One year ago, today, I had my very first spinal injection of Spinraza— the first-ever FDA approved treatment for my disability, Spinal Muscular Atrophy (SMA). Life was a lot different one year ago. First of all, I was worth a hell of a lot less money at that point. My spinal fluid didn’t have 6 vials of super-sonic, super-expensive Spinraza floating around inside of it. You know, the way the miniaturized Dennis Quaid floated through Martin Short’s body in the 80s movie, Innerspace? One year ago, I was a body that was decidedly pre-bionic. Dennis Quaid’s tiny spaceship would not fly out of my nose if I sneezed. Now, however, I wouldn’t be surprised if my boogers had diamonds inside. Yes, I’m that pricey now.
As I have shared here previously, it was a long, hard-fought battle to gain access to this drug, and I’m lucky to have a spectacular medical team at Stanford Neuroscience that helped to make this possible. I wish I could say that all adults with SMA have such outstanding advocates for care as I do. But, we still have a long way to go to make this current treatment, and all the upcoming treatments coming down the pharmaceutical pipeline, available and accessible to all those living with my rare, genetic condition.
But, my Spinraza journey didn’t end at the point of that lumbar puncture needle one year ago today. Rather, it really had just begun. Given the complexities of getting the long needle through my crooked, and fused anatomy, each injection since that July day has been a tiny battle of wills. A mental and physical game where I prepare like a seasoned warrior. A soldier that knows that the upcoming battle could be a smooth victory just as easily as it could be a giant shit show. You know, like a Trump/Putin press conference?
However, these hardships (and there have been many!) have been worth it. In the 365 days since that magic vial’s liquid have begun to do their work, I have had measurable improvements. Given that this neuromuscular disability is progressive, even merely slowing or halting the natural deterioration is a victory. To have improvements, like I have seen, is more than I could have hoped to achieve. Especially as an adult with SMA. I had never thought I’d live to see a treatment that could help me. It’s hard to mentally process… to put your brain around. You know, just like it’s hard to process pickle-flavored ice cream, self-driving cars, and why the hell we Americans can’t figure out the metric system.
I look forward to what the future holds for my Spinraza journey, yet, I eagerly anticipate what medical science has in-store for those of us, of all ages, with SMA. I’ve heard that there are more treatments currently in the trial and research phase. Perhaps, one day, I will have additional cause to celebrate.
Until then, if I sneeze, please excuse the diamonds.
For more updates, don’t forget to subscribe to my blog in the sidebar.
Some Mondays can exist on their own cosmic plane. An alternate reality where weird stuff happens more frequently than other days of the week. It’s a day for hangovers, short tempers, and trying to get all the things done that you should have done over the weekend — if you hadn’t been rewatching the latest season of The Crown while still wearing your pajamas. By the way, Princess Margaret would have definitely approved of pajama-wearing after 2pm. Her sister, the Queen, however? Not so much.
Earlier this week, I had one of those Mondays. It was the day of my 6th injection of Spinraza and I went into it primed, pumped, and ready. But, the signs presented fairly early on that day that things were gonna be just a little bit weird— like a Kanye West interview.
The drive to Stanford is always arduous— and traffic-ridden. With the number of cars and trucks that are trying to push through the freeways from the Central Valley to the Bay Area, it’s like trying to pass a rump roast through a shower drain.
It always amazes me that so many folks make this long commute on a daily basis. It boggles my mind. I’d have a serious mental breakdown if I had to do that. The kind that would make me unable to enjoy the mythical 4,000 square-foot suburban house that I could maybe afford, but never have time to live in.
On this particular Monday, the traffic, surprisingly, wasn’t too bad— meaning that it was only moderately heinous. You know, like rice cakes or gender reveal parties for unborn babies. It was tolerable, but not something you’d voluntarily go out and do.
Anyway, despite the flowing traffic, the mood of the drivers was decidedly grim. And, frequently, downright hostile. Dozens of horns were honked. Many cars were aggressively passed. And a slew of motorcycles were cutting off cars at each opportunity. There was more tension on that freeway than in the last episode of The Bachelor.
A case of the Mondays was in full-form.
We arrived to the Neuroscience building earlier than expected (shockingly!!), and the nurses took me back to the room to prepare for my lumbar puncture. It wasn’t long before one of the doctors came to go over the last details of the procedure. Given that Stanford is a teaching facility, they work in pairs— an attending (teacher) with a fellow (student). There’s also no way of knowing which doctors will be on duty on a particular day, either.
Having a lumbar puncture is always a tricky business, but when you have complex anatomy, like me, it’s even more precise. I lay on my side and they use a fluoroscopy machine (like an x-ray) to monitor their progress as they move the five-inch needle around my spinal rods and into the small space in my vertebrae to access my spinal fluid— where the Spinraza must be injected. It’s like a playing a game of darts in a really sterile bar— only the target is me, and I’m awake and not a cork board.
Given this complexity, there are lots of factors that can determine how quickly and easily the procedure will go. The experience of the doctor. My position on the table. And, frankly, a good amount of luck.
The fellow (student) worked the needle into position in my spine and all seemed okay… but, the spinal fluid wouldn’t drip out the needle (how you verify that you are actually in the right spot).
Remember when we talked about the Mondays? Yeah, well, it wasn’t finished with me, yet.
The fellow readjusted the needle, back and forth. In and out. Making small centimeter-sized adjustments to try to yield the spinal fluid. But, it WOULDN’T FUCKING COME OUT.
Meanwhile, with each move of the needle, tiny nerve pains were boomeranging around my back and hip. They even tried tilting the table so that gravity might help the fluid to dribble out.
But, no, it didn’t work. Isaac Newton’s Law of Gravity was a piece of shit. I don’t care what they teach us in physics class— it doesn’t always work. Especially in the alternate reality that is a Monday.
After this went on for a while, the attending doctor (the teacher), pushed aside the fellow (the student) and proceeded to give it a go himself. Frankly, if Isaac Newton had been in the procedure room in that moment, the attending doctor might have kicked him in his 17th century balls. A few minutes later, though, he was finally able to get it done. Gravity be damned.
I was so relieved. And so were the sore muscles in my shoulder, and the nerves in my hip and back. I daresay even both doctors were relieved.
About an hour later, I was back in my wheelchair and ready to load up in the car for the ride home. Just as we were opening the doors to my van, another vehicle with a disabled placard began aggressively revving their engine behind us, trying to hurry us into leaving the parking spot so they could take it themselves. It reminded me of all those angry folks on the road from earlier in the morning.
Then, a few seconds later, the driver rudely waved at us— as if hand gestures were like spells from Harry Potter that could magically make me load up into my van, strap me (and my wheelchair) securely inside, all in five seconds.
My friend, Edith, that had accompanied me on the trip, raised an eyebrow as I drove my wheelchair up my van’s ramp, “I think we need to make this car-loading-up thing take much longer than usual. What do you think?”
I grinned, “Oh, yes.”
No one likes tests. Whether they’re in school, at the doctor, or at the DMV— they are generally un-fun. You rarely hear someone yell, “Yay, a test! I’m SO happy.” If a person did say that, you’d probably question their mental stability.
For a lot of folks, tests bring out an anxiety— a stress to perform well, which, ironically, is made harder by the stress itself. It’s a terrible Catch-22.
I have to do well on this test or I’ll never go to college!
I have to pass this exam or I can’t get my license!
Will that marijuana I smoked a month ago show up on this urine test!?
As a worrywart, high-achieving student, I generally would experience some anxiety before tests, especially the big exams— like the AP test, the LSAT, and all those personality tests on the internet. I’d fret for days beforehand, wondering how it would all turn out. Would I score well enough on the LSAT to get into law school?… Would the online test sort me into Hufflepuff or, worse yet, Slytherin House?!
These thoughts would consume me.
It shouldn’t be surprising that when it was time for me to have another evaluation to check my progress on Spinraza, I worried about it. A lot.
While I had felt positive changes, and experienced measurable improvements previously, would it still translate to results this time?
It was a question that loomed over me… like the Hindenburg right before it exploded.
I’m sure reading this, you’re probably thinking, “Girl, calm down. Don’t stress. Just do the best you can.”
I wish it were that simple. Given the high price tag associated with nusinersen treatments, there are many insurances and government agencies that are looking to limit who has access to the drug. They want to put parameters on who can get it and who can’t. And a major factor they are looking at is age.
As an adult in my thirties with Spinal Muscular Atrophy, I am considered old. Not old in the way that Betty White is old, but at least old in a moderate way… like Jane Fonda or Donald Trump.
While there aren’t THAT many of us that have lived this long with SMA, there are still plenty of us adults out there that need access to this drug. So, we have to continue to prove that this treatment works for adults. That it produces results.
That’s a lot of pressure. Especially for something that a person can only do SO much about. I can do stretching, breathing exercises, and increase my protein to help things along, but that’s about it. I mainly have to see if the magical Spinraza droplets do their work.
Leading up to my evaluation at Stanford earlier this week, I was very anxious about it. On the drive over, I listened to the Spinraza mixed CD I had made and tried to gear myself up. It worked pretty well… after all, track #2, Eye of the Tiger, is always a solid choice.
Upon arrival to the Neuroscience Center, I only had time to inhale half of a tuna sandwich before they called me back to begin my evaluation. The next three SOLID hours passed in a blur of respiratory and physical therapists, nurses, research assistants, and stress sweat (good thing I put on extra deodorant!).
I wasn’t finished with one test before another person was hovering nearby to begin the next. I didn’t even have time to eat my homemade graham cracker and peanut butter sandwiches. (And you know how much I love peanut butter!)
The grueling afternoon reached its peak when the physical therapist asked me to open up one of those clear round Ziploc containers with the blue lid. Previously, I hadn’t even been able to attempt this task. Not even close. But, this time, I felt that I might be able to do it. I pulled, groaned, heaved, and nearly cried. But, after five minutes of desperately trying (and nearly doing it), I ran out of steam. I felt defeated. And pissed off. I told the PT, “I’m gonna buy one of these fucking containers and practice this at home. Next time, I will do this.”
Yeah, I’m that kind of person.
While that moment was very disheartening, I’m happy to say that my results showed some improvements. I was able to lift a cup with a weight inside all the way up to my mouth. The strength in my arms and hands increased. And, lastly, but most awesomely, there’s a respiratory test that measures the diaphragm muscle. Before Spinraza, I got a 50. At this evaluation, I got a 72.
By the time all of this was done, I was exhausted. I wanted to curl up in bed with hot chocolate and watch TV forever. All the shows. Even the stupid ones on Bravo… Like The Real Housewives of Beverly Hills.
Thankfully, I get a little break now. I don’t have to head back to Stanford until next month for dose #6. I’m looking forward to the respite… and the break from all these tests.
Although, if I get bored, I’m sure there is a personality test online I can find. Like… If you were a dog, what breed would you be?…
A border collie. Definitely.
For more updates, don’t forget to subscribe to my blog in the sidebar!
It’s been a little over a month since my 5th injection (first maintenance dose) of Spinraza. As I was getting over a respiratory virus at the time of the injection, it took a little longer to feel the effects of this latest dose. But, about 10 days ago, I felt a little zing… the burst of feeling when my three SMN2 genes decide to be mini versions of The Hulk— turning from nerdy Mark Ruffalo into a green, CGI shirtless monster.
The muscles in my arms and torso were more responsive and almost… tingly. I often feel the same way if I drink too much red wine— only this time I didn’t have a purple-stained mouth as a memento.
I noticed new abilities. In the winter months, my feet and legs are always cold. So, when I get into bed at night, I have to use a heating pad to warm them up. To stop a person from scalding themselves or setting their bed on fire, my particular heating pad as an “auto-off” feature that activates after about 45 minutes. This is exceedingly annoying. While I’m appreciative of the consideration for my safety, it takes me longer than 45 minutes to warm up. So, I have to press the button on the cord to turn the heating pad back on again.
The past few years, I’ve had a hard time reaching the cord and pressing the button. But, last week, I noticed that I was able to grab the cord more easily, and to press the button more firmly. My icy toes were super stoked by this development.
I also grew hungry again — similar to what I felt at the beginning of my Spinraza journey. I wanted to eat. And I specifically wanted protein. Meat, beans, yogurt, eggs— and oh-so-much peanut butter. I would have slathered peanut butter on a steak if my inner foodie hadn’t cried out in horror, “You aren’t a kookie pregnant sidekick in a romantic comedy! No one wants to see you put Skippy on a filet mignon!”
This burst of energy coincided with the arrival of the Winter Olympics. If you know me at all, you’d know that I’m a die-hard fan of the Olympics. It doesn’t matter if it’s the summer or the winter games, I love it all. I watch it ALL DAY. And this isn’t hyperbole. From dawn until dusk, that’s what I do. My life practically stops. I’m like Donald Trump with his Twitter account. Nothing else of any importance happens in my life.
So, this week, I’ve been glued to the TV. I’m not sure if it’s because of the endless hours staring at the LCD screen while listening to the Olympic music, or all the extra protein grams floating around in my body, but I’ve started having delusional thoughts.
“What is wrong with that figure skater? Landing a quad jump can’t be that hard.”
“Every Norwegian baby comes out of their mother’s uterus wearing tiny skis.”
“I bet with just two or three more years of Spinraza, I could totally do Olympic Curling.”
Now, this doesn’t make any sense. And it has no basis in reality. But, this doesn’t mean that I didn’t think it.
Perhaps it’s a good thing that the Olympics only come around every couple of years. These delusions aren’t good for me. Frankly, if they continue much longer, I might become convinced of something truly crazy. You know, like that North Korea is a magical place where a man named Kim Jong Un gives hot fudge sundaes to everyone that comes to visit.
Unfortunately (but, secretly, amazingly!), my friend Joahn sent me this Olympic scarf two days ago in the mail— which has only fueled my obsession. I wear it around the house while I watch the Olympics and eat hummus. If you look close enough, you might see crumbs on it.
I think I’m a lost cause.
For more updates, don’t forget to subscribe to my blog in the sidebar!
It’s been well-documented that, like many with Spinal Muscular Atrophy, I’m a verifiable germaphobe. If a person could earn a certification in Cootie-Evasive-Techniques, I’d have a damn Class A license. The kind that people stand in line at the DMV for 3 hours to get.
Strike that. There’s no way in hell that I could go to the DMV and stand in line for three hours. I once heard someone cough up their gallbladder at the DMV. There are enough germs in those offices to keep the Centers for Disease Control busy for at least 6 or 7 months. Eww.
Anyway, if you’ve been following my Spinraza journey, you’d know that the date for my first maintenance dose had been fast approaching. Given the importance of these spinal injections, and the set schedule of receiving them, it’s vital that nothing interfere with a dose.
Not even a plague of locusts, a doomsday asteroid or a Buy One Get One Free sale at the GAP.
Seriously. I don’t care how much I love those long-sleeve tees.
Given that we’re in the midst of a raging cold & flu season, I’d been paranoid that I would catch a bug prior to my Spinraza treatment. This fear paralyzed me for weeks. I avoided going out in public places, I tried limiting my interactions with family and friends. I basically became a hermit. Like the Unabomber. Only I didn’t try to build explosives out of fertilizer, chicken wire and cherry-flavored bubblegum. (Yes, I remember the show MacGyver.)
But, then, Christmas happened.
And, I really, really like Christmas.
You can probably guess what happened next.
Yup— I caught a respiratory virus.
The symptoms started about 10 days before my Spinraza injection. It wasn’t long before I had a river of fluid coming out of my nose. I’m sorry to be so graphic, but I have very little filter when it comes to these things.
After consuming my first box of Kleenex, I began to notice something. I felt a burst of joy— and not all of it was because of my low-grade fever.
You see, I was able to blow my nose harder than I had been able to do for a very, very long time. Like years. At least since the Bush Administration (the 2nd dude, not the 1st dude). I was able to blow so hard into the Kleenex that my ears actually popped.
This may not seem like a big deal, but before Spinraza, I couldn’t do this. Nasal drainage would simply slither down into my lungs, and I would struggle for weeks to get all the damn stuff out. This was a striking difference.
This development invigorated me. I wasn’t going to let this piece-of-shit virus get in the way of my Spinraza treatment. After all, I clearly needed more of the stuff so I could keep ejecting all those boogers out of my nose.
So, armed with antibiotics, breathing treatments, and a fuck-ton of garlic (seriously, I smelled SO bad), I made it through that week to Spinraza Day.
The early morning arrived and we were greeted with the first rainy day in, like, months. So much rain hit California that all the dirt decided to just turn into rivers and carry folks away. Not cool.
Luckily for me, even though the visibility was dodgy at times, we slowly made our way over to Stanford— safely. Upon arriving, I began to get nervous.
Could I sit still during the procedure without having to cough or blow my nose? After all, moving or twitching while two doctors inject a giant needle into your spinal fluid really doesn’t sound like a good idea. Just like buying sushi from the back of a van isn’t a good idea.
After checking-in and getting settled into the room, the nurse told me that the Spinraza Gods had blessed me once again. The same amazing duo that did my last procedure were back for the day. It felt like the rainy heavens had opened up and a damn Puccini opera was playing just for me.
In case you’re wondering why I was so excited, here’s the deal: there’s no way to know which doctors will be on-call that day. Stanford is a teaching hospital, so the rotations are random and unexpected. The time this procedure can take varies widely— depending upon the doctors. The longer the procedure takes, the more painful and arduous it becomes.
And these two doctors didn’t disappoint. They had the needle in so quickly, that I didn’t even have time to cough or blow my nose. It was glorious.
The rainstorm continued on the drive home, but I was so delirious with relief that I didn’t even mind. I was exhausted. Spent. Relieved. And, yes… slightly full of snot.
I get a little break before my injection, so I will enjoy these months— hopefully without viral invaders.
Wish me luck!
For more updates on my progress, don’t forget to subscribe to my blog in the sidebar.
One year ago, the folks at Biogen and Ionis dropped a festive holiday morsel that created a firestorm in the neuromuscular community. They had marketed and developed the first-ever treatment for Spinal Muscular Atrophy… and the FDA had approved its use for all ages and types of the rare, genetic condition. It was like Santa Claus had dropped a major bag of loot down the chimneys of families and individuals with SMA.
If you’ve been following my blog this past year, you’d know that I am one of the lucky ones blessed with these really fucked up genes. Since I lack copies of the SMN1 gene, my body doesn’t produce enough of a specific protein that allows for muscle growth and maintenance– hence, why I have Spinal Muscular Atrophy. Instead, my body has 3 copies of an alternative gene, called SMN2, which can produce very limited amounts of this essential protein. These backup genes are called ‘backup’ for a reason— they aren’t very good at their job. They work at a slow, meager, and inconsistent rate. Like politicians in Washington D.C.
Yet, these backup genes are why I am currently still alive… and why I didn’t meet St. Peter as a toddler sometime during the Reagan administration. Yet, I am one of the fortunate ones… this shitty, progressive disease has taken many young infants, children, and adults over the years— including my own brother as a baby.
This is why the development of Spinraza (nusinersen) was so revolutionary. Science had finally offered a treatment for what had previously been untreatable. You know those sad, pathetic backup genes I mentioned earlier? Well, Spinraza targets those SMN2 genes and BAM! tricks them into producing more protein. It’s like giving them steroids— only it won’t result in a shrunken penis… or expulsion from the upcoming Winter Olympic Games (yes, I’m talking about you, Russia!).
It’s important to note that Spinraza isn’t a cure. But, when you live your life knowing that with each year that goes by, you’ll get weaker, even the prospect of stability and maintenance of strength is a victory. That could add years to our lives and make everyday just a little easier— you know, just like GPS and the Swiffer Mop.
I began my Spinraza journey soon after the announcement of its FDA approval. Yet, it still took months to navigate all the hurdles to qualify for this treatment. It was a stressful, hopeful time— like the gestation of a baby… or waiting for a poop to come out of your butt after you’ve been constipated. But, in July, the magic day finally came— my very first injection at Stanford Neuroscience.
Very quickly after that first treatment, I began to feel little changes and improvements. Neck and torso muscles tightened… My voice grew louder, more robust… cuss words were easier to shout— I could even trail them together with appropriate adverbs. It was awesome.
My grip and range of motion in my hands improved. And, when I had my follow-up evaluation after my 4 loading doses, the numbers did show that these improvements weren’t just all in my head. (Even though a great many other things are in my head!)
As I’m an adult in my thirties, my results will never be as dramatic as those currently being seen in younger kids and teens. But, as my neuromuscular specialist, the estimable Dr. John W. Day at Stanford, told me, “Our goal with adults like you is to halt progression of the disease. With SMA, that is a victory. Anything above simple stabilization is icing on the cake!”
I head back to Stanford in a few weeks for my first maintenance dose of Spinraza. I feel fortunate to be able to receive this treatment. More fortunate than many realize. After all, there are many, many others with SMA (both here in the US, and around the world) that have not been able to do so.
Given the specialized nature of this treatment, the exhaustive research that went into it, and the limited number of folks with SMA, the price for the drug is very high. The drug companies have to recuperate their expenses and make some kind of profit— otherwise research into rare diseases, like mine, won’t ever happen. And, as we all know, money makes the world go ‘round.
The price tag for the first year’s doses of Spinraza, at $750,000, more closely resembles that of a really large house… or the salary of a mediocre NFL player. (Unlike NFL Commissioner, Roger Goodell, who has somehow convinced people to pay him hundreds of millions of dollars for doing nothing more than making Americans spend their time and money on a sport. A game where grown men wear stretchy pants and slam into each other until they get too many concussions and eventually have to retire to eat soup through a straw.)
Anyway, the high price of Spinraza has caused American insurance companies, and international government health organizations, to limit access to the treatment. They are using a variety of parameters to reduce the numbers of eligible recipients— including age, SMA Type, SMN2 gene copy number, and hair color. (Okay, I might have made that last one up.)
But, the more folks they deny, the more money they save.
This has been devastating to families and individuals with Spinal Muscular Atrophy that have been unable to receive Spinraza. To know that this drug exists (the only treatment available), and to be unable to get it, is a mindfuck of epic proportions. And, with each day that goes by, these individuals will get weaker. And some of them will die.
As we mark Spinraza’s anniversary, I am left with a couple final questions…
What is the value of a life? What is the value of a life… like mine?
I don’t know the answers to those questions… and I’m not sure if they should even be answered. But, plenty of bureaucrats seem to be doing that right now.
And many folks with SMA are falling short of the price.
Earlier this week, I made the journey over to Stanford for my post-Spinraza-loading-doses evaluation. The neuromuscular team wanted to check my progress after beginning treatment. They are closely monitoring every aspect of my condition for their records— and to prove to insurance companies and other doctors around the world that, yes, Spinraza works on adults, too (not just kids). That way the insurance companies can stop being discriminatory, money-grubbing, ageist fuckheads so doctors can do their jobs and TREAT THEIR PATIENTS!
Whew, sorry. I got a little worked up there. Usually, I only get this riled up when Starbucks is out of caramel sauce… or I see motorcycles cutting people off between lanes in traffic… or I have to listen to Donald Trump speaking words together in clusters (i.e. sentences).
Anyway, at the beginning of the entire Spinraza process in February, I had an entire battery of tests. I saw physical therapists, respiratory therapists, occupational therapists— basically every kind of therapist that exists, except for the psychiatric kind. Which was unfortunate, because considering how drawn-out and stressful this whole thing would end up being, perhaps seeing a psychiatrist at the outset wouldn’t have been a bad idea. Maybe then I wouldn’t have needed as much Xanax, Netflix, or chocolate fudge ice cream.
They measured everything that could possibly be measured. The strength of my muscles and lungs. The flexibility of my joints and limbs. My fine motor skills. My ability to do long division. And if I knew the difference between their/they’re/there.
Okay, I might have made those last two up.
But, I think they should have tested that.
Anyway, this week, I had to repeat all those benchmarks again. As I am a high-achiever, obsessive-type, I had begun prepping for these tests at home. If there was going to be a test, I would get a good score. If there was a gold star or a happy face sticker to be earned, I wanted two of each. Maybe three. Yes, I am that person. That person in your high school class that always wanted to earn a higher score than you did.
In the days and weeks leading up to my follow-up evaluation, I did stretches at home, lifted small weights, exercised my hands with a squeeze ball, and did deep breathing. I was determined to score better than last time.
Upon arrival, I was weighed. I discovered that I had gain several pounds since February. While I’d like to think this was muscle weight gain, I suspect it’s more likely due to the extra chocolate fudge ice cream.
One of the last tasks in my first evaluation was to lift a pound weight from my lap to a table. I couldn’t complete the task last time. I couldn’t even move the weight at all. The cuff weight just sat in my lap like a useless lump as I poked at it with my tired fingers.
This failure haunted me. I’m sure Kim Jong Un feels the same way each time one of his rockets crashes into the sea.
So, I worked on this maneuver at home. I found a 16-ounce bag of dried split peas in the pantry and practiced lifting it from my lap to my desk. After a few days, I could do it quite easily. I was ecstatic. On the day of my recent evaluation, this was the test I was ready to tackle. I wanted to OWN it. And, in celebration, I promised myself I’d have a margarita on the rocks— with lime.
Unfortunately, I had to do nearly ALL the other tests first before this one. I showed off my slightly stronger biceps, triceps, my increased grip, and the wider range of motion in my hands. I was working muscles that hadn’t worked this well in a few years.
The downside to all this (you knew this was coming, right?), was that by the time we approached the lap-to-table weight test, I had begun to tire. I was able to lift the weight into the air (which I couldn’t do months ago)… but I didn’t have enough oomph to get it on the table.
I began to panic. I tried again. And again. And forced the physical therapist to stay longer in the exam room so I could try again. I could feel the gold star slipping through my fingertips. I did NOT want my damn rocket to self-destruct over the Sea of Japan. No, no, no.
I knew the physical therapist had other patients to get to and I could tell she was annoyed with my obsession with completing this one particular task. I was like a dog with a bone. I wouldn’t LET. IT. GO. I was like Donald Trump still obsessing over Hillary Clinton. I just couldn’t move on.
But, the physical therapist had had enough. When she left the exam room, I nonetheless shouted after her as the door closed, “If I can do this task on video will you give me the points for the task?!? Will you?!? Will you?!?”
Yeah, I was that person.
It didn’t seem to matter that I went on to ace my pulmonary function test… that each measure of my respiratory ability had improved. I was still obsessing about the goddamn weight test. I wanted those points.
After a short rest, I had my friend start videoing me… I managed to lift a weight from my lap to the table in the exam room. Inside, I cheered… HELL, YEAH! I did it. I had proof. However, the physical therapist was gone by then.
But, if we’ve learned anything about me so far, it’s that I don’t give up easily. Upon leaving the neuromuscular department, the occupational therapist came over to chat. Before we parted ways, I burst out, “oh, and could you please tell Tina that I got a video of me putting the weight from my lap to the table?! Could you?!” I took a breath and added in a desperate rush, “I want those points!”
Yeah, I was that person.
Despite that emotional hiccup, everything else went well. And I was happy with how things had unfolded. The whole evaluation took nearly three hours, though, so I was exhausted by the time we loaded up in the car.
But, on the entire 2+ hour drive home, I thought about the celebratory margarita I’d have later that evening. I had moved that weight from my lap to the table. I had video proof of it, even though it may not have counted. And that’s all that matters, right? That margarita would be mine.
I think I deserved it.
It’s been an intense 9 months. Between qualifying for the nusinersen treatments, waiting to get the ‘greenlight’ to begin injections, and then enduring the hectic schedule of getting my first four loading doses, it’s been a crazy ride. While Hurricane Spinraza was slow to arrive, it gained speed at the end and has packed quite a wallop. A positive, encouraging wallop… but, a wallop just the same.
Perhaps it’s too soon after Harvey, Irma, and Maria to make hurricane metaphors— but, I’m going to do it, anyway. If President Trump can pitch paper towels into a crowd of desperate Puerto Ricans like a carnie at a state fair, I guess anything goes.
Physically, receiving these injections isn’t easy. There’s discomfort, soreness, — and yes, sometimes pain— from the actual treatments themselves. There’s the exhaustion from the long traffic-ridden drives to and from Stanford. It’s a physical challenge. Surprisingly, though, an additional improvement that I noticed after beginning my injections? My residual soreness/pain resolved itself more quickly with each and every treatment. My body felt just a little tougher each time.
Despite the physical challenges, very few people discuss the emotional challenges of this Spinraza journey… especially for those of us that have lived with spinal muscular atrophy all of our lives. So much energy, especially by medical professionals, is focused on our physical health, that our emotional health can be neglected. But, these needs are just as important. The last nine months have taught me that.
Those of us with SMA are expert jugglers. We balance many things in our lives… work/school, family obligations, friends and the management of our care needs— all while keeping track of the pregnant Kardashians. Which is seriously hard work. I keep waiting for one of the reality stars to put up a live pregnancy camera in their house. You know like the zoos do when they have a pregnant female panda? And everyone livestreams the zoo camera online in the days leading up to the birth in the hopes of catching a glimpse of the pink squirming glob that comes out of her panda uterus?
(Think of the ratings, Kris Jenner… just think about it.)
Anyway, we SMAers can handle a lot. It’s challenging to juggle our usual tasks— but, we do it. Because we have to. But, when you add in the giant, heavy ball that is Spinraza, it’s impossible for us to keep all the other stuff in the air without dropping some shit on the floor. This is unfortunate because I literally can’t pick up any of the shit I drop on the floor. I have to wait until someone comes and picks it up for me.
After my last loading dose, I physically and emotionally crashed. I went into hibernation— like one of those mama bears that Sarah Palin rattles on about. For about a week and half, I didn’t want to do ANYTHING. I wanted to drop all my juggling balls and sit in the corner and watch episode after episode of the Gilmore Girls. Because if anyone could fix my problems, it was Lorelei Gilmore.
So, I wallowed. And stewed. And fretted. And took some Xanax.
This went on for a while. But, then it started to fade away. I felt the muscles quivering in my arms— reminding me that I had $500,000 swirling in my spinal fluid. It was time to get to work. I had fucked around for long enough.
I started exercising my arms, wrists and hands— with stretches and small weights. I began lifting things more easily than I had done in a while. While in the car, I held a water bottle to my mouth and drank (without a straw!). I lifted a one-pound bag of dried split peas from my lap to my desk— something I hadn’t been able to do at my physical evaluation at Stanford in February.
Things were happening.
So, now I’m trying to make these things part of my routine. I sit at my laptop (while I’m watching Netflix), and do my weird exercises. I’ve discovered that music isn’t my motivator… but, good o’ streaming TV does the trick!
Just maybe not a live Kardashian cam…
There’s only so much a person can handle.
(Please “follow” my blog for more updates…)