Hurricanes, Pregnant Pandas & Kardashians

Standard

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…)

Advertisements

Commuters, Gladiators & Me

Standard

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

(For more updates and fun, subscribe to my blog…)

Spinraza, Turkey Burgers And The Voices In My Head

Standard

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

The Magic Little Bottle

Standard

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.

IMG_4263 blog

 

xoxo

(To follow my progress, please subscribe to my blog…)

The Waiting Game

Standard

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.

A Stanford Valentine

Standard

On a typical Valentine’s Day, one might expect a day of romantic gestures— giant teddy bears, Papa Murphy’s heart-shaped pizzas, and overpriced jewelry from the neighborhood mall. Since my boyfriend is of the fictional variety, I don’t have to worry about pretending to like the “chocolate diamond” necklace he bought for me at Zales.

This week, my Valentine’s Day definitely wasn’t a typical one— I got to spend the most romantic day of the year at the Stanford Neuroscience Building for my Spinraza evaluation. Upon arriving, we proceeded to not be able to find any handicapped parking in the garage. This is the irony of going to a place where a good number of the patients are crippled just like you. Handicapped parking becomes a valued and scarce commodity— like Stradivarius violins, raw uranium ore, and politicians with integrity.

The first item on the agenda was a lumbar spine CT-scan. Spinraza has to be administered into the spinal fluid, so the neuromuscular doctors need to verify that there is a pathway available in the lumbar region for their mega-pointy needle. For folks like me who have scoliosis and spinal rod fusions as a result of our Spinal Muscular Atrophy (SMA), this isn’t an easy task. You know Pin The Tail On The Donkey? Imagine playing that, blindfolded, with a donkey that has anatomy that closely resembles the tornado from Wizard of Oz. Try to pin the tail on that donkey’s ass and you might hit Glinda the Good Witch instead.

Anyway, after my lumbar CT-scan, I went to see the team of specialists. After taking my vitals, the first thing they wanted to do was find out my weight. This was not as simple as it sounds.

If Elizabette’s wheelchair weighs X pounds without her sitting in it, and it weighs Y pounds when she is sitting in it, how much does Elizabette weigh?

This problem would have been easy if I had known my wheelchair’s weight without me sitting it in— aka, the tare weight. Which, of course, I didn’t. So, we had to do it the hard way. But, I am now pleased to report that the tare weight of my wheelchair is 377 pounds— this does not include me and my backpack full of random stuff. That’s super heavy. Therefore, I’d like to apologize, in advance, if I ever accidentally run over your foot. Or, even purposefully— which I might do if you’ve pissed me off enough.

Anyway, after that was completed, I met with two neurologists, a few nurses, a physical therapist, a respiratory therapist, a blood-draw technician and a circus juggler. (Okay, I might have made that last one up.)

Everyone seemed professional and I was encouraged by their thoroughness. Although, the physical therapist and respiratory therapist were kinda bossy— in a good way that brings out my competitive nature. If anything is turned into a game or a test of skill, I will win, goddamnit.

They tested my pulmonary functions and my physical strength using an assortment of cool gadgets. These will be the benchmarks they will use to measure potential progress moving forward.

All in all, it was a tremendously long, but encouraging, day. The preliminary lumbar CT-scan radiology report indicates that I have a potential open spot on my L5 vertebrae, which is a promising sign. I also learned that the radiologist thinks I have a really big bladder. This is not surprising. I can comfortably go quite a long time without peeing. I suspect that my bladder can currently hold more water than the Oroville Dam.

I’m grateful to the family that joined me on the trip to Stanford and fed me peanut butter and jelly sandwiches. An influx of sugar makes anything more tolerable. If everything falls into place, I’ll try to get Spinraza. It’s still a long journey ahead and I have lots of hoops to jump (or wheel) through, but I’m on my way!

img_3731