Masks, please.

This blog post will be a little more serious than my usual ones. There will be fewer cuss words, fewer jokes about politicians, and fewer references to pumpkin spice products. But, please keep reading, anyway.

A few weeks ago, I was invited to speak at a conference about my disability— Spinal Muscular Atrophy. This conference is a way for medical professionals to learn the newest research and collaborative ways to improve patient care. As a person with SMA, they wanted me to offer my perspective and feedback to the attendees.

I quickly agreed. Highlighting the lived experiences of disabled people is something that is often missing from events like these. Nothing annoys me more than having nondisabled people talking about how best to “care” for us without any effort to elevate our disabled voices. So, any chance I get to promote disability awareness– I am definitely going to take it.

So, I was all-in on speaking at the conference. After all, the knowledge that I have as a person that has lived with SMA my entire life is just as valuable (if not more so) than a doctor with four framed degrees on their office wall.

I started planning what I would say. What to wear. What topics seemed important to me. How I needed to make sure not to use any four letter words– which I tend to do, even when I don’t mean to. (I’m working on this. Really.)

However, after I happily agreed to participate, I found out something that changed everything. Masks were voluntary at the conference— they were not required. And the only way I found this out was that I had to directly ask an organizer about this safety protocol. If I had not asked, I would have shown up to the event unaware of this.

So, in summary:

A medical conference about Spinal Muscular Atrophy (one of the most complex, deadly genetic diseases in existence) seemed to be okay with not making masks a requirement to attend their event.

The reason for this decision? The conference organizers didn’t want to “impose additional requirements” on attendees. (The targeted demographic for the conference being medical professionals— who are nondisabled).

Yet, it didn’t seem to register that they were now hosting an SMA conference where an actual SMA person doesn’t feel comfortable attending.

Now, I know I’m not in PR, but this doesn’t have good “optics.”

In addition, many of these medical professionals will interact with high-risk SMA folks regularly. They don’t think a masked event is a good idea? I’d think the only thing they’d want to bring home from the conference is a free T-shirt and a stale poppyseed muffin from the breakfast bar— not an infectious disease.

To be perfectly honest, when I learned this, my stomach dropped. I felt nauseated. I felt tears prickle the corner of my eye. (It’s ableism at its most basic— prioritizing the wants/whims of the nondisabled to the disabled’s exclusion.)

Yet, a part of me was not surprised given how the world is trying so hard to return to normality. But, for those with SMA (and other disabilities) we’re endangered when society tries to act like the last 2.5 years didn’t happen.

Disabled people, especially disabled people of color, have had the highest mortality rates for COVID-19— and the lowest access to health care. This pandemic has been real to us in a way that it hasn’t been for other people.

The trauma of this continues for us each and every day. Yet, while society is now trying to “live with the virus,” disabled people are the ones more likely to die from it.

As a result, within the disability community, masking is seen as a form of accessibility. Like elevators, ramps, ASL interpreters, closed-captioning and large-print. Masking (along with testing and vaccines, of course) allows high-risk people to participate in society more safely, securely and fully. It is a way to say, “I care about you. I see you. I want you here.”

Therefore, despite my initial enthusiasm, I won’t be attending this SMA conference. I am disappointed and sad. Disability can be isolating, and it cuts a little deeper when society displays such obliviousness.

So, I am left with this final suggestion: if you are planning a public event, please consider the disabled when you do so. By going beyond the basic policies of your city/school/company, you can ensure your event is more accessible and inclusive. (After all, those policies were made for political/economic reasons that suit the nondisabled masses. Not people like us.)

Don’t forget: the disabled will never be able to take a seat at the table unless we feel safe to do so.

It’s up to you to help make that happen.

UPDATE: Upon reading this blog post, conference organizers changed the policy to make masks mandatory. As a result, I decided to participate in the event. While I am happy they reconsidered, I’m also disheartened by the lengths that disabled people like me have to go simply to have our needs met. This extra labor is exhausting— especially when many of these accommodations should already be in place. —E.

A Time Traveler’s Guide To Judgment Day

Since beginning my Spinraza journey over two years ago, I have been exposed to some pretty high-level scientific stuff. The Principles of Motor Neurons. Genetic Sequencing. Genetic Manipulation. And a whole bunch of really smart people geeking-out on how to turn cripply SMA people like me into X-Men.

People often ask me what it’s like, during this stage of my life, to describe what Spinraza has meant to me. What it has done for me. Most of the SMA patients out in the world are adolescents, children. Many are babies. After all, only a small portion of those with SMA make it into adulthood. So, those of us that are adults can give a perspective that children simply aren’t able to do. We have more life experience, and frankly, we’ve got the big-adult vocabulary to articulate what this is really like. We’ve got detailed analyses with really large words. SAT words. Oxford Dictionary words.

So, do you want to know what it’s really like?

You know the classic H.G. Wells novel, The Time Machine? The story that popularized the concept of time travel and launched an entire genre of science fiction? When Wells first published the book in 1895, it sparked the imagination of millions. Over a century later, the concept of time travel is still intoxicating.

Everyone (including you!) probably has a secret idea of what they’d do if they had the opportunity to travel in time. While most would do something selfish, like get winning numbers from a future Powerball drawing, some folks would do something more altruistic. Something that would benefit mankind… like killing Adolf Hitler or castrating Harvey Weinstein.

But, all these scenarios are fictional. Because time travel is impossible. Right?

Well it is… Unless you’re me.

If you really want to know what the Spinraza process has felt like to me— what is has meant to me— this is the way I describe it.

I am now a time traveler.

A time traveler that is revisiting a part of her life that she thought was gone forever.

Spinal Muscular Atrophy has always been a progressive condition. I was stronger at 5 than I was at 15. At 15, I was considerably stronger than I was at 35. And, by 55, there was a good chance that I would be dead.

But, with Spinraza, a crazy thing happened. The mad scientists at Stanford thrust me into their Interventional Radiology room— injected some liquid into my spinal fluid… and turned back time.

I began to live in own past. I could do things that I hadn’t been able to do in years. I was living in the past. Just like H.G. Wells wrote about 124 years ago. And, just like the novel, it’s been a wild, and frankly exhausting, ride.

It’s important to note that time travel isn’t free— and Spinraza is one of the most expensive drugs in the world. So, to continue to qualify, I must undergo thorough physical and respiratory evaluations every few months to measure my progress.

Given there aren’t many adults like me receiving the treatment, the data gained from our experiences is especially valuable. These measures and outcomes will hopefully offer the evidence needed to expand Spinraza access here in the US, and around the world. I want more time travelers with SMA. But, to do that, we’ve got to illustrate the benefits with cold, hard data.

No matter how you slice it, though, this added pressure is intense. While I’m constantly told not to be anxious about it— to simply do my best— I do worry about it. I fret about the messages I get from people with SMA around the world longing for treatment. I fear they won’t get to travel back to their past.

I feel guilty that I do.

Not all of us long to do something bold with a time machine. We don’t all want to be heroes that shoot Nazis. Some of us just want to travel to a time where we could talk a little louder… laugh a little harder… and pick our own boogers.

A few days ago, I had yet another full day of these assessments. It’s a day that I truly dread. Each time. It’s long, exhausting, and I usually always have at least one moment when I long to scream or curl into a corner and cry.

I care so much about these tests that it’s almost all-consuming. I do frequent physical therapy in preparation. I have even sought support from a counselor to work through the emotional challenges of this arduous process. These assessment exams are often all I can think about. Literally. All. I. Can. Think. About.

It’s like how Donald Trump must feel about Twitter.

I came into this assessment day— judgment day, frankly— feeling unsure. Feeling like perhaps I had plateaued. That I hadn’t noticed a change from the last time. I was resigned to this idea. I accepted it. It was okay (and expected) to have a plateau because SMA, by nature, is progressive. Stabilization is a win, too. Stabilization means more time available in the time machine.

But, I had a surprise. My respiratory measures showed additional small gains. My physical assessment was the most solid— yet.

The time machine was still working.

While I made it through this judgment day, in six months, I will have to do it all again. And again… and again. Because, in some way, time still manages to march on.

But, I’ll be waiting for it.

“Looking at these stars suddenly dwarfed my own troubles and all the gravities of terrestrial life. I thought of their unfathomable distance, and the slow inevitable drift of their movements out of the unknown past into the unknown future.”

— H.G. Wells, The Time Machine

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84 Minutes… and Counting

I recently got a letter from the Social Security Administration asking me to participate in the National Beneficiary Survey (NBS) for those on Social Security Disability. The supposed goal of the survey is to improve programs that Social Security offers and to see how existing services are being utilized. I was “randomly selected” to participate in the anonymous survey. I think this is meant to make me feel special. As if I’ve won some kind of prize— like a free donut.

According to the letter, the policy research company tasked with facilitating the NBS offers a $30 gift card to participants who complete the phone survey. This line caught my attention because I like gift cards. And the fact they were going to give me a gift card for giving my opinion was very enticing. I am a person with many opinions. Further, giving suggestions and feedback is one of my favorite things to do— even when my opinion isn’t wanted, needed, or welcome. So, the fact that they wanted me to give my opinion made this a Win/Win situation for me.

So, I gave them a call. After waiting on hold for a few minutes, I was informed that the survey would take around 50 minutes. That’s a lot of time. And it’s especially a lot of time when you’re a disabled person like me that takes nearly 2 hours to get up, showered, and dressed each morning. But, I did have opinions to share. Lots of them.

I told Lucy, the survey company employee, to proceed. She began by asking me about my disabling condition, which then resulted in me having to spell out Spinal Muscular Atrophy because no one knows what the hell it is. Then, she asked:

“When did you first get a disability that inhibited your ability to work?”

“During the Reagan Administration.”

“Pardon?”

“I mean, I suppose it wasn’t Ronald Reagan’s fault, directly, but I born and diagnosed with SMA while he was president.”

As the questions proceeded, it was very clear that the survey wasn’t written with people like me in mind. This is odd because I’m über-disabled. If you had to give an alien from outer space a stereotypical example of a disabled person, I’d be your girl. But, this doesn’t mean that Social Security sees it that way.

“When do you believe that your disability will improve enough for you to get off of SSDI?”

“Not even the Vatican can make something like that happen.”

“How about in 2 years?”

I launched into a detailed genetic description of SMA, not fully satisfied until I could hear her nails clicking speedily on the computer keyboard in the background. I was determined to make my survey answers the most thorough and detailed she had ever encountered– like damn Tolstoy’s epic War and Peace. I wanted every word I said entered in the record. I was going to earn that $30 gift card.

“What could we do to help you return to work full-time? Job training?”

“I don’t need job training. I have a university degree. I graduated with honors. The main problem here is that Medicare & Social Security Disability Insurance need to cover home-based personal care attendants. The barrier of inadequate home-based care is what prevents many of us with the skills and abilities to work from doing so. How can we work if we don’t have someone to help us get dressed? How can we work if we don’t have someone reliable to drive us there? The faulty poverty-driven Medicaid system inhibits us from bettering ourselves by punishing us for even small advances we make by stripping away the meager benefits we receive. This needs to change. You want more disabled people to work? Make home-care coverage part of the Medicare program. I guarantee you’ll see results from that investment.”

“So, would a job coach help you return to work?”

I began to feel a headache nag at the corner of my temple. I began to seriously regret doing this survey. Just like a woman always regrets getting bangs. While at the salon you think it’s great, you’ll inevitably realize that those bangs will never resemble the rest of the hair on your head. This survey was turning out just like that.

Then, Lucy began asking me about my physical abilities and other aspects of my life. And that’s when the survey became more interesting.

“Can you climb a flight of stairs?”

“I assume you mean without Inspector Gadget’s help.”

“Can you stand and bear weight?”

“Nope.”

“When was the last time you could stand and bear weight?”

“Please don’t make me bring up Ronald Reagan again.”

“Have you served in the Military?”

“HAHAHA! Wait— is that a serious question??”

“Have you participated in the Ticket to Work Program?”

“No. But, I want to talk about the military thing some more.”

“Umm— okay.”

“Can you imagine if I went into a military recruiter’s office and tried to enlist? They would freak the hell out.”

*The sound of Lucy’s typing ceases and she begins chuckling.*

“I should do it. I’m really good at driving my power wheelchair— which is operated by joystick. Therefore, I could pilot a military drone airplane without a problem. So, that means that I could kill innocent civilians just as easily as an able-bodied person… maybe better.”

I glanced at the clock. I had been on the phone for 84 minutes—and counting. The questions went on for a few moments longer, but my attention started to fade until the very last question:

“Is there anything else you’d like to add?”

“Yes, actually. And I want you to write this down. Are you writing this down?”

“Yes.”

“There’s a bill in Congress right now that could address some of the issues I’ve raised in this interview. It’s called the Disability Integration Act. H.R. 555. Those of us with disabilities want to live productive and independent lives. Please help us make that happen.”

She thanked me for participating in the interview, and wished me all the best. Then, she asked me if I wanted the $30 gift card mailed to me, or someone else. Someone else?? I actually had to have her repeat the question because it was so insane.

Does anyone spend 84 minutes on a survey and then give the only good part about the entire experience away to someone else? No. Definitely not. I may be disabled, but I’m not a communist.

Ronald Reagan would be so pleased.

Red Tape, Duct Tape & The Wheelchair Vortex

When you’ve got a rare and complex disability like Spinal Muscular Atrophy, time can be a very precious commodity— like diamonds, Labradoodle puppies, or a really good Wi-Fi signal. Since many everyday tasks can take us longer to complete (like showering, eating, and getting dressed), we tend to budget the rest of our time wisely and carefully. It’s an important skill to cultivate when you have a disability— otherwise you’d never get that term paper done, never balance your checkbook, and never have time to buy laundry detergent at the store.

Along with these everyday tasks, life with a complex disability comes with a lot of hidden bureaucratic demands that take up time. Between filling out on-going forms for Social Security and state services, to coordinating between various caregivers, doctors and therapists, to following up on medications and equipment, it’s a vortex of bureaucracy that keeps our lives in motion.

The time it can take to attend to this red tape is daunting. You know the hours it can take to prep and cook an entire Thanksgiving dinner? That’s a LOT of time, right? Imagine having to do that several times a month— even when you’re tired, sick, or just want to lay in bed and watch people get decapitated on Game of Thrones. That’s what it is often like for us. Plus, we don’t even get any leftover turkey out of the deal (which is totally lame because I love leftovers!).

Recently, I’ve had an extra bundle of fun (can you sense my sarcasm?) added to my usual list of bureaucracy. This task is at the top of my list of The Most Dreaded Of All Tasks. In fact, I daresay it is the veritable Mount Everest of tasks a cripple like me must climb. And I don’t say this lightly because I literally can’t climb anything at all.

It’s the Trying-To-Get-A-New-Power-Wheelchair-Vortex-Of-Hell.

Sure, it might sound exciting to get a new custom power wheelchair— to get a bright shiny new model with fancy features and leather upholstery that smells like a pretentious Italian shoe. But, a new wheelchair ain’t a Prius (even though it can cost nearly as much). They are also not so interchangeable as to be simply a matter of budget, taste and style.

As I’ve written on this blog before, these wheelchairs are built to our specific measurements and our medical need. They are designed to maximize independence and health. They are the very tool of life. And, when you have a complex disability like SMA, a small change in a wheelchair design or an error in a measurement for the seating can mean the difference between sitting comfortably to having a pressure sore on your buttocks. It can mean the difference between being able to drink a glass of water independently to not being able to reach the table at all. So, a lot can ride on these devices and making sure they are fit appropriately.

That’s a lot of stress— and a lot to add to our already-busy plate. So, it’s not surprising that we’re often not eager to get a new wheelchair when we’ve still got one that works well. But, as time goes on, it can become difficult to get parts and do repairs. Plus, I can’t speak for every disabled person, but I’d rather not go too long with a decrepit wheelchair that’s only held together by loose wiring, duct tape, and human stubbornness. (I think we’ve got enough of that in the White House).

So, every 7 or 8 years, I begin this process anew. Given the price tags on these custom wheelchairs, insurance companies don’t make it easy to qualify for coverage. There are many hoops that a person must jump through to get a power wheelchair. This is ironic because most of us that need these devices literally can’t jump at all.

First, my doctor must reestablish my need for a power wheelchair and reverify my disability. This is despite the fact that I’ve been this way for 37 years. It’s as if they fear that my genetic condition is going to suddenly disappear into thin air— like Lori Loughlin’s career.

Second, I must be seen by a special neuro physical therapist that will do a head-to-toe functional assessment— which must corroborate my doctor’s findings and include an analysis of whether my mental state is good enough to operate a wheelchair without driving myself off a cliff.

(Even though this process makes that very, very tempting.)

After that, my doctor has to verify the special neuro-physical therapist’s evaluation and that it’s in full agreement with his recommendations. He then sends it all to the DME company, which is in charge of fitting me for the new wheelchair and making sure it’s functional.

I’m fortunate that I have a long and positive relationship with my local DME (durable medical equipment) provider, Alan. He has been very accommodating and helpful to me over the years. He’s put up with my tears of frustration, my harassing phone calls, and my complaints about how ugly all their wheelchair paint color options actually are.

Insurance companies don’t make this process easy for DME providers, either. To be frank, they make it a giant pain in the ass, which has driven many DME companies out of business, which in turn makes it hard for disabled folks like me to get our rickety wheelchairs repaired or replaced.

It’s the red tape nightmare that just keeps on giving— like a bad case of shingles.

Anyway, Alan came over a few days ago and we came up with an initial plan for my new wheelchair. This plan basically consisted of me pleading desperately:

“Alan, make the new chair exactly the same as this chair. Seriously. Like not even a centimeter difference. Okay? You know the old fable, ‘The Princess & The Pea?’ I am the Princess. Trust me, I will know if there’s a pea under the mattress…..Umm, why are you laughing? I’m not kidding. I am the Princess. There will be no PEA in this WHEELCHAIR!”

Realizing I sounded slightly deranged, I coughed, and added:

“By the way, would you like a cup of coffee? Perhaps you might need it now.”

And that’s basically how that initial consult went.

It’s now up to Alan and his team to gather all his notes, the paperwork from my doctor, the assessment from physical therapist, and to combine it all together to submit for insurance approval. Every “T” must be crossed, and every “i” must be dotted. If it isn’t, I will have to begin the process again— the idea of which makes me want to cry.

Here’s hoping the duct tape will hold out until then, though.

Wish me luck — And stay tuned for updates!

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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.)

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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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Further Adventures in Physical Therapy

A few months ago, I reentered the world of physical therapy after a 20+ year hiatus. Sure, I’ve had the occasional physical therapy assessment during the course of my Spinraza treatments for my Spinal Muscular Atrophy the last couple years. But, nothing on a regular basis since childhood. Over twenty years ago. I know this hiatus was long. Like really long. The kind of hiatus you usually only find in the careers of really famous people. Like Barbra Streisand. Or Betty White. Or, Roseanne Barr before she dumped her career in the toilet again.

I admit, of course, that I brought a lot of emotional baggage with me to my return to physical therapy. I had a lot of painful ghosts in my closet that I didn’t enjoy examining again. (Just as I don’t like remembering all the baggy printed sweatshirts I wore in 1993.) But, I suppose that despite all of that, the important thing is that I returned to physical therapy in the first place. Even if it took a long time. (There’s no forgiving those ugly sweatshirts, though.)

Since the autumn, I’ve been going to sessions with a local PT once a week. Given my eccentric crippliness, I’ve been an academic challenge for my physical therapist, Robbie. Spinal Muscular Atrophy is not a run-of-the-mill knee replacement. Or a case of whiplash. Or a strained shoulder. It’s an all-over-the-body kind of weirdness. It’s like trying to teach music to a kid that can only reach 12 of the 88 keys on the piano. Coming up with a song to play under that kind of limitation takes some skill and imagination. It goes without saying that Mozart definitely won’t be in my repertoire. But, I’d settle for a little… Do Re Mi, maybe?

Luckily, my PT has risen to the occasion. He did some research. He thought outside the box. And, he developed a therapy program that is as eccentric and odd as I am. It features a random assortment of objects and tasks— seemingly like some shit he just grabbed from his junk drawer at home on his way to work. After all, everyone has a drawer like this at home. The kind with paper clips, dried pens, rusty green pennies, and silly putty from 1987.

From the look of my therapy table that first day, though, Robbie’s junk drawer had a different collection of crap. Wooden clothespins. A small cloth Easter basket with a yellow chick on it. A rubber tube that resembles a giant Twizzler. And a travel coffee mug. Junk, nonetheless, but higher-quality junk than I tend to find in my drawer.

Anyway, each session he lines up the clothespins on the edge of the Easter basket and has me pull them off one-by-one. This may sound easy, but I’d like to say for the record that it is not. A surprising number of muscles have to work together to pinch a clothespin, remove it from a rim, and then place it inside a basket. So many tiny muscles that don’t always want to work in unison. It’s hard. I have dreams about these clothespins and that damn Easter basket. The perky baby chick taunts me when I fail. It’s very annoying.

Luckily, the other tasks aren’t so aggravating. We do range-of-motion stretches and exercises to strengthen my torso and neck. Then, we do bicep curls with the giant rubber Twizzler. At the end of the session, I practice lifting the travel coffee mug to my mouth to simulate taking a sip. Robbie puts water in the mug beforehand— but he doesn’t tell me how much so I get challenged by the weight.

Some folks may lift hefty barbells… but, it seems like I can lift an $11 dishwasher-safe container.

The other day, though, my PT had an addition to our session. To work on my neck and torso muscles further, he rigged a red laser pen light on the bill of a baseball cap. He put the cap on my head and then put a bullseye target on the wall. Keeping my posture as straight as possible, I aim the laser onto the target, and work to keep it there as long as I can. It’s way more challenging than you’d think. Imagine a low-tech version of Nintendo’s Duck Hunt. Yet, it’s just as satisfying. Plus, there’s no pixelated dog on the screen to smirk when you lose.

I look forward to seeing what else is in store in the coming weeks. This overall experience has been more fun than I expected at the outset… and not just the moments when I threaten to blind Robbie with the laser. It’s also been challenging, but in a good way… a competitive way. Like when I read War & Peace by Leo Tolstoy a few years ago. It was long and massive, but when I finished it, I was proud of myself. I gave a cry of triumph, “I MAY NOT BE STRONG ENOUGH TO LIFT THIS DAMN BOOK… BUT, I READ IT, ANYWAY. HA!”

If all this wasn’t enough to recommend this experience, though, I’d also like to report that my physical therapist has noticed some subtle improvements as I’ve progressed through the last few months— which is really encouraging. My arms stretch higher. My neck muscles hold longer. And I can lift a whole lot of water in that mug. So, stay tuned for more updates in the coming weeks…

Until then, watch out for my red laser!

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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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Beyond the #PAWECMHAA

The other day, our heater was on the fritz for about 48 hours. Under normal circumstances, this would not be that big of a deal. You could just throw on an extra sweater or drink some warm tea. You could snuggle up on the couch with your cocker spaniel or a random stranger. Whatever you’re into, I don’t judge.

Or, perhaps— more likely— you’d say to me, “Get a grip, girl. You live in California. The gallon of milk in your refrigerator lives in a colder climate than you do.”

When you have a disability like SMA, though, this isn’t so simple. Because many of us are in wheelchairs, our circulation isn’t the best. So, our bodies are often super sensitive to fluctuations in temperature. Putting on more layers of clothing can also become physically cumbersome. Meaning, if I wear that giant Christmas sweater on top of the thick fleece hoodie I bought at GAP, there’s a good chance I won’t be able to move my arms to do pretty much anything. Like grab the phone to call for help. Or, eat a bowl of warm soup. Or, even effectively pick my nose. I would be like the crying little brother in The Christmas Story that flails around in an immovable snowsuit. It wouldn’t be pretty.

So, when my heater went out, I quickly thought, “FUCK, this isn’t going to go well for me.” I immediately messaged the HVAC repair man and he said he’d try to make it out in the next couple of days. Then, I quickly consulted my weather app while I still had enough warmth left in my fingers to operate my iPhone. It said that we were in for a stretch of below-normal temperatures— lows around 34°. I felt my weak little muscles shrivel at the numbers— just as an insecure man’s penis shrivels up in the presence of a confident and capable woman. (I could easily make a reference to Donald Trump at this point. But, I won’t.)

Anyway, I can hear non-California residents guffawing at this forecast. And, before you claim that even hairless kittens can survive that kind of cold, I’m going to tell you that I really don’t care. IT’S STILL COLD!

At that moment, it became a race against the clock— to see how much I could get done before the temperature inside my house dipped too far down. To the Point-At-Which-Elizabette-Can’t-Move-Her-Arms-Anymore. Also known as its scientific acronym, PAWECMHAA. If you are curious, this measure is roughly around 56° Fahrenheit. So, in a whizzing flurry, I dashed around my house doing all the things that I had been procrastinating from doing for a while. I balanced my checkbook, updated my Christmas card list, wrote some emails, and then plucked a few stray hairs from my chin. I was more productive in those few hours than I had been in days.

But, eventually… as the day progressed, the PAWECMHAA was reached.

I pulled out my rechargeable hand warmer and held it in my palms like a fragile premature infant. It was wonderful. I praised it. Cooed at it adoringly. It was a cozy bubble of warm bliss.

However, I quickly discovered that it only worked for about 1.5 hours on one charge.

As the heat faded from my palms, I cursed the cradled device, “This baby is a piece of shit. Argh!”

So, I had to come up with a new plan. Drinking tea helps me think, so I had some. And while my hands were cupping the warm mug, I had a thought.

I would get the largest coffee mug in the cabinet and fill it with water. Then, it could be microwaved for two minutes until the water grew super hot. Due to the magical properties of water to retain heat (yay, science!), I could use it like my rechargeable hand warmer. But, it would only take two minutes to rewarm the water— a major plus when dealing with extended PAWECMHAA temperatures.

I started by holding the outside of the cup… then, as the water slightly cooled, I began dipping my fingers inside the water. The plan worked brilliantly.

The next day, as the temperatures continued to dip, my morale grew low. And as I sat in my kitchen dipping my fingers into my giant pumpkin mug of hot water, I had a dramatic realization. I would never survive in a cold climate. Never. In fact, if I had been a member of the Donner Party that attempted to cross the snowy Sierra Nevada Mountains in 1846, I would have been the first one dead. Without question.

And then they would have eaten me.

To be frank, they wouldn’t have even had to wait until I was dead, because once PAWECMHAA was reached, I couldn’t put up much of a fight, anyway.

So, it was in this state that the HVAC repair man, Ricky, soon found me: bundled in a knitted blanket, dipping my fingers in a mug of hot water and muttering about nineteenth-century cannibalism.

It was a good thing he came when he did.

Within an hour, or so, Ricky had located the issue, and got the heater running again. As I heard the whoosh of hot air burst from the vents, I felt a tear of joy on my cold and numb cheek.

And, after a short while, the thermostat climbed beyond the PAWECMHAA. I said a prayer of thanks.

I would not be on the menu tonight.

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Adventures in Physical Therapy

Given that I’ve had a disability my entire life, you’d think that I’d be fully-immersed in all medical-related things. But, that’s just not true. For example, despite my love for WebMD and Wikipedia, I’m not familiar with every pharmaceutical brand on the market. I don’t know the best medication for type 2 diabetes or psoriatic arthritis. But, from the commercials that I’ve seen on television, each of the available medications have potential side effects that include temporary blindness, liver damage, suicidal thoughts and/or heart failure. It’s an uplifting assortment of options.

Anyway, while I do know a lot about stuff related to Spinal Muscular Atrophy (SMA), there’s a lot of other things that I don’t know. Stuff that, as an outsider, you’d think would be an everyday part of my life.

Like physical therapy.

This statement might come as a bit of a surprise. You might be thinking, “You mean the crippled girl doesn’t go to physical therapy every day? What a slacker! How does she expect to get better if she doesn’t do that??”

Here’s the thing: until I began Spinraza treatments last year, the expectation that I could maintain or improve my strength was pretty much a non-starter. An impossibility. A don’t-even-dream-about-it kind of thing. You know, like thinking that Kanye West will ever not be super weird?

This isn’t to say my physical therapists in the 1980s didn’t try their damndest to make this not be so. Defying the laws and biology of SMA, my childhood therapist pushed and shoved me into standing boxes to straighten my curving back. They placed heavy, painful weights on my legs and hips to try to reverse my irreversible muscle contractures. They tried to push my body into doing things that it just simply couldn’t do. They weren’t intending to be cruel, but their understanding of SMA at this time just wasn’t very advanced. They pushed more than they should have. They expected more from me than I could physically deliver. They were doing what they thought was best, though. But, these good intentions didn’t mean that this period of my life still wasn’t an utter hell on earth.

My confidence took a beating at this time, as well. It was hard not to feel that way when my inability to continue to participate in these physical therapy sessions was seen as a result of my laziness– and not the natural progression of my disability. The fault was with my motivation and my drive. Not the fact that it was an excruciating torment to endure… with no progress to show for it. In fact, the opposite happened. I progressed anyway. As all of us with SMA have done. It’s biology. Not laziness.

It’s perhaps not surprising that I eventually became withdrawn during these physical therapy sessions… and then increasingly uncooperative. Finally, it got to the point where during designated therapy time, I would hide in corners of the elementary school so the therapist couldn’t find me.

It was the biggest act of rebellion I had ever pursued up until that point. And it was also a moment in my life when I felt the most unheard.

Those gut-clenching moments remain with me to this day. I’ll always be that anxious little girl in the school physical therapy room, even though it was over twenty years ago.

So, it is with the baggage of those memories that I’ve recently reentered the world of physical therapy. My revolutionary Spinraza treatments have led to some small improvements in my strength and stamina. Which, given the progressiveness of SMA, is fucking awesome.

As I’ve written here before, I’ve been doing some informal exercises at home since beginning treatment. And it’s been very helpful. But, my Stanford medical team advised me that it was time to take it up a notch. So, I was referred to a local physical therapist to develop a custom exercise plan.

Yesterday was my very first consultation. I’m not gonna pretend that I wasn’t nervous. That I wasn’t worried that the physical therapist was going to push me too far beyond my ability because he didn’t have an understanding of SMA (like my old therapist all those years ago). Or, that I wasn’t dreading the whole experience… the way Trump dreads a wind tunnel.

So, what did I do? I did what any self-respecting Type A person like myself would do. I obsessively prepared for my appointment by typing up an information sheet for the therapist about me, about SMA, and why I was there in the first place. I also harassed the receptionist with warning calls that I would perhaps be a high-maintenance patient. Not like the everyday car accident victims and torn rotator cuff patients that they saw everyday. I would be a whole bundle of crippleness. The person that, when presented with a new patient form, would circle the ENTIRE BODY as an indicator of where I’m having trouble.

I’m sure my neuroses were quite evident in my voice on the phone. And I’m certain that this left an impression on the poor receptionist because when I rolled into the office, she instantly called me by my first name— despite the fact we had never met.

I filled out more medical history forms. And I had to resist adding smart-ass remarks in the margins. How could I not answer the question “How long have you had this issue?” with a sarcastic reference to the Reagan Administration?

I only have so much willpower, after all.

When the physical therapist, Robbie, came over a few minutes later, though, he was remarkably calm. And, despite my fears, he didn’t look like a deer in the headlights when faced with probably the most disabled patient he had ever encountered.

I wasn’t a truck driver with a case of whiplash. I was a whole-lotta-weird with muscles that behaved in ways you wouldn’t expect. (Come to think of it, also kinda like Kanye West.)

Robbie quickly admitted that he had never encountered SMA before. (Most medical professionals actually haven’t– this is not unusual.) But, that fact didn’t stop a gleam of excitement from entering behind his eyes. I recognized the same look in his gaze that I would get back in 1990 when I got a brand new set of Legos. The look that Ina Garten still gets when you flop a freshly-caught, raw sea bass on her kitchen table.

The look that says: “Oh, boy! This is way more fun than a hip replacement!”

I’m happy to report that he asked all the right questions… he was engaged and interested. And incredibly collaborative. It was just the experience that I had been hoping it would be.

I was being heard.

And, for that, I was so very grateful.

For the time being, I’ll be heading to physical therapy once a week, so stay tuned for more updates on my progress. I can’t believe I’m saying this, but I’m strangely looking forward to it!

I do wish there was a way that I could have told that to my ten-year-old self, though. That the experience didn’t have to be the way I endured it… and that it could have been much, much better.

Maybe then I wouldn’t have had a reason to hide.

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elizabette… unplugged

muscular dystrophy