1990: Revisited

Standard

With the passing of Barbara Bush earlier this week, the news has been awash with memorials of her life and the presidency of her husband, George H.W. Bush— or, as I not-so-secretly call him, “Old Man Bush.” I realize that calling the 41st president by that moniker sounds ageist and mean, but given we had another president with LIKE EXACTLY THE SAME NAME, how else am I to differentiate the two?? I suppose, in some ways, though, it’s better to be “Old Man Bush” than it is “Little Bush” — which is what I called his son.

Anyway, in all honestly, my recollection of the years when George & Barbara Bush lived in the White House are decidedly hazy. I was only around 8 at the time, so anything that wasn’t in the shape of a Lego really didn’t interest me. But, nonetheless, I do have vague flashbacks of Barbara with her shock of white hair and her bright suits the color of a Troll doll’s hair. Seriously, those suits were bright.

I bet she even glowed in the dark.

You know… it’s easy to imagine George and Barbara playing hide-and-seek in the White House. ‘Cause, if anyone were to do it, it would probably be those two lovebirds.

Bar, ready or not, here I come!” A few minutes of scrambling later, and then you’d hear George exclaim, “Come here, you saucy minx, I can see you glowing all the way from the Lincoln Bedroom!

Anyway, I do remember Barbara’s literacy programs in my elementary school, but as I was a certifiable bookworm already, Barbara was truly preachin’ to the choir with me. I don’t think it was possible for me to read any more books— after all, I had already made my parents broke by forcing them to buy me the entire series of The Babysitters Club. (I wish I was kidding.)

But, despite my early ambivalence to politics, I do remember one landmark moment during the presidency of Old Man Bush (sorry, I still can’t seem to help myself). It was that moment in 1990 when George signed The Americans with Disabilities Act (ADA) into law.

I didn’t know it at the time, but that legislation had been a long time in coming. Many disabled activists had endured many trials and hardships to make that moment possible. Even though I was young, I could still feel the importance of that revolutionary document. On the news that day, I saw folks in wheelchairs at the White House sitting next to the president. I had never seen that before. They were people like me. (Although, in all honesty, they were mostly male and super white. At the time, of course, diversity was an unnecessary concept, not an actual reality. You know, like women CEOs and food allergies.)

The ADA would nonetheless go on to shape the civil rights movement for disabled people all over the world. It was a giant leap forward for accessibility, inclusion and equal-access. But, as amazing as the legislation was, it’s still an imperfect document. It has loopholes, exclusions, and falls short in various areas that could further improve the lives of people like me. So, I can say without hesitation that we still have a long way to go. There are still many barriers that must fall.

Despite this, there has been a movement recently to try to erode away some of the protections of the ADA. Earlier this year, the House of Representatives passed H.R. 620, a bill misleadingly named “ADA Education and Reform Act of 2017.” By removing the reasons for businesses to proactively comply with the ADA, H.R. 620 attempts to chip away at the rights of a disabled person to fight for the removal of barriers to access. It makes it more difficult, and nearly impossible in some cases, for an aggrieved disabled person to seek accommodation. Nonetheless, the shitty bill has moved on to the Senate, where it sits right now.

With the passing of Barbara Bush, it’s made me reflect on that moment when her husband first signed the ADA. Often more vocally progressive than her husband, I’m sure that moment in 1990 brought Barbara much pride.

Now, all these years later, we shouldn’t be looking to scale back the ADA, we should be working to expand it. Time marches forward, after all.

Unless you can’t walk. Because then you might not even be able to get in the building.

ADA

(Old Man Bush signing the ADA in 1990. Photo via Associated Press)

Alexa Saves The Day

Standard

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.

IMG_4697
For more updates, don’t forget to subscribe to my blog in the sidebar!

buymeacoffee

Test Anxiety

Standard

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

For more updates, don’t forget to subscribe to my blog in the sidebar!

buymeacoffee

A Cure for Cold Feet

Standard

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.

olympic

For more updates, don’t forget to subscribe to my blog in the sidebar!

buymeacoffee

How To Survive A Shutdown

Standard

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…

IMG_4628

 

buymeacoffee

Boogers, Rainy Days & Surviving Dose #5

Standard

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!

For more updates on my progress, don’t forget to subscribe to my blog in the sidebar.

xo

dose5

Spinraza: One Year Later

Standard

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.

IMG_4573

A Germaphobe’s Guide to the Holidays

Standard

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!

xoears2

Margaritas & How To Stalk A Physical Therapist

Standard

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.

IMG

 

buymeacoffee