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

Friday, July 31, 2015

Chapter 14: The 9.5 Year Phase

Wouldn’t that be so nice? If there were actually an entire textbook dedicated to HIBM? One with an index where I could look up what to expect a year, two years or 10 years from disease onset? With chapters like, “How Much Exercise is Okay?” Or, “What is the Role Hormones Play in Disease Progression?”

Because HIBM is so ultra-rare, there are relatively few scientific groups in the world working on it . We simply don’t have enough information on HIBM to fill up a textbook. Maybe a thick pamphlet. And though we have some general guidelines and a basic understanding of this disease, what each of us desperately seeks to know is the rate at which our own personal bodies will deteriorate. And no one can tell us that. There is only one line we patients get to read over and over again in terms of statistics and numbers; hard facts. It’s the one that’s been haunting me for these almost 9 1/2 years: “On average, HIBM leads to severe incapacitation within 10 to 20 years of onset.”

Tonight, while starting to wind down from a long, busy, active week, I had what I guess I would call an epiphany. I realized that for these last 9 1/2 years, without conscious awareness, I have been holding my breath. Not to the point of respiratory distress, just to the point of not being able to fully inhale and exhale. In eight months from now, I will officially enter into that feared chronological phase. And yet, though I can’t guarantee it, it appears extremely unlikely that I will fall into that category, the one of “severe incapacitation.” Doesn’t mean I never will. And doesn’t mean I won’t even within 20 years. It simply means that at the 10 year mark- the one I’ve been dreading all along, the one I can just barely make out in the distance- I will not be where I so desperately feared I would be, back when this all started.

Let me tell you what “severe incapacitation” has meant to me all these years. The vision is one of me sitting in a wheelchair with my hands folded on my lap, needing to have someone feed me and having to use a water bottle with an extra long straw so I don’t have to bend my neck. I don’t know if I imagine this scene because it’s my own personal worst-case scenario or because I’ve actually seen HIBM patients progressed to such a state. When I have, those moments have been traumatic. And if you know me, you know I don’t mean that in any offensive or disrespectful way. I mean it in the most honest, authentic, compassionate way.

I am slowly yet suddenly becoming conscious that these images have been flashing in my mind for the last 9.5 years. And so it helps me explain why tonight, in this moment, I feel almost a kind of elation. An intense surge of gratitude. It may be the first time that I don’t feel angry at the fact that at age 40, I have to feel grateful for not being physically incapacitated. Instead, I feel joy. Relief. It feels as if I am finally fast-forwarding through that vicious soundtrack. The one that’s been on repeat: “But I shouldn’t have to deal with this disease in the first place.” I think it feels like true acceptance.

I do have this disease. That part already happened. It’s over. This is what’s happening to me, now. It’s time to let go.

I can not tell you how many times a day I use the word “acceptance” with patients. And so often they look at me, quizzically. “But how do I do that? How does that happen?” I don’t blame them for the confusion. It’s vague and abstract and there’s no how-to guide. But I always offer the same response, “you have to mourn the loss first. You have to hold the feelings, look at them, let yourself feel them and then move on. Set them free.” I tell them that only then will they will no longer distort their thoughts. They will no longer engage in as many cognitive distortions, all the catastrophizing and “fortune-telling” that triggers the panic and anxiety. I can’t promise them it will work. But internally, I can vouch for it.

We do not have crystal balls. We cannot know the future.
This is not my opinion. This is not me trying to make a convincing argument.
This is simply a scientific fact.

Tonight I am just where I am.
It’s that plain and simple.
And it feels really good.

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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