What it's like to live with a progressive neuromuscular disease

Thursday, September 1, 2016

Coming clean

Tonight the Neuromuscular Disease Foundation hosted a dinner party to celebrate its 10 year anniversary. It was an opportunity to bring together scientists, researchers, NDF board members and patients living with HIBM. It was not a fundraiser, but rather an evening to thank everyone who’s been involved over the years and to share all of the progress that has been made towards raising awareness and finding a treatment. It came at the end of the first day of a two day symposium on HIBM. The symposium has become an exciting annual event organized by the NDF. Scientists share the status of their clinical trials and patients get to ask them questions directly. It’s an invaluable opportunity. This year, patients flew in from as far away as Japan and Portugal. It all took place a mile from my house at UCLA. It could not have been more convenient. Especially since it wasn’t even a work day for me.

I would tell you what it was like to be there, but I can’t. Because I didn’t go. Not to the symposium nor to the dinner.

I wasn’t “under the weather” and I did not have more pressing plans. I simply chose not to go. My logical, rational self RSVP’d “yes” right after receiving the invitation. My emotional self tried to follow suit, but simply couldn’t.

It feels hard to come clean and confess this. The feelings around it are so messy. They range from shame, embarrassment, and guilt, to pride for empowering myself to make my own choice and compassion for my own personal process. It feels confusing. I am bombarded by the “shoulds,” though I know there is no place for them in the battle for emotional survival.

I should go because I may learn something new. Something hopeful and inspiring.
I should go because there are so many incredible patients whom I see on Facebook and with whom I could connect and bond.
I should go because the NDF was founded by my own family on my behalf 10 years ago.
I should go so I can show my support and gratitude for all that they’ve done.
I should go because so many other patients are going and they don’t seem to have much ambivalence about it.
I should go because if I don’t, what will people think of me? How will they judge me?

The noise gets so loud that I sometimes can’t hear what’s coming from within. The whispers of fear, vulnerability, and sadness. I’m scared. I’m terrified. I don’t want to go and listen to lectures that seem to always end with, “there’s so much more we need to learn and do.” I don’t want to sit in a room looking around at my future self in a wheelchair, needing help using the toilet, having trouble feeding myself. The closer my body moves towards the once distant disease outcomes that sit at the core of my own dread, the farther away I want to run. In the old days, when I saw patients who were 10 years in, I could reassure myself I’d never get there. The science would surely rescue me. I’d be spared. But now, how can I even hold onto that shield when I seem to be headed down the same path? Especially when it’s happening right in front of me? I write this paragraph knowing that it shows my own lack of acceptance and my own learning curve about disability and identity. I write it because it would be inauthentic to leave it out, not because it feels good to acknowledge that seeing other patients makes me fearful for myself.

But then I wonder how is it that all the other patients seem to be able to handle it? Am I weak? Am I not as resilient as I thought it was? Do I just talk the talk of acceptance, but when it comes down to it, am I really just all the way back at the beginning of this grieving process?

It feels like every day it takes everything I’ve got to not sink into a depression. I am fighting the physical battle on the outside and an emotional one on the inside.

Every time I go to pick up my kids from school, I am fighting for one of the only two disabled parking spots. I am competing with grandmothers who seem to have all the time in the world to arrive an hour early to secure a spot and a mom who hops out of her car wearing platform wedges and carrying a toddler. I’m fighting to not judge them, to wonder if maybe they have some sort of “invisible disability” but it’s hard not to bring the rage and feel the injustice in that moment. Especially when I seem to be the only one wearing leg braces and using a cane.

I am fighting back tears when the very well-intentioned, not so psychologically-minded, long-standing crossing guard at the school mimics my gait, yelling out, “I wish I could strut my stuff like you do with your hips like that!”

I am fighting with all the curbs and all the steps out in the world that don’t have railings.

I am fighting with the hallway from my parking space to my office door which seems to somehow be growing longer and longer.

I am trying not to lose it every time I struggle to pull a tight shirt up and over my head. When I can’t put the backing of an earring into its post. When I can’t hold my children’s hands while trying to cross the street because I have a cane in one hand. When I can’t pick something up off the floor with my left hand because I have almost no strength left in those fingers and my right hand is holding onto my cane. When I try to do a modified push up on a bar at the gym and my muscles give out and I end up with a bloody nose.

It is exhausting. All of it. It wears on me. It is relentless and cruel. And it won’t stop here. That’s the most terrifying part. It will just keep getting harder and harder. These are the non-negotiable moments that make up my every day.

And then there are the negotiable ones. Like this symposium. Though in the end I opted out, I was still curious. What was I missing? So I logged onto the live streaming that was being offered. The first slide I saw was one I’d seen too many times before. The very first time I saw it years ago, I looked away. I hate it. It traumatizes me every single time. It shows a photo of the legs of a patient who is quite progressed. It looks like the photo of the legs of a holocaust survivor. I quickly logged off. I looked down at my deformed calves. My once healthy and strong calves that I foolishly used to complain were “too muscular.”

I took some time away and then I logged on again later in the day. I couldn’t help myself. This time speaking was one of the scientists running the NIH clinical trial: “The hoped for outcome of our treatment is to slow progression, not actually reverse the disease. We are still not able to regenerate muscle tissue.” Of course, I’m well aware of that scientific fact. But it doesn’t mean it’s not an emotional hit every time I hear it. That’s when I start to beg and pray and bargain that it only stays this bad. That I get to just have to deal with the losses and hardships of today. But then tomorrow arrives. As it always does. And I’m that much closer to worse.

Tonight was billed as a celebration. I’m not in much of a celebratory mood. I don’t want to celebrate the fact that there may be some novel new treatments coming down the pipeline. I’ve been there and done that. I’ve gone to that party so many times. I’m kind of done showing up.

10 years. Ten years ago I let myself believe that in 10 years time, there would surely be some kind of treatment or cure. I held onto that. I relied on that. I showed up for it. And it’s not here. I’m not saying it won’t arrive one day. I’m not saying it’s anyone’s fault. I’m not saying people aren’t doing everything they possibly can. I’m just saying now is when I need it.

Please visit the NDF website for more information about HIBM and how you can help fund a cure

Neuromuscular Disease Foundation

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