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

Saturday, May 31, 2014


What does it mean to be doing “fine?” So often people ask us how we’re doing and we offer that up as a simple, basic response. Sometimes without even thinking. A good friend recently asked me how I was doing and I reflexively said “fine” because after all, I haven’t been feeling depressed, I haven’t been crying and I haven’t been living in my dark, scary “vortex.” So, presumably, I’m fine. But as I thought about it later, in a more reflective and honest way, I realized that although I haven’t been experiencing any overtly negative emotions, the truth is, I haven’t really been “fine.” At least not in a way that feels okay to me.

A few weeks ago, a fellow HIBM patient who has become a dear friend came to visit. Last year she moved to a new city for work, a city she chose specifically because of its world renowned physical rehab center. She shared with me what it had been like to be a patient there. She was disappointed. She said they spent too much time pointing out her limitations and not enough time focusing on all that she still can do and that was discouraging. She mentioned that they expressed some concerns about her lung capacity. Those two words lingered in the air. Lung capacity. She continued on about having been referred to a pulmonologist and getting expensive tests done, etc. And though I sat there calmly and listened, in that moment, my mind entered into a different dimension. Everyone knows about HIBM’s debilitating effects on major skeletal muscles, but what most people don’t know is its effect on smaller skeletal muscles, like the intercostal muscles between the ribs which are responsible for allowing our lungs to expand and contract when we breathe. This was not new information to me. When I was first diagnosed, (and before I knew what was good for me in terms of coping) I would go on-line and read about the disease and its progression.  I distinctly remember reading somewhere about late-stage symptoms, including difficulty with the cough reflex and pulmonary function. Though I had been able to process, to a certain degree, the muscle wasting stuff- the pure physical deterioration from the disease- I couldn’t wrap myself around problems with breathing, a basic physiological function. That was something, I told myself, that I “could not handle.” So I locked it up in a box and buried it way down deep. I told myself in a very rational, calm way that I had no need to even go there. After all, at the time I was only walking with a slight limp and potential breathing difficulties had to be decades away. Chances of me ever even getting that progressed were slim to none considering there’s bound to be some form of treatment available by then. Right? And so for years, I never even let myself look for the box.

But in this past year, it seems the key has slowly made its way into my hands. I have started noticing, in the most mindful (observing without reacting) way, occasions when I’ve run out of breath: on Friday nights when I am singing the Shabbat blessings for my family, in the pool when I’m swimming laps, in my office when I’m leaving long voicemails for patients and in the car with my sons, when we’re having a contest over who can chant a longer ohm. Noticing a new need to have to pause for air in between words or come up for a breath where I didn’t have to before. Sure, I’ll be the first to note that shortness of breath is a primary symptoms of anxiety. But this was all before I was feeling anxious about it. Before I imagined it could even be related. Then, in this past month, I heard not one, but two separate patients mention it. Neither of these patients would be considered to be in late stages of their diseases. So where does that leave me? How do I interpret/ process that?  How do I rationalize through this one? How am I to cope? 

My usual pattern is to let all the fear and anxiety wash all over me. Let it in, then up, then out. But not this time. No way. I was going to remain very rational and logical about it. I was not going to let my emotions get involved. Because once again, I “could not handle it.” In fact, I was fine. If anything, I was feeling happy, energetic, totally in touch with all of the amazingness that is my life without HIBM. It started with wanting to see Billy Joel in concert at the Hollywood Bowl. So many people were posting on Facebook what a good show it was that I decided that it would be a totally fun experience and that I needed to go. In fact, I became rather obsessed with going- compulsively searching for tickets on-line and seeking out friends who wanted to accompany me. When that didn’t happen, I moved on to needing to see Bruno Mars and Pharrel at the Hollywood Bowl. I  scoured Craigslist and though the tickets were outrageously expensive, I didn’t care. I had to have them. I was going to carpe diem. Seize the day. Who cares that the Hollywood Bowl is an exhausting uphill venue and difficult to navigate? Or the fact that I really only know a few Bruno Mars songs? I then started thinking about my 40th birthday (in a year from now). I excitedly told my husband that maybe we should go back to Italy where we took 3 romantic, semi-spontaneous trips to during medical school. Wouldn’t that be amazing? We should totally do that, right? Let’s stay up tonight and watch a third Breaking Bad episode in a row. Let’s delay sleep for just a little bit longer. Let’s learn how to do stand-up paddling this summer. My quads are still strong and it would be a good work out, right?

Escape. Do. Go. Don’t feel. Don’t let it in. Don’t let it get too quiet. Keep the noise volume on high. Have all the experiences now.  Distract. Maximize this moment. Take advantage. Fill the void. Don’t think about it. Don’t slow down. 

Coping mechanisms. They are how we try to keep the anxiety in our psyches at a minimum.  Strategies to avoid the emotional pain and discomfort. For some, it involves drinking or getting high, for others it’s shopping or working or socially isolating. For me, in these last couple of weeks, it’s been doing. Anything to preoccupy me, distract me from opening that box. It’s exhausting and though I’m fully aware of what it is that I’m doing and the fact that it’s not sustainable, the insight into it doesn’t make it any easier. When life feels out of control, we do everything in our power to try to regain control somewhere, anywhere. The irony is that we often only end up feeling that much more out of control. Crazed even. On the one hand, I come down harshly on myself. Judging myself for not being able to hold the feelings in a more calm or measured way. After all, I tell myself, I am a psychiatrist.  I spend my days holding other people’s difficult feelings and teaching them how to cope with them in healthy ways. And on the other hand, I have compassion for myself. When I step out and look in, of course I understand  why I’m doing what I’m doing. I’m simply trying to survive. I’m trying to take in and digest, little by little, the reality that is unfolding in front of me while still moving through my life and participating in it in a way that is maximally fulfilling and meaningful. A life that if full of so much awe and wonder. And punctuated by moments of such intense grief and pain. And somehow, I am handling it. We all do. It’s how we are wired. To survive.
I started the above post yesterday afternoon, after I had finally let all of the feelings come up and out. One of the recipients of those feelings was my best friend, Gabrielle, whom many of you know independently or through my blog. I shared with her my desire to try to write about what’s been going on for me but also my fears in doing so. As I was writing, I received an email from her. Below is what she wrote. It’s what she wanted to say to you. And I think it’s the blog post I was too afraid to write myself:

I met Jen for coffee today. It had been too long since we made time to just sit down and be together.
She recently told me that she thinks her intercostal muscles are getting weaker.  I had never heard of intercostal muscles, so I googled them. They are the muscles that run between our ribs that allow our lungs to expand when we breathe. I’m used to thinking about Jen’s loss of mobility. I’m used to seeing it. I’m used to hearing about that mourning. By “used to,” I only mean that it has become familiar. It never gets less painful – hardly. Familiarity is a weird beast when processing something that is progressively degenerative. Familiarity takes the form of structure and expectation. It shapes the grieving process. But it doesn’t make it easier. In fact, there’s a separate grieving about the familiarity itself.  But breathing. This feels unfamiliar in some way. Quite honestly, I stopped breathing when she said it. And then she reassured me, as she always does, giving me the reasons why it will be okay. And I drank them. And then she put it in a box and locked it up and a couple of weeks went by.
But as Jen always says, there is no real way to lock up truth. And so she opened up the box and took another look and shared with me today that the intensity of this reality, this progression, has made her turn to a familiar coping strategy.  In order to stay “fine,” by which she means get through the days without entering the vortex of dark feelings, she has been manically chasing down concert tickets, making plans to live every moment in some kind of carpe diem way that involves lots of planning and logistics and busy-doing-not-thinkingfeeling.  She tells me it’s exhausting.  That it feels “crazy” because she knows what she is doing. She knows she is looking for control in an out-of-control, almost compulsive, way because she is losing control of her body. 
She tells me that she wants to blog about it. About her breathing. About her coping strategy. About willing herself to walk across sand dunes and then taking a solo swim in the ocean to feel alive. Please stop right now and imagine what that means, what that takes for Jen. Have you drawn from a reserve you don’t actually have and willed yourself to a moment of feeling alive? I haven’t, so you should hear it from her. I asked her if she paid a price for it later.  She said without hesitation, “I’m always paying a price.”
She wants to write about it, to tell you, to tell you that it’s okay to find your own coping strategy and see it for what it is and survive another day because of it.  But then she tells me that she wants to give you a take-away positive message and she can’t pin that down.  She wants to wrap it up with a bow for you. And that’s the moment in our coffee when I start to cry. So I ask myself why that moment? I could have cried with her when she told me that it’s sometimes hard to take a full breath. But I didn’t.  Because I know, and Jen knows, that I will sit with her in that pain forever, hear it, feel it, but give her space to find her own voice in it. But when she wants to take care of you or me because maybe we can’t handle it, then I cry.
I ask her why. She says she worries that this is too much, that you won’t know what to do with it because it’s too heavy, that she will alienate herself from people because it will be too hard, too scary for you to take it in and then still know how to be with her. I hear her say that and I want to make promises about human nature, but I know she’s right. That maybe only a handful of people will know what to say or how to react or how to just be in that reality with her. And I’m not talking about comments on Facebook or emails. That kind of community love is beautiful and so important to her in its own way. But I’m talking about sharing space with her.  I’m talking about being able to sit in her pain without spinning it for your own inspiration. Be inspired by yourself.  Be inspired by resilience. But be open to sitting with Jen in her pain and just being with her. That’s what we all want, right? For someone to keep us company in a way that makes us feel heard and loved without the need to cover or hide or inspire or care-take. Just hang out and be. Jen’s really good at that.

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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