A Summer Recap

Today is the last day of August, which means it’s the last day of Spinal Muscular Atrophy Awareness Month, the last day I need to feel guilty about consuming pumpkin spice flavored products, and the last day of the eighth month of 2020— the year that seriously NEVER FUCKING ENDS.

As I write this, I’m sipping on a Starbucks pumpkin spice latte that was dropped off at my door by a mask-wearing friend— a drink that I re-heated before consuming with my own straw (because COVID, duh). My guilt about enjoying this autumnal beverage is now at a very low level. I love pumpkin spice and I’m not afraid to admit that considering I’m less than 24 hours away from turning the calendar into a month that ends in “-ber.”

Reflecting back on August, and the summer as a whole, I’m just glad to get this season behind me. It’s been long. It takes a lot of emotional bandwidth to be a very high-risk person during a global pandemic. To have to sequester yourself in your home for months at a time (with no end in sight) because the virus is so tricky, and not enough people in the nation, and in the community, are taking this risk seriously. To know that if you get COVID19, it will probably kill you. To know all of those things, but still try to keep a routine, and stay as safe as you can. It’s a lot to juggle.

Those of us with SMA are often expert jugglers, thankfully. It’s something that Spinal Muscular Atrophy Awareness Month should definitely highlight— which it really doesn’t. We can balance home, school, work, medical, and care attendant schedules so efficiently that often people don’t even see us doing it. We’re like ninjas. Crippled ninjas with great organizational skills. Yet, oddly, society often mistakenly thinks disabled people like me don’t have anything to do. That we sit at home all day watching reality television shows and FoxNews. Which is weird. If I wanted to do that, I’d just be President of the United States.

This August has also been hot. And smoky. Having a disability that impacts your respiratory system isn’t ideal when a giant wildfire is burning just a couple dozen miles from your house. The apocalyptic ash falling from the reddened sky is not a great visual for a year that has already turned into a real-life Hunger Games.

Thankfully, the summer did have some bright spots. I got my 13th Spinraza injection at the end of June— which was the first time I had really ventured out of my house since March. The injection went smoothly and it was comforting to be in a place where people weren’t whining about wearing masks like little assholes.

The summer brought more good news on the SMA front— the FDA approved another treatment for my condition. It’s called Evrysdi, which unfortunately sounds more like the name of a magical elf from Lord of the Rings than it does a medication. I suppose it really doesn’t matter what the name is… just as long as they don’t manufacture it in a tree. Evrysdi is an oral suspension drug taken daily, unlike Spinraza which is administered via intrathecal lumbar injection every 16 weeks.

I’m excited that there is now another option available for our SMA community. Just five years ago, I never would have imagined that this would be the case. I never would have thought that pharmaceutical researchers would have bothered to take the time, or use the resources, to develop one, let alone THREE treatments for SMA. The third being Zolgensma— a gene therapy for infants up to 2. (No offense to the Zolgensma people, but your drug name sounds like a StarTrek villain that wants to destroy the universe with a laser. #SorryNotSorry)

Note: I should probably add at this point that I am available to any pharmaceutical companies out there that need help coming up with names for your future SMA drugs. It might save you considerable headache if you just ask me first. Because I will make fun of the name. I just can’t help myself. Just like I can’t help myself from imagining that Spinraza was the name of a 1920’s showgirl that waved around a 6-foot feather boa.

Anyway, that’s it for now. I think it’s best to wrap this up because I’m getting down to the bottom of my pumpkin spice latte. And it will take all of my concentration to slurp up the last orangey bits of nutmeg swimming at the bottom.

So, with that said… Cheers to all of you. xoxo

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#ThanksForDistancing

Under the advisement of my doctor, I have been in self-quarantine for the last 58 days. In that amount of time, I’ve only been ONE place, and that was to receive a life-sustaining Spinraza spinal injection with my SMA neuromuscular specialist. To be honest, I would have taken these precautions even without my doctor’s advice. After all, I did have an awareness that humans are reeeally great at spreading germs around— just like monkeys love to throw their poop. I knew in my gut that COVID19 was coming— just like Randy Quaid knew that the aliens were going to attack in Independence Day.

As someone that is high-risk, not just for COVID19, but for other respiratory illnesses, I have a well-developed radar for danger. I’m like one of those police dogs that can sniff cocaine in a Toyota Corolla parked 9 blocks away.

By the end of January, I started to get a twinge. An inkling. I started to feel something looming on the horizon. I had seen the news reports from China. I knew it was impossible to contain a virus in this modern, fast-paced world. It was going to spread. It probably already had. Ever try keeping an entire litter of kittens contained inside of a box? Good luck— because at least one little bugger is going to sneak out of the box when you aren’t looking.

When I went into self-quarantine 58 days ago, I began to mentally prepare myself for the long-haul. I knew that this virus wouldn’t go away quickly. It would linger and I would have to be careful for many, many months. Possibly over a year. It was a lonely & isolating thought— removing myself from the world with no reasonable end in sight. Yet, I had some experience in this regard, thankfully. Cold & flu season is dangerous for me… so quarantining and socially-isolating is not a new thing for me. I do it at various points each year. Never to this extent, though. Not by far.

But, I had been training my entire life for this. It would be my personal Mount Everest Moment. My Reese Witherspoon Trying To Do The Pacific Coast Trail Moment. My Donald Trump Pretending To Not Want To Fire Dr. Fauci Moment.

But, as the virus spread (which I knew it would), and many grew sick and hospitals became overwhelmed, people actually began to take the threat seriously. Cities issued stay-at-home orders. States & nations shutdown. Governments finally responded. And, most amazing of all, people learned how to wash their hands. To be honest, I was surprised that these measures were actually being undertaken. And I was even more surprised that they were being followed.

This isn’t to say that I don’t think these measures were valid. Yes, they were— and still are! But, I guess the skeptic in me didn’t think our society had it in us to do something like this… to take drastic life-altering steps like this. To buy hand soap… and to stay home— on a massive scale.

It’s a huge deal.

As someone that has experience with quarantining, and living life within physical restrictions, I understand how difficult these times can be. Logistically. Financially. And especially emotionally. It is a mental hurdle that is not easy to surmount— especially when you have no experience doing so. So, I want to acknowledge that.

The stress of all of this is real. The burden of all of this is real. For the young. For the old. And for all the ages in between.

But, the steps we’ve taken (and continue to take) have given me hope. The curve is flattening, and many lives are being saved. We are buying time for science to catch up with this virus. We are giving hospitals time to prepare. On a personal note, the murky specter looming of many months of isolation now feels less gloomy because of what society has done… what you have done.

And I’m so very thankful.

To help get through the days until communities are able to loosen the restrictions in place, I thought it might be helpful to share some survival tips that I’ve honed through the years. I am a veteran Quarantiner, after all.

1. Create A Routine — this is an essential component of surviving shitty times. For reals. Don’t be going to bed at 2am one day, and 7pm the next. Eat meals/snacks at a set time. Schedule Zoom sessions with friends. Write down a schedule. And stick to it. This helps regulate your nervous system & your anxiety.
2. Create Benchmarks In Time — Having something to look forward to, however small it might be, is key to getting through each day. I recently instituted “French Toast Fridays.” Each Friday, I have homemade French Toast for lunch. It’s simple, but it’s something I look forward to because I get to put a mountain of whipped cream on top.
3. Create Small Daily Goals — Often people think that a feeling of achievement can only happen when it’s something big. This is not true at all. Small achievements, even arbitrary ones, can help occupy the mind & give it direction.
4. Create Gratitude — At the end of each day, write down (or say out loud) three things you are grateful about that day. It could be something serious, or something silly & inconsequential. For example, you could say “I’m thankful for… 1) my home… 2) my family… 3) the mute button on the Zoom app.”
5. Create Your Castle — A “castle” is a safe-space. A place that protects you from harm. Instead of thinking of your home as a place you are confined to, think of it as your “castle.” A castle can also be something smaller than a home. It can be a bedroom, a corner of the living room, or even the 15 minutes spent alone in the shower. It’s a space, or a time, where you can just BE.
6. Create Moments of Joy — Despite what you may think, little moments of happiness can be manufactured. You can conjure them up from anywhere— like a Patronus charm. For example, about a month ago, my friends & I decided that our group text messages to each other must be written in rhyme. (Shel Silverstein can kiss my ass.)

I think it’s important that we all are aware, though, that many people live in unsafe environments— whether it be from abuse, domestic violence, or extreme poverty. So, during these times, we must be aware of the struggles of others and offer compassion, aid & understanding when we can.

We can be kind,
If we just set our mind.

❤️

[If someone you know is struggling… the National Suicide Prevention Lifeline (1-800-273-8255) & the National Domestic Violence Hotline (1-800-799-7233)]

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Icebergs, Firefights & Winning #9

Getting sick when you have SMA is not a fun experience, as I’ve discussed previously on numerous occasions. The tiniest cold can turn our lungs into a swamp of mucus, and recovering from, even such a minor illness, can take longer than a steamship voyage across the Atlantic. And that’s assuming there are no icebergs lurking in the water that could sink the ship— or, worse, yet, send us to the hospital for IV antibiotics. It’s a dangerous ocean out there, even without German U-Boat torpedoes.

So, it’s easy to imagine that if the illness is more than a cold— things can become serious very quickly. Recently, I was threatened with such an iceberg. A Titanic-sinking hunk of Influenza— the most dreaded of respiratory viruses. The little bastard that attacks during the night, overwhelms your defenses, and turns Leonardo DiCaprio into a blue icicle.

As soon as it hit, I knew exactly what it was— and the danger it posed to me. I had prepared for this moment, and didn’t take it lightly. Keeping my lungs clear was my top priority. I would sacrifice sleep and rest (the two things most needed when you are ill), just to keep those airways pliable and open. I would do anything to get the mucus out. Literally anything.

So, I leapt into action. I pulled out all my respiratory devices. CoughAssist. BiPAP. Nebulizer. And I started my emergency influenza protocols. Tamiflu. Zithromax. Prednisone. Albuterol. I had trained well for this moment. I was like those Paris firefighters that protected the Notre-Dame Cathedral. I was not going to let the whole structure fall apart. Sure, I knew my roof was made of really old wood covered in medieval pigeon shit. That just meant I was going to have to work extra hard to save the good bits of the building. But, it was definitely worth saving. If cranky French revolutionaries couldn’t defeat me in 1789, I wasn’t going to let a fire do it. Mon Dieu!

The following days passed in an exhausting whirlwind of respiratory care. Given the complexity of SMA, we can most often receive better care at home than in the hospital. You might find this surprising, but most local medical professionals have little to zero experience with a patient with a rare neuromuscular disability, like SMA. So, hospitals can be very dangerous for us. We have learned this the hard way. So, we have to become our own advocates and our own resources. And our doctors become our partners and colleagues… we teach them sometimes more than they can teach us.

It wasn’t long before my bedroom began to resemble a respiratory care unit. And I personally resembled the little green Mucinex monster from the commercials on TV. I was green, gross and contagious. Frankly, I was my own worst nightmare.

And, I was exhausted. Coughing and keeping my lungs clear became a full-time job. The kind of job with lots of overtime and no weekends off. The really, really sucky kind. In fact, I was so exhausted that I took to blending my food so that I wouldn’t have to waste energy eating and chewing. Every ounce of my strength was devoted to respiratory care and I needed to maintain the nutrition and calories to do it. Yes, I really was that committed. And, yes, blended food actually tastes exactly the same as non-blended food. For example, a blended Chile Relleno still tastes exactly like a non-blended Chile Relleno. (Unfortunately, it does look like baby poop, though.)

As my efforts slowly yielded positive results, this didn’t stop the mental toll from weighing heavily on me. The anguishing rotating schedule of BiPAP, CoughAssist and Nebulizer— in between drinking my meals and trying to rest— was relentless. There wasn’t even much time for Netflix, which says a lot because I can always find time for Netflix.

Emotionally, I struggled. I cried, even (which wasn’t wise because it made me even more snotty and gross). I had nightmares about really scary things. Like going to the hospital… being put on a breathing tracheotomy that took away my ability to speak… and, worst of all, Donald Trump tweeting even more than he already does.

After two weeks, I began to see the light. I could go stretches of time without coughing. My doctor noted that my lungs were improving. I was eating solid food again. I stopped dreaming that Robert Mueller was kidnapped by Boris and Natasha.

Things were looking up.

And then, last week, I made it to Stanford to receive my 9th injection of Spinraza. It was just the boost that I needed. A physical boost, but perhaps even more importantly, an emotional win.

In the days since, I feel my strength slowly returning. It will take time for me to get back to my pre-iceberg self, but I just need to be patient.

Wish me luck!

(PS: If you need to find me, I’ll probably be watching Netflix.)

influenzalife

Assessments, Zombies & The Cost of a Life

Every few months, as part of my Spinraza journey, I go to Stanford Neuroscience for a full day of physical and respiratory assessment tests to measure my progress— and my overall health. These tests are important for research purposes and to learn more about Spinal Muscular Atrophy and how it may respond to these revolutionary new treatments, like Spinraza. I’m fortunate to have an outstanding medical team at Stanford and they are really “writing the book” on SMA treatment protocols for adults. In the past, there wasn’t much that could be done to help the underlining medical cause of SMA, but science is currently progressing more than I’d ever thought I’d witness in my lifetime. So, it’s an exciting time to have an obscure condition that’s caused by a few fucked-up genes.

People finally seem to have the know-how to address the complicated science behind it. And it’s not theoretical. It’s not science fiction. It’s real— unlike many of the things that we spend so much time worrying about these days. Fake things. Like a zombie apocalypse… Alien abductions… Donald Trump’s tan.

Despite all of these advancements in science, and my excitement to be a part of it all, this doesn’t mean that I don’t get tired of being a cog in the large medical machine. That I don’t secretly (or not so secretly) dread these periodic assessments I must endure. Because I do. I dread it a great deal. Just like I dread how my ass will feel when I’ve eaten too many tacos.

As I’ve shared on this blog, I always get stressed and anxious before these tests. Even though I know much of this pressure is self-inflicted, there’s still the very-real fact that my results, compiled with the results of others, will impact the ability of other SMA adults in this country, and around the world, to have continued access to Spinraza, and future treatments that are coming down the pipeline.

I worry about that. A lot. Because I know that as a result of the high cost of these “orphan drugs,” payers and insurance companies are looking for any reason to limit who gets access to the treatments. Even though the pool of those of us with SMA is already so small, they’d still like to make that tiny pool even smaller. When scientific advancement meets economics, economics wins every time.

Insurance and pharmaceutical companies are already in negotiations about the next batch of SMA treatments and their potential costs. They are calculating the cost of an SMA life. The value of an SMA life. They are using terms in their reports like “quality-adjusted-life-year.” And “value-to-healthcare-systems.” And “how-to-keep-saying-these-mercenary-things-without-sounding-like-assholes.”

Access to these treatments is being evaluated on cost, not on who may benefit. Not on the value of the life itself. The looming pressure of this is very real. And, it cuts to the very heart of one central question: What is a disabled life worth—like mine?

Because, in most common societal measures, a disabled life is not worth as much as an able-bodied one. Society reinforces this view every day. And it has for many, many years. It’s been done in subtle ways. Like government policies, lack of community inclusion and accessibility, and in forms of everyday discrimination (which is called ableism). Unfortunately, some of these reminders are actually not-so-subtle.

In fact, sometimes, they slap you in the face.

It wasn’t long after I began writing about my Spinraza journey on this blog, that I received a comment from an anonymous reader. This gentleman lashed out at me for having the audacity and selfishness to think that my life was worth the cost of my treatment. That, because I’m disabled, my life isn’t worth it. That my contributions aren’t worth the added cost.

His ramblings took a nonsensical turn as the message progressed, so I was able to dismiss it and not internalize it as others may have done. But, his overt message spoke to the “unspoken” belief that many have about disability. He said the thing that many may believe, but don’t have the guts to say. After all, this idea is deeply woven in society. It’s why ableism is one of the most common, yet least talked about, forms of discrimination.

All of these thoughts weigh on me with each assessment that I endure. Each weight that I lift. Each paper that I tear. And each measurement that is taken.

In those moments, it suddenly becomes the time for me to “prove my worth.”

My Stanford team has kindly attempted to dissuade me from these anxieties before my assessments. They’ve urged me to not put this pressure on myself. Part of me completely agrees with them. It’s ridiculous to carry such a burden. Just like it’s ridiculous to believe it’s possible to die in a zombie apocalypse.

Yet, despite these assurances, given the society we live in, with its “quality-adjusted life years” being used to measure the cost of my life, and all the outside forces attempting to devalue lives like mine, how could I feel any differently?

It’s really hard not to do so. But, damn it, anyway, I have to try to shed that burden. To push it off my back. Dismiss it. You know, the way a dictator ignores human rights laws? (I’ll let you decide which dictator I’m talking about. There are so many to chose from.)

So, it was with all this in mind, that I rolled into the Neuroscience building yesterday. It was dark and dreary outside, and as the rain threatened to fall, I felt some of the wind come out of my sails. I would just do the best I could. And that’s it. I just needed to keep reminding myself of that.

Four hours later, I was feeling exhausted and ready to head home. I was spent. I had been measured, and then measured again. I was able to lift a small weight and move it in a way I hadn’t been able to do before. I couldn’t tear the paper, though, because unfortunately it was done near the end of the assessment when I had begun to tire. Normally, this would have really annoyed me, but I managed to contain myself. I listened to that new voice in the back of my head that said, “Drop the burden, girl. Just do your best.”

And so I did.

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Quarantines, Clipboards & Spinraza #8

I’ve made it no secret that I am abhorrently afraid of cold & flu season. When you have SMA and your respiratory system is total shit, even a simple cold can turn nasty. You know the way a mother’s ear may alert to the sound of a crying child? My ears instead perk to the sound of a cough, a sneeze, and the tiniest sniffle. I can even detect the slightest change in the tenor of a person’s voice that may indicate secret congestion. I don’t mean to brag (okay, maybe I do), but I am the Sherlock Holmes of Cootie-Detectors. I’m so damn good at it that I may know you are getting sick before you do.

When I was scheduled for my 8th dose of Spinraza, I knew that I was entering a dangerous portion of the cootie-season. The post-Thanksgiving/pre-Christmas cornucopia of Microbial Abundance. So, I knew that this year I was going to have to employ every germ-avoiding tactic in my arsenal so that I could receive my Spinraza treatment on-schedule and unimpeded.

Thus, about 10 days before my injection, I announced to EVERYONE THAT I KNEW that I was going into quarantine. Literally everyone. Even the mailman knew that he had better not sneeze on my Christmas cards.

I became a certifiable hermit. I turned down invitations to holiday gatherings. I avoided crowds and eating food that wasn’t prepared at home. I threw away all my romaine lettuce.

The more isolated I became, the more I felt myself turning into one of those wingnuts that sits in their house and angrily begins to believe conspiracy theories that they read on the internet. That the Moon Landing was a hoax… that Barack Obama was born in Kenya… that Donald Trump’s hair is actually grown on his head.

The bitterness became real. And the further I got into my self-imposed exile, the more my anxiety grew about someone breaching my quarantine and infecting me. I knew that if that happened, I was truly inches away from turning into the actual Unabomber. Like I would send a bomb to that person’s house and curse their familial line for 46 generations.

As my procedure day approached, I became more and more tense. Xanax wasn’t quite covering the heights of my nervousness. My left hand began to twitch. Hallmark Christmas movies even stopped having their sedative effect on me.

I simply COULD. NOT. GET. SICK.

It was a horrendous amount of pressure. It consumed me. So, when the magical day arrived, and I woke up at 3:00am feeling okay, I nearly cried with relief.

And then I remembered that my reward was having a five-inch needle poked into my spine.

Strangely, this thought didn’t scare me as much as getting sick did. I think that speaks volumes to the depths of my emotional neuroses… and how bad Hallmark Christmas movies really are.

By the time I arrived to the Neuroscience Center, I was actually calmer than I had been in days. After being settled in my room before the procedure, the resident doctor performing the ‘needle poke’ came to introduce himself. As it’s a teaching hospital, there are different teams of doctors— one resident & one attending. You never know who you will have until the day of the procedure. It’s like playing roulette… but with your spinal canal.

Right away, I had a good feeling about the new doctor. He seemed competent and excited— which jived with my mood of the day. I also sensed his competitive nature when he asked:

“So… uhh…how long did it take them to do this procedure last time?”

While this may seem an innocent question, as a competitive person, myself, I instantly recognized the subtext to his question. What he really meant was the following:

“I want to do this better and faster than last time. I will win this game.”

And, I must say, the guy did deliver. He had the needle in my back so quickly, that I didn’t have time to begin daydreaming about lunch (my favorite way to pass the time). The whole procedure was done in thirty minutes.

As he removed the giant needle, the panicked mania of the past days all seemed worth it to have Spinraza floating in my cerebral spinal fluid like magic minions. But, as exciting as it was, I was ready to go home. To get the hell out of there. I was spent. It had been a tough week trying to not turn into a domestic terrorist.

Just as I was about to get ready to leave, though, a research fellow came into the room with a clipboard. He cheerfully asked, “I’ve got a few questions for you.”

A little voice in my head whimpered, “Oh, fuck.”

A clipboard is never a good sign. If there are enough papers to warrant a clipboard to hold them, that’s too many papers.

I gazed longingly at my wheelchair and wished I was sitting in it instead of the hospital bed. It would be so much easier to zoom away from this man and his clipboard. But, then my conscience reared up and decided that I needed to be the scientifically-responsible person and submit to the questions.

This was a big mistake.

The questions went on. And on. And on.

He asked me questions that I had already answered on previous appointments. Questions that should have already been in their records.

I began to resent his perky face and his clipboard. And his inability to read my medical records. My mind began to race— does anyone take the time to read anything anymore?? Do they??

The minutes ticked by. Finally, he said, “Well, I think that’s it.”

I sighed with relief and gazed at the clock. His survey had literally taken longer than my entire procedure.

Paper rustled on the clipboard, “Oh, wait, I’m missing a page!”

If I could have physically banged my head on the wall in that moment, I would have done so.

“Just a second…”

He located the paper and rattled off a few more questions. I would totally tell you what the questions were but, honestly, I wasn’t even listening by this point. He was like the teacher in Charlie Brown— all sounds, but no actual words.

When he finally went away, my nurse came back in, “My goodness, I thought that was never going to end.”

I burst out, “I know, right??? Holy crap.”

She clapped her hands together, “Let’s get you out of here, shall we?”

“Yes, please. I’ve got some Hallmark Christmas movies to watch.”

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August Awareness and Lots of Stretchy Pants

August is known for many things. Even though most summer vacations are ending, it’s still a month when the heat is blistering and the yucky air has that palpable, tangible quality… like an dog’s fart. Also, schools open this month and all the pumpkin spice food products will begin to appear in stores.

If you think it’s too early for it, you aren’t alone. Even though I have a well-developed love for pumpkin spiced lattes, I don’t want to drink one while it’s still 100 degrees outside. I don’t care if there’s a chance that my sweat could smell like an autumnal wonderland. It’s still not worth it.

August is also Spinal Muscular Atrophy Awareness Month. The month that celebrates and brings awareness to the 1 in 10,000 babies born (including me!) with a really peculiar— and often deadly— genetic glitch called Spinal Muscular Atrophy (SMA). While the condition is rare, 1 in 50 people running around this earth are actually genetic carriers. But, since it’s a recessive condition, it takes two of these carriers getting together to produce a child with SMA. Even then, only 25% of children of those unions will even have the condition.

If you are confused by this scientific explanation of recessive genetics, you weren’t fortunate enough to have had the late Mr. Eugene Field as a biology teacher at Patterson High School. I feel sorry for you if you missed out on his amazing greatness. I guess you’ll have to make due with looking it up on Wikipedia, instead.

Anyway, until very recently, a diagnosis of SMA was practically a death sentence. While advancements in medical care have allowed many of us to beat the odds and thrive long into adulthood, there was really nothing science could do to treat the condition itself. But, that is changing. For the past year, I’ve been receiving a gene-splicing treatment called Spinraza that boosts my production of a protein that my body is lacking. I’ve written extensively about my treatment journey on this blog. I’m happy to report that more treatments for SMA are on the horizon in the coming years, too.

Awareness months, like this one for SMA, serve an important purpose. They garner attention to the cause and provide a catalyst for fundraising. Many other medical conditions and diseases have their own awareness months, too. I’m sure many of you have taken part in such events. And that’s fantastic. After all, these are vital tools for generating donations for research. But, while raising money for a “cure” is very important, we mustn’t forget that we have to also support those living with these conditions, too.

We have to make sure that there are services and infrastructures available to help those currently living and fighting these conditions— and their families. As someone on the other side of this, I must say that I occasionally cringe a little when I see so many fundraising efforts with simple, pithy titles about running and walking for cures. Sometimes I feel like they miss the point. After all, before we can get to the moment when someone could possibly be “cured,” there will be a lot more time spent with the person needing support and helpful resources.

Let’s be honest, from a practical point-of-view, a “Walk for a Cure” isn’t really going to do a patient much good if they don’t have a way to get to their doctor appointments. Let alone if they don’t have personal care assistance in their homes or even nutritious food on their table.

And, I don’t mean to be a killjoy, but you know that 5k Run for Cure you did last year? The one where you wore those new $85 running pants from Lululemon? Yeah, that run is probably not going to help that rural woman in Kansas afford the pharmacy copay on the anti-nausea pills that she needs during chemo.

I don’t mean to imply that these fundraising efforts are useless. Nothing could be further from the truth. These events and funds are incredibly important. So, keep on running in your crazy expensive stretchy pants. But, while you’re doing it, remember that there’s more that we can do.

Awareness means more than a “cure.” It’s about living in community— together. Helping each other. Whether it’s August, or any other month of the year.

Until then, Happy August… and happy running.

xoxo

Happy Spinrazaversary to Me!

One year ago, today, I had my very first spinal injection of Spinraza— the first-ever FDA approved treatment for my disability, Spinal Muscular Atrophy (SMA). Life was a lot different one year ago. First of all, I was worth a hell of a lot less money at that point. My spinal fluid didn’t have 6 vials of super-sonic, super-expensive Spinraza floating around inside of it. You know, the way the miniaturized Dennis Quaid floated through Martin Short’s body in the 80s movie, Innerspace? One year ago, I was a body that was decidedly pre-bionic. Dennis Quaid’s tiny spaceship would not fly out of my nose if I sneezed. Now, however, I wouldn’t be surprised if my boogers had diamonds inside. Yes, I’m that pricey now.

As I have shared here previously, it was a long, hard-fought battle to gain access to this drug, and I’m lucky to have a spectacular medical team at Stanford Neuroscience that helped to make this possible. I wish I could say that all adults with SMA have such outstanding advocates for care as I do. But, we still have a long way to go to make this current treatment, and all the upcoming treatments coming down the pharmaceutical pipeline, available and accessible to all those living with my rare, genetic condition.

But, my Spinraza journey didn’t end at the point of that lumbar puncture needle one year ago today. Rather, it really had just begun. Given the complexities of getting the long needle through my crooked, and fused anatomy, each injection since that July day has been a tiny battle of wills. A mental and physical game where I prepare like a seasoned warrior. A soldier that knows that the upcoming battle could be a smooth victory just as easily as it could be a giant shit show. You know, like a Trump/Putin press conference?

However, these hardships (and there have been many!) have been worth it. In the 365 days since that magic vial’s liquid have begun to do their work, I have had measurable improvements. Given that this neuromuscular disability is progressive, even merely slowing or halting the natural deterioration is a victory. To have improvements, like I have seen, is more than I could have hoped to achieve. Especially as an adult with SMA. I had never thought I’d live to see a treatment that could help me. It’s hard to mentally process… to put your brain around. You know, just like it’s hard to process pickle-flavored ice cream, self-driving cars, and why the hell we Americans can’t figure out the metric system.

I look forward to what the future holds for my Spinraza journey, yet, I eagerly anticipate what medical science has in-store for those of us, of all ages, with SMA. I’ve heard that there are more treatments currently in the trial and research phase. Perhaps, one day, I will have additional cause to celebrate.

Until then, if I sneeze, please excuse the diamonds.

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Test Anxiety

No one likes tests. Whether they’re in school, at the doctor, or at the DMV— they are generally un-fun. You rarely hear someone yell, “Yay, a test! I’m SO happy.” If a person did say that, you’d probably question their mental stability.

For a lot of folks, tests bring out an anxiety— a stress to perform well, which, ironically, is made harder by the stress itself. It’s a terrible Catch-22.

I have to do well on this test or I’ll never go to college!

I have to pass this exam or I can’t get my license!

Will that marijuana I smoked a month ago show up on this urine test!?

As a worrywart, high-achieving student, I generally would experience some anxiety before tests, especially the big exams— like the AP test, the LSAT, and all those personality tests on the internet. I’d fret for days beforehand, wondering how it would all turn out. Would I score well enough on the LSAT to get into law school?… Would the online test sort me into Hufflepuff or, worse yet, Slytherin House?!

These thoughts would consume me.

It shouldn’t be surprising that when it was time for me to have another evaluation to check my progress on Spinraza, I worried about it. A lot.

While I had felt positive changes, and experienced measurable improvements previously, would it still translate to results this time?

It was a question that loomed over me… like the Hindenburg right before it exploded.

I’m sure reading this, you’re probably thinking, “Girl, calm down. Don’t stress. Just do the best you can.”

I wish it were that simple. Given the high price tag associated with nusinersen treatments, there are many insurances and government agencies that are looking to limit who has access to the drug. They want to put parameters on who can get it and who can’t. And a major factor they are looking at is age.

As an adult in my thirties with Spinal Muscular Atrophy, I am considered old. Not old in the way that Betty White is old, but at least old in a moderate way… like Jane Fonda or Donald Trump.

While there aren’t THAT many of us that have lived this long with SMA, there are still plenty of us adults out there that need access to this drug. So, we have to continue to prove that this treatment works for adults. That it produces results.

That’s a lot of pressure. Especially for something that a person can only do SO much about. I can do stretching, breathing exercises, and increase my protein to help things along, but that’s about it. I mainly have to see if the magical Spinraza droplets do their work.

Leading up to my evaluation at Stanford earlier this week, I was very anxious about it. On the drive over, I listened to the Spinraza mixed CD I had made and tried to gear myself up. It worked pretty well… after all, track #2, Eye of the Tiger, is always a solid choice.

Upon arrival to the Neuroscience Center, I only had time to inhale half of a tuna sandwich before they called me back to begin my evaluation. The next three SOLID hours passed in a blur of respiratory and physical therapists, nurses, research assistants, and stress sweat (good thing I put on extra deodorant!).

I wasn’t finished with one test before another person was hovering nearby to begin the next. I didn’t even have time to eat my homemade graham cracker and peanut butter sandwiches. (And you know how much I love peanut butter!)

The grueling afternoon reached its peak when the physical therapist asked me to open up one of those clear round Ziploc containers with the blue lid. Previously, I hadn’t even been able to attempt this task. Not even close. But, this time, I felt that I might be able to do it. I pulled, groaned, heaved, and nearly cried. But, after five minutes of desperately trying (and nearly doing it), I ran out of steam. I felt defeated. And pissed off. I told the PT, “I’m gonna buy one of these fucking containers and practice this at home. Next time, I will do this.”

Yeah, I’m that kind of person.

While that moment was very disheartening, I’m happy to say that my results showed some improvements. I was able to lift a cup with a weight inside all the way up to my mouth. The strength in my arms and hands increased. And, lastly, but most awesomely, there’s a respiratory test that measures the diaphragm muscle. Before Spinraza, I got a 50. At this evaluation, I got a 72.

By the time all of this was done, I was exhausted. I wanted to curl up in bed with hot chocolate and watch TV forever. All the shows. Even the stupid ones on Bravo… Like The Real Housewives of Beverly Hills.

Thankfully, I get a little break now. I don’t have to head back to Stanford until next month for dose #6. I’m looking forward to the respite… and the break from all these tests.

Although, if I get bored, I’m sure there is a personality test online I can find. Like… If you were a dog, what breed would you be?…

A border collie. Definitely.

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Boogers, Rainy Days & Surviving Dose #5

It’s been well-documented that, like many with Spinal Muscular Atrophy, I’m a verifiable germaphobe. If a person could earn a certification in Cootie-Evasive-Techniques, I’d have a damn Class A license. The kind that people stand in line at the DMV for 3 hours to get.

Strike that. There’s no way in hell that I could go to the DMV and stand in line for three hours. I once heard someone cough up their gallbladder at the DMV. There are enough germs in those offices to keep the Centers for Disease Control busy for at least 6 or 7 months. Eww.

So, yeah.

Anyway, if you’ve been following my Spinraza journey, you’d know that the date for my first maintenance dose had been fast approaching. Given the importance of these spinal injections, and the set schedule of receiving them, it’s vital that nothing interfere with a dose.

Nothing.

Not even a plague of locusts, a doomsday asteroid or a Buy One Get One Free sale at the GAP.

Seriously. I don’t care how much I love those long-sleeve tees.

Given that we’re in the midst of a raging cold & flu season, I’d been paranoid that I would catch a bug prior to my Spinraza treatment. This fear paralyzed me for weeks. I avoided going out in public places, I tried limiting my interactions with family and friends. I basically became a hermit. Like the Unabomber. Only I didn’t try to build explosives out of fertilizer, chicken wire and cherry-flavored bubblegum. (Yes, I remember the show MacGyver.)

But, then, Christmas happened.

And, I really, really like Christmas.

You can probably guess what happened next.

Yup— I caught a respiratory virus.

The symptoms started about 10 days before my Spinraza injection. It wasn’t long before I had a river of fluid coming out of my nose. I’m sorry to be so graphic, but I have very little filter when it comes to these things.

After consuming my first box of Kleenex, I began to notice something. I felt a burst of joy— and not all of it was because of my low-grade fever.

You see, I was able to blow my nose harder than I had been able to do for a very, very long time. Like years. At least since the Bush Administration (the 2nd dude, not the 1st dude). I was able to blow so hard into the Kleenex that my ears actually popped.

This may not seem like a big deal, but before Spinraza, I couldn’t do this. Nasal drainage would simply slither down into my lungs, and I would struggle for weeks to get all the damn stuff out. This was a striking difference.

This development invigorated me. I wasn’t going to let this piece-of-shit virus get in the way of my Spinraza treatment. After all, I clearly needed more of the stuff so I could keep ejecting all those boogers out of my nose.

So, armed with antibiotics, breathing treatments, and a fuck-ton of garlic (seriously, I smelled SO bad), I made it through that week to Spinraza Day.

The early morning arrived and we were greeted with the first rainy day in, like, months. So much rain hit California that all the dirt decided to just turn into rivers and carry folks away. Not cool.

Luckily for me, even though the visibility was dodgy at times, we slowly made our way over to Stanford— safely. Upon arriving, I began to get nervous.

Could I sit still during the procedure without having to cough or blow my nose? After all, moving or twitching while two doctors inject a giant needle into your spinal fluid really doesn’t sound like a good idea. Just like buying sushi from the back of a van isn’t a good idea.

After checking-in and getting settled into the room, the nurse told me that the Spinraza Gods had blessed me once again. The same amazing duo that did my last procedure were back for the day. It felt like the rainy heavens had opened up and a damn Puccini opera was playing just for me.

YAAAASSSS!

In case you’re wondering why I was so excited, here’s the deal: there’s no way to know which doctors will be on-call that day. Stanford is a teaching hospital, so the rotations are random and unexpected. The time this procedure can take varies widely— depending upon the doctors. The longer the procedure takes, the more painful and arduous it becomes.

And these two doctors didn’t disappoint. They had the needle in so quickly, that I didn’t even have time to cough or blow my nose. It was glorious.

The rainstorm continued on the drive home, but I was so delirious with relief that I didn’t even mind. I was exhausted. Spent. Relieved. And, yes… slightly full of snot.

I get a little break before my injection, so I will enjoy these months— hopefully without viral invaders.

Wish me luck!

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Dimensions, Dictators, and a Whole Lot of Weed

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

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

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

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

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

Seriously.

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

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

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

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

So, I suppose this is an improvement.

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

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

Just watch out for those black holes.