Since beginning my Spinraza journey over two years ago, I have been exposed to some pretty high-level scientific stuff. The Principles of Motor Neurons. Genetic Sequencing. Genetic Manipulation. And a whole bunch of really smart people geeking-out on how to turn cripply SMA people like me into X-Men.
People often ask me what it’s like, during this stage of my life, to describe what Spinraza has meant to me. What it has done for me. Most of the SMA patients out in the world are adolescents, children. Many are babies. After all, only a small portion of those with SMA make it into adulthood. So, those of us that are adults can give a perspective that children simply aren’t able to do. We have more life experience, and frankly, we’ve got the big-adult vocabulary to articulate what this is really like. We’ve got detailed analyses with really large words. SAT words. Oxford Dictionary words.
So, do you want to know what it’s really like?
You know the classic H.G. Wells novel, The Time Machine? The story that popularized the concept of time travel and launched an entire genre of science fiction? When Wells first published the book in 1895, it sparked the imagination of millions. Over a century later, the concept of time travel is still intoxicating.
Everyone (including you!) probably has a secret idea of what they’d do if they had the opportunity to travel in time. While most would do something selfish, like get winning numbers from a future Powerball drawing, some folks would do something more altruistic. Something that would benefit mankind… like killing Adolf Hitler or castrating Harvey Weinstein.
But, all these scenarios are fictional. Because time travel is impossible. Right?
Well it is… Unless you’re me.
If you really want to know what the Spinraza process has felt like to me— what is has meant to me— this is the way I describe it.
I am now a time traveler.
A time traveler that is revisiting a part of her life that she thought was gone forever.
Spinal Muscular Atrophy has always been a progressive condition. I was stronger at 5 than I was at 15. At 15, I was considerably stronger than I was at 35. And, by 55, there was a good chance that I would be dead.
But, with Spinraza, a crazy thing happened. The mad scientists at Stanford thrust me into their Interventional Radiology room— injected some liquid into my spinal fluid… and turned back time.
I began to live in own past. I could do things that I hadn’t been able to do in years. I was living in the past. Just like H.G. Wells wrote about 124 years ago. And, just like the novel, it’s been a wild, and frankly exhausting, ride.
It’s important to note that time travel isn’t free— and Spinraza is one of the most expensive drugs in the world. So, to continue to qualify, I must undergo thorough physical and respiratory evaluations every few months to measure my progress.
Given there aren’t many adults like me receiving the treatment, the data gained from our experiences is especially valuable. These measures and outcomes will hopefully offer the evidence needed to expand Spinraza access here in the US, and around the world. I want more time travelers with SMA. But, to do that, we’ve got to illustrate the benefits with cold, hard data.
No matter how you slice it, though, this added pressure is intense. While I’m constantly told not to be anxious about it— to simply do my best— I do worry about it. I fret about the messages I get from people with SMA around the world longing for treatment. I fear they won’t get to travel back to their past.
I feel guilty that I do.
Not all of us long to do something bold with a time machine. We don’t all want to be heroes that shoot Nazis. Some of us just want to travel to a time where we could talk a little louder… laugh a little harder… and pick our own boogers.
A few days ago, I had yet another full day of these assessments. It’s a day that I truly dread. Each time. It’s long, exhausting, and I usually always have at least one moment when I long to scream or curl into a corner and cry.
I care so much about these tests that it’s almost all-consuming. I do frequent physical therapy in preparation. I have even sought support from a counselor to work through the emotional challenges of this arduous process. These assessment exams are often all I can think about. Literally. All. I. Can. Think. About.
It’s like how Donald Trump must feel about Twitter.
I came into this assessment day— judgment day, frankly— feeling unsure. Feeling like perhaps I had plateaued. That I hadn’t noticed a change from the last time. I was resigned to this idea. I accepted it. It was okay (and expected) to have a plateau because SMA, by nature, is progressive. Stabilization is a win, too. Stabilization means more time available in the time machine.
But, I had a surprise. My respiratory measures showed additional small gains. My physical assessment was the most solid— yet.
The time machine was still working.
While I made it through this judgment day, in six months, I will have to do it all again. And again… and again. Because, in some way, time still manages to march on.
But, I’ll be waiting for it.
“Looking at these stars suddenly dwarfed my own troubles and all the gravities of terrestrial life. I thought of their unfathomable distance, and the slow inevitable drift of their movements out of the unknown past into the unknown future.”
— H.G. Wells, The Time Machine
I’m nearly two years into my Spinraza journey, so you’d think I’d be relaxed about the entire process. That I would have a ‘been-there-done-that’ attitude about the spinal injections, and the subsequent physical and respiratory evaluations that follow. That I’d be that chill kid that doesn’t get emotionally twisted into knots at the sight of the familiar 500g weight… or the large plastic mouthpiece that gets shoved under their lips to test their pulmonary capacities. That my vast experience would allow me to stay cool under these pressures. You know, like Robert Mueller when faced with a nation that can’t seem to read a 448 page document that they forced him to write in the first place?
But, no, I’m decidedly not that unshakable.
I had my 9th Spinraza injection several weeks ago, after recovering from a nasty bout of influenza. This illness took its toll, and I was worried about the effect it could have on the physical and respiratory assessment results to follow. So, in the days leading up to my assessment, I began to physically and emotionally prepare for the grueling day of tests. I doubled-up on my physical therapy. I increased my protein intake. And, most importantly, I talked to my counselor about breathing exercises to try to keep me from slipping into ‘lizard brain’ during the stressful moments of the day.
If you aren’t familiar with ‘lizard brain,’ please know that I’m not making this concept up. It’s not about a colony of salamanders on Animal Planet. Rather, the term refers to a frenzied state of mind that can occur when extreme stress causes all reasonable thought to vanish— and you are left with the instinctual urge to fight, or run away. This part of our brains is very elemental. Prehistoric. And it traces back into the beginnings of life. The ‘lizard brain’ hibernates inside all of us— but, if you are Donald Trump, it’s activated 87% of the time.
I am a naturally competitive person. As I’ve discussed on this blog, I often push myself to my limit. I want the A on the test. I want the gold star. I want to be able to say that I tried my damned best. When my ‘lizard brain’ is activated, my instinct is to fight. Generally speaking, this drive is a good thing. On these physical and respiratory assessments, it means that I give a lot of effort. If my brain calculates that there’s a 51% chance that I can lift that 500g weight, I have a hard time letting that task go. I want those points. I want to see them write it down on their damn little clipboard. My lizard brain can go from ‘off’ to ‘on’ in that exact moment. And it won’t back down until I’m red-faced, shaking— forcing the physical therapist to pry the 500g weight from my sweaty fingertips.
This isn’t necessarily a good thing. A reasonable, normal brain would realize that I shouldn’t waste energy that I will need for the rest of the assessment. But, ‘lizard brain’ makes unwise decisions. It does stuff that’s just not rational. This is why someone needs to remove the Twitter app from the president’s phone.
At Stanford this week, I really tried to keep that part of my brain contained. I came into evaluation day prepared. I was going to be focused. Methodical. I was energized— and ready. The lizard was going to be On. A. Leash.
I ate a good breakfast. My friend, Edith, brought me a Toffee Nut Latte from Starbucks to sip on the drive over to Stanford. It was a size grande. This is an important detail because I generally only order a size tall at Starbucks because my naturally high-strung personality can only handle so many shots of espresso. (This fact will become important as this story moves forward).
About halfway to Stanford Neuroscience, I got the sudden urge to listen to rap music. I never, ever listen to rap music because my taste in music tends to be more similar to a 92-year-old Jewish man than the Millennial hipster that I actually am. But, it felt like I needed to hear that music in that moment. As I started belting out the lyrics to Petey Pablo’s Freek-A-Leek, Edith grew concerned:
“How much of that latte have you drank?”
“Uhm, only like a third of it so far. But, I feel really good about how this day is going to go.”
The music continued. As we approached the bridge into Palo Alto, I started counting overpriced Tesla cars that I’d see on the freeway. They were easy to spot with the pretentious ‘T’ on their trunk. I briefly pondered if the company’s founder Elon Musk liked rap music. Then, I remembered that I needed to worry about my lizard brain, so I did a few breathing exercises and counted fourteen more Teslas. Then, I felt a hot flush run through me:
“Ooo, can you open the window? I want to feel the bay’s cool air on my face on the bridge.”
“Did you finish your drink?”
“Nope. Only half so far. But, it’s really yummy. The Toffee Nut Latte is almost as good as the Chestnut Praline Latte. You know, it’s probably not as good because instead of two nuts in it, it only has one nut. Like Lance Armstrong.”
We parked in the garage, and as the cool air hit my skin, I realized that, for some reason, I was already sweating.
“Fuck, I’m sweating.”
“I should have got you a decaf.”
“Look, my undershirt is stuck to me.”
After checking in to the reception area and drinking some water to hydrate, I waved at my doctor’s nurse practitioner across the foyer. When she came over and hugged me hello, I hoped she couldn’t smell my sweaty and toffee-nutted body.
I was taken back into the exam room and my vitals were taken. When my blood pressure was within the normal range, I was shocked. Seriously surprised. So, my lizard brain decided that it was a good idea for me to drink the rest of my latte.
The respiratory therapist came in a did a full pulmonary function test. Right away, I could tell that all my hard work during my influenza recovery, and afterward, was paying off. The Spinraza was doing its job. The numbers with each respiratory test were coming in higher than before. I was on a respiratory and caffeine high. My lizard brain was secretly tweeting:
“This is the most huge thing ever. I’m the best pulmonary test-taker in history.”
After I caught my breath from those tests, I finished my latte. As my straw was slurping the last toffee bits on the bottom of the cup, the physical therapist came in for the physical assessment tests. With the caffeine pumping in my blood, I felt especially focused. All the world fell away until it was just me and that test. I watched her hand making notations on her clipboard. I started to mentally tabulate points like a card-counter at a blackjack table in Vegas. I was going to bust the dealer. She was the dealer. I would not be stupid enough to take the bust card.
“If I lift this cup this way, will I get more points?”
Five minutes later.
“I know I only get three tries to do the task successfully and get my points. But, if I don’t set the item down, it still counts as one try, right?”
I don’t know if the caffeine was doing it, or all the preparation I had done, but I felt a zing of excitement as, one by one, the tasks came easier than I had expected. My mental tally was telling me that my score was higher than last assessment. And the therapist agreed:
“You’re doing really well. I think you’re ready to try an additional task we haven’t done before: putting you on the end of a table to see if you can sit upright all on your own. Without any support.”
“Will I get more points for that?”
“Then let’s do it!”
I’d like to report that I did get those points. I did sit on the edge of that table all on my own. First, I did it bracing on my own hands. Then, I did it without the support of my hands for even more extra bonus points. It was an amazing moment. Edith even cried a little because it’s a task we’d never thought I’d be able to do.
I’d like to say that this achievement was emotional for me in that moment, too. That this milestone was the culmination of two years of treatment that paid off in a tearful crescendo.
But, it didn’t happen in that way. And you know why it didn’t? Because my lizard brain was too busy shouting:
“GIVE ME ALL THE POINTS!”
It was still all worth it, though.
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.”
Given that I’ve had a disability my entire life, you’d think that I’d be fully-immersed in all medical-related things. But, that’s just not true. For example, despite my love for WebMD and Wikipedia, I’m not familiar with every pharmaceutical brand on the market. I don’t know the best medication for type 2 diabetes or psoriatic arthritis. But, from the commercials that I’ve seen on television, each of the available medications have potential side effects that include temporary blindness, liver damage, suicidal thoughts and/or heart failure. It’s an uplifting assortment of options.
Anyway, while I do know a lot about stuff related to Spinal Muscular Atrophy (SMA), there’s a lot of other things that I don’t know. Stuff that, as an outsider, you’d think would be an everyday part of my life.
Like physical therapy.
This statement might come as a bit of a surprise. You might be thinking, “You mean the crippled girl doesn’t go to physical therapy every day? What a slacker! How does she expect to get better if she doesn’t do that??”
Here’s the thing: until I began Spinraza treatments last year, the expectation that I could maintain or improve my strength was pretty much a non-starter. An impossibility. A don’t-even-dream-about-it kind of thing. You know, like thinking that Kanye West will ever not be super weird?
This isn’t to say my physical therapists in the 1980s didn’t try their damndest to make this not be so. Defying the laws and biology of SMA, my childhood therapist pushed and shoved me into standing boxes to straighten my curving back. They placed heavy, painful weights on my legs and hips to try to reverse my irreversible muscle contractures. They tried to push my body into doing things that it just simply couldn’t do. They weren’t intending to be cruel, but their understanding of SMA at this time just wasn’t very advanced. They pushed more than they should have. They expected more from me than I could physically deliver. They were doing what they thought was best, though. But, these good intentions didn’t mean that this period of my life still wasn’t an utter hell on earth.
My confidence took a beating at this time, as well. It was hard not to feel that way when my inability to continue to participate in these physical therapy sessions was seen as a result of my laziness– and not the natural progression of my disability. The fault was with my motivation and my drive. Not the fact that it was an excruciating torment to endure… with no progress to show for it. In fact, the opposite happened. I progressed anyway. As all of us with SMA have done. It’s biology. Not laziness.
It’s perhaps not surprising that I eventually became withdrawn during these physical therapy sessions… and then increasingly uncooperative. Finally, it got to the point where during designated therapy time, I would hide in corners of the elementary school so the therapist couldn’t find me.
It was the biggest act of rebellion I had ever pursued up until that point. And it was also a moment in my life when I felt the most unheard.
Those gut-clenching moments remain with me to this day. I’ll always be that anxious little girl in the school physical therapy room, even though it was over twenty years ago.
So, it is with the baggage of those memories that I’ve recently reentered the world of physical therapy. My revolutionary Spinraza treatments have led to some small improvements in my strength and stamina. Which, given the progressiveness of SMA, is fucking awesome.
As I’ve written here before, I’ve been doing some informal exercises at home since beginning treatment. And it’s been very helpful. But, my Stanford medical team advised me that it was time to take it up a notch. So, I was referred to a local physical therapist to develop a custom exercise plan.
Yesterday was my very first consultation. I’m not gonna pretend that I wasn’t nervous. That I wasn’t worried that the physical therapist was going to push me too far beyond my ability because he didn’t have an understanding of SMA (like my old therapist all those years ago). Or, that I wasn’t dreading the whole experience… the way Trump dreads a wind tunnel.
So, what did I do? I did what any self-respecting Type A person like myself would do. I obsessively prepared for my appointment by typing up an information sheet for the therapist about me, about SMA, and why I was there in the first place. I also harassed the receptionist with warning calls that I would perhaps be a high-maintenance patient. Not like the everyday car accident victims and torn rotator cuff patients that they saw everyday. I would be a whole bundle of crippleness. The person that, when presented with a new patient form, would circle the ENTIRE BODY as an indicator of where I’m having trouble.
I’m sure my neuroses were quite evident in my voice on the phone. And I’m certain that this left an impression on the poor receptionist because when I rolled into the office, she instantly called me by my first name— despite the fact we had never met.
I filled out more medical history forms. And I had to resist adding smart-ass remarks in the margins. How could I not answer the question “How long have you had this issue?” with a sarcastic reference to the Reagan Administration?
I only have so much willpower, after all.
When the physical therapist, Robbie, came over a few minutes later, though, he was remarkably calm. And, despite my fears, he didn’t look like a deer in the headlights when faced with probably the most disabled patient he had ever encountered.
I wasn’t a truck driver with a case of whiplash. I was a whole-lotta-weird with muscles that behaved in ways you wouldn’t expect. (Come to think of it, also kinda like Kanye West.)
Robbie quickly admitted that he had never encountered SMA before. (Most medical professionals actually haven’t– this is not unusual.) But, that fact didn’t stop a gleam of excitement from entering behind his eyes. I recognized the same look in his gaze that I would get back in 1990 when I got a brand new set of Legos. The look that Ina Garten still gets when you flop a freshly-caught, raw sea bass on her kitchen table.
The look that says: “Oh, boy! This is way more fun than a hip replacement!”
I’m happy to report that he asked all the right questions… he was engaged and interested. And incredibly collaborative. It was just the experience that I had been hoping it would be.
I was being heard.
And, for that, I was so very grateful.
For the time being, I’ll be heading to physical therapy once a week, so stay tuned for more updates on my progress. I can’t believe I’m saying this, but I’m strangely looking forward to it!
I do wish there was a way that I could have told that to my ten-year-old self, though. That the experience didn’t have to be the way I endured it… and that it could have been much, much better.
Maybe then I wouldn’t have had a reason to hide.
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!”
Tearing a folded piece of paper is not something that most people put much thought into. In fact, folks probably do it all the time without thinking of the physical effort that such a motion takes. Especially if it’s thick computer paper— the fancy kind that you can only buy at an office store. The tangible, professional-grade that big banks, mega-corporations and white-collar criminals use right before fucking over a bunch of middle-class homeowners. Or stealing the identities of poor old people that don’t know that Windows isn’t just something that you cover with drapes.
For those of us with SMA, tearing a folded piece of paper may actually be hard… if not impossible. Prior to beginning my Spinraza treatments, it was a task that I had not been able to do in a very long time. Not even the thinner type of paper that you buy at the dollar store. The kind they sell next to the cheap neon highlighters that smell like meth.
But, this ability is tested during the very-important PT assessments that measure my progress with Spinraza. While it seems an odd thing to test, it’s actually a good measure of hand strength and changes in grip. I’ve had two assessments so far, and I could not complete this particular task on either try— which royally pissed me off. As I’ve demonstrated before, I’m not the kind of person that does well with failure. If there’s an exam, I had better get an A. And if I don’t, I will not be happy about it and I will work myself into a damn tizzy to score better the next time. If you know me at all, you’ll understand that this is not an exaggeration. In fact, you’ve probably also worried that at some point I’m going to give myself an ulcer.
Next month, I will undergo another full PT assessment, which means that I will be confronted with that piece of paper. And, I really don’t want to fail that task once again. I don’t want to be a sad loser like the Mets or Hillary Clinton. So, yesterday, I began to practice this task. Fiendishly. Surprisingly, after about seven minutes, I achieved victory. I tore that damned piece of paper in half. And then, about twelve minutes later, I did it again. I was so happy that it didn’t seem to matter that I was sweating through my Secret Powder Fresh deodorant.
Today, in the time it took to watch two episodes of Comedians in Cars Getting Coffee, I tore a piece of paper FIVE FUCKING TIMES. If you don’t believe me, here’s a picture of the paper:
If you’re wondering how long it actually took me in real-time (not Netflix-time), it was about 30 minutes. So, roughly six minutes per tear in the paper. Although, I did two of the tears in less than 30 seconds— which, interestingly enough, is the same duration of President Trump’s attention-span.
I’ve got several more weeks to prepare for my next assessment, so wish me luck. Maybe, if I keep at it, I will no longer be the world’s slowest confetti-maker.
A girl can dream…