way I’ve really been able to sleep with it is if I medicate myself either with Ativan or Ambien first. Even then, I wake up fairly often. I find myself constantly checking the machine to see if I’ve hit four hours of use yet so I can pull the mask off and sleep comfortably.
This machine has taught me what my actual limit for life is.
As I lie in bed struggling to breathe, I know that when this becomes my existence, and not just an artificial effect produced by a machine, I am going to be done. I cannot live that way. It is abjectly miserable, and it is impossible to do anything else when you are struggling so hard just to get air into your body. That is not going to be a life worth living. When the machine is doing my breathing for me, and there is nothing in my brain except an animalistic fight for control over the oxygen coming in and out of my lungs, that will be the time to say goodbye. Because that is not a life.
For now though, I have the ability to pull the mask off. I counts down the time until four hours has passed, when I can pull the mask off and sleep properly. For now life is still great. I have ideas and plans. Including a very important plan to call the respiratory specialists and see if there is some adjustment that can be made to make me a little less abjectly miserable when I’m trying to sleep. To make sleep possible at all without taking potentially addictive medications. Friday I will take the van in and try to get it limping along for a little while. I literally just needed to last a couple of years. Sunday, I get to do something really really awesome I am super excited about. I have things to do. People to see. Life to live.
One breath at a time.
Now that ALS has started to incorporate itself into my subconscious, more and more of my symptoms and new realities have started showing up in my dreams. I can usually still walk. My hands usually still work. ALS isn’t tied into my subconscious self on a permanent basis, yet; it hasn’t become fundamentally part of who I am in my dreams. But occasionally ALS will tiptoe into my dreams and I’ll be in a wheelchair, or I’ll have that dream where you’re trying to run but can’t, but it’s not panic inducing because …of course I can’t.
My current reality with Radicava has apparently assimilated itself into my subconscious as well. In real life, I have a port which is attached to a tube that snakes its way into my arteries. Once a month a nurse comes, and stabs a bent needle through my skin into that port, from which an IV line can be run. We attach medication to that IV line, and thus my infusions are done. Gravity plays a hefty part in this role and the flow can go both ways; we found out the hard way that if there is not medication running into the line, then blood can run out. There has been a couple of times that we’ve been a little delayed in cutting off the flow at the end of the infusion while unhooking the bag, and dark arterial blood starts running up the line. Blood is *supposed* to be able to travel up the line, it’s one of the things they look for when they first access the ports, to know that there is good flow. That’s probably because the first couple of times I’ve accidentally bled into the line he freaked out a little bit. Now we are super casual about it, because it’s honestly no big deal. We just have to push a little saline down the line to get my blood back in my body, and throw that line into the biohazard ban instead of the trash. NBD.
…It is still a little unnerving to know that if I really wasn’t paying attention and things went wrong, when the port is accessed, I could just…bleed out.
I guess my subconscious thought so too. Last night I dreamed that J was helping me with the infusion while we were in some hotel, and somehow the IV bag came off and we didn’t notice. The end of the line just spilled out into nothingness, and I had bled out over a pint before either of us noticed. In my dream J instantly went into panic mode, and started furiously trying to clean up the blood once we clamped off the line, while I assured him that a pint of blood is practically nothing, and people donated this much all the time, and I was fine. I conceded that cleanup was definitely in order, the place looked like a murder scene a little, though. I was more worried about explaining blood stains in the carpet to the people that actually owned the place.
And then the cop showed up.
Suddenly in my dream I’m explaining to a very nervous and suspicious policeman about ALS, and what it is, and what the infusions are all about, and how this was a medical mishap and is not an attempted murder, actually, despite what it looks like, while J is furiously scrubbing blood out of the carpet. I think in the end I convinced the cop that everything was okay, but J was still panicking and worried that he was going to get arrested for trying to murder me.
So yeah, apparently Radicava is normal enough to me now that it’s showing up in my subconscious dreams. That’s a new one.
So uhhh I have my port installed. It was …an adventure. Lemme tell you all about it!
When last we left our intrepid heroine, she had a really nasty reaction to the adhesives on her PICC line dressing. We moved to a different type of dressing that didn’t adhere for shit. The day I was done with the 14 day initial run, my awesome home infusion nurse came by to change the bandaging. She recommended we get the PICC pulled ASAP so I could get the dang adhesives off my skin.
It took a little convincing to get the nurse to refer me for a port; she was concerned that if I had a reaction to the adhesive as it was, having a port would be just as bad. I was concerned about not having tubes sticking out of me at all times; at least the port would give me a 2 week break every month. We eventually secured the referral, and my surgery was scheduled for 12:30 on December 27th, with a separate appointment that morning at 8:30 to get the PICC removed. When I told my home infusion nurse, she arranged to come by that evening (after her company holiday party, even!) to pull the PICC so we could get the wrappings off me and let my skin recover a bit. We love Deena, she’s rad. It was surreal to see her pull the line…it kind just kept coming out, like a magician’s scarf of surgery and horror. It was MUCH longer than I’d thought. And it was friggin’ DELIGHTFUL to have all that out/off. And the shower I was finally able to take was goddamned MAGICAL.
I received a call from St. Vincent’s hospital the next day – the surgeon I’d been scheduled with wasn’t actually going to work that week, could I go to Providence Portland instead? At 9:30am? Sure, no problem. The next day, they called again – sorry, could we make it same time, but back at St. Vincent’s after all? No problem. J worked that day, so I asked my sister-in-law-of awesomeness Jeanette to be my ride.
And then I made a terrible mistake.
OK so I’m a smart person, and with intelligence comes curiosity, and I wanted to know exactly what I was in for with this port. I watched a video of a woman demonstrrating how to access a port; no big deal, kind of persnickety with regards to keeping everything absolutely sterile, but whatevs. And thennnnnnnnnnn I watched a video of a port install surgery. OK, I am not really squicked out by surgery, and I found the whole thing fascinating until the phrase “BLUNT DISSECTION” came up, and the surgeon LITERALLY TORE A HOLE IN THE PERSON BY JAMMING HIS PINKY IN THE INCISION AND WIGGLING IT AROUND. HOLY FUCKBALLS. My brain went NOPE, FUCK THAT, FUCK THIS, AND FUCK YOU FOR SCHEDULING THIS MOTHEFUCKING MEDIEVAL-ASS SHIT. Intellectually I knew it was fine – MORE than fine, because I was gonna be hopped up on Versed and remember NONE of it and get some good drugs afterwards – but the actual crux of the mistake had been watching this video righe before bedtime, so my brain just did donuts in the parking lot for awhile.
The morning of the surgery, I got a call from the hospital – would I mind coming in early? It was 8:30 and I was scheduled to check-in at 9:30, so I was a bit confused, but I told them I’d be there as soon as I could. We parked too far away and I was a sweaty mess when we arrived at check-in, where I was told…I was scheduled at the OTHER hospital, still. I explained the conversations, they luckily sorted it out. I went upstairs, put the goofy little gown on, and waited. The nurses came in, partly to take vitals and start an IV and make sure I wasn’t pregnant, and partly to play peek-a-boo with my adorable niece. The doctor came in to introduce himself and ask if I had questions. he was delighted that I referred to the Huber needle I’d be using to access the port as “the crooked needle”. I was delighted by his use of the phrase “twilight sleep” to describe the conscious sedation I’d be under. They’d give me benadryl in my line along with the sedatives and a dose of antibiotic, he said, hopefully I’d just sleep through the whole thing.
I remember them wheeling me into the surgery, and seeing the GINORMOUS fucking monitor he’d be using to make sure he was placing the port properly, with x-ray. It was weird to see my name there. The several nurses had their A-Game banter going, and we were having a great time explaining names and tattoos to each other. One nurse thought I had an accent, and asked how long I’d been a Yankee. “My whole life?” A male nurse/technician/whathaveya scrubbed me down with a blue solution, making me the world’s least convincing Smurf. I was freaking cold, despite the warmed blanket they’d put over me. The IV nurse told me he was putting the antibiotics in my line now, and I felt the cold sting. he told me he was starting the sedative now, and then I…
I do not remember a thing after that.
I woke up with a very large image of my x-ray on the monitor, showing the line running into my artery. I was shivering. They wheeled me back to the recovery room, and I slept a bit. Jeanette had taken my niece Jewel to get some lunch or something. They brought me a turkey sandwich, which I devoured. They had put the dressing on a bit shitty; I couldn’t raise my head all the way, or turn my head much because of the way my skin was taped up. It itched a bit. I was given discharge instructions and no pain killers (“Just take some Tylenol”). My sister came back, I got dressed, she took me home, and I slept some more.
The next morning I was aware something was up. I was naughty and pulled the dressing off a bit to allow myself the ability to raise my fucking head, and where the dressing had been tugging was now an angry red wound. I was very itchy. All over my chest. Angry red hives, all over my chest, up my neck, and across my chin. Tylenol, it turned out, did not fucking suffice, so I took some of the hydrocodone I had leftover from breaking my ankle that the doctor hadn’t wanted to give me either because ibuprofin should be fine. YOU JUST TORE A HOLE IN ME BIG ENOUGH FOR A 1 INCH SQUARE MEDICAL DEVICE AND ALSO CUT MY NECK TO SHOVE WIRES INTO MY VEINS – NEWSFUCKINGFLASH TYLENOL AND IBUPROFIN ARE NOT SUFFICIENT FOR THIS. BLUNT. DISSECTION. BITCHES. I slept some more. I woke up to even angrier hives, annnnnnnnnd my eye was a swollen bag of fluid.
So uh…yeah. I was having an allergic reaction to something. It couldn’t be the dressing itself, since the hives were everywhere. It got worse and itchier over the night. I had a different IV home nurse visit that day, to change the bandage since they’d thoughtfully left a needle in the port for me to start the infusions, but I’d bled a bit so there was gauze under the dressing which needed to go. She was very concerned about the reaction, namely that it might be a reaction to the port itself. My delightful friend Lizzie was coming over that day with delicious food for me, and she kindly picked up some Benadryl on her way in. Luckily I had a doctor’s appointment already scheduled for the next day, for an unrelated issue and holy FUCK will I rant about that in a bit. I took Benadryl, ate delicious home made food, and slept a lot.
My PCP is a cool guy, and didn’t mind dealing with the reaction as well as the initial problem we were scheduled to deal with. He suggested two more antihistamines in addition to the Benadryl, and to contact the hospital if it didn’t clear up by Tuesday (Monday was New Year’s). Luckily it did, mostly. I think the problem was the smurf shit they scrubbed me down with, since the hives pretty much appeared everywhere they used it. I have no explanation for my eye becoming a blister, tho.
We’ve now completed the second round of infusions, the port works beautifully, and we pulled the needle out last night. I am once again dressing-free for two weeks. The scar is already nearly healed up, and the itching has almost entirely subsided now that I’ve got no more adhesives on me. I can feel the port under my skin, and it’s weird.
Now I am become cyborg. beep bloop bleep.
So! Today is the last day of the first run of Radicava. I’m confident at this point that any side effects are tolerable, and I want to continue. The only potential things I’ve noticed is that there MAY be an uptick in the frequency/severity of my headaches, but it’s nothing I can’t tolerate, and there’s been a few times when there’s been a weird panick-attacky feeling, where my heart is beating in my throat, but that always goes away. Labs will be drawn to make sure I’m not experiencing anything serious, of course, but my own internal feeling is that everything’s GO for continuing.
Whether this is even doing any good, I won’t know until March, the next Clinic Day. But it’s not hurting anything, so we’ll keep it going. This also means I’ve asked to go ahead with the port install. It will be a vast relief to no longer have these tubes coming out of my arm that need babying, not least of which is because the adhesives that protect the PICC line itch like a MOFO.
Since my fall last Tuesday, I’ve noticed my hip hurting a bit, one spot particularly. I kept expecting a bruise to form, but it never did. Two nights ago, I was pushing on the spot, to figure out exactly where it hurt, and my fingers found a hard lump that rolled around a bit under my skin at the joint. And I remembered wayyyyyyyyyyy back at the beginning of my Godzilla Disease diagnosis attempts, how we initially thought the problem was in my hip, and then I got an MRI, and the MRI showed a small tumor thing in my hip meat. It was deemed medically uninteresting, though, and ruled out as the cause of my woes. I was told that it had probably been there, like, forever, and wouldn’t be a problem, but maybe keep an eye one things and recheck it in a few years.
I guess, yeah, it’s been a few years, so it’s time to get the dang thing rechecked. So THAT will be fun, as I can’t really get up into MRI machines these days. I’ll make an appointment soon. One more damn thing. I’m sure it’s fine, but dang, man.
I’ll let you all know when I get the port installed. 😀
Tuesday was not a good day.
It was the urology follow up, and the day began with a clear reminder of why the appointment was needed, followed by massively uncomfortable prep, and then the Lyft ride to the hospital. I was half an hour early, thankyewverymuch. Once inside, I don’t even know exactly what happened, only that my walker was moving faster than I was, and I didn’t have the strength to pull it back under me, and suddenly I was kneeling on the ground. Two older men noticed my plight, and asked if I needed help.
“Yes please?” The question mark was because I had serious doubts these two were going to be able to get me off the ground. Doubts well founded. They were not. A third, younger man assisted and we finally got me seated on the walker. They all left with my profuse, if a little breathless thanks. I wasn’t hurt at all. After catching my breath, I stood a little shakily (yay adrenaline!) and made my way to the urologist, who has a very heavy office door. A gentleman inside noticed I was having a hard time pushing the door open, and opened it for me.
…Only I was leaning on the door knob at the time. With my support pulled away from me, I lost my balance and landed on my back in the hallway.
Please ask if someone needs help before ‘accommodating’ them? This is the first time it’s actually caused a fall, but I’ve NEARLY been pushed or pulled off balance many times by someone trying to help. Please offer to help, but wait until I am aware of you and ready before doing the thing. Thanks.
I was surrounded by noisy, concerned people, including the really bitchy admin from last time who was actually quite sweet this time. One of the people who helped me up this time was one of the same older gentlemen from before. “If I’d known you were coming here, I’d have waited with you.” We got me seated again, and I was left to rest in the lobby, blissfully left to my own devices once everyone was sure I was OK.
The appointment itself was…uncomfortable. All sorts of probes and wires were inserted or otherwise attached, then my bladder was filled and i was instructed to bear down. Nothing leaked, which in retrospect was hardly a surprise because it’s only when I get UP that problems happen, but I didn’t have the presence of mind to point that out. The wires were disconnected, the doctor came in and looked at my results, and…instructed me to pee more often.
That’s her whole plan. Just get up every three hours and pee, maybe you won’t leak. Oh and keep doing those Kegels with muscles you no longer possess. She clearly does not speak ALS.
I left angry and frustrated, and took a Lyft home, only to find Amazon had delivered a package while I was gone. Right in front of my door. My case of Monster. My very heavy 24 count case of 20 ounce cans, right in front of the door. Annnnnnnnnnnd in trying to move it enough to get inside the door, I uh..yeah I fell again. Badly this time. Pain shot through my previously damaged knee and my freakin’ ankle that was broken. My mom, alerted by the noise of the fall (but not the swearing, oddly enough, she didn’t hear me yell), came out to try to help, but there wasn’t anything she could really do. Once I caught my breath and got my legs out from under me, I was able to shove the Amazon box out of my way and crawl inside. It was good to have my mom there this time; even though she could in no way help me up, she was able to get the walker out of my way and put our stepstool by my bed so I could use it to haul myself up.
Thank God I had taken an ativan that morning before leaving, or I’d have been a blubbery mess. As it was, I took a well deserved nap and was pretty sadbrained the rest of the day. Yesterday I was pretty dang sore all over and worked from home. Landing on your ass three times in 2 hours takes it out of a girl.
BUT! Yesterday was also the completion of my first week of Radicava! I think it’s going well. It maaaaaaayyyyyyyy be exacerbating my headaches though; I’m keeping an eye on it. It’s been an eventful and stressy week, so hopefully the coming one is kinder and a more useful baseline. I won’t know really if the meds are helping anything until March, the next Clinic day. I’m committed to keeping it going at least until then.
So now you’re up to date, and I’m gonna go play on the internet. <3
Step 1. Gather the required materials.
One box of Radicava, which contains two 100ml bags. Two saline flush syringes. One package of IV tubing. At least two alcohol prep pads. One IV pole.
If we were taking labs, or redressing the PICC line, there would be so many more components. This, though, is the minimum. There will come a time when this is routine and second nature, but for now, everything is still double and triple checked. We lay things out like we’re preparing for surgery. It’s not far from the truth.
Step 2. Wash your hands.
There’s hand sanitizer, which also gets liberal use, but there’s no substitute for scrubbing your hands with soap and hot water. I find it almost impossible to wash my hands well these days, and drying is a nightmare, so I typically opt for the sanitizer only. If your hands work, though, go wash them.
Step 3. Open all the packaging.
The box of Radicava contains two trays of 100 ml bags of medicine, each with a silica bag and a little pink oxygen indicator. We peel back the lids on the bags, which each have a plastic seal over the input valve. We leave those intact until the last minute. We unwrap the two saline syringes, but leave the caps on. We open the first alcohol prep pad and set it aside. We unwrap the IV line and hang it over the IV pole. OK. I think we’re ready.
I’m appalled every day at how much waste this generates.
Step 4. Prep the line and bag.
We close the IV line, either through the clamp or dial, depending on the tubing style. Removing the bag’s seal and uncapping the IV line’s spike, we push the spike into the gray seal on the bag as straight as possible so as not to split the bag. It takes a surprising amount of force to do this. Because the line is clamped, nothing happens yet. We squeeze the tube’s chamber to fill it about half full of medicine. Carefully, slowly, we unclamp the line and allow the medicine to flow through the tube, stopping it just before it spills out of the end. We clamp it off and set it aside, dangling harmlessly from the IV pole for now.
Step 5. Sanitize the input valve on the PICC line.
The alcohol pad is scrubbed over the PICC’s blue input valve for 15 seconds. It doesn’t have a cap, so it’s out in the open all the time, well, tucked up under the sock against my skin, and must be cleaned carefully. We let it air dry.
Step 6. Saline Flush to clear the line.
Uncapping the saline syringe, the plunger is pushed juuuuuuust a little bit to push the air out. Or, you know, accidentally create a beautiful arc of saline in the air if you push too hard. The syringe is then coupled to the blue port on my PICC, twisted in place to secure it, and the saline injected a push at a time, to the rhythm of a heartbeat. Bublump. Bublump. On the second push, I feel the cold liquid in my vein, and a moment later, I smell and taste the saline in the back of my throat. It’s a hospital smell. The rest of the syringe is injected, decoupled, and disposed of in the biohazard bin.
Step 7. Insert tubing into PICC and begin infusion.
The IV tube is pushed into the blue PICC port, the little plastic collar screwed tight to secure the connection. The tube is unclamped or undialed, and the Radicava begins to flow into my vein. It feels like nothing. It’s not cold, like the saline, it has no taste, no burning like some antiseptics and anisthetics. There is no sensation at all, and the only reason I know I’m getting medicine is to watch the IV chamber steadily drip drip drip drip…
Step 8. Swap bags when the first one is empty.
The tricky part to this is allowing all of the medicine to drain out of the bag without letting the chamber empty, which will allow air down the line. Air in the line is bad. I have remedy available, if that should happen; it’s easy enough to use a saline syringe at the bottom of the line to force liquid back up into the chamber and clear air out. But it’s best to not let that happen. We swap the bags and sit back to wait some more. This is supposed to be a 1 hour infusion, but it’s taking closer to 2. A lot of the reason for this is an extension of the PICC line we added so that I can reach the ports myself, which bottlenecks the flow AND adds extra distance for the medicine to have to travel.
I can do a lot of this myself, except the coupling of the IV line to the PICC. Both ends are very floppy and you can’t touch the ends without having to start over and resterilize. Doing all these things, though, cause my hands to cramp up a lot and then be completely useless for the rest of the day, so I happily leave it to others when I can. J’s been doing it the last couple days, which is marvelously helpful.
Step 9. Bleed the line carefully.
Once the bag is empty, we play a game of chicken with the medicine, allowing it to drain down the line until the air is allllllllllllllmost to the PICC line. We don’t want air in my line, obviously, but we also want every last drop of the insanely expensive medicine in my veins and not in the trash. Once the line’s drained as far as we dare, the line is clamped, decoupled, and the entire bag and IV line is discarded. More trash.
Step 10. Saline flush.
Hands are washed and sanitized again. After another scrub of the port, the second saline syringe is rid of air pockets, attached to the blue port, and screwed in. More heartbeat push, more cold, more hospital smell and taste. Yummy. The syringe goes in the biohazard bin, even though it never really touched my fluids. Quite the opposite.
Step 11. Redress and cleanup.
We’re done! Everything is thrown out, the IV pole is collapsed and stowed, and the PICC line is coiled up and tucked under the itchy fishnet sock around my arm keeping everything covered.
Today will be day 5. Fortunately, it is not time sensitive in application like an antibiotic might be, so as long as I do it sometime on that day, it’s fine, it doesn’t need strict scheduling. Last night’s dose began around 8; tonight will be closer to 10 or 11, my first ever dose was 9 AM. For the second round, I have to do 10 infusions over 14 days, and I can also pick and choose dates and times. If I want to do Monday through Friday and skip weekends, I can. If I want to do all 10 days up front and get it done, I can. It’s up to me.
The main problem I’ve had so far has been dealing with the PICC, in that it’s inconvenient as hell (I miss showering – I can only shower if I take these huge complicated precautions to wrap it all up watertight and I just can’t pull it off on my own) and itchy AF. The bandage tape is itchy, the sock is itchy, the lines are tickly and like to snake their way out of the sock and say hi to the world from under my sleeve. It’s convenient as hell for not having to have an IV poked in every single time, but everything else about it sucks. I’ve already decided to get the port-a-cath installed once I’m done with this round.
I’m tolerating the drug just fine. I *think* it’s exacerbating my headaches, but nothing unbearable, and it may be causing night sweats, but I have to research that to find out if it’s even a thing that might happen. Otherwise I’m doing good. We’ll find out in March if it’s affected my decline in the least bit.
So that’s everything so far about radicava, darlings. It’s going well. I’m still terribly excited about all of this.
I met yesterday with my PT specialists and a sales rep to pick out my new wheels. I’m getting one of these!
I opted for the midwheel drive, it’s got a smaller footprint and pivots around corners rather than steering like a car, so it’s easier to drive. I’m still totally gonna run into things. Count on it. I got the USB charging port (instant popularity!) and a cup holder and..yah! I also opted for the seat elevation feature that will let me look people in the face when sitting in the chair, even though insurance is proooooobably not going to cover it. But I think being able to see over tall counters is important.
Tomorrow I start Radicava infusions. The nurse is coming over around 11 to teach me how to do it, and then Friday s/he’ll come back to watch me do it and make sure I learned properly. They’ll also change out the dressing tomorrow, for which I am grateful. It’s itchy.
This has been your baby update.
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.
I can’t wait to keep you guys posted.
Ok seriously, this is a TMI warning. There be candid, unglam talk about pee and even some butt stuff. So uh. Yeah. Proceed with discretion.
Soooooooooo the main reason I have not been posting is two-fold, with the reason being DEEEPRESSSSHUNS, stemming from 1) my stepfather dying and now my mom lives with me, and 2) a new symptom which is KICKING MY ASS in all kinds of fun physical, mental, and emotional ways. My surprise roommate situation – that will get its own blog, don’t you fret. Things are actually settled and pretty ok on that front now; that’s the GOOD part about avoiding blogging during a crisis – you get to walk in at the end of the story!
Ok so I just checked the archives for the first time I wrote about this thing and it was March of 2016. So I need to stop calling it a new symptom. Duly noted, self. Well. It was kinda a one-off thing, it happened a couple of times? But the last few months it’s been a serious deal and I am seriously not dealing well.
As you may have surmised, it’s about this:
“Urge incontinence”. Only…there’s no “urge” part anymore. It’s closer to say that it will occur to me that I haven’t peed for awhile and should prolly do that, or I kind of have to go, and then before I can get to the toilet, I am just peeing. Everywhere. I’ve had to leave work early because I had to change my clothes, I’ve had to change clothes I literally just put on. And it’s not like I’m peeing in my chair, oh no, it fucking WAITS until I am alllllllllmost to the toilet and then just lets itself out all over me and the bathmat in front of my toilet before I have the chance to undress, much less sit the fuck down. Unless I physically cross my legs to contain it – and often, even then – there’s a mess.
I’m 42 and I wear incontinence pads every day now. As I posted previously, a lot of the articles about ALS say that usually bladder and bowel function aren’t affected. I took a lot more comfort in that than I realized. Like..sure I might be choking on my own spit someday, but at least I won’t be sitting in a puddle of my own urine. But no, I’ve not even begun to have speech or swallowing problems yet, but I have left trails of pee from my room to the toilet – while WEARING a fucking pad. They only hold so much, and I’ve peed through even the overnight Poise pads more times than I can count.
Fun fact – Poise pads are rated by flow, just like menstrual pads, onle they don’t use words like “light, medium, and heavy”. They use words like “drips, dribbles, spurts, and gushes”.
Additional fun fact: menstrual pads and incontinence pads are NOT THE SAME THING. Ask me how I found out! At work!
So, last clinic, I brought this up, and we started the road to Figure Things Out. It has NOT been a good road.
Like, at all.
We started the easy path, with a medication. That did nothing. We upped the dose. Nothing. I was referred to a urologist. He had me pee in a cup to make sure I didn’t have any weird infections – this is not easy any more. You kinda need functioning hands to maneuver this, and remember this point. It becomes VERY important later. He then did an ultrasound on my bladder to make sure I was emptying it completely when I peed. I do. He shrugged and gave me samples for a new med to try. I did. They did nothing. He gave me another months’ supply in samples, and said he was previously going to do a couple of tests that day, but given my whole situation, he instead referred me to his colleague, who could do an ENTIRE workup. He said they’d call to schedule that; it’s an hour and a half appointment that involved probes and a scope up my urethra, soooooo be prepared for that I guess.
A week later, I get a call to make the appointment, and they send me a pamphlet of what to expect, and a sheet of instructions on how to prepare. The word “enema” is involved. Two enemas, exactly. One the night before, and one the morning of. The informational pamphlet says they’ll be sticking a scope into my bladder, to take a look, they’ll also be doing a flow test which means I sit on a commode and pee to determine…something, I guess. How fast I pee? And then they’ll be filling my bladder with sterile saline and stick a probe in both ends, and then have me do some tests like bearing down, and standing up. I guessed there would be puppy pads all over the room for that part. I was not looking forward to this, like, at ALL.
And so, the weekend before the appointment, I had to muster the courage to ask J to take me to the store so I could buy a freakin’ enema. He’s actually been amazing about listening to the really awful details of this whole bullshit Godzilla Disease, and took it in stride, and sympathized about the scope part cause he’d had that done. We bought what I needed, and then the night before the appointment, I set forth to do the thing.
OK. Here is where it is important to remember the part about “you kinda need functioning hands”. If you’re not familiar with an enema, it is essentially a flexible little bottle of saline with a thoughtfully, pre-lubricated plastic tip. You insert the thoughtfully pre-lubricated tip into your butt, squeeze the contents of the bottle in, and then wait for nature to do its thing. The bottle, for reference, is smaller than a standard soda bottle by a lot. It’s also full of water, which is heavy. Sooooooo don’t actually picture this, because gross, but…entertain a brief thought about what kind of difficulties a person with ALS may have in this situation. Especially when she is also overweight, and seated on a narrow, raised toilet seat with bars that prevent her from say, separating the knees as far as one might wish to get access to that business.
If your imagined, hypothetical scenario included dropping the bottle in the toilet more than once, and then ultimately only managing to squeeze maybe a third of it in? Congratulations. Now do that again in the morning.
The night before the appointment, after dealing with the ahem..effects..of the ordeal, I have a worrying thought. The clinic has more than one urology office location. There’s the one at the hospital in NW Portland, where I’ve been seeing this dude, but they ALSO have a location in NE Portland, where Dr. Goslin is. The urologist just mentioned a colleague. Same office? I scoured the paperwork I had, nothing had an address on it. It was Monday night at 10. I couldn’t call anyone. I checked the web portal for the clinic, and they had NOTHING about upcoming appointments anywhere. I could check every fucking thing else in my medical files with them, but nothing about an upcoming appointment. They hadn’t called with an appointment reminder.
I took my chances and went to the same clinic. And…yeah, you already guessed it was the wrong fucking hospital. Amazingly, they had another opening at the right hospital the very next week, so I made that appointment. After the month I’d been having, and the whole enema ordeal and the humiliation of that and the frustration of my hands just not fucking cooperating enough to do this, and the nightmare scenarios of thinking I’d ever have to ask someone for help with that, and paying $30 on a Lyft to the hospital and knowing I was going to have to spend another $30 to go home….I went into the hospital bathroom and sobbed a lot. Then I went home and took a nap.
The next week, I had better ideas about how to do the prep work. It went better, but also involved having to do the thing twice, since I could still only get a half dose in. The morning of, I had a complete incontinence issue and peed alllllllllll the fuck over m,y bedroom carpet and the bathroom tile and the bath mat and the toilet seat and everyfuckingwhere. And then after I cleaned it all up, I had to deal with the second dose of enema funtimes, and then got dressed and called my Lyft. It is 20 miles from my apartment to the hospital, mayyyyyybe 45 minutes with traffic. I left around 7:30 for an 8:30 appointment. There was a 25 minute traffic delay. I got to the hospital at 9AM. When I realized I was going to be 10 minutes late, to a 90 minute appointment, I thought about calling them to apologize but didn’t think I’d missed the appointment It was an hour and a half, and they always keep you waiting 10 minutes in the lobby anyway. When Waze bumped the arrival estimate to 8:45, I contemplated having him just turn around and take me back home. When the arrival time hit 9AM, I just kinda…turned off and knew I was showing up just to reschedule with the receptionist. I got to the office, explained what happened to the Eastern European lady, told her I realized at this point I was probably going to have to just reschedule the whole thing, and she looked at me like I was stupid.
“You have to PLAN for these things,” she told me like I was a child. “You can’t think traffic isn’t going to happen.”
“I planned a 15 minute buffer,” I told her. “It took 45 minutes longer than it should have.”
“You have to think about traffic,” she shook her head at me, exasperated. I guess she realized I was very, very close to tears, because then she said “I’ll see if they can get you in. But I don’t think so.”
Cue about 8 minutes of me leaning against the counter while she talked to the other office folk about how I seriously expected to be able to show up 30 minutes late and then a muffled conversation around the corner with the nurses, and I was JUST about to tap on the glass and remind her snarky bitch ass that I had ACTUALLY apologized and asked to fucking reschedule in the first place when I GOT THERE when she came back and said they could do PART of the appointment today, and maybe if the next person doesn’t show up for their appointment, we could get it all done.
We did not, in fact, get it all done.
Wanna guess which part we did not get done? The part that required the enema prep? OH WELL DONE YOU GET A PRIZE. We DID get the part done where they put numbing gel in my ladybits and then jammed a camera scope in there. I got to see the inside of my apparently healthy bladder, and even watched my kidney spit a blurble of pee into it. I did the pee-over-a-cup-on-a-scale test, too, and then sat in the room alone with no underwear on while we waited to see if the next guy showed up. When he did, I was ushered out to the lobby to reschedule without being given a chance to put said underwear back on, and then stood at the counter with a breeze up my dress while I waited for Ms. Thing to get me another appointment. At least she was equal opportunity bitch, because another dude came out of the office and needed some followup something, and she barked at him to just sit down and she would get to him in a minute. And then told me that she just couldn’t STAND when people just HOVERED like that. And then she tried to be sweet and called me darling when she found an appointment a month away “so much quicker than I thought for you” and made a point of scheduling me for the LATER time, and wrote my appointment time as 15 minutes earlier for arrival on the reminder card. Like…yeah, I GOT it, bitch. I WAS LATE. And then I went outside and got a Lyft home that cost $40, like the ride in, and I guess the worst part is how everyone assumes I have a magical support network for free that can help with enemas and free rides where I need to go so it’s just an inconvenience to THEIR asses when I get caught in traffic or they send me to the wrong fucking hospital in the first place.
Oh, and this was on Halloween.
So now, I get to wait a month, do enemas again, and in the meantime just continue peeing myself all the time because it’s not at all disruptive to my life? And then pay another $80 in Lyft fare to have probes shoved in me? And in the meantime, the urologist’s advice was to do some Kegels. IF I COULD CLENCH THOSE MUSCLES IN THE FIRST PLACE, LADY, WE WOULD NOT BE TALKING TODAY. DO YOU KNOW WHAT THE FUCK ALS EVEN IS. It’s when you try to use a muscle and CAN’T. BECAUSE THE MUSCLE IS GONE.
So yep. I’ve not been living my best life lately. This has been really hard. And humiliating. And a big fucking mess. In every sense of the word.
Next time I’ll tell you about clinic day and all that. This was a difficult post to write. I’m going to go look at some cat pictures or watch some jellyfish for awhile.
My cats knocked my depression meds into their water dish and I was completely unable to do anything about it, because it’s a heavy ceramic fountain. So not ONLY did they ruin half my monthly supply, they poisoned their water. Assholes. Insult to injury, it was the day after my friend Lizzie had come over and thoroughly cleaned the fountain out while she was helping me with cleaning the apartment (we love Lizzie a lot). She expressed dismay that she’d JUST cleaned the damn thing out, and I told her that it was okay, I’d strongarm J into helping me.
She replied in an email, “If you had strong arms, you wouldn’t have to ask J!”
And I laughed a lot.
She had replied in email instead of comment, because she wasn’t sure it was too far. It wasn’t. Gallows humor keeps me able to deal with this, and I realize that sometimes even my own jokes are ‘too far’ for some people – like recently when someone asked me how my new tattoo’s white ink was going to fade, and I told them I’d be dead before I had to worry about it.
Some day, someone will say something that goes too far. probably. Maybe. I dunno. I’m pretty fucking dark. It’s beyond gallows humor…guillotine humor? Firing squad humor? Saying it out loud a lot of times as a joke makes it easier to take it seriously. The concept of your own mortality is a bitter pill to swallow, so I need to wash it down with humor.
At least for as long as I’m able to swallow.
I have Clinic next Monday, and one of the things I need to talk to them about is maybe upping my depression/anxiety meds. I’ve been having severe bouts with SadBrain lately, and while I have hella circumstances that warrant being sad, I don’t like being crippled by ennui just because a cat video looked at me wrong.
Last night, even though I KNEW BETTER, I watched a documentary about coral on Netflix. I knew it was going to deal with the devastation on our reefs caused by climate change, but I love coral and wanted some beautiful imagery. I wasn’t disappointed on either front, and when one of the guys involved with the project started crying over the devastation of his beloved corals, so did I. And I cried for a long time.
And I thought, not for the first time and CERTAINLY not the last, that I’m a little bit glad I’ll be dead sooner than later. I don’t want to live on this planet anymore. And I honestly couldn’t tell if that was SadBrain or Cynicism talking.
Either way, both them bitches need to shut up.
..and the backlog of words waiting to be written backed up and I EXPLODED!
Okay, not really, but I’ve worked myself into that awful spot where updates are long overdue, but I can’t tell you about THAT because first I have to tell you about THIS, but it’s dependent on this OTHER thing for context, and I wanna talk about THIS but it needs to be a video but I really need to vlog about the cruise first, and then the wake…
And so for weeks I’ve posted nothing at all. Which is DUMB. So let me sum up some things, and then when I feel like I wanna say something, I can do that, and then fill in the back story as I can. The Cliff Notes version:
Clinic Days: Progressing as normal. Last time my breathing capacity was down a little, but it was still a strong normal. My hands continue to degrade. I made an appointment with Deb the Wicked Awesome PT who made me a Wolverine glove to hold my fingers up. I now have a wheelchair at home to get pushed around in.
Home search: Nothing. Despair.
Support Network: Lizzie is amazing and helps clean my place and I am VERY much enjoying the strengthened friendship that’s resulted out of the hangouts. She’s keen. Puce has become a freakin’ CHAMP-EE-UNNN in my life, to the point where he pushes me in my walker from the car to my desk every day. He’s amazing. Every dang day I am grateful for the people in my life who just kinda stepped into the roles I need, and I’m not at all sure what I did to deserve any of it.
Cruise: So much fun. You should do a cruise if you can.
Awake Wake: I literally don’t have the words. So many people, and so much love, and so much good food, and creativity, and hardly ANY crying, and SO MANY PEEPS OH MY GOD. My favorite part was sitting in the corner, watching all of my friends greet other mutual friends they haven’t seen in too long. It was the most uplifting thing I’ve ever experienced, and I’m so freaking grateful to everyone who came.
Vitamin shots: Don’t seem to be doing anything except make me pee pink, but I’m continuing them until next clinic day anyway.
Radicava: cautiously optimistic, but holy HELL is that expensive and complicated and..yeah. Every time I hear about it I think of Rikki-tikki-tavi.
Politics: Don’t even get me started. He wants to completely defund the ALS registry, which is the single most important tool we have to finding a cause and therefore a cure. I get angrier and more depressed with all of it every day, so I spend my days actively avoiding all of the news. It seeps in through my friends feed anyway, and I try to not be hateful and bitter. The world seems like a very ugly place right now, and I actively work to remain ignorant so that I can remain sane and functional. Bleh.
ALS Sucks: Someone else I knew with ALS died recently. I know his wife better than I knew him, and she’s an amazing person (seriously, caregivers are the unsung, underappreciated heroes of all time), but it brings the total number of people I know with ALS to….one. This is why I avoid the hell out of ALS forums. They’re seemingly all “EVERYTHING SUCKS” or “RIP So-andSo, who lost the fight with ALS today…” Meh. There’s only one cure for this disease, and it sucks.
Settling Affairs: Yeah, speaking of which I still need to finish that all up. It’s hard. I’m glad I don’t actually own anything of value.
Voice banking: Done! I have my digital voice and it is some serious Uncanny Valley stuff and I can’t wait to show it to you.
Work: I still have a job, I’m working from home two days a week now because it’s hard to do much of anything, and even getting out of bed and putting civilian clothes on and wrestling with myself to get in to work is a freakin’ challenge. But I still have to keep doing this because see: Home search.
So, that’s the quick (!) update. A lot. Realllllly need to post more. Soz. Soon. <3 I hope you're doing excellent things today.
One thing I love about being one of Dr. Goslin’s goslings is that she is super, wicked smart and stays on top of the latest research. Any time someone sends me an article about some new breakthrough or other, I am completely confident that she has already seen it and researched it to pieces. The upshot of this is that, when she suggests I try something, I know it’s a very well-considered proposal.
In our last Clinic Day, she told me about this article. Massive megadoses of B12 have been tentatively shown to maybe possibly potentially help with some of ALS’ stupid symptoms. She gave me the article, asked me to mull it over, and let her know if I’d like to try it.
Roadblock number one? Insurance won’t cover it. She told me it ran between $250 – $300. I could either get it in a vial with needles, or pre-filled needles, for more dollars. Now, I can manage to afford that now, while I’m unemployed, but when I’m not? Sucks to be me, if it works. I had me a nice angry meltdown on facebook about how stupid that is, sulked, and researched some more. My friends assured me that if it worked, they’d help me fundraise to get it, so don’t let that stop me.
Roadblock number two? Self. Administered. Intra. Muscular. Injections. Now, I’m tattooed, have multiple piercings, I am in NO fear of needles. But I didn’t know that I’d have the nerve to stab myself every day in the thigh. In the morning! And then there was the problems of mechanics, having enough hand control to push the plunger in. So, a mental AND physical challenge.
I decided to try; they recommend one month at least. I decided to go for it all three months until next Clinic, and I’ll likely still be employed all that time to afford it. I sent Dr. Goslin an email, she gave me the prescription, and thus began the Wacky Comedy of Errors. Holy crap.
First of all, only several pharmacies compound the stuff. For some perspective, the average over-the-counter supplement is 2.4 micrograms. This injection is 25 MILLIGRAMS. That is more than TEN THOUSAND TIMES the dose. Understandably, there’s a limited number of folks who make it that strong. So I had to *gulp* CALL A PHARMACY. IN PERSON. They got the prescription from Dr. Goslin. Then they called me to get my personal information. Then they called me back with pricing. A vial of it would run me $215, plus $30 shipping because it has to be kept refigerated and mailed cold. Oh. But they can’t ship it to Oregon; they’re not licensed to ship there, did I know someone in California or somewhere I could have them ship to, who could forward it to me? For another whatever-it-costs for overnight shipping to keep it cold? LUCKILY I have my dear friend Amanda in Vancouver, which is not so far away, and she was happy to recieve the package on my behalf AND dose it out into the syringes for me. Two days later, she got my vitamins.
With no syringes.
No big deal, she went to a pharmacy. …Who would not sell her any without a prescription. They gave her 4, though, so I could get started while I waited over the weekend for my doctor to send in a prescription. Doc Goslin was in a conference, turns out, so she turned the task over to her nurse, who mistook the instructions and sent in a scrip as though the injections were WEEKLY, so they only gave me 11. And to the wrong pharmacy, but that wasn’t her fault, DocGos didn’t tell her that part of my request. So I sent in an email to get it corrected and to the right pharmacy, only insurance now wouldn’t cover it because I was trying to fill the scrip too soon. You’d think the fucking things already have heroin in them, with how hard it’s been to get hold of some. FINALLY we’ve got it sorted and I can go pick up the rest of them tomorrow.
I started the shots a week ago. You have to keep it refrigerated, take the shot out of the fridge 20 minutes prior to administration, and keep it in a dark place while it waits, because B12 is light-sensitive. And THEN you may stab yourself. It took a couple of tries, mind you. 1mL is a LOT of liquid to get in there. The initial stab isn’t bad, unless I hit a nerve, but sometimes it takes some doing to get the plunger all the way in. And sometimes some of the liquid comes back out when I withdraw the needle, which sucks because it’s a blood red liquid that stains. I was warned that it gets metabolized quickly, and I’ll pass whatever doesn’t get readily absorbed, so my urine miiiiiight turn reddish or pink.
It totally does. So, thanks for the warning.
I haven’t noticed a difference in anything yet. I’m still experimenting with where to do the injection, as there’s not a LOT of muscle left in my thighs, and it’s blanketed with fat. Shooting my bicep though, feels like an immunization shot and leaves my arm sore all day. I’m sure it will get easier. It’s still taking one or two false starts before I manage to work up the nerve to stab myself though.
I’m not sure what I’m hoping for, with this. If it does something, then I’m tethered to $245 payments a month out of pocket. And eventually finding someone to stab me with a needle every day. If it doesn’t, then I’ll be out a lot of money with nothing but soiled cupcake band-aids and self inflicted puncture marks to show for it. I mean, of COURSE I’d like it to do something, even if it does mean weighing the pros and cons of perceived improvement vs. cost and hassle.
I guess I’m just saying, this disease complicates everything. At all times. For everyone. It really SHOULD come with a secretary and a kitten.
And someone to do the shots for me.
I’ve had depression most of my life. I’m really, really lucky in that it’s a super high functioning depression; most of the time I can still convince myself to Get Shit Done. I know many, many people who aren’t that lucky. Most days, I can get out of bed even though I don’t want to and my brain asks what is the point, even, and my anxiety tells me a million lies a day that I can usually push aside and do things anyway. A lot of folks with depression are like this; we’re not all like the commercials show you.
Some days though.
Some days it really IS like that. The days you call in sick because you literally just….can’t. The days you cry, the whole day, for little or no reason at all. When you spoon food in your mouth and it sits there, unchewed, for like five minutes. The days when your cat looking up at you and meowing (as he has a million times) is suddenly the worst thing ever and you just shake in frustration because you don’t know what to do. About the meowing, about standing in your kitchen, about being alive at all. And then you go to bed and the next day it’s fine, and it’s like you were possessed. If you’re lucky and female, sometimes you realize that the depression is PMS in disguise and somehow just knowing that takes the sting out. It’s temporary. It’s going to be okay, even if you don’t feel like it right now. Which of course is the same thing you tell yourself the OTHER days, too, but with nothing concrete to point at, you never believe yourself.
Depression and terminal diseases are tricky. Because you have a PERFECTLY legitimate reason to be sad, but you know in those slumps that it’s not why you’re crying. When they talk about your meds, and ask how you’re doing, of COURSE you’re low; you have a terminal fucking disease. Separating the mind disease from the physical disease becomes a very demanding and complicated thing, and of course you won’t get it right all the time. You don’t want to bump up the meds and become a zombie if your uptick is just cause you’re quite reasonably sad; it’s only for the sadness you can’t help, the depression that is there for no other reason than your chemistry is off and your brain hates you. The I-have-hella-circumstances depression can be medicated too, but I don’t like the idea of taking something all the time for something that’s legitimately situational and not just chemical. I like having an as-needed med for those times.
Wednesday was one of those times.
I think it was triggered Tuesday night; I found my newt dead in his tank. Now, the newts were always just above furniture, the same as a fishtank; they hated to be looked at, much less TOUCHED. They were low maintenance, you top off the water when it evaporates and toss in a couple of frozen bloodworm cubes once in awhile. I wasn’t particularly emotionally attached to these animals. The cats found them enchanting, I called it Newt TV and it was Molly’s favorite show. I always felt a little guilty for not getting more enjoyment out of them, surely there was some kid out there who would love these neat little pets more than I, but they were perfectly happy being completely ignored. They looked like pissed-off old men, and I named them after the old heckling muppets, and we coexisted. I was upset when Molly somehow pulled the screen off the tank and she either killed one of them outright or put it on the floor and it dried up and died outside of its tank; it seemed like it was an easily preventable death and I should have noticed he was missing from his tank before he had a chance to mummify in my living room. The last newt, I’m pretty sure died of natural causes – there was water in his tank and he’d CERTAINLY gone longer without being fed before – but I failed to notice until he’d had time to partially decompose in there. It was a warm week, probably didn’t take long for that to happen but I was still horrified with myself. Not guilty, he didn’t die because of neglect, just…I should have noticed that a living thing in my care was no longer living before then. I felt shaky and weird, horrified at his little corpse that I just couldn’t bring myself to fish out of the tank just yet, and went to bed after taking an Ativan.
Wednesday was work from home day. My stomach felt…off..so I called off the housecleaner. And then at some point during the day, sadbrain kicked me in the head. Everything was wrong. Work was frustrating and seemed hopeless. I checked Facebook to distract myself, but that turned out to be the absolute WORST thing, because not only were several friends having terrible things happening to them, but the world was full of screenshots of a dead black man bleeding in the street next to his car. And then I lost my shit. And cried and cried. And then went to sleep for a bit, and woke up crying, and everything was the worst. For the rest of the day, I couldn’t stop crying. The slightest thing set it off, and when you have ALS and the slightest things are stupidly difficult already, the world just seemed …too much. I had social obligations that night, and begged off instead, because I didn’t know if I’d ever stop crying. And then I watched television to distract myself, and HOLY SHIT WAS THAT A DUMB THING I DID.
OK. So. Something about me and my broken brain. This sounds stupid, but, welcome to how my personality disorder works. Look up Avoidant Personality DIsorder, and read all about my dumb brain. I have a really hard time watching new shows, because they’re an emotional risk. I just don’t know how they’re going to make me feel, so I have to be REALLY REALLY brave to try something new. I usually have some kind of an “in” – it’s recommended to me by a friend who knows about my broken brain, it’s by a writer whose work I trust, it’s so dang silly it couldn’t possibly be harmful. Otherwise I stick to ‘safe’ shows, like nature specials (Sir David Attenborough is legit one of my favorite people on the planet), cooking shows, How It’s Made.
So I picked this show that had just been added to Netflix:
Dream Knight (드림 나이트)
Alternate titles: 玩偶骑士
Starring Song Ha Yoon and Im Jae Bum (JB)
Though she’s constantly bullied, orphaned high schooler Joo In Hyeong (Song Ha Yoon) refuses to let life get her down and fills her little home with positive vibes from her favorite boy band. But fandom hits the next level when she discovers the ability to call upon four mysterious hotties (played by GOT7), who turn her world topsy-turvy with magical and hilarious antics, including JYP artist cameos. No matter how tough life gets, she’ll get by with a little help from her friends, especially with dreamy knights!
HOW COULD THAT HAVE GONE WRONG. I mean, it even had wacky sound effects and live-action cartoon antics. Only…she lives in a trailer because her mom died suddenly. Ok, I’ve seen anime like that before, that doesn’t HAVE to be depressing; it can lead to wacky misunderstandings involving four boys unsupervised in a single woman’s home. Classic harem anime formula. Four gorgeous guys show up, but they’re really magical dolls born from her tears of despair, here to make everything better! And what she wants most in life right now is to win a dance competition so she can dance with her favorite idol! Only she can’t really dance because she’s clumsy! THIS IS A COOKIE CUTTER FORMULA. Throw in the “oh noes, when her wish comes true the magical dolls will disappear!’ trope that ALWAYS FINDS A SOLUTION (hint: she falls in love and true love’s kiss saves him!) for good measure. Why not. Oh hey, loophole that if they kill her, they can remain human! O NOES (whatever, they totally won’t betray her).
Only she lives in the trailer because her aunt fucked her out of her mom’s fortune. Only she’s clumsy because she actually has myasthenia gravis! What’s that? OH ONLY A MOTHERFUCKING PARALLEL DISEASE TO ALS THAT CAUSES MUSCLE WEAKNESS AND EVENTUALLY PARALYSIS. No big deal, not fatal, right? Nothing to be upset about as a viewer? Oh, what’s that? Her disease is progressing quickly and she’ll be paralyzed within a year? Is that her and her knight finally falling in love even though the other knights have decided to betray her after all and she doesn’t know about any of this, including the fact that they’re not human? Is that her praying to her dead mother to give her the strength to dance really well, this one last time, with the man she loves? And then afterwards, she is going to break up with him to spare him a lifetime of taking care of a cripple? Oh, is this her winning the competition, everything is happy, wait a minute ARE YOU FUCKING KIDDING ME THEY ACTUALLY DO DISAPPEAR FOREVER AND THAT IS THE END OF YOUR SHOW YOU ASSHOLES.
After triggering a lot of ALS/terminal disease buttons, you’re not even going to give me a happy ending to your stupid boy band television live action cartoon?
ARE YOU FUCKING KIDDING ME.
….so yeah I cried until I nearly threw up, cried until I gave myself a migraine, called in sick the next day and cried that whole day too. Zootopia was released on Netflix, but I knew it was a not-even-bothering-to-veil-this analogy for race, and after sobbing in despair for a couple of hours about race relations ALREADY the previous day, I avoided that trigger. And just avoided the internet best as I could. And slept. And I don’t menstruate anymore so I couldn’t even lie to myself that it was temporary, and I thought about just not showing up to life ever again, and slept some more, and took more ativan in three days than I’ve taken in the last six months. And slept. And Friday came, and I was no longer crying, but so bone-tired that all I could do was sleep some more.
And the tricky part is looking back at that and trying to figure out what was Depression, and what was Disease. My feelings had a reason; their intensity did not, necessarily. Because I need to decipher what the situation really was, what were the triggers, in order that I might avoid them in the future and not lose three days of my life to crying and sleeping the next time. The dead man on my feed, that was obviously a real trigger, and there is most decidedly some very real buildup to that breaking point – you’ve read the news or failed to avoid it as much as I have. I had reason to cry over that. Maybe not so long. Friends’ issues that came up, I don’t know that there would have been tears to go with the empathy otherwise. Not sure. The frustration that my hands cramped up when I tried to eat something, real. Intensity, probably uncalled for. Etcetera. I have to unpack all of these things, examine them carefully, and put up traffic cones around the ones likely to make me slip again. There is certainly an element of the single straw that broke the camel’s back, here; a lot of kinda shitty things have been going on lately, a lot of micro-stresses, and the weight of the major ones combined, and the dam broke. I was way overdue for a cathartic cry. But not so hard, not so long.
ALS has added a layer of difficulty to this process. I can’t just shrug it off and say fuck it, I had a breakdown, maybe it’s time to try a new med. I’m paying much closer attention to all of this, for as much as I could easily play the “I’m Dying” card when I freak out and withdraw, I don’t WANT to unless it’s true. I don’t WANT to give myself permission to ignore causes and allow myself to drown in slumps like this without trying to figure out how to never do that again. My life is too short to allow whole days and weeks to be wasted if I can do something to avoid that. I quite literally…do not have time for this.
And if I’m being honest? Neither do you. Please look after your mental health, babies.
So! I had a follow up with Dr. Goslin about all the things that came up from clinic day, notably meds changes, follow up with the physical therapist, and a general check-in. I found that I had lost 5 pounds, which I am not at all unhappy about. She was unconcerned about that, telling me as usual that maintaining my weight is important; as long as I’m not dropping weight quickly it’s not a problem. Even though I miss being 60 pounds lighter, I suppose that will come in time, and that will be a problem. But until then I am fat and alive.
As far as the physical therapist goes, we didn’t find any braces that helped more than the ones I currently have. Most of them actually exacerbated the problems, particularly on inclines and declines. Instead we’re going to look into some sort of knee brace, as my knees are usually what fails when I fall. We’re thinking some sort of neoprene brace with metal supports; maybe that won’t make it quite so meat-stilty when I walk. We will see. Basically anything that keeps me walking as long as possible is a good thing, in my opinion. Ideally, also not tripping over the cats would be good.
Regarding the meds conversations, we had started me on something to help with the… er.. accidents… And something to may be kickstart the antidepressants again, as they didn’t seem to be doing a whole hell of a lot lately. Lab bladder control made is working out awesomely, we’re going to slightly increase the dosage on that. The other med doesn’t seem to be helping but it also doesn’t seem to be detrimental, so we are going to slightly increase the dosage on that. Maybe we’ll get a reaction. We are also increasing the dosage on Nuvigil, to see if we can’t kickstart my energy levels some more. In that same light, we are decreasing the gabapentin because I’m not sure it’s doing much of anything anymore, and it’s known to cause drowsiness, and I really hate taking something three times a day. Hopefully I can taper off that altogether. The antidepressant kick starter was also prescribed hopefully to reduce the migraines; I haven’t had one in a couple of months and I’m not sure if that’s working, or if it’s just that the Botox is no longer in my system.
Related to the headaches, we had a conversation – again – about the Cefaly device. When she had first talked to me about it, she had explained that she KNOWS that it looks like snake oil and seems super fake. (Seriously so fake.) The science she assured me, was sound and it had potential to be effective. Since then, she had actually tried the device and bought a few for her patients to try; a few of them reported up to 50% fewer migraines. It worked so well that none of her patients have returned it. So she wrote me a prescription for one, and almost $400 later, it should be arriving soon. The crappy part about it is that insurance will not cover it so this was out-of-pocket. Expect future updates when I finally get my hands on it. LIVE FROM THE FUTURE WITH MY SPACE HEADBAND OF MIGRAINES -2 !
We also spoke about medical trials. She hadn’t been able to attend the research symposium, so I told her about my conversation with Dr. Beckman and how frustrated he seems with his complete lack of progress. She shared my sense of dismay and agreed that an actual trial is probably not forthcoming anytime soon. HOWEVER, she told me, there was ANOTHER trial about to start. LITERALLY about to start. Like, in August. She was positively geeking out about the potential for this trial, super excited about the potential, and in fact said she felt BETTER about this than the copper trial. It’s centered on inflammatory behavior with ALS degeneration; previous trials had stopped the progression of ALS in patients with those inflammatory markers while they were taking this drug. Only 35 percents of ALS patients have those markers, though. It’s an IV administered drug, and a six-month trial; I think she said five visits for the first two months and three visits a month for the rest of the duration. I’m not exactly sure what it entails in detail, but I suppose I shall find out if I am selected for this trial. Because of course when she asked if I was interested, I said HELL YES. The trial coordinator is supposed to be giving me a call very soon to give me more information and arrange to have me tested to see if I have those markers.
So THAT’S exciting.
She also suggested, which in retrospect seems OBVIOUS, that I go to the physical therapist to learn how to fall gracefully, and how to get back up. It’s the getting back up that’s the problem, usually. So I’m going to go do that. Soon. In the meantime she gave me materials about various “med alert” type devices. Which, I realize I really SHOULD be looking into, but every time I think about it, the phrase “I’ve fallen and I can’t get up” also goes through my head. It’s one of those stupid associations, one of those steps that feels like surrender, no matter how practical and ultimately necessary it may be. I’m working on getting over that.
So that’s the haps. You are now fully updated. I’ll make a goofy video once I get the Cefaly. I’m sorry I haven’t been updating lately, the world those outside and inside has been in that sees stupid, dramatic, and sad. So I’ve been in full out capital avoidant mode, sitting in bed with my cats eating candy and watching nature TV. It’s so much easier.
I hope life is treating you kind. I hope you are safe, and happy.. And I hope it stays that way.