Commuters, Gladiators & Me

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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, mIMG_4323y friends!

xoxo

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Spinraza, Turkey Burgers And The Voices In My Head

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I had my 2nd injection of Spinraza earlier this week, so I’m pleased to report that I’m now worth a cumulative quarter million dollars. The little cells and neurons in my spinal fluid are so high-class that I fear that soon they’ll be too cool to spend time with me anymore. What if they forget their humble origins and do something snobbish— like adopt a British accent or befriend a Kardashian?!

The second injection was a little more painful than the first. Getting a needle through the labyrinth of my spine is no easy task— I’ve got metal rods in there to help with my scoliosis, some bony fusions and twisty vertebra. So, the doctor must slowly and carefully insert the needle, making microscopic adjustments and realignments as he/she goes. It’s like playing the classic board game Operation— one wrong move and the buzzer goes off. But, instead of a buzzer, they’ll hear me yell, “Argh! What the $&@#!

So, yeah, it’s kinda fun.

But, the excellent neuroscience team hit the bulls-eye— even though it took a little longer than I would have liked. I lay on the table, on my left side, for over an hour as they worked their magic. I stared at the wall and tried to ignore the pain in my shoulder from maintaining the position they require for the injection. I tried thinking of things that would distract me— what food I’d order at the restaurant later on… why I seemed to be drooling so much on my hospital-issue pillow… and when would the pumpkin spice lattes finally return to Starbucks this autumn?

As I was approaching the end of my tether, I felt the tears slide in and I began to cry. Not the cute kind of crying, of course, but the wet, snuffly kind. The kind reserved for Hallmark commercials and cheesy movies like The Notebook and Rambo.

But, then, I heard a voice in my head… a teasing, yet urgent voice that was surprisingly insistent, “Come on now… NO PAIN, NO GAIN!” I recognized the booming voice instantly as my late uncle, John. It was comforting to hear his voice and it helped me push through that moment. I’m not a person that generally hears voices (I may have many other medical issues, but that ain’t one of them)… yet, that gravely, stubborn encouragement was just what I needed.

The end result makes all the pain and discomfort worth it, though. At the beginning of my journey, my neurologist told me that our goal was stabilization— to halt the progression of my Spinal Muscular Atrophy. That would be a victory. That would add years to my life. Any gains, even minuscule ones, would be icing on the metaphorical cake (if that cake cost $125,000 a slice).

As I mentioned in my last blog post, I began to see improvements very quickly after my first treatment. In the days since my second, I’ve felt tightening in the muscles of my back, legs and shoulders. I also managed to pick up a hefty turkey burger (something I couldn’t do easily before) and shove it in my mouth like a starving Chris Christie. I was so proud of myself. It didn’t seem to matter that I got meat juice and condiments all over myself— that wasn’t the point. The point is, I ate it without using a fork and a knife. I OWNED that turkey burger. That burger was my bitch. It was awesome. I bet Donald Trump felt the same way about Chris Christie during the election, too.

IMG_4299Anyway, soon I’ll be headed back to Stanford for Dose #3… stay tuned for more Spinraza fun!

xoxo

Two Weeks Later…

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It’s amazing how much can change in two weeks. Wars have been fought and won in a shorter span of time and, if you are a believer, than the entire world was created by the Almighty in a mere 7 days— including, Adam, Eve, snakes, assorted shrubberies, and the Fuji apples you can buy at Savemart for $1.29 a pound.

And if all that wasn’t enough excitement, I also made the front page of the estimable Patterson Irrigator newspaper― instead of my column’s usual spot on page 5, or 7. So, yeah, a lot can change very fast.

Two weeks ago, I had my first injection of Spinraza. (For those of you that haven’t been following my journey, you can read my past writings on this topic HERE.) It wasn’t long after that initial treatment that I first began to feel that magical little $125,000 serum at my work in my spinal fluid— like busy Oompa Loompas toiling at Willy Wonka’s factory over a steaming vat of marshmallow cream.

The night following the injection, I woke up at 2am absolutely starving— like I hadn’t eaten anything for days. If you know me at all, you’d know this is an impossibility. There are few things in this life I love more than food. And if I could think of what those things actually are, I would write them right here.

I spent the next 3 days basically eating everything that wasn’t nailed down— especially things with protein. I was like a mama grizzly bear that hadn’t seen food since she went into hibernation last December. A real grizzly― not the fake ones that Sarah Palin seems to find everywhere she goes.

To give you an example, after already consuming breakfast, lunch and three snacks, one afternoon around 4pm I got hungry again and began casting my eyes around my kitchen. My radar settled upon a pouch of cashew nuts on the counter. For a few minutes, I attempted to open the reusable zippered fastener on the pouch. But, I quickly grew impatient and annoyed when my not-strong-enough fingers couldn’t open the lip. Undeterred, I grabbed my purple Crayola kid scissors (the only kind that I can use without hurting myself) and proceeded to desecrate the thick pouch until I had wormed a two-inch hole into the plastic. As the soothing scent of roasted cashews wafted up to my nose, I knew that victory was at-hand. THE CASHEWS WERE MINE!

This inexplicable hunger, this rabid feeding frenzy, culminated in a fluttering feeling in the muscles of my neck, my upper arms and my right hand. I began to notice that things were just a touch easier to do. My Sonicare toothbrush felt lighter. I was typing faster on my computer. I was able to grab my water bottle just a bit more smoothly. My voice sounded stronger. My respiratory numbers were up. I could text jokes about Mr. Trump to my friends even more swiftly than I had two weeks ago.

Things were happening.

It’s difficult to fathom that the little vial of muscle juice was already working. But, while a great deal of things are “all in my head,” I guarantee than this definitely wasn’t. Just because I could convince 11-year-old me that Santa was real, despite all the evidence to the contrary, this doesn’t mean that I’m imagining that the Spinraza was already helping. While my imagination is incredibly well-honed, it’s not that good.

This week, I head back to Stanford for Dose #2. I never thought I’d be this excited to get another shot into my spine. Never. Just as I thought I’d never live to every meteorologist IN THE WORLD go apeshit over an upcoming solar eclipse.

Seriously, though, this hype is way too much— just like the Twilight movies. Once the solar eclipse is actually done, everyone is going to forget all about it… just like they forgot how they once found Robert Pattinson brooding and charming.

Anyway, please wish me luck for Dose #2.

Who knows what the next two weeks will bring?

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The Magic Little Bottle

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I never imagined that one tiny glass vial could ever consume so much of my time, my thoughts… my efforts. My quest for this magic little bottle—this miracle drug— has been months in the making. But, on a recent July day— a resplendent blue-skied morning— it finally happened.

Spinraza is now real.

For those of you that haven’t been following my journey, you can read my past writings on this topic HERE. But, if you’re one of those people that used Cliff Notes or Spark Notes in school and are actually too lazy to go and read these posts, here’s a recap…

Right before Christmas, the FDA approved the very first treatment for my rare genetic condition— Spinal Muscular Atrophy (SMA). This progressive neuromuscular disease is the #1 genetic killer of children under two years of age— but there’s a small segment of us that manage to survive into adulthood. There is no cure. Due to a missing gene on my 5th chromosome, I am not able to produce a vital protein for muscle growth and maintenance. Instead, I must rely on alternate genes in my DNA to produce this protein. But, these alternate genes aren’t very reliable or productive— just like dial-up internet, a really stoned teenager, or the entire US Congress.

This revolutionary new medication tweaks my alternate genes, allowing them to produce more protein than before— like when Peter Parker was bit by that weird spider that changed his DNA and turned him into superhero. Don’t worry, though, I won’t be climbing walls or spewing webs from my wrists like Spider-Man. This is a treatment, not a cure. But, gaining just a little strength would make a big difference in my life.

In all honesty, I never thought I’d live to see the day when there was a real treatment for my disability. Just like I never thought I’d live to see an orange-tinted, reality television star become President of the United States.

So, yeah, I guess anything can happen.

Since the FDA approval in December, I’ve been laboring to get this treatment, having to surmount many obstacles. For example, there were tests of all varieties— physical and pulmonary exams, blood tests, genetic screenings, a polygraph test, and a breathalyzer.

Okay, I might have made those last two up.

I also had to contend with the insurance hurdles to get this very-expensive medication covered. At $125,000 per injection, Spinraza is an orphan drug— which means that it is so incredibly specialized that only the few of us with SMA can actually use it. Drugs like these are years in the making, so if only a small number of people can use them, each dose has to be very pricey to recuperate the costs.

Last month, the excellent team at Stanford Neuroscience called that I had been given the “green light” to begin treatment. It was one of the happiest days of my life. Just like the first time I drank a pumpkin spice latte and the day I first got an iPhone.

So, this week, we headed over to Palo Alto for my first lumbar spinal injection of Spinraza. The sky was blue with promise and there was anticipation crackling in the air. It took over an hour for two doctors to carefully maneuver the tiny needle into my spinal fluid— dodging the complexities of my scoliosis (the side effect of my SMA) as they went. But, with the help of live x-ray guidance, they did it.

When the nurse brought out the magic little bottle of Spinraza, I felt tears of joy, not pain, rush into my eyes. And when she finished injecting the vial into my spinal fluid, she said, “Elizabette— it’s in.

Even though I had gone through a lot to get to this moment, I knew in an instant that my journey was really just beginning.

So, stay tuned, folks.

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xoxo

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The Waiting Game

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I’m sure some of you may be wondering how my quest for Spinraza— the first treatment for Spinal Muscular Atrophy (SMA)— is faring. Obtaining this treatment isn’t for the faint of heart, it takes will, perseverance and just a little desperation— like climbing Mt. Everest, running a marathon, or taking a job in the Trump Administration.

As I’ve documented here, I’ve been examined by several Stanford doctors, I’ve had DNA tests, and I was approved and prescribed Spinraza… two and a half months ago.

But, I’m still waiting to receive the treatment. If I was a female chipmunk, I could have already given birth to nearly three litters of babies in the amount of time I’ve already been waiting. That’s a lot of chipmunks.

Insurance companies around the country have a wide range of policies on Spinraza. While the FDA approved the medication for all ages and types of SMA, at $750,000 for the first year of injections, it is in the insurance companies’ financial interest to limit access to the treatment. So, they have come up with a wide variety of parameters that they are using to approve/deny payment for the drug. Some insurances say you have to be over 15 years old, other insurances say you have to have less than 3 copies of the SMN2 gene, while some say you have to have over two copies of SMN2. Odder still, some are saying that that they won’t pay for those who already use a wheelchair… or a ventilator… or have a last name that ends with “r.”

I have Medicare, so my insurance situation is even stranger. Medicare is requiring something called “Buy & Bill.” Administering hospitals must purchase Spinraza themselves and then Medicare, in theory, will reimburse it. However, under this current set-up, the financial responsibility/burden is on the hospital. And most hospitals are understandably hesitant to assume that risk because they know there is a chance that Medicare will not reimburse 100% of Spinraza’s cost.

Would you fork out nearly a million dollars for a medication if you weren’t sure you’d get paid back for it? Uhm, HELL no. And no one wants to be the bad guy that sends on that kind of bill to the poor cripples in the wheelchair. I may not be in marketing, but even I know that’s not good PR. So, because of this policy, those of us with Medicare are in limbo. We’ve got prescriptions, but no way to get the drug— it’s like having a ticket to the moon, only NASA is broke and all the space shuttles are in museums.

I’ve been told by folks at Biogen (the distributor of Spinraza), and at Stanford, that talks are happening behind the scenes to figure this out. But, as time goes by, all of us with SMA get weaker.

Being patient is difficult, especially for someone like me that doesn’t have a lot of patience from the start. After all, I can barely wait for a grilled cheese to come out of the pan before I take a bite. I hover over the stove like a drone— and I burn the roof of my mouth EVERY. DAMN. TIME. Who can wait for that melty cheesiness? I know I can’t. You’d think that the resulting soreness in my mouth would deter me from doing it again the next time, but I’m just not that smart.

I’m sure some of you reading this are aghast at the price tag associated with Spinraza— and are thinking that there is no need for the treatment to be that expensive. But, please don’t descend upon the makers of this drug with torches and pitchforks on my behalf.

A specialized medication of this caliber is years in the making. There are many costs that go into creating something like this. So, they need to recuperate what they have invested in it. Further, this isn’t a medication for diabetes or high cholesterol— only a very limited number of people can use Spinraza. So, each dose has to be expensive to make it worth their time. If there is no financial incentive to research rare conditions, like Spinal Muscular Atrophy, then no one would bother doing it. That’s a fact. Kindness and altruism doesn’t make the world go around— money does.

In the meantime, today I received this Spinraza welcome packet from Biogen in the mail. It’s a treatment organizer— like the planner I had in high school that I rarely used. This organizer also came with a decidedly cheap pen— emblazoned the Spinraza logo.

spinrazapicNo offense, Biogen, but it’s 2017. Who writes with an actual pen, anymore? And who carries a planner? No one.

And I’m going to be frank… Don’t be like Clark Griswold’s cheap boss that bought him that crappy gift in National Lampoon’s Christmas Vacation. At $125,000 an injection, you could buy me an iPad, instead.

I’ll be waiting. You know where to find me.

Summer, Flip Flops, and Tony Danza

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Ugh. As I sit here typing this, the thermometer is registering 95°F. It’s barely May and only 4 days ago I still had flannel sheets on my bed. And now I’m sweating and my jeans are sticking to my ass. Why couldn’t the summer heat arrive gently? Like the slow bloom of a flower… or water dribbling down a clogged drain. Why, instead, does it have to be so blunt and nasty? Like a bull, or Steve Bannon, in a china shop?

I HATE SUMMER! There, I said it. I don’t like the heat, the sun, or even the clothes. I much prefer the styles of the cooler months— my cabinet full of scarves is a testament to that. Plus, I can’t wear most summer clothes, like shorts, because I sunburn in mere minutes. This isn’t dramatic hyperbole. One time I got a sunburned in the time it took to go from the front doors of the shopping mall to my handicapped parking space— which was IN THE FRONT. Yes, I really am that white.

Now that we’re on the subject of summer attire, I also don’t understand flip-flops. They seem unnatural and highly dangerous. Why would you want to walk around on something so unstable? It’s a shoe that’s barely attached to your body. You are one thin plastic strap away from disaster. It’s a good thing that I can’t walk, because if I had to wear flip-flops in the summer, it wouldn’t end well for me. First I’d get a bad sunburn on the top of my foot, then I’d fall and break my nose on the nearest object, like a ficus plant. It would be like an episode of The Three Stooges, only less funny and less Jewish.

If all that weren’t enough, the slapping sound flip-flops make is also decidedly unappealing. Do you intentionally want to sound like a walrus flapping their hands together? Because, I hate to be the one to say it, but you really do sound like that.

Anyway, thankfully I have air conditioning to help me during these trying times. It’s currently humming in a soothing way that reminds me of those noise-canceling machines that they used to sell at Bed, Bath & Beyond next to the display of soda-making kits that NO one ever buys. The same ones that have been on sale since 2005. If you are thinking of buying one, you might want to reconsider; I’m sure those flavored syrup pods expired back during the days when Bush Jr. lived in the White House.

Those were simpler times, though, weren’t they? We didn’t have a president with orange skin and hair… and Michael Jackson and Whitney Houston were still alive— so we could listen to their music without the sad pang of nostalgia. Those also were the days before the word taxi had been replaced by Uber. Before long the nerdy folks at Oxford will just drop that word from their dictionary entirely… and a hundred years from now little children won’t even know what a taxi is, let alone that it was yellow and usually driven by strange foreign men with accents— or Tony Danza.

IMG_3914It’s only May, though, so I better get used to this heat. If you need me, you can find me sitting in front of the fan, grumbling… and not wearing flip-flops.