Cripples, Loonies & Richard III

I’m a sucker for a historical tale— as long as it’s full of drama, intrigue and at least one insane king. If the story also has two or three loony royals, that’s even better. Especially if one is suffering from a questionable mental disease that could have been easily prevented if his parents hadn’t been cousins or he hadn’t shagged every woman in sight.

I have a decided preference for true stories— because, most often, the facts are way more interesting than anything anyone could make up. (Err— perhaps someone should tell this to Donald Trump.)

History is full of stories that are, frankly, unbelievable. If you think Game of Thrones is exciting and awesome, you should pick up a book about the Plantagenet family. Those folks were CRAY. Loco en la cabeza. The kind of crazy where one minute they are achieving remarkable victories for England, and the next they are trying to secretly (or not so secretly) murder all their younger brothers before they had a chance to grow hair on their testicles.

Yeah, I’m not kidding.

If you think George R.R. Martin conjured all of his Game of Thrones storylines from his own mind, you don’t know enough about the Lancasters and Yorks. During their Wars of the Roses, the English crown exchanged hands so many times that you’d think it was a fucking game of Hot Potato. The kind you play on rainy days at school during recess. Well, if “recess” were a battle that you had to wear sixty pounds of armor, that is.

I like reading books or watching documentaries about intense periods of history, like these. In fact, I just spent the weekend reading a book by Dan Jones, my favorite medieval historian. Some people like to go to the beach on their days off, I like to read about revolutions, multi-generational family feuds and all the kings named Henry.

Good times.

Anyway, as I turned the last page after Henry Tudor had emerged triumphant over one of history’s favorite villains, Richard III, I started thinking about poor Richard. Sure, he did lots of bad things… but, frankly, so did many other people at the time. Yet, somehow, in the years since, he has emerged as the great supervillain. The Lex Luthor of the Middle Ages. A grasping Voldemort that would lock young Harry and Ron in a tower.

But, what really separates Richard III from all the other medieval baddies? Of which we have many to choose from? He wasn’t the only one to knock off a relative, or two. He wasn’t the only one to steal a crown that wasn’t his. He wasn’t the only one to turn into a paranoid egomaniac.

But, Richard III did have one distinguishing feature. He had an orthopedic disability that caused him to have a serious case of scoliosis. This made him excellent villain material. It’s not surprising that William Shakespeare took that particular trait and ran all the way to the bank with it. It’s super easy to demonize a dude with a crooked and hunched back.

As a person with a disability (and also, scoliosis!), I’m left wondering… if Richard III hadn’t had this medical condition, would history’s recollection of him have been different? Would his contemporaries (and those in the decades following), have had such an easy time shoving him into the “EVIL” category?

At the time, those with disabilities weren’t looked upon kindly. Abnormalities, birth defects and other medical conditions were often seen as a “Curse from God” and punishment for inherent evilness and other wrongdoings. These prejudices persisted for much of human history, and can even sometimes be witnessed today (as much as I wish I could say otherwise).

The disability of Richard III, without question, influenced others’ perception of him. It’s easy to imagine a man that has been “cursed by God” to be capable of really nasty things. Especially in the medieval world where understandings of medicine, the human body, and religion were best left unexplored— and unquestioned. Even William Shakespeare penned these words for old Richard III, leaving us no doubt about how we should view the man’s nature: “I am determined to prove a villain.”

I can say one thing for certain. I’m really glad that I was not born during this crazy period. And that’s not just due to all the beheadings, the wars, and the general miserableness. You see, a girl with a medical condition, like Spinal Muscular Atrophy, would definitely not have lasted for long.

Seriously.

In addition to all the other preventable diseases that could kill me, all it would take is one tiny sniff of Black Plague and I would have been dropped in a patch of dirt outside the churchyard. You know, where the unblessed and cursed are left to rot? After all, sanctified ground within the churchyard is reserved for way better folks— like those that put the decapitated heads of their enemies on spikes outside the village gates.

In truth, I bet my medieval dirt nap would have been met with some relief. “Yay, the cursed girl is gone! Time to get back to sharpening my axe collection.”

Ahh, history. What tangled tales you weave!

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After Hawking

“The downside of my celebrity is that I cannot go anywhere in the world without being recognized… The wheelchair gives me away.”

— Stephen Hawking (1942-2018)

The passing of theoretical physicist & cosmologist Professor Stephen Hawking earlier this week leaves a big, gaping hole in the scientific world. A black hole, surely, of infinite size, depth and velocity— so powerful that probably only Dr. Hawking, himself, could describe it. I won’t pretend to attempt it because my scientific knowledge is limited to shit I’ve read in National Geographic Magazine. And random stuff I’ve Googled on Wikipedia.

So, yeah, I’m not very sciencey.

Professor Hawking was also probably the most famous person in the world that happened to use a wheelchair. A real-life Professor Xavier from X-Men… only with glasses and a better haircut.

For the majority of his life, Dr. Hawking had ALS— a progressive motor neuron disease. Due to advancing medical science, and the top-notch home care he had available to him, Professor Hawking’s lifespan exceeded that of many others with ALS. (Which speaks to the importance of self-managed home care for those with disabilities and why it should be more readily available— and covered!)

His life was even the subject of the 2014 biopic, The Theory of Everything, starring Eddie Redmayne. Eddie won the Academy Award for his portrayal of Hawking, which was not surprising because everyone knows that the best way to win an Oscar is to play a disabled character. Of course, the caveat being that the actor, himself, can’t—under any circumstances— be disabled in real life. Because that would be stupid.

I thought the movie was fairly decent up until the end… when a brief scene actually made me yell aloud in disbelief: “As if!”

While at a lecture to receive an award for his many, many achievements, the filmmakers inserted a moment where Professor Hawking witnesses a student at the lecture dropping a pen. The writers have Hawking daydream that he can stand up out of his wheelchair and pick it up.

Really??

A man that thinks in fucking lightyears and units of measure that literally NO ONE ELSE UNDERSTANDS is going to pause while receiving an award, to muse, “Gee. Screw science and my accomplishments. If only I could pick up this pen…”

I call bullshit.

But, you see, that’s what society would believe that he’d be thinking. Because to be seriously disabled is so scary to most people that they can’t even fathom it. To someone able-bodied, of course, Hawking would yearn to pick up that pen. But, in reality, he probably was thinking nothing of the kind.

As someone disabled, I can honestly say I’ve never seen someone drop a pen, and then lament, “Gosh, my life would have SO much more meaning if I could pick up that pen off the floor.”

Anyway, I seem to have digressed.

In the days since Professor Hawking’s passing, the internet has been awash with obituaries and tributes to him. His scientific achievements merit such accolades, that’s without question. While many of these tributes are extremely well-meaning, they miss a key element of the man that was Stephen Hawking…

He achieved everything he did with his disability, not despite it.

Stephen Hawking didn’t overcome his disability. He lived with it— achieving great things in the process. His infamous wheelchair wasn’t a trap, or a road block… it was a vital tool of life.

I’ve seen several cartoons and graphics circling the internet this week, showing Hawking drifting out of his wheelchair to stand and walk among the stars. Implying freedom. That he’s free from his wheelchair now. Free from the bounds of his disability.

Many seem to find this touching. Which is why these images have been shared thousands of times. But, when I see that, it makes my gut clench.

No matter how much Stephen Hawking had accomplished in his life, his story wasn’t complete without this magical metamorphosis. Floating free from his bonds to stand upright. To walk among the cosmos on two working legs. Because, heaven forbid anyone drive a wheelchair through the stars, instead.

While I’m not anywhere close to Hawking’s genius and epic achievements (by lightyears), as a disabled person, I’ve got a few things in common with him. His passing, and the response to it, has made me reflect upon what others may think about me.

When I die, will people think that I’m free? That I no longer have my wheelchair and disability to bind me?… burden me?… hold me back? Has my life boiled down to that?

I can say one thing for certain. If you draw a cartoon tribute of me floating out of my wheelchair after I die… or say that I’m “free now”… I will personally haunt you until the end of your able-bodied days. Not a cute haunting, either. A really annoying one where you hear whispers of “Fuck you” whilst you drift off to sleep. You’ll also never find your favorite pair of socks and your coffeemaker will always seem to malfunction when you are tired and/or hungover.

You will RUE. THE. DAY.

In fact, if it were theoretically possible, I’d come back to life, like Jesus Christ, just so that I might run you over with my wheelchair. Yes, I would definitely do that. Resurrection is a small price to pay for revenge.

So, your best bet? Don’t be comforted by thoughts that I’m “free.” Rather, please be sad that I’m dead. Okay?

Something tells me that Stephen Hawking would agree with me.

xoxo

Stephen Hawking NASA 50th

(photo via NASA)

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Alexa Saves The Day

When you’re disabled like me, technology is interwoven into the very tapestry of our everyday lives. While most folks can’t set aside their addictive iPhones long enough to take a shit, at least those devices aren’t hinges of mobility. Tools of survival.

In order to fully function, our gadgets must be in complete synchronicity… like an orchestra playing well in tune. If one instrument goes awry, the whole concert could be a total flop. Like Lady-Friendly Doritos or Kevin Spacey’s career.

I’m not a novice when it comes to technology fails. Just weeks ago, I grappled with a wheelchair shutdown that, while eventually resolved, led me to have anxiety for nearly a fortnight. I’m glad to have that behind me, but the delicate balance of our disabled, gadget-rich lives always teeters on the edge of the precarious.

This morning I had one of those moments. When the balance shifted decidedly out of my favor. It was an ordinary day and I was doing ordinary things. Drinking my late-morning coffee. Attending to various tasks. When suddenly, the perfect storm happened.

My dominant arm, which I use to support myself, fell out of place on the armrest. This, in turn, caused my chest to topple forward and my neck to get thrust down. Often, I’m able to extract myself from the situation with various wheelchair maneuvers. Like gunning the throttle to thrust my body backwards. Or, shoving my chest up against a table. But, this time it all went spectacularly wrong.

When I attempted to push my chest backward against a high table, my arm slipped further and activated my wheelchair’s tilt seating system so that it lowered and pinned me against the table. Unfortunately, my arm was stuck on the tilt button, keeping it activated and running, even though it was fully lowered. When the tilt is activated, the wheelchair is immovable, meaning the control box was useless. Meaning I couldn’t reverse myself to reach the iPhone that was resting on my lap. Meaning that I couldn’t text or call for help.

I was well and truly fucked.

I began to panic as the seconds ticked by… and the seating tilt motor ground forward in an endless mechanized rhythm. I couldn’t get it to stop. I knew that if it continued for an extended period of time, I could burn out the electronics.

While you might think that I was most concerned about my own current discomfort, no… alas… I was thinking about my wheelchair. And how disastrous it would be if the tilt system went kaput.

The panic grew overwhelming, and my breathing grew labored in the awkward, cramped position I was in.

Although I was currently alone, I knew someone would be popping in within the next hour. But, I didn’t think my panic or my seating system tilt mechanism would last that long.

All sorts of crazy thoughts were accelerating through my head like a runaway locomotive… or those commuter trains that always seem to be derailing in movies starring Denzel Washington.

How long will I have to wait here?

How much would a new tilt cost?

Will the insurance pay for it?

Will it take weeks to be fixed?

What if I panic and die?

What if I panic and die while Donald Trump is still President?!?

I was a righteous mess. I tried to calm myself. Stop the pounding heartbeat I could hear inside of my head.

And then, I remembered it.

ALEXA!

My Amazon Echo Dot (similar to the Google Home Mini) was perched 8 feet away. It had been given to me by NMD United, a peer-run non-profit organization that serves adults with neuromuscular disabilities. It’s voice-activated tools are extra helpful to those of us with mobility issues.

It’s always just waiting to do helpful things… like tell me the weather forecast, play me songs by Katy Perry… or… call people for me!

I called out, “Alexa, call someone!”

She replied, “Who do you want to call?”

I began to grow irritated, “I don’t care!”

“Donna Karan is not in your address book.”

“ARGH!”

I pulled myself together, “Alexa, call 2, 0, 9, 6…..”

After a pause, she asked, “Would you like me to call Daddy Cell?”

“Yes!!!” I exploded.

When the Echo Dot began to ring and I heard my dad’s voice answer, I collapsed against the countertop in relief. I think I even drooled a little.

I’m thankful to say that my dad arrived before any noticeable damage was made to my wheelchair. Although, perhaps time will tell on that front. Luckily, even though it felt like an eternity on my end, I think the entire episode only lasted about 10 minutes. And I’m doing A-OK now.

Technology is a fickle, fickle business. And our hold on it is incredibly tenuous. But, sometimes, when one aspect fails, another may save the day.

Thanks to Alexa.

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Test Anxiety

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.

neuro

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How To Survive A Shutdown

I really wish this was an article about the government shutdown. It would be a lot more satisfying to spend the next 1200-odd words comparing members of Congress to the small, misshapen toadstools you find in the pond next to a toxic waste dump. I mean, they can’t expect us to praise them for failing to do their actual job, right? Last time I checked, if a person couldn’t demonstrate any real skill or talent, they’d get fired. (*This rule doesn’t apply to Kardashians, or other reality television stars— including Donald Trump.)

No, this isn’t about a government shutdown. Rather, this is about another shutdown of a far more frightening sort. The kind that makes your hair turn white and causes you to question your own mortality.

Yes, it’s a wheelchair shutdown.

I have one of those custom motorized wheelchairs— the kind with oodles of special features designed to maximize my comfort, independence and mobility. This thing has a personalized seating system, a reclining feature, and six tires that allow me to turn in a small enough space to fit at least 4 or 5 Olsen twins.

This is handy so that I can get into smaller areas like a bathroom or a pantry— where I can grab a box of Cheez-Its without waiting for someone to do it for me. Anything that makes it easier for me to grab food to stuff into my face is a huge, valued part of my life.

Anyway, these wheelchairs are designed specifically for each patient. From the dimensions of the seating system to the height off the ground— it’s all perfectly designed to me. In fact, even the NASA-inspired honeycomb seat cushion is created to fit my buttocks. It’s like a designer Gucci purse for my ass.

While this may sound extravagant to some, if you had to spend 12 hours a day sitting in one chair, it had better be amazing. Not some piece of shit you bought at a garage sale.

These specialized chairs are not interchangeable. If something goes wrong with my wheelchair, I’m majorly… well… fucked. I can’t borrow a wheelchair to use until mine gets fixed. There’s no Hertz Rent-A-Car for custom wheelchairs.

I think you can sense where I am going with this, right?

A couple of weeks ago, a fault message appeared on the screen of my joystick— “Right Motor Fault.” I had just gotten into my chair and the morning had been bright with promise. I had a caramel vanilla coffee waiting for me and a whole list of things planned for my day. It was going to be GREAT. The kind of day where I accomplished a lot of paperwork— yet still had time to make a pot of chicken noodle soup and watch two or three episodes of The Crown on Netflix. Yeah, it was supposed to be that kind of day.

But, upon seeing that error message on the screen, my mood immediately plummeted. It went from GREAT DAY to… JESUS, MY LIFE IS OVER.

You see, my chair would not move.

Heart pounding, my mind began to race. I turned off the power, let the wheelchair sit for a moment, and took 3 deep breaths so I wouldn’t hyperventilate. Then, I tried the chair again. This time, the motors activated and moved.

While you may think I was ecstatic, relieved, joyous— I decidedly was not. My relief was measured, cautious— for I knew that a motor fault error was a sign of impending doom. Like a meteor heading to Earth or a Black Friday sale at Best Buy. Someone—somewhere— was going to get screwed over by a 60’ LCD television for $180. And that person was me. It was inevitable.

This was the third set of motors I had installed on my wheelchair— even though the chair is less than seven years old. So, I knew all the signs. The cheap toys in a McDonald’s Happy Meal have a longer shelf life than my shitty motors. You’d think that a manufacturer of a beautifully designed wheelchair could manage to put well-engineered motors on it, too. But, no.

I guess we cripples can’t be choosers.

For the next couple of days, the specter of malfunction hung in the air— I knew the motor error would happen again, it was only a matter of time. So, I did what any organized, thoughtful person would do. I called my local wheelchair company to give them a heads-up that sometime in the next week, or so, my life was going to go down the toilet.

Then, I called my doctor to have him fax a prescription for “motorized wheelchair repair” to the aforementioned wheelchair company. Yes, the prescription really does say that. Who knew that prescriptions weren’t only for antibiotics and Lipitor… or, if you’re Bill Cosby, then Quaaludes?

These repair parts take time to come in, so I knew I needed to get the order in pronto. Stat. ¡Muy rápido!

I also knew that there was no way in hell that my current motors were going to last until their replacements arrived.

And, I was right. A few days later, after sporadic functionality, my chair stopped for good. ‘Right Motor Fault’ had won.

I had to be pushed around in my chair like a giant cart of bottled water at Costco. Or one of those pathetic drivers that runs out of gas and gets stranded on a freeway.

I couldn’t do anything.

My life stopped.

You know the old saying that sailors have a potty mouth? Well, even the shadiest pirate in 1790 had nothing on me at this point. I was a bundle of anxiety and curse words. I couldn’t say one sentence without at least two to three versions of the word ‘fuck’ in it. As a verb, adjective, adverb— I’m not sure there was a part of speech I didn’t use.

Then, once I had exhausted myself, I called the local wheelchair company in tears a couple of times. It wasn’t pretty.

Some old smart British dude once said, “Necessity is the mother of invention.” I think anger and rage also are, too. After a hunt in my garage, we found an old set of motors that had been leaky (but functional). So, we swapped the leaky right motor for my dead one and said a prayer to the Broken Wheelchair Gods.

It worked. And the chair continued to work for another week until the new motors arrived from the shitty motor factory in The-City-Shall-Not-Be-Named, Ohio.

But, that week was still pure torture. I’d get up in the morning, get into the chair, and I’d feel my heart rate go up by about 20 points before turning on the joystick. Each time the motor fault error didn’t appear felt like Christmas morning all over again. Not the Christmas morning of recent years (you know, as a boring adult), but the Christmas morning of childhood— when Santa brings you a big box of Legos or a My Little Pony with glittery, purple hair.

Yes, it really was that good.

Now that this current crisis is behind me, though, it means that I must start thinking about the process of getting a new wheelchair sometime soon. Given how precise and perfect the seat and chair must be, you can understand how I might approach this with dread.

I’m sure I’ll be writing about the process in the coming months… so, stay tuned.

Keep your fingers crossed that these motors don’t die first, though.

At the rate I’m going, it’s not looking promising…

Boogers, Rainy Days & Surviving Dose #5

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.

So, yeah.

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.

Nothing.

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.

YAAAASSSS!

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!

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dose5

Spinraza: One Year Later

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.

Dimensions, Dictators, and a Whole Lot of Weed

I’m not going to sugar-coat it, roll it in cinnamon, or top it with frosting. I’m going to be honest. The kind of honest that you usually only see from small children or really elderly relatives— but, rarely see from politicians or men that run around grabbing women by the ass.

Okay, here goes… The year 2017 was… just… straight-up weird. So weird that it made me wonder if we had somehow veered off into a parallel universe. I know you might think I sound loony. But, stick with me for a moment.

Stephen Hawking, the super-genius, theoretical physicist, writes a lot about this concept. He says that black holes could be portals to other countless universes— very much like our own world, but, just a little different. So, there could be infinite planet Earths… countless other cities of Patterson… and many versions of me.

This is a troubling thought. I wonder if all versions of me are as scared of spiders? Or, like pumpkin spice lattes? Or like to drive so fast in their wheelchairs?

I also wonder if all the versions of Patterson have quite as many palm trees? Or, as many pizza places? Because I don’t know if it’s possible for a town to have as many locations to buy pizza as we do right now.

Seriously.

Anyway, what if the Earth was swallowed by a giant, unknown black hole, and we slipped into another dimension? Maybe that would explain why 2017 was so weird… and it would also explain why Patterson suddenly seems to want so many marijuana dispensaries.

Between the record-breaking hurricanes, deadly earthquakes, and massive fires, it could be argued that Mother Nature didn’t care much for 2017, either. Nonetheless, we mustn’t forget the victims of these disasters— many organizations are on the ground now helping those that are affected. Donate time or money, if you can. Don’t be a Scrooge.

It’s important to note that not everyone has had a difficult year, though. North Korea’s Kim Jong Un spent the year honing his missile launching and nuclear skills. After scattering hardware all over the Sea of Japan and threatening the West with death and destruction, he still found time to attack a village of Hobbits with a potato cannon. So, all in all, it was a pretty solid year for dictators. Just ask Vladimir Putin.

We also found out that Americans can become obsessed with solar eclipses— so much so that they will call-in sick to work, and drive hundreds of miles to watch the shrinking sun through a peephole in a modified box of Honey Nut Cheerios. I suspect that many of these same people spent the prior year, 2016, chasing cartoon Pokémon on their smartphones.

So, I suppose this is an improvement.

Scientists made several discoveries this year, too. A new species of orangutan, a close primate cousin to humans, was recently discovered hiding in an isolated forest in Indonesia. It’s been a century since a new species of great ape has been found. So, this is an incredibly exciting— and surprising— development. After all, who knew that Donald Trump wouldn’t be the only new orange-colored primate to burst into the international scene this year?

As we say goodbye to 2017, here’s hoping that the year to come will be a healthy, safe and happy one.

Just watch out for those black holes.

A Germaphobe’s Guide to the Holidays

I love everything about the holidays. The music. The food. The carbs. The festive spirit that makes even the dumbest Hallmark movie seem quaint and charming. While I eschew The Hallmark Channel for the other 11 months of the year, for these few weeks, I tolerate the weak plot lines, terrible acting, and the tons of synthetic snow they import from China. I suspend my cynicism and convince myself that this stuff is A-OK— you know, just like Matt Lauer did with his overactive penis.

Anyway, while this season heralds many wonderful things (the extended holiday selection at Starbucks being one of my particular favorites), not everything about this time of year is so great. Yes, I’m talking about all the cold & flu viral cooties that float around faster than Hallmark’s plastic snowflakes. For the average person, this is only a minor annoyance. Perhaps some sniffles here and there, and odd sick day from work. Nothing that Tylenol Cold and a shot of whiskey can’t handle.

But, for someone with spinal muscular atrophy, like me, a minor cold can turn into fucking Armageddon. Like the kind with Bruce Willis and that damn asteroid. Or the kind that wipes out all the dinosaurs on Earth—except for Barney… and Larry King.

So, to call me a germaphobe would be a vast understatement. It would be like calling Einstein merely ‘clever’ or saying that Donald Trump just ‘somewhat likes’ using hairspray.

I am a full-fledged germ freak. If I hear someone cough or sneeze, my ears suddenly morph into the radar of a Navy submarine. I quickly determine the distance between me and the sick person, and if I need to undertake any evasive maneuvers like Sean Connery in The Hunt for Red October. I will burrow into the ocean floor if need be. Don’t think I won’t.

If they’ve done something especially stupid, like cough directly into their own hand (instead of the crook of their elbow), I’ll glare at them maliciously while I catalogue every surface that they touch with their virus-ridden hand.

Yes, I really am that bad.

And, yes, it really is stupid to cough or sneeze into your own hand. You should always cover your face with your arm, instead. Less chance of spreading the virus to others.

Anyway, given the respiratory weakness of those with SMA, it is very difficult for us to keep our lungs clear. It is harder for us to cough. Harder for us to blow our nose. So, the drainage that might only be an annoyance to you, can become dangerous to a person like me. It can settle in our chest and potentially cause serious issues.

If I do get sick, I have to be very diligent. I vigorously use my respiratory devices (BiPAP, nebulizer, and CoughAssist) to prevent any complications. Under the best of circumstances, it can take me at least 10 days to 2 weeks to get over a mild cold. More serious illnesses can knock me out for even longer.

As happy and joyful as the holiday season is for me, it can be difficult, too. To the average person, an invite to a holiday cocktail party is immediately accepted. After all, who doesn’t like eggnog and a free selection of crackers and salami?

But, for me, deciding to attend the party would be a gamble. Like playing Russian Roulette or marrying a Kennedy. As much as I love eggnog (which, I do!), I must weigh that against the fact that at least one or two dipshits will probably attend the party even though they are sick and should stay home. Do I want to risk that they won’t sneeze near the salami? Do they know how to properly wash their hands?? What if they actually try to hug me???

Oh, the horror.

This kind of analysis runs through my head with every holiday invite that I receive. Before accepting anything, I quickly consult my calendar to make sure I have nothing important to do for the following two weeks after the event on the off-chance that some fuckhead gets me sick.

You can imagine why it might be easier for me to sit at home this time of year and watch badly-written Hallmark movies, instead. Fake snowstorms are far more palatable than hacking up part of a lung.

Nonetheless, it also isn’t healthy for a person to hide away in their house like the Unabomber. So, I try to venture out from time to time… armed with plenty of Purell, of course.

But, if I turn down an invite to your holiday event, please don’t take it personally. This doesn’t mean that I don’t like you, or that I hate salami… or eggnog. It just might mean that I’m worried your other guests might be carriers of the bubonic plague or some other horrible disease.

So, yeah… nothing personal.

Happy Holidays to you all!

xo ears2

Nutcrackers, Holiday Decorating & All The Lies We Tell

Around three weeks ago, I was in a store and holiday music was already playing. Even though I snorted in disgust at the prematurity of it all, that didn’t stop me from admiring a collection of miniature Christmas trees roughly the size of Kevin Hart. You’ll be happy to note that I didn’t buy one, but I seriously considered it.

That set my mind into motion— into thinking about the holidays and when I’d put up my own decorations this year. I always tell myself that I’m going to wait until after Thanksgiving, but that never seems to happen. It’s more what I tell other people if they ask me when I put up my Christmas decorations. I always chirp, “Oh, I wait until after Thanksgiving.” But, in reality, I’m secretly hunkering down in my house around November 15th with empty nutcracker boxes strewn all over my dining room table and a rim of peppermint mocha residue around my lips.

It’s one of those secrets that we all keep and then lie to others about. You know, like how many times a day we floss (which is never), how many times a week we empty the lint compartment in the dryer (which is not enough) and if we wash our hands after we blow our nose (which should be all the time, but never is!).

When you are disabled, like me, you have to rely on others to help you put up your Christmas tree. You have to cajole and charm someone into climbing into the recesses of your garage to pull out the 7.5 foot plastic tree crammed in a cardboard box large enough for Kim Jong Un to stuff at least two dead bodies.

I’ve had the same artificial tree for quite a few years now. I don’t like having a real tree. It’s too much commitment. I have a hard enough time remembering to water my two houseplants, I definitely don’t have time to attend to a needy spruce tree. Plus, I don’t want something that will drop needles and crap all over my living room floor. If I wanted that, I’d just borrow a toddler.

Each year, there’s always a big moment of dread right before turning on the Christmas tree lights for the first time. That sinking feeling of wondering if this will be the year that it finally takes a giant poop. I’m sure I don’t need to describe this feeling any further. After all, if you’re a San Francisco 49ers fan, you feel this nearly every week.

Anyway, the last couple of seasons, my tree had begun to show its age. The lights began to dim, and large segments of the tree would randomly go dark, only to perk up again hours later. So, this week, I decided to take the plunge and get a new one. My new tree seems okay so far, but the branches were so smashed from being in the box on the long journey from China that the branches required considerable fluffing to stop them from resembling large marijuana joints. I suppose that’s one benefit to real trees, though— they are already fluffy.

However you may spend this holiday weekend, have a safe and happy one. If you need me, I’ll be here fluffing my tree, consuming large quantities of stuffing, and canoodling with my nutcrackers.

IMG_4507 (2) Happy Thanksgiving!