[susana-m]

Hi and congrats on getting Evrysdi! I didn’t realize there was an age limit, that’s nuts.

I know the anxiety well. Starting with possible side effects to the question of how much hope is reasonable, what can you expect to improve.

Historically, I’ve always had constipation but my body’s first defense is nausea. You never know with a new Rx so I planned on staying home with backup help. We left the transfer sling for my Hoyer lift under me as that would eliminate one time consuming step if there was a mad dash to the bathroom. It’s demoralizing when you don’t make it to the toilet in time.

One day, two days, nothing dramatic.

Had some nausea, tried taking the Rx at least 1/2 hour before food on an empty stomach and clear sailing from then. I’ve always been restless and prone to migraines, didn’t notice any significant changes there.

What I haven’t seen is anyone talking about the emotional process. The overwhelming anticipation and underwhelming reality DeAnne mentioned.

I was about 10-12 years old when I verbalized that I was not going to get better, that a “progressive neuromuscular disorder” only had one prognosis: slow decline without any real brakes. The sun rises in the east, the earth is round and SMA does not improve. Allowing yourself to hope for change means risking crushing disappointment. And then I peeled a tangerine, something I had not been able to do for 2 years. My doctors mentioned the volume of my voice had risen. These small changes mean the progression has paused and they’ve found ongoing improvements.

What’s been most impactful for me has been focusing on what I can control. I’m very deliberate with my food choices, I meditate daily, and am engaged in projects. Keep in mind, these are strategies that have worked for me but your mileage may vary.

The purpose of fear is to point out dangerous terrain, to keep you alive. You’re asking those who’ve gone before.

We get it.
We’ve lived it.
We got your back.

[susana-m]

I feel pretty fortunate to be in Miami where STS has been operating for over 30 years. Each county accepts bids from transportation companies who manage the day to day operations. It’s got its limitations like scheduling in advance, problematic (sometimes nonexistent) accountability, range, and the likelihood you’ll detour to pick up other passengers. Considering how many ways those fluid circumstances can go wrong, they’ve been fairly reliable. They’re not limited to healthcare transportation or to financial qualifications so school, work and socializing is accessible.

But you’re right,

it shouldn’t be this hard.

It shouldn’t be this expensive.

For a group that has historically lived on or close to the poverty level, the cost of living’s astronomical

[susana-m]

Ari, the genius in what you said is that you recognized how you put yourself in that position, you saw yourself as the patient with all that comes with it: the uncertainty, the powerlessness, the preconceptions, the fear, the actual crisis itself, all placed on the altar of medicine while looking for a solution.

It’s so easy to forget that what we bring to the table defines our experience, defines how we allow ourselves to experience everything. I’ll say one thing: people with SMA are sassy AF fighters. God help you if you get in our way whether you’re an insurance company, our own fear, or SMA itself.

Crack bone, suck marrow, be compassionate with yourself and decide who you are in the equation.

[susana-m]

We were in the clear, one week before my scheduled vaccination when my PCA gave me – us – Covid.

I was shocked at the utter lack of information or real medical guidance available at the time. Keep in mind this was over a year ago in February 2021. We monitored our oxygen levels, drank fluids and slept a lot. It was like being kidnapped and held hostage by the Covid Apocalypse.

We recovered but we’re more sensitive to heat, short of breath if we do too much, and Covid brain is a frustratingly real thing.

Was starting to think I was the only SMA who got sick. Notice any other long term symptoms?

[susana-m]

It’s the fading independence that’s toughest for me. Sure, I get a little sad when I know there’s camping and I can’t join. Going to wild spaces is like going to the moon, admired from a distance. I wasn’t able to have a private vacation, driving was as independent as I could manage for a short period of time but that’s long gone now.

I miss putting on my own makeup. You know how it is: spend the first part of your life figuring out who you are and how you want to present yourself then *BAM* you’re trying to explain how much mascara is enough. And not being able to feed myself, that was a sucker punch to the ego.

Most people aren’t aware how the very help we need can often demote functioning adults into needy children, at least in the eyes of those helping. (In ourselves, too.) There’s a biological aspect we gloss over: the mechanics of assistance. When you physically look down there’s an underlying assumption the shorter person is young and helpless, somehow lesser. We see it with kids, the elderly and the disabled. I’m not saying everyone does it but it’s part of every exchange that’s worth discussing

[susana-m]

We’ve heard a lot about healthcare workers and their struggles with burnout but patient burnout is also a thing. Or, if it’s not yet recognized, it should be. I used to think it was just me, now I know it’s ever present in people with long term health issues. Especially progressive disorders.

You know what it feels like: dragging fatigue, resigned to test #57, another doctor who is going to confirm what you already know. Let’s not forget the energy it takes to be present, ask questions and keep some autonomy in the decision making process itself.

All of that is secondary to the shifting symptoms and changing landscape of SMA itself. That can be really challenging when your baseline strength drops, suddenly you can’t do a task anymore. It’s exhausting, both physically and emotionally.

F**k adulting.

When I get to this moment I try to remember a few simple strategies. Go outside. Get your cell and take a spin around the block. Research has shown just looking at a tree for 5 minutes lowers blood pressure. Vitamin D deficiency has been linked to depression, fatigue, muscle pain, weakness and stress fractures. Vit D also helps build calcium in your bones so that’s a win on a lot of levels.

Another strategy is becoming ultra present in the now. Instead of grieving for what’s already happened or what might happen in the future, I stop, look around my space to anchor in this place and focus on the task at hand. It’s like the ‘work vacation’ you mentioned in your article.

This is probably obvious but therapy can be tremendously helpful with the right counselor.

It’s important to cut ourselves some slack. It’s easy to find compassion for others but giving it to ourselves is like pulling teeth. (That’s not an SMA thing, it’s a human thing) These last years have been traumatic for everyone, it’s ok to have a bad day. Grab a cookie or some chocolate, turn off social media and watch a movie or go to a park.

This too shall pass.

[susana-m]

Warding off SMA? I’m intrigued. Would you share what that looks like for you?

For me it means weekly acupuncture, eliminating refined sugar, eating less meat and dairy, some gentle range of motion, trying not to do too much. Anything that is proactive gives the added bonus of feeling empowered to make choices that matter in your well being