Getting sick when you have SMA is not a fun experience, as I’ve discussed previously on numerous occasions. The tiniest cold can turn our lungs into a swamp of mucus, and recovering from, even such a minor illness, can take longer than a steamship voyage across the Atlantic. And that’s assuming there are no icebergs lurking in the water that could sink the ship— or, worse, yet, send us to the hospital for IV antibiotics. It’s a dangerous ocean out there, even without German U-Boat torpedoes.
So, it’s easy to imagine that if the illness is more than a cold— things can become serious very quickly. Recently, I was threatened with such an iceberg. A Titanic-sinking hunk of Influenza— the most dreaded of respiratory viruses. The little bastard that attacks during the night, overwhelms your defenses, and turns Leonardo DiCaprio into a blue icicle.
As soon as it hit, I knew exactly what it was— and the danger it posed to me. I had prepared for this moment, and didn’t take it lightly. Keeping my lungs clear was my top priority. I would sacrifice sleep and rest (the two things most needed when you are ill), just to keep those airways pliable and open. I would do anything to get the mucus out. Literally anything.
So, I leapt into action. I pulled out all my respiratory devices. CoughAssist. BiPAP. Nebulizer. And I started my emergency influenza protocols. Tamiflu. Zithromax. Prednisone. Albuterol. I had trained well for this moment. I was like those Paris firefighters that protected the Notre-Dame Cathedral. I was not going to let the whole structure fall apart. Sure, I knew my roof was made of really old wood covered in medieval pigeon shit. That just meant I was going to have to work extra hard to save the good bits of the building. But, it was definitely worth saving. If cranky French revolutionaries couldn’t defeat me in 1789, I wasn’t going to let a fire do it. Mon Dieu!
The following days passed in an exhausting whirlwind of respiratory care. Given the complexity of SMA, we can most often receive better care at home than in the hospital. You might find this surprising, but most local medical professionals have little to zero experience with a patient with a rare neuromuscular disability, like SMA. So, hospitals can be very dangerous for us. We have learned this the hard way. So, we have to become our own advocates and our own resources. And our doctors become our partners and colleagues… we teach them sometimes more than they can teach us.
It wasn’t long before my bedroom began to resemble a respiratory care unit. And I personally resembled the little green Mucinex monster from the commercials on TV. I was green, gross and contagious. Frankly, I was my own worst nightmare.
And, I was exhausted. Coughing and keeping my lungs clear became a full-time job. The kind of job with lots of overtime and no weekends off. The really, really sucky kind. In fact, I was so exhausted that I took to blending my food so that I wouldn’t have to waste energy eating and chewing. Every ounce of my strength was devoted to respiratory care and I needed to maintain the nutrition and calories to do it. Yes, I really was that committed. And, yes, blended food actually tastes exactly the same as non-blended food. For example, a blended Chile Relleno still tastes exactly like a non-blended Chile Relleno. (Unfortunately, it does look like baby poop, though.)
As my efforts slowly yielded positive results, this didn’t stop the mental toll from weighing heavily on me. The anguishing rotating schedule of BiPAP, CoughAssist and Nebulizer— in between drinking my meals and trying to rest— was relentless. There wasn’t even much time for Netflix, which says a lot because I can always find time for Netflix.
Emotionally, I struggled. I cried, even (which wasn’t wise because it made me even more snotty and gross). I had nightmares about really scary things. Like going to the hospital… being put on a breathing tracheotomy that took away my ability to speak… and, worst of all, Donald Trump tweeting even more than he already does.
After two weeks, I began to see the light. I could go stretches of time without coughing. My doctor noted that my lungs were improving. I was eating solid food again. I stopped dreaming that Robert Mueller was kidnapped by Boris and Natasha.
Things were looking up.
And then, last week, I made it to Stanford to receive my 9th injection of Spinraza. It was just the boost that I needed. A physical boost, but perhaps even more importantly, an emotional win.
In the days since, I feel my strength slowly returning. It will take time for me to get back to my pre-iceberg self, but I just need to be patient.
Wish me luck!
(PS: If you need to find me, I’ll probably be watching Netflix.)
Every few months, as part of my Spinraza journey, I go to Stanford Neuroscience for a full day of physical and respiratory assessment tests to measure my progress— and my overall health. These tests are important for research purposes and to learn more about Spinal Muscular Atrophy and how it may respond to these revolutionary new treatments, like Spinraza. I’m fortunate to have an outstanding medical team at Stanford and they are really “writing the book” on SMA treatment protocols for adults. In the past, there wasn’t much that could be done to help the underlining medical cause of SMA, but science is currently progressing more than I’d ever thought I’d witness in my lifetime. So, it’s an exciting time to have an obscure condition that’s caused by a few fucked-up genes.
People finally seem to have the know-how to address the complicated science behind it. And it’s not theoretical. It’s not science fiction. It’s real— unlike many of the things that we spend so much time worrying about these days. Fake things. Like a zombie apocalypse… Alien abductions… Donald Trump’s tan.
Despite all of these advancements in science, and my excitement to be a part of it all, this doesn’t mean that I don’t get tired of being a cog in the large medical machine. That I don’t secretly (or not so secretly) dread these periodic assessments I must endure. Because I do. I dread it a great deal. Just like I dread how my ass will feel when I’ve eaten too many tacos.
As I’ve shared on this blog, I always get stressed and anxious before these tests. Even though I know much of this pressure is self-inflicted, there’s still the very-real fact that my results, compiled with the results of others, will impact the ability of other SMA adults in this country, and around the world, to have continued access to Spinraza, and future treatments that are coming down the pipeline.
I worry about that. A lot. Because I know that as a result of the high cost of these “orphan drugs,” payers and insurance companies are looking for any reason to limit who gets access to the treatments. Even though the pool of those of us with SMA is already so small, they’d still like to make that tiny pool even smaller. When scientific advancement meets economics, economics wins every time.
Insurance and pharmaceutical companies are already in negotiations about the next batch of SMA treatments and their potential costs. They are calculating the cost of an SMA life. The value of an SMA life. They are using terms in their reports like “quality-adjusted-life-year.” And “value-to-healthcare-systems.” And “how-to-keep-saying-these-mercenary-things-without-sounding-like-assholes.”
Access to these treatments is being evaluated on cost, not on who may benefit. Not on the value of the life itself. The looming pressure of this is very real. And, it cuts to the very heart of one central question: What is a disabled life worth—like mine?
Because, in most common societal measures, a disabled life is not worth as much as an able-bodied one. Society reinforces this view every day. And it has for many, many years. It’s been done in subtle ways. Like government policies, lack of community inclusion and accessibility, and in forms of everyday discrimination (which is called ableism). Unfortunately, some of these reminders are actually not-so-subtle.
In fact, sometimes, they slap you in the face.
It wasn’t long after I began writing about my Spinraza journey on this blog, that I received a comment from an anonymous reader. This gentleman lashed out at me for having the audacity and selfishness to think that my life was worth the cost of my treatment. That, because I’m disabled, my life isn’t worth it. That my contributions aren’t worth the added cost.
His ramblings took a nonsensical turn as the message progressed, so I was able to dismiss it and not internalize it as others may have done. But, his overt message spoke to the “unspoken” belief that many have about disability. He said the thing that many may believe, but don’t have the guts to say. After all, this idea is deeply woven in society. It’s why ableism is one of the most common, yet least talked about, forms of discrimination.
All of these thoughts weigh on me with each assessment that I endure. Each weight that I lift. Each paper that I tear. And each measurement that is taken.
In those moments, it suddenly becomes the time for me to “prove my worth.”
My Stanford team has kindly attempted to dissuade me from these anxieties before my assessments. They’ve urged me to not put this pressure on myself. Part of me completely agrees with them. It’s ridiculous to carry such a burden. Just like it’s ridiculous to believe it’s possible to die in a zombie apocalypse.
Yet, despite these assurances, given the society we live in, with its “quality-adjusted life years” being used to measure the cost of my life, and all the outside forces attempting to devalue lives like mine, how could I feel any differently?
It’s really hard not to do so. But, damn it, anyway, I have to try to shed that burden. To push it off my back. Dismiss it. You know, the way a dictator ignores human rights laws? (I’ll let you decide which dictator I’m talking about. There are so many to chose from.)
So, it was with all this in mind, that I rolled into the Neuroscience building yesterday. It was dark and dreary outside, and as the rain threatened to fall, I felt some of the wind come out of my sails. I would just do the best I could. And that’s it. I just needed to keep reminding myself of that.
Four hours later, I was feeling exhausted and ready to head home. I was spent. I had been measured, and then measured again. I was able to lift a small weight and move it in a way I hadn’t been able to do before. I couldn’t tear the paper, though, because unfortunately it was done near the end of the assessment when I had begun to tire. Normally, this would have really annoyed me, but I managed to contain myself. I listened to that new voice in the back of my head that said, “Drop the burden, girl. Just do your best.”
And so I did.
I’ve made it no secret that I am abhorrently afraid of cold & flu season. When you have SMA and your respiratory system is total shit, even a simple cold can turn nasty. You know the way a mother’s ear may alert to the sound of a crying child? My ears instead perk to the sound of a cough, a sneeze, and the tiniest sniffle. I can even detect the slightest change in the tenor of a person’s voice that may indicate secret congestion. I don’t mean to brag (okay, maybe I do), but I am the Sherlock Holmes of Cootie-Detectors. I’m so damn good at it that I may know you are getting sick before you do.
When I was scheduled for my 8th dose of Spinraza, I knew that I was entering a dangerous portion of the cootie-season. The post-Thanksgiving/pre-Christmas cornucopia of Microbial Abundance. So, I knew that this year I was going to have to employ every germ-avoiding tactic in my arsenal so that I could receive my Spinraza treatment on-schedule and unimpeded.
Thus, about 10 days before my injection, I announced to EVERYONE THAT I KNEW that I was going into quarantine. Literally everyone. Even the mailman knew that he had better not sneeze on my Christmas cards.
I became a certifiable hermit. I turned down invitations to holiday gatherings. I avoided crowds and eating food that wasn’t prepared at home. I threw away all my romaine lettuce.
The more isolated I became, the more I felt myself turning into one of those wingnuts that sits in their house and angrily begins to believe conspiracy theories that they read on the internet. That the Moon Landing was a hoax… that Barack Obama was born in Kenya… that Donald Trump’s hair is actually grown on his head.
The bitterness became real. And the further I got into my self-imposed exile, the more my anxiety grew about someone breaching my quarantine and infecting me. I knew that if that happened, I was truly inches away from turning into the actual Unabomber. Like I would send a bomb to that person’s house and curse their familial line for 46 generations.
As my procedure day approached, I became more and more tense. Xanax wasn’t quite covering the heights of my nervousness. My left hand began to twitch. Hallmark Christmas movies even stopped having their sedative effect on me.
I simply COULD. NOT. GET. SICK.
It was a horrendous amount of pressure. It consumed me. So, when the magical day arrived, and I woke up at 3:00am feeling okay, I nearly cried with relief.
And then I remembered that my reward was having a five-inch needle poked into my spine.
Strangely, this thought didn’t scare me as much as getting sick did. I think that speaks volumes to the depths of my emotional neuroses… and how bad Hallmark Christmas movies really are.
By the time I arrived to the Neuroscience Center, I was actually calmer than I had been in days. After being settled in my room before the procedure, the resident doctor performing the ‘needle poke’ came to introduce himself. As it’s a teaching hospital, there are different teams of doctors— one resident & one attending. You never know who you will have until the day of the procedure. It’s like playing roulette… but with your spinal canal.
Right away, I had a good feeling about the new doctor. He seemed competent and excited— which jived with my mood of the day. I also sensed his competitive nature when he asked:
“So… uhh…how long did it take them to do this procedure last time?”
While this may seem an innocent question, as a competitive person, myself, I instantly recognized the subtext to his question. What he really meant was the following:
“I want to do this better and faster than last time. I will win this game.”
And, I must say, the guy did deliver. He had the needle in my back so quickly, that I didn’t have time to begin daydreaming about lunch (my favorite way to pass the time). The whole procedure was done in thirty minutes.
As he removed the giant needle, the panicked mania of the past days all seemed worth it to have Spinraza floating in my cerebral spinal fluid like magic minions. But, as exciting as it was, I was ready to go home. To get the hell out of there. I was spent. It had been a tough week trying to not turn into a domestic terrorist.
Just as I was about to get ready to leave, though, a research fellow came into the room with a clipboard. He cheerfully asked, “I’ve got a few questions for you.”
A little voice in my head whimpered, “Oh, fuck.”
A clipboard is never a good sign. If there are enough papers to warrant a clipboard to hold them, that’s too many papers.
I gazed longingly at my wheelchair and wished I was sitting in it instead of the hospital bed. It would be so much easier to zoom away from this man and his clipboard. But, then my conscience reared up and decided that I needed to be the scientifically-responsible person and submit to the questions.
This was a big mistake.
The questions went on. And on. And on.
He asked me questions that I had already answered on previous appointments. Questions that should have already been in their records.
I began to resent his perky face and his clipboard. And his inability to read my medical records. My mind began to race— does anyone take the time to read anything anymore?? Do they??
The minutes ticked by. Finally, he said, “Well, I think that’s it.”
I sighed with relief and gazed at the clock. His survey had literally taken longer than my entire procedure.
Paper rustled on the clipboard, “Oh, wait, I’m missing a page!”
If I could have physically banged my head on the wall in that moment, I would have done so.
“Just a second…”
He located the paper and rattled off a few more questions. I would totally tell you what the questions were but, honestly, I wasn’t even listening by this point. He was like the teacher in Charlie Brown— all sounds, but no actual words.
When he finally went away, my nurse came back in, “My goodness, I thought that was never going to end.”
I burst out, “I know, right??? Holy crap.”
She clapped her hands together, “Let’s get you out of here, shall we?”
“Yes, please. I’ve got some Hallmark Christmas movies to watch.”
Since I’ve begun my Spinraza journey, I undergo periodic assessments that measure my muscle and respiratory function. These assessments are quite exhaustive and make even the SATs look easy— you know, like one of those “personality quizzes” that you find online? The ones that tell you whether your personality resembles a baked potato or a goat cheese flatbread?
Anyway, these assessments can be brutal. Every tiniest movement and tiniest muscle is measured. And then measured again. This thoroughness isn’t just meant to drive a person loony (which it can), it’s for scientific purposes. Mainly to see how this crazy-expensive treatment is actually working.
I’ve seen measurable improvements since beginning treatment, that’s indisputable. This is amazing given the progressive nature of Spinal Muscular Atrophy. After all, merely slowing or halting progression is also a substantial goal. So, seeing an improvement is a bonus.
But, with the need for these assessments comes a unintended side effect for the patient— stress, worry and anxiety. When you’ve fought long and hard to receive the drug (while many wait all around the world to have access to it), you’re terribly frightened of having it taken away. There’s an unspoken need to prove yourself during these assessments. To prove that you are worth this expensive treatment. To prove that all of us with SMA are worth it. That’s a lot of pressure. Especially to a person like me that is already competitive. A person like me that breaks out into hives if they aren’t scoring in the 90th percentile.
My outstanding medical team has attempted to calm these fears and anxieties. They’ve told me to not put pressure on myself. But, I know that the data from assessments, like mine, are being compiled into a study that will be used to potentially help (or hinder) adults with SMA around the world in their fight for treatment. So, I know it does matter. Despite how much they may try to allay my anxieties.
Recently, I headed to Stanford for another assessment. Like a prizefighter, I had been prepping for weeks beforehand. There were a few tasks that I had been unable to perform in my prior assessments that I was determined to master this time around. Mainly, tearing a folded piece of paper and opening a small plastic container with a snug lid.
The looming nature of those tasks were swimming around in my mind in the waiting room as I nibbled on my turkey sandwich.
Please let me tear that fucking piece of paper. I’m gonna be so pissed if I can’t do it.
What if I get performance anxiety? There’s no Viagra for paper-tearing.
Ugh, what if I can’t open that container, either?? Dude, if that happens I’m gonna run over the damn thing with my wheelchair. Just see if I won’t.
This internal dialogue continued in my mind until, thankfully, the physical therapist came to take me for my first assessment. In the PT room, all the assorted gizmos were laid out on a table. At the sight of the tiny weights, containers and charts, my heart started to beat faster— making me exceedingly glad that I had taken a tiny Xanax thirty minutes before (in between bites of my aforementioned turkey sandwich).
But, before the therapist could give the first instruction, I blurted out: “I’m really nervous about this. I even took a Xanax a few minutes ago. I’ve been practicing opening a container and tearing a folded piece of paper at home. I really, really want points for those tasks.”
The therapist arched an amused eyebrow, “Oh, I definitely remember how you feel about getting points.”
During my last assessment, I nearly held her hostage until she allowed me one more chance to earn the one point I was determined to get. It wasn’t my proudest moment. There might have even been tears (not sure if they were hers or mine, though). It’s a good thing she has a good sense of humor. Otherwise, I think I would have seemed pathetic.
“It’s going to be fine. You really shouldn’t worry—”
I interrupted her, “I brought with me a stack of paper that I’ve already torn at home as evidence that I can actually do it. It’s in my backpack if you’d like to see it.”
Laughter pulled at the corner of her mouth, “Uhm… yeah…” She paused and looked at me as if she was suddenly REALLY glad that I did take that Xanax, after all. “I’ll just pretend you didn’t say that.”
“Are you sure? I mean, I can totally show you—”
“No, no. Let’s just not.”
And so, the assessment began. My range of motion was checked. I was asked to squeeze and poke various technical devices— each measuring the strength in various muscles of my arms and hands. I was asked to lift coins and weights of various sizes. I was asked to pull a rabbit out of a hat. (Okay, I might have made that last one up.)
Things were going really smoothly when she grabbed the dreaded plastic container. “Okay, now it’s time for this,” she waved it in front of me as if the motion could magically stop the sweat from collecting in my palms.
I grabbed it. Yanked. Pulled. Strained.
And nothing happened. I began to panic—the litany of anxiety returning to pound into my head like the clanging of a 1950s typewriter.
Sensing my emotions, the therapist took the container and set it on the table in front of me, “How about we come back to that?”
She then folded a piece of paper and handed it to me to tear in half. My attention was still so focused on the plastic container that I had the paper torn halfway down the middle before I realized that I had actually done it.
My heart raced in joy as I finished tearing the paper. Then, I slammed the two halves down on the table like Rocky Balboa.
“Ha!” My cry was loud and victorious. Sylvester Stallone could kiss my butt. (Actually… Eww, no. Maybe not. He’s old and he’s taken way too many steroids over the years.)
Feeling more confident, I demanded, “Give me that container back so I can try again!”
And you know what?
Nothing fucking happened.
I still couldn’t open it. My mood plummeted as I watched the physical therapist write a “0” on the assessment for the task of opening the container.
My inner soul cried, “NOOOOOOOOO!”
I hadn’t been this upset since the original DuckTales animated series was cancelled back in 1990. I was despondent. Even though I had torn the paper, it wasn’t enough. I wanted that container. I needed that container.
The assessment was over. As the physical therapist walked away briefly, I glanced once more time at that damn piece of plastic. With my sorrow, I felt all the muscles in my body relax.
I was defeated. With a sigh, I absently grabbed the container. And, before I knew it, I had removed the lid.
My mouth dropped open in shock. I had done it.
But, the physical therapist was on the other side of the large therapy room. She didn’t see me do it. It was like scoring a perfect 10. Only not at the Olympics where it truly mattered.
I sputtered, “OH MY GOD, GET OVER HERE NOW! LOOK AT THIS!”
When the therapist returned, I quickly did it again. I was suddenly a Ziploc Plastic Container Opening Machine.
“I’m going to get points for this, right? Right?” I knew I sounded frantic, but I didn’t really care.
She chuckled, “Yes, you will.”
I wasn’t fully satisfied. Not after all I had put myself through. “While I’m not saying that I don’t trust you, I’d really like to see you write it down. Right here in front of my face where I can see you do it.”
“If it will make you feel better?”
“Oh, it will. I promise you.”
She grabbed her pen and changed the “0” to a “1.” It was the most beautiful number I had ever seen.
“Ha!” My cry was loud and victorious.
Maybe Rocky could kiss my ass, after all.
I had my 7th dose of Spinraza this week. You’d think I’d be a pro at this by now— but, unfortunately, receiving a complex lumbar puncture isn’t something you can train for… unlike a marathon or a really competitive game of backgammon. I actually don’t even know how to play backgammon, but it sounds like something that sophisticated people would do. Like playing bridge. Or committing white collar crimes.
Anyway, when you have a wonky and complex anatomy like mine, each injection is its own story. Its own event. A prior injection can’t, in any way, predict the outcome of the next. Just because one injection was easy, that doesn’t mean that the next can’t be a fucking disaster. I’m sure if you’re the parent of a toddler you will completely understand this concept. After all, while your child may behave on one trip to Target, that doesn’t mean that the next time he won’t pull an entire display of Febreze on top of his head.
As I shared on this blog previously, my last injection was a challenge. So, I went into this treatment with open eyes, and the reasonable expectation that there could be copious tears at some point in the day. (Just hopefully not the doctor’s.)
Anyway, the drive to Palo Alto was decidedly uneventful— the sky shifting from Central Valley smoke and haze, to a Bay Area overcast. But, as we got off the bridge and arrived to the Neuroscience Center, the morning sun opened up and a cool, slightly crisp (dare I say, even fall-like?!?) air met my skin. If you know me, you’d know how much I love the autumn. So, I tried not to let this omen get my hopes up for how the day would turn out. I told myself, “Elizabette, calm down. There’s still plenty of time for you to be the kid with the Febreze on their head.”
While waiting for the procedure, a research associate came and asked me if they could save the spinal fluid they collect from me for scientific purposes. I clearly like science, so I signed the paper and said YES. After all, it’s not like I am going to take my spinal fluid home and put it in a jar by my bed. That would be creepy. Besides, I have no room for it on my nightstand, anyway. That’s where I keep all my photos of George Clooney.
By this point, I really started to have to pee. As I have to hydrate a lot in the days before the injection, my bladder was holding a crap ton of urine. When I say that, I’m actually not kidding. Lucky for me, I think I was given the bladder of a much larger primate. Like a gorilla. Or Tom Hanks from A League of Their Own. You know that scene in the movie where he takes a drunken piss in the locker room urinal? And his pee goes on for over a minute? Yeah, I can totally do that.
Anyway, I was secretly hoping that the research associate would hurry up and go away so that I could get to the point of the morning where I got to use the restroom. If she didn’t skedaddle when she did, I would have probably signed away my own pancreas just to get her to leave. I think I could have found a way to make due without it.
Thankfully, it didn’t come to that, so I was able to go empty my mega-bladder in fairly short order before being taken back to the procedure room. After I was laid on the table, on my left side, the doctors began taking measurements on my back— to try to locate the ONE open spot in my spine. The only part that isn’t obstructed by the two scoliosis rods nestled along my vertebrae. They fired quick x-rays to line up their needle approach.
Watching the big fluoroscopy machine as it swiveled and swung around me, gathering images, and listening to the doctors strategize on their game plan, I started to feel a rumble in my stomach. Even though it was only 10am, lunch was beginning to sound really good to me.
Thinking about food is one of my favorite things to do. In fact, I daresay I like it even more than pumpkin spice lattes and making jokes about Donald Trump. And that should say a lot since I like those things VERY much.
It shouldn’t come as a surprise that, at this point, my mind began to wander. So consumed by the thought of food, I didn’t notice the giant needle slide into my spine and the sting of the lidocaine that numbed the passage. Instead, I imagined the In N Out Burger that I suddenly wanted to eat. And the stack of smothered “animal style” fries that I would get along the side. Over the scent of hospital antiseptic, I could taste that damn special sauce that I love so much.
It wasn’t long before I started drooling on the sterile pillow. I wish I could say that I made this last bit up, but I didn’t. I literally left a puddle on the light blue fabric. It was kind of gross.
Anyway, so distracted by my thoughts, I gave a jolt when the needle penetrated into my spinal canal and a zing of cold sensation flew down my right hip. Immediately afterward, the attending doctor proudly announced, “Here it is” as my spinal fluid dribbled out of the needle— evidence of the procedure’s success. They collected some fluid (for their aforementioned research purposes) and then injected in the Spinraza.
Seconds later, the procedure was done and the needle was out. Dazed by the speed with which it had all happened and still in a haze of pickles and grilled onions, I asked, “Wait, how long have I been here??”
The nurse laughed, “Only about 20 minutes. This is a record time!”
I was thrilled. I was definitely NOT going to be the kid with Febreze on their head this time. My stomach grumbled loudly, again, sensing that lunchtime was suddenly going to be a whole lot sooner than usual.
They helped me off the procedure table, and as they wheeled my bed back to my room, the nurse asked, “So, what are you going to do with the rest of your day?”
It was the question I had been waiting to answer.
“I’m going to In N Out!”