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!

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

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.

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!

Introducing… SPINRAZA GIRL!

Margaritas & How To Stalk A Physical Therapist

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.

Hurricanes, Pregnant Pandas & Kardashians

It’s been an intense 9 months. Between qualifying for the nusinersen treatments, waiting to get the ‘greenlight’ to begin injections, and then enduring the hectic schedule of getting my first four loading doses, it’s been a crazy ride. While Hurricane Spinraza was slow to arrive, it gained speed at the end and has packed quite a wallop. A positive, encouraging wallop… but, a wallop just the same.

Perhaps it’s too soon after Harvey, Irma, and Maria to make hurricane metaphors— but, I’m going to do it, anyway. If President Trump can pitch paper towels into a crowd of desperate Puerto Ricans like a carnie at a state fair, I guess anything goes.

Physically, receiving these injections isn’t easy. There’s discomfort, soreness, — and yes, sometimes pain— from the actual treatments themselves. There’s the exhaustion from the long traffic-ridden drives to and from Stanford. It’s a physical challenge. Surprisingly, though, an additional improvement that I noticed after beginning my injections? My residual soreness/pain resolved itself more quickly with each and every treatment. My body felt just a little tougher each time.

Despite the physical challenges, very few people discuss the emotional challenges of this Spinraza journey… especially for those of us that have lived with spinal muscular atrophy all of our lives. So much energy, especially by medical professionals, is focused on our physical health, that our emotional health can be neglected. But, these needs are just as important. The last nine months have taught me that.

Those of us with SMA are expert jugglers. We balance many things in our lives… work/school, family obligations, friends and the management of our care needs— all while keeping track of the pregnant Kardashians. Which is seriously hard work. I keep waiting for one of the reality stars to put up a live pregnancy camera in their house. You know like the zoos do when they have a pregnant female panda? And everyone livestreams the zoo camera online in the days leading up to the birth in the hopes of catching a glimpse of the pink squirming glob that comes out of her panda uterus?

Yeah… that.

(Think of the ratings, Kris Jenner… just think about it.)

Anyway, we SMAers can handle a lot. It’s challenging to juggle our usual tasks— but, we do it. Because we have to. But, when you add in the giant, heavy ball that is Spinraza, it’s impossible for us to keep all the other stuff in the air without dropping some shit on the floor. This is unfortunate because I literally can’t pick up any of the shit I drop on the floor. I have to wait until someone comes and picks it up for me.

After my last loading dose, I physically and emotionally crashed. I went into hibernation— like one of those mama bears that Sarah Palin rattles on about. For about a week and half, I didn’t want to do ANYTHING. I wanted to drop all my juggling balls and sit in the corner and watch episode after episode of the Gilmore Girls. Because if anyone could fix my problems, it was Lorelei Gilmore.

So, I wallowed. And stewed. And fretted. And took some Xanax.

This went on for a while. But, then it started to fade away. I felt the muscles quivering in my arms— reminding me that I had $500,000 swirling in my spinal fluid. It was time to get to work. I had fucked around for long enough.

I started exercising my arms, wrists and hands— with stretches and small weights. I began lifting things more easily than I had done in a while. While in the car, I held a water bottle to my mouth and drank (without a straw!). I lifted a one-pound bag of dried split peas from my lap to my desk— something I hadn’t been able to do at my physical evaluation at Stanford in February.

Things were happening.

So, now I’m trying to make these things part of my routine. I sit at my laptop (while I’m watching Netflix), and do my weird exercises. I’ve discovered that music isn’t my motivator… but, good o’ streaming TV does the trick!

Just maybe not a live Kardashian cam…

There’s only so much a person can handle.

striped

xoxo

(Please “follow” my blog for more updates…)

Fully Loaded

My Spinraza journey has been one of highs and lows. I’ve had laughter and joy— but, also some tears and anxiety. It’s been a whirlwind of emotion that has frankly surprised me. If my journey were a story, I’d say that each injection introduced a new plot twist. You know, a plot twist like a long-lost, evil twin brother in a really good telenovela? Or, a sudden beheading on Game of Thrones? Or the content of a press conference at Trump’s White House? Because, in all those examples, where you think the plot is headed is never where it ends up.

Earlier this week, I had my fourth, and final, loading dose of Spinraza. After these initial doses, I will just have to return to Stanford four times a year for a single dose to maintain the level of drug in my body. Like topping off a tank of gas. Only this gas is worth more per ounce than plutonium. Just don’t tell Kim Jong-un about this stuff. Knowing him, he’d probably try to fling a vial of it at Japan.

My dose last month went incredibly smoothly, as I documented here. I had a confident, gladiator doctor that had the needle in the target zone so quickly that I thought he was joking. After all, it ain’t easy to negotiate the long lumbar needle through my spinal rods, even with the live x-ray guidance. So, this time, I figured that world-record speed would be hard to beat.

But, I was wrong. Upon arriving to the radiology department at the Neuroscience Center, I was informed that the A-Team was going to do my injection that morning. I felt a jolt of excitement. I get the same feeling when I get a coupon for 25% off at Bath & Body Works.

When I met the doctor, it wasn’t Mr. Gladiator… Rather, it was like if Dr. Meredith Grey had suddenly developed an undetermined-European accent and appeared at my bedside. Her friendly smile and slightly wavy dark blonde hair were straight from one of my favorite TV shows.

She was calm, capable and prepared. And she had the needle inserted so quickly that I didn’t even feel one damn thing. Like nothing. Not a twinge. Not a zing. Like Robert Redford was to Horse Whisperers, she was to lumbar punctures. She was the Lumbar Whisperer. I think she should put that on her business card…

“Doctor. Neuroradiologist. Lumbar Whisperer.“

It has a nice ring to it.

It wasn’t long before I was ushered to the recovery area. Feeling so relieved to have the injection complete, I quickly guzzled some water and an entire caffeinated Mountain Dew to ward off any potential spinal headache. (Spinal headaches are a very common side effect of this procedure— and caffeine and hydration can help prevent it.)

It was all going swimmingly… until the nurse returned to take my blood pressure and the numbers too closely resembled the final score of a record-breaking NBA basketball game. The excitement and caffeine had hit my bloodstream like a locomotive— and my blood pressure proved it.

They told me to relax, which is the one thing that is impossible to do when you’re a hypochondriac like me. If you tell me to relax, the exact opposite thing is going to happen— I’m going to panic.

My palms began to get sweaty and I became convinced that I was going to have a stroke and die— taking my expensive plutonium-loaded spinal fluid with me to the grave.

They observed me for over 45 minutes, waiting for the blood pressure to reduce. Which, of course, it didn’t. How could it when I had 4 sets of eyes watching me and telling me to calm down?

It’s no surprise that I began to cry. All the stress from the last few months— the waiting for the Spinraza, qualifying to get it, all the insurance hurdles, going through the discomfort of getting the injections… all of it… it hit me like a wave, pulling me under. I bet Trump gets the same feeling each morning when he wakes up and realizes he’s still president.

Soon after that, the nurse took pity on me and let me go home. My relief was enormous. I don’t remember much of the drive back home to Patterson— I was in a daze of relief, exhaustion and, yes, joy, too.

For you see, I had done it. I had survived my loading doses. I wouldn’t have to do another injection again for 4 months. It seemed like bliss.

Stay tuned, though, for more updates on my progress… I will be chronicling everything here (lucky you!). Most of all, thank you for your support on this journey… it has meant so much.

Commuters, Gladiators & Me

After my experience with my last Spinraza injection, I came into Dose #3 like a soldier ready for battle. If there was a potential contingency, I had already thought of it— at least twice. I was like fucking General Dwight D. Eisenhower in advance of the invasion of Normandy. I was mentally and physically prepared. I had taken a mini Xanax and hydrated like an Olympic marathoner. I was ready. Fear me and my Spinraza greatness!!

On the morning of the injection, I woke up at 3:45am— a time when most reasonable folks are asleep. But, if you are President Donald Trump, it’s your favorite time to sit in the dark and send weird messages to your 36 million Twitter followers.

I got dressed, and we ventured out into the pre-dawn morning to make the trek from Patterson to Stanford. We encountered a purplish sunrise and about 765,983 damn commuters clogging the roadways.

I was distracted by my upcoming procedure by contemplating the utter horror all those drivers endure each day on the roads. How do they do it? How do they manage to survive that commute each and every day? Maybe they were the ones that needed the Xanax— not me.

We arrived well in advance of my appointment and managed to snag one of the coveted handicapped spots right in the front of the Neuroscience building. Getting one of those parking spots is like winning the lotto, only way better because you actually have a chance of winning. (Yes, I’m talking about you, feverish Powerball players. Let’s be real — the more millions in the jackpot, the less chance you will win. And spending more money to win isn’t a guarantee, either. Just ask Hillary Clinton.)

For each of my Spinraza injections thus far, I’ve had a different team of doctors performing the procedure. Stanford is a teaching institution, so there’s a rotating group of doctors eager to plunge that needle into my wonky, curvy spine. This time, my team was extra-confident. They strode into the room like Russell Crowe in Gladiator. After examining my scans, one doctor proudly announced, “I’ll get this done in 20 minutes.“

My last procedure had taken over 90 minutes. In my mind, I thought, “Bullshit, Turbo. You’ve never met a spine like mine. I’m your damn Kilimanjaro. I’m fucking Mount Everest.“

But, I stayed quiet. I didn’t want to squelch his enthusiasm. I also didn’t want to piss him off since he was the one with the really big needle.

I got into position on the table and they began. I listened to their low, confident chatter behind me as I let my mind wander. I thought about food— as I often do, generally. I was hungry since I had to fast for the hours prior to the procedure. I contemplated lunch….. and then I felt a zing of heat down my left leg.

“Whoa– what was that?” I called out as my nerves quivered in response.

“I’m in,” Dr. Confidence remarked behind me― like a member of the DAR coolly ordering a Cobb Salad at a country club.

My brain stalled for a moment. I looked up at the clock on the wall in front of me… a mere 20 minutes had passed. I had difficulty computing what he had said. I sputtered, “Wait— what?! Are you for real?“

“Yes. I’ve already begun to collect the spinal fluid before we inject the Spinraza.“

Like a Looney Tunes cartoon character, my eyeballs began to bug out of my head, “Holy shit! You weren’t kidding at the beginning, were you?“

“Nope,” The Radiological Gladiator replied.

Elation flooded my veins. I felt like it had suddenly become Christmas Morning and Santa had brought me a really great present. Like a My Little Pony. Or an Easy Bake Oven. Or a $125,000 injection administered pain-free and in World Record Time. SWEET JESUS, SANTA IS REAL!

I was wheeled out of the fluoroscopy room just a few minutes later, my face wreathed in a smile, while clutching another, empty magic little vial in my fist. VICTORY WAS MINE!

Like Russell Crowe, I wanted to yell out, “My name is Maximus Decimus Meridius, commander of the Armies of the North, General of the Felix Legions, loyal servant to the true emperor, Marcus Aurelius. And I will have my vengeance, in this life or the next!!“

I didn’t yell that, of course, because I didn’t want them to think that the Spinraza was making me psychotic.

So, instead, when I returned to the room, I gulped down some water, and a caffeinated Mountain Dew to ward off the spinal headache. Then, I scarfed down a sandwich and sent a flurry of texts to my loved ones— my more-nimble fingers flying across the screen like super-sonic gnats.

Yes… Spinraza works, my friends!