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

Friday, February 24, 2017

Even in basketball gym parking lots

This year, my 6 year old son decided he wanted to play basketball on a team. He is no doubt the sportiest among us. We signed him up willingly, but secretly dreaded getting up and out for games on a Saturday at 9AM. We haven’t been a part of the whole group sports subculture until now and we weren’t sure it was for us. But turns out, the whole experience has been so much fun. Sure, it’s been tricky for me physically- trying not to get knocked over when the doors open to the gym and people come rushing in and out, or trying to hobble my way quickly enough across the court to get a seat before balls go flying – but those discomforts have been in the service of something so comforting. As silly as it may sound, going to and from basketball practices and games every week has been quite normalizing for me. As in, it’s given me an opportunity to feel like everybody else. It’s what so many moms and dads do. I get to feel typical, regular, just like them. All of the feelings that HIBM has taken from me over the years.

I’ve been savoring the normalcy. Until last Wednesday. I was walking back to my car with one of my new favorite mom friends and our kids after practice. We were discussing where we were taking the kids to dinner (another simple parenting act that, though increasingly challenging, I’m so grateful to still be able to do). And all of a sudden, just like that, I didn’t fully clear my foot on the ground and I tripped and fell. Hard. I’m used to the bruised knees and scratched elbows that come with my falls. But this time, I tried to put my hands out in front of me to no avail. The next thing I knew, my forehead knocked right onto the concrete curb. I didn’t lose consciousness, but the pain was intense. Any time I fall in front of my children, my first instinct is to try to keep it together. I don’t want to scare them. I don’t want them to worry. This time, there actually weren’t even tears to suppress. Maybe because I was so stunned. Maybe because I was with a relatively new friend who hadn’t yet experienced the extent of my disability and I was afraid to be too vulnerable. Would I scare her off? Would it be too awkward? Would I put her in the uncomfortable position of feeling like she had to know what to say? She right away bent down to help me up and calmly asked me what I needed and how she could help. I said I was fine, as I always say right after I fall in public. I casually explained that falling is just a part of my life. I gathered myself together and we kept walking.

Then we noticed my elbow was bleeding. She insisted that I pull around to her car so she could tend to my small wound with her first aid kit. She also said she would take all the kids to dinner in her car. I told her she was being silly, but she wouldn’t take no. I pulled around and put down my window and as she was putting neosporin and a bandaid on me, she suggested in the kindest, most compassionate way that maybe I shouldn’t put my head down when I fall. I realized that she wasn’t aware that doing so wasn’t intentional. I then explained that my neck is so weak that I no longer have much control over it under the force of gravity. And then, of course, I started crying. She told me not to be upset, she reminded me that I have an amazing husband and two beautiful children, that I am alive and healthy. She said it with such love. I felt it. And then I cried even more. She then said she would take all four kids to dinner and that I was not allowed to join. She insisted that I take some time to be with my husband. I protested for a bit, and then I allowed myself to receive her compassion.

I drove away and cried and screamed and cried some more, all the way home. My right brow bone was throbbing and I could feel the swelling. I cried because of the pain. I cried because of the fear of what’s to come. I cried because I didn’t get to pretend any more that I was like everybody else. And I cried because of the kindness.

I came home that night to my husband holding an ice pack at the door. I sat down at the dining room table and iced my eye for an hour, terrified of having a scary looking bruise above my right eyebrow at work the next day. I wish I could have explained to my husband that I fell because I wasn’t using my cane or because I wasn’t watching where I was going. But I no longer have the luxury of those options. The scary truth is that there’s nothing I could have done differently. This is just where my body is right now. I didn’t fall trying to climb stairs or do something heroic. I was just trying to do the (no longer) simple task of putting one foot in front of the other.

Once I finally stopped crying, the humor came. I joked with my husband about how grateful I was that I didn’t rip the knees of my beloved jeans that I got on crazy sale last year. I joked that maybe I should just walk around in a helmet from now on, pretending not to notice if people stared. We laughed that I should have a consultation with a plastic surgeon and draw up plans for my dream nose so that if I fall and break my nose, I’ll be ready to go. (But then he ruined the fantasy by saying, knowing my luck, I’d get a nose job and then trip and fall on it and waste all that money.)

After the tears and after the jokes came the hardest part- taking a serious look at what it means to have a fall like that and how best to move forward. Where is that line? How far do we patients push the boundary of what’s safe before we accept that we can’t keep doing what we’ve been doing in the same way? Is it when I get a concussion? A laceration on my forehead requiring stitches? If you’re a very logical, practical person, perhaps you’d say the time is right now. Why be foolish, right? And if I were you and I were reading this, I’d probably agree. But if you were me, you’d know how impossible it feels to yet again confront the reality that HIBM is, as advertised, a progressively debilitating disease. What will be next? Will I start using two canes? Will I go straight into a walker? And what about a wheelchair? For how much longer can I get by doing the things I enjoy in the way I enjoy doing them? For how much longer will the benefit outweigh the cost? And just how scary is that cost?

We are living in dark times. The ugliest underbelly of humanity is being exposed. The most vulnerable among us, including people with disabilities, feel threatened and live with increased fear for our futures. And though we can make our voices heard through marching and social media, etc., there is an extremely uncomfortable feeling of powerlessless that makes it harder to hold onto hope. I’ve looked for light in kindness and compassion. It’s one thing we can each control about ourselves. It is so powerful. We can be vulnerable, and we can allow ourselves to receive love. We can actively fight against divisiveness by being intentional about forming deeper human connections. Even in basketball gym parking lots. I don’t like tolerating more help in my life, but at the same time, I increasingly depend upon exactly the kind of compassion I want to put out in the world. We need to hold each other up with it.

Friday, January 6, 2017

Rubber Bands and Trapezes

Three and a half years ago, I booked a family trip to Club Med, Ixtapa. It sounded ideal – an all-inclusive beach resort with non-stop activities for the kids, an easy direct flight from LA and a time change in the right direction. My sister and her own family had gone for years and raved about it. I wanted in on the action too. When I told my sister about our plans, she gently warned me that it may be hard for me to get around such a large resort. I initially dismissed her concern, in part because that’s always my initial reaction when confronted by yet another disease-imposed limitation, but also because I figured (hoped) she was just staying true to her lovingly over-protective big sister self. But after a few weeks of thinking more seriously about it, I canceled the trip. It wasn’t a major tombstone to add to the cemetery of losses, but giving up on a vacation because of this disease was a tombstone nonetheless.

When I let go of the things I want but can’t have, the wanting part almost always lingers. Once I’m done grieving the initial loss, I start to imagine alternative routes to the same destination. The most extreme example of this process was when I tried to let go of wanting a second child. Only after fully mourning the fact that I could not safely carry another pregnancy was I able to consider other ways of fulfilling my dream of having two kids. (For those who don’t know, we used a gestational surrogate to have a 2nd child. For the record, it was, hands down, the best decision I have ever made in my life.)

It’s the process of finding this line between the emotional and the rational, between how desperately I want it to be and how it actually is. It’s the line between living in sorrow on the one extreme and denying emotional pain on the other. Finding that line determines to what degree this disease interferes with my participation in my life.

So, when I started thinking about our winter vacation this year, Ixtapa was still nagging at me. You might imagine that with the downward progression my body has experienced in the last 3.5 years, I would have crossed it off the list. But in that same time, the ways in which I navigate my body in the world has changed. I now use a cane full-time and I have learned how to tolerate the extreme emotional discomfort of being wheeled around in places like the airport, in the name of practicality and efficiency. So I reconsidered whether I could make this vacation work.

When I called to make a reservation, I asked if they had a wheelchair I could use while there. They said they did (with a warning that it was a bit old). That was enough for me to decide to go for it. I felt proud of my rational self and my willingness to take advantage of available resources, despite how depressing it was to face the reality that I’ve actually reached this stage in my disease.

But a few weeks before our departure date, that pride turned to anxiety. Did I really want to be dependent on my husband pushing me around the resort in a wheelchair the whole time? How would that affect me emotionally? I started to fantasize about finding some sort of sporty stand-up travel scooter, like the one I have at home. When I’m zipping around on my scooter at 15 mph, picking up the boys from school, I actually feel kind of cool. Adults stop to ask me from where I got it. Kids point and exclaim how “awesome” it is. Most importantly for me, I can interact at eye level with everyone else.

My on-line search for a portable, collapsible, stand-up travel scooter turned up nothing. Sure there were plenty of more traditional sit-down electric mobility scooters available, but I wasn’t willing to consider them. I rationalized that I’d be fine with the hotel wheelchair. Of course, there’s a difference between rationalizing and being truly rational. And I wanted to be rational. Then I remembered that a while ago, a dear friend of mine saw a woman riding on what essentially looked like an adult electric tricycle. She stopped her on the street, asked her the name of the scooter, and then texted me a picture of it right away. (True friendship love.) It was called the Travel Scoot.

Ten days before we were set to leave, I mustered up the courage to look into the Travel Scoot. Sure, it was portable and lightweight and had a very cool (relatively speaking) design, but it didn’t meet my criteria of being able to stand up (i.e. feel “less disabled”). Plus, it cost far more than I was willing to spend on something I technically didn’t need. It is hard for me to accept or tolerate more help in my life – whether from a mobility device or other people – until I feel like I really need it. Even if it would make my life easier or more comfortable. But then I think about a moment I had a bar-mitzvah a few months ago, talking to the grandmother of the bar-mitzvah boy. When we got up to take our seats at the table, me with my cane, she said wished she had brought hers too. When I asked her why she didn’t, she replied, “I’m too vain.” It struck me as somewhat fascinating that even someone twice my age, with presumably all that extra life experience, was letting her feelings of discomfort or embarrassment or vulnerability interfere with making a rational logical decision to be safe and more comfortable. I didn’t want to be her.

So, I went from being unwilling to even consider purchasing this scooter to ordering it within 2 days. And in those 2 days, I broke down and sobbed 3 different times. Once on the phone with my best friend in the bathroom at work while she was at her work waiting to take a deposition, once at home with my loving husband, and once by myself. All the usual thoughts flooded in behind the tears: “I wasn’t supposed to ever get here. I can’t believe this is really happening to me. I don’t know how to find the strength to keep adjusting and adapting. I’m not sure how much more of this I can take.”


My scooter arrived 3 days before we left. My husband, my most rational cheerleader of all, thought it was an awesome design from a physics and aesthetics standpoint and was excited to try it out. My kids thought it was cool and had fun scooting down the hall. And I just sort of resigned myself to full acceptance.

I wish I could tell you that making the decision to buy the scooter was the hardest part. But of course, the hardest part was then actually being on vacation with it. Now, it wasn’t nearly as miserable or impossible as it would have been without it. I know that. But I hated being a spectacle everywhere I went. Especially when I tipped over and got a huge bloody gash on my knee. I hated getting stuck in the mud and having to ask a stranger to give me a push. And then unrelated to the scooter itself, there was the realization that I can no longer walk on the sand without the help of my husband. I literally can’t take a step in any direction on my own without falling down. And even with his help, it’s a whole production getting to and from the ocean. And in front of an audience of hotel guests on the beach. The level of helplessness I feel when stranded (after all, he does have our two boys to tend to as well) and the degree of dependence I now live with at baseline is something to which I imagine will take me a very long time to adapt. I couldn’t even navigate the buffet on my own because with my one good hand holding onto my cane, I don’t have another strong hand to hold my plate of food. Even the resistance of the metal serving tongs were too much for my “good” hand to be able to properly serve myself food. (A sweet teenage girl saw me struggling and helped serve my veggies one night.) All these emotional hits make me never want to go anywhere out of my comfort zone.

I want to acknowledge that it’s not lost on me that I’m sharing about navigating a beautiful beach resort and struggling at a bountiful buffet. Especially in the context of the current global suffering of humanity, I get that I am beyond privileged and blessed to have these opportunities. Even to be able to afford a mobility scooter. (I was shocked to learn that across the board, insurance will only cover one if you can’t physically get to the bathroom in your home unassisted.) But just as I compassionately remind my patients who judge their struggles as “first world problems” or defend against their pain by arguing, “but things could be so much worse,” I remind myself that it’s never either/or. I can make room for the feelings of pain and sadness alongside the feelings of gratitude. I can feel so lucky to have access to this scooter and these resources and feel so terribly unlucky that it has to be a part of my reality.
I refuse to let HIBM restrict and narrow my life. It is like a thick rubber band constantly tightening around me and threatening to diminish my quality of life. Because I don’t have the physical might to stretch it out, I can only use sheer will. If I let my emotional (or physical) discomfort dictate how I live my life, I will never expand it.

On this trip, expansion for me meant meeting two lovely families, one of whom goes to our school back home and with whom we plan to maintain a friendship. It meant daily swims far out in the ocean where I could feel liberated and at peace. It meant watching my boys cultivate their independence, running around the resort by themselves, meeting people from around the world. It meant being able to go to and from my room without needing anyone to accompany me. And finally, my personal favorite, it meant trying my hand at trapeze. Yep, did that. Despite the long and treacherous (and quite inelegant) climb up the ladder, more people than I cared to notice staring up at me, and barely being able to hold onto the bar, the moment of expansion I experienced while swinging for those few seconds was exhilarating. I guess sometimes you have to climb the tallest ladder for a few seconds of joy. It’s painful and crazy and, ultimately, worth it.



And here I am in full expansion mode: club-med-trapezing



Sunday, December 4, 2016

Survival of the Fittest?

The first time I ever went dancing with my now husband, he called me Elaine. Of course, as a fellow avid Seinfeld watcher, I knew exactly what he meant. I wasn’t offended, because if there’s one skill I always had confidence in, it was my dancing. If you’re not familiar with that particular Seinfeld episode, it’s the one when Elaine gets a company party going by showcasing her hilarious, ridiculously uncoordinated dance moves. I think what perhaps my husband was tapping into at that moment was my complete lack of self-consciousness on the dance floor. (Or so I liked to believe.) You know that bumper sticker that says, “Dance like no one is watching?” Well, before I ever read that, I never even knew that was a thing. Was I supposed to feel self-conscious when I danced?

Dancing has never really felt optional to me. It’s almost like an involuntary reflex. I cannot hear a good beat or feel a soulful rhythm and sit still. I just don’t know how to do that. I’ve never understood people who sit in their seats at a concert. I’m not judging, it’s just not something I can relate to. I can’t even sit still in my car when a good song comes on.

I did a lot of dancing up through my mid-20s. But then, between residency and having kids, life just didn’t really allow as much room for it. Plus, the times I did try to go to a club or a bar in my 30s, I either didn’t recognize the music or the dancing didn’t start until far later than my chronically tired adult self could handle. That’s partially why for my 40th birthday, a year and a half ago, I had a dance party with my very own DJ who could play my very own favorite songs. On some level, I knew it was maybe going to be my body’s last chance to “dance” in a way that still felt somewhat familiar to me. I had a blast that night. I didn’t care so much that I was “dancing” with my cane. Or that I couldn’t move my body in the way that I used to. The joy I felt being surrounded by so many good friends and such good energy took over. I felt satisfied that I had one last all out dancing hurrah.

I hadn’t attempted to dance since that night (not counting in my car or with my boys at home). But then last night a dear friend of mine celebrated her 40th birthday with a dance party. I could not have been more excited to go. I love her deeply and even swore to her on the phone hours before that I would be the first one on the dance floor and the last one to leave. Sounds kind of silly now writing it out loud, but I genuinely believed it. I went in with that exact intention in my mind and in my heart. But, as is often the case lately, my body had different plans for me.

I was so excited when the lights dimmed and the first good song came on. I jumped up and made my husband come with me. Not just because I love him and would always prefer to be with him than without him. But also because I now need him if I’m going to try to do some semblance of dancing and expect to stay upright. I was actually quite impressed with myself. In spite of the fact that I had to hold on to at least one of his hands the whole time, I was still able to get lost in the music. I felt happy. Pleasantly surprised. But of course, I couldn’t keep it up for very long. I started to fatigue. And no matter how good of a sport my husband is, if you know him, you know that a crowded party and loud hip hop music is not at the top of his list of favorite things. We sat down back at our booth. We had spent most of the night sitting at that booth simply because I can’t stand for very long. I’m actually amazed that most people seem to be just fine standing all night at a party. We didn’t know many people there which was fine, but small talk’s never really been my thing and because we were sitting and most everyone else was standing, we weren’t doing much interacting.

My husband slowly drifted into his phone and I just sat next to him. People were really starting to get down on the dance floor. Then some of my high school favorites came on- Beastie Boys, Run DMC, Black Sheep. Music that made me not just nostalgic for my youth, but for my pre-diseased body. The body that would’ve hopped up, with or without my husband and joined the gaggle of girls dancing with the birthday girl. The body that would have felt sweaty and alive and carefree. The body that could have expressed her celebratory feelings in the most disinhibited way. I initially took so much pleasure in seeing other peoples’ pleasure in dancing, seeing them shimmy and shake and put their hands up in the air. But after a while, all-of-a-sudden, it started to feel like too much. I wanted so desperately to be just like them. I wanted to partake in that kind of fun. I wanted to have my body back. Just for one night. And just like that, sitting in that booth, staring onto the dance floor, I started to cry. I desperately tried to hold it in, to distract myself, to focus on how happy the birthday girl was, but it wasn’t enough. I didn’t even want my husband to see. I didn’t want to “ruin” the night for us. The kids were sleeping at my mom’s. This was going to be a super fun night. I looked at him, made some snide comment about him being on his phone and then let it out. He cradled me into his chest and I let in all the pain. It hurt. On top of it, I felt sad that the collective joy of the night wasn’t enough for me to keep it together. I felt foolish that I didn’t recognize the role denial was playing until it was too late. (Which is silly, because that’s exactly how subconscious defense mechanisms operate.)

I lasted long enough to sing happy birthday and watch her blow out the candles. Then we snuck out and I continued to cry the whole way home. Tears for the past, present and future. For all the upcoming dance parties I wouldn’t be able to enjoy. My husband lovingly and jokingly reminded me that I probably didn’t have to worry too much about future dance parties since we don’t really get invited to that many these days. I laughed because he was right. And then I cried some more.


I haven’t blogged much lately. Not because I haven’t had anything to share, but because the more depressing the losses, the harder it feels to put them out there. I am experiencing changes in my body that are becoming increasingly harder to manage. People used to ask me if I had pain associated with my disease, and they (and I) took comfort in the fact that I didn’t, but now I do. I suppose it was only a matter of time before I experienced the kind of chronic pain so many other patients have described. I am starting to experience more fasiculations in my larger muscle groups, primarily my hamstrings. Fasiculations are involuntary muscle contractions that can be normal in people with healthy muscle tissue, but in someone with a neuromuscular disease, it indicates a dying muscle’s last-ditch attempt to contract. I now have enough swelling in my feet to warrant considering wearing compression stockings. That might not be so bad except for the fact that my weak fingers won’t even be able to pull them on. The other day my younger son asked me to come outside and play basketball with him. He and I both know I can’t really move around, but that day I discovered I no longer had the upper body strength to throw a basketball over head.

I don’t have a satisfying message here. I don’t have a silver lining in any of this. There is simply only one thing I can rely on and that’s the purely biological process of adaptation. I recently read a quote modifying Darwin’s theory of evolution: “It’s not the strongest or the fittest who survive, but those who can adapt best to change.”

I have to believe the same thing I tell my patients all the time-we are biologically wired to survive, to move through and adapt to loss and change. I have to believe that though my circumstances may never change, the intensity of my emotional response to them will.

I have to believe that. Because I simply don’t know how else to move forward without it.

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.

Wednesday, April 27, 2016

emetophobia* (of a different kind)

It’s like that feeling you get when you know you are going to throw up, but you don’t know when. You try to convince yourself that the nausea will pass. You distract yourself. You wonder if maybe your body’s just being hypersensitive. You are desperate to avoid the extreme unpleasantness that comes with vomiting up your insides. And yet, you know that you’re inevitably going to have to endure it if you want some relief. Once it’s over, your stomach will eventually settle. But it’s so scary. And so uncomfortable. You hate it. And there’s nothing you can do to stop it.

I’ve been trying to avoid the metaphorical throwing up for a few weeks now. In other words, I’ve been desperately clinging to denial. It sounds something like this…Am I really getting worse? No, I’m probably just feeling weaker because I’m recovering from the flu. Am I really having trouble lifting my arm up to hang clothes on the higher rack in my closet? No, this jacket is probably just particularly heavy. Am I really going to need to start using a cane full-time? No, it’s just the shoes I’m wearing today that are making it difficult for me to walk. When I get home, I’ll be sure to add them to the pile of shoes that no longer work for me.

I have become a master at this. I will go to great lengths to avoid coming to terms with the truth. I will distort my perception of my reality for as long as I can to avoid feeling the pain that comes with confronting it.

When I’m comfortably in denial, it usually means that my disease progression has slowed for a bit. It means I have moved through grieving my most recent loss of function and adjusted to my new limitations. It is during these glorious times of disease stability that my frenemy, Hope, pays me a visit. She says to me, “Sure, this is bad, but you can live with this. And, let’s not forget that there is always a chance that you won’t get any worse than this.”

I try not to get too close to her because I know she can hurt me, as she has so many times before, but I also feel reassured by her presence. She’s the one who helps me turn up the volume of everything else when I’m trying to drown out the voices of truth. The voices that yell out at me when I stand up from the our living room sofa, not wearing my leg braces, and I literally cannot take a step in any direction without losing my balance.
“Turn it up! Louder!” we say.

She’s the one who tries to help me breathe when the biologically-programmed panic of not being able to fight, flee or protect my young sets in. She tells me that even though I may feel as if I’m vulnerable prey, there are no predators to fear in my modern day life. I can and will continue to adapt. I will be responsible about enlisting help. And ultimately, I will survive.

But over time, I notice she’s not hanging around as often. She’s not there to help me in my quest to convince myself that it’s going to be okay. Changes start to set in. Painful experiences become harder to look past. New social situations present themselves and I realize I’ve lost any chance of trying to “fit in” or not have my disease be obvious. Those days are over. And eventually, the whispers become increasingly louder. That feeling sets in. I know it’s coming. I know I’m going to have to start to feel the sadness again. The pain. I don’t want to. I’ve just gotten comfortable here. I don’t want to feel sad. I don’t want to cry. I don’t want to live in the truth. It feels so bad. It feels so icky. Repulsive almost. I’ll do anything to avoid it.

And yet, I’ve been through this cycle enough times to know that if I want to get past it, if I want to reach the land of acceptance, there is only one way to get there.

We try to do everything we can to bypass the journey or even just make it more tolerable- binge on TV shows or food, get high or drunk, lose ourselves in work or just try to fall asleep and hope that when we wake up, we’ll have arrived.

But nothing can ever quite do the trick.
I know I have to let it all come up and out.
I know I have to stay in it until it’s over.
I know that despite it getting exponentially harder to settle back into the new baseline, there is no other way to keep moving through.

I also know that most of my blog entries are just collections of (sometimes overdone) metaphors trying to capture the pain that comes with moving through my own personal 3 stages of grief: denial-depression-acceptance. Sometimes, in these raw, non-distorted moments of truth, it feels as if the writing is the one thing I have to hold onto. So, I guess I’d just like to thank you for holding onto it with me.


*emetophobia: an intense phobia that causes overwhelming anxiety pertaining to vomiting

Thursday, March 10, 2016

Finally, I’m here. I’ve arrived!

When I was first diagnosed with HIBM (or as it’s now often called, GNE myopathy) almost 10 years ago, I couldn’t even say the name of the disease out loud. I could barely accept that I had it. I didn’t want anything to do with it. I didn’t want to read about it, I didn’t want to talk to any other patients, I didn’t want to attend any fundraisers and I most certainly did not want to meet any other patients. It was all I could do to cope. I was barely hanging on. It was all too much. Too scary. Too painful. Too real. So I didn’t push myself. I went at my own pace. I knew it was to the dismay of some loved ones who thought it would benefit me to reach out to other patients for support. Especially those who seemed to be doing quite well living with the disease. But I wasn’t ready. I admittedly felt a bit self-conscious of my process. It seemed that other newly diagnosed patients were able to jump right in and access any and all resources that were available to them. But I wasn’t.

In the early years, I cried all the time. I cried not only about what was happening in the moment, but also about what I realized was never going to happen in my future.

I cried every time I thought of not being able to dance at my kids’ weddings. (For whatever reason, that one came up a lot at the beginning). Then, as I continued to progress, I cried when I thought about possibly not being able to stand up on the bimah at their bar-mitzvahs.

I cried (and became angry and defensive) the first time somebody suggested I consider surrogacy as a way to have a second child to complete my family.

When I watched my mother and mother-in-law act as night nurses for my boys when they were babies, helping me throughout the night, voluntarily exhausting themselves for the love of their grandsons, I cried because I knew I’d never be able to do that for my own children and their babies.

I cried myself to sleep the first time I went to the snow with my 10 month old (who will be turning 9 in May) because I knew then that I’d never be able to ski down the mountain alongside him the way my parents did with me. And when someone so lovingly told me there were adaptive ski programs at most mountains, I became angry and defensive because I wasn’t interested in some special program to help me. I was interested in having my old skiing body back.

I used to cry a lot. And I still do my fair share of crying, but it’s different. My mind travels to all of those same places in the future, 10 or 20 years down the road, but when it gets there, it sees something very different from what it saw back then. I was wearing different lenses then. I couldn’t see through the pain and the loss. It was cloudy and dark. I was too angry to try to envision any other scene. I simply couldn’t.

But of course I couldn’t. I hadn’t yet honed any coping tools at the start of this journey. I couldn’t possibly imagine tolerating where I am now when I was all the way back there. Quite frankly, it would have been odd if I had been able to embrace it. I just wasn’t there yet. And no matter how many people tried to reassure me that I would eventually figure it out or that it would be okay, I didn’t buy it.

Here’s where I am now…

For the last few years, I have taken my family to the snow and managed to ski standing up, with some simple modifications, for a few hours each trip. The last time I did, I lost control and gently crashed into a group of young girls in ski school, one of whom said so adorably and sympathetically, “It’s okay, don’t worry, we were all beginners once!” As I smiled at her sweetness, I felt that familiar pang in my heart that comes with every new loss. I knew that was the last time I would try to ski standing up.

Spring break is approaching. My younger son is turning 5 and he is ready to get serious about skiing. And so am I.

A strange thing has transpired. I am excited. I am excited to enter into this new world. I am giddy just imagining myself skiing down the mountain with my family. I spent 30 minutes on the phone with the head of the Mammoth Mountain adaptive ski program yesterday. She was awesome. Who knew there were so many options? A ski bike? A mono-ski? A bi-ski? I’m so curious to see which one will work for me. I am so excited to just get on the mountain. To show my boys what I can do. To feel the cold and work up a sweat. To get to earn my apres-ski hot chocolate. It’s strange. It almost feels as if I’m betraying my younger, more skeptical, more defiant self. As if I shouldn’t be okay with this. And yet, it also feels as if I’m giving her a precious gift. One I couldn’t receive until now.

I wish I could give this gift to newly diagnosed patients. I wish I could say to them, “Look at me! I’m doing it! Don’t worry, you’ll figure out a way too! Your own way!” But if they are anything like I was then, they will likely just cry while reading this. Or feel angry. Or hopeless. And I wouldn’t blame them.

Everyone has his or her own process. I am only here to share mine. Mine doesn’t always feel as easy or as fast as I would like, but it got me here. I’m here. I made it! Right now.

And for that, I am so grateful.



Monday, February 15, 2016

Pain is inevitable. Suffering is a choice.

I’m coming up on 10 years of knowingly living with this disease. An entire decade. One fourth of my life.

Throughout these past 10 years, I have mourned losses, adjusted to new baselines, adapted new ways of coping, advocated on behalf of patients and invested a tremendous amount of emotional energy in trying to keep perspective and stay in touch with the gratitude for all my blessings. I am proud of myself for tapping into resources within me that I never knew I had.  I am proud of myself for learning how to hold the pain and the joy in one hand. But also, I am exhausted. It feels like I’ve been at it for long enough. Too long. I want to be done with the mourning and just focus on the coping. I want this part to be over.

It would be so nice if that’s how it worked. You put in your time, you pay your dues, you demonstrate your unexpected resilience in the face of adversity and then you get to be done. And by done, I don’t mean done with all the challenges and disappointments that are inherent in life. Those are inevitable. I mean done with some to allow room for others. Because there are always others.

Life as an adult is hard. It’s hard to navigate the complexities of parenthood. It’s hard to grow older and confront the losses of loved ones as our mortality becomes more real. It’s hard to navigate the waters of the work-life balance and not feel exhausted by the swim. Add extra stressors to the mix and it can feel overwhelming. And then add ones that are chronic and it can feel despairing. That is what I think I have been in touch with lately, the despair.

I’m sick of the process. I’m sick of having to talk myself down from the panic that sets in when I get a glimpse of what’s coming based on what’s already here- more frequent and more serious falls, more difficulty taking heavy things out of the fridge, more difficulty getting out of a chair. I am tired of not knowing whether I should renew my office lease for another three years, tired of rushing to plan vacations despite them not being the ideal time/stage for my family simply because my physical abilities are on a ticking clock. I’m exhausted by constantly trying to pull myself back into the present moments. In each of those moments, I have to run through the five stages of grief at an accelerated pace. I have to constantly hope that maybe this disease won’t ever get as bad as I think it will. As bad as I know it can get.

But that hope comes with a cost. It’s increasingly harder to hold onto it in the face of continued progression. So rather than invest in the hope that I won’t get exponentially worse, I am going to try to start shifting my resources towards hoping that despite how much worse I get, I will still be able to hold onto me.

That’s what I’ve been afraid of all these years. Losing me. My smile, my upbeatness, my desire for adventure, my spontaneity, my silliness, my capacity to feel carefree, my ability to have fun. I have feared that I will become so disabled that I will no longer have the capacity to feel truly happy. And so as a result, I have desperately held onto a need for me to not get worse in the service of maintaining my emotional health. But that in itself is exhausting. The holding on.

I am going to try to loosen my grip. Let go of what I think I require to be happy and wonder if maybe there’s more to it. I will stop trying to convince myself that my body is going to be “okay” and instead accept that maybe it won’t be.

I do not perceive this as a defeat. I do not see this as me not having a positive attitude or me giving up on hope. It feels empowering, like an act of self-compassion, a way to preserve my strength and my emotional energy.

I am tired. I want to stop struggling to stay afloat in this sea of unknowns and instead gently turn over and try floating on my back for a while. I want to catch my breath. I want to feel the calm. I want to see where it takes me.

Friday, November 27, 2015

Thank you

I was going to post yesterday, but I chickened out. I was going post about how, for someone who feels in touch with gratitude on a regular basis,  it was disappointing to not feel it to the degree I normally do on Thanksgiving.  Thanksgiving is like my gratitude Superbowl. It’s when we are all so in touch with all the ways in which we are blessed.  But I haven’t been feeling as grateful in this past month.  It sounds so taboo. I guess it’s not that I haven’t been feeling grateful,  it’s just that it’s been harder counteract all that I’m not  grateful for. And yesterday,  I didn’t want to be a buzzkill. Or even offend anyone. So today,  I wanted to share. I wanted to tell you about some experiences that have felt hard in this last month. And where I have ultimately found the gratitude.

In October, my husband and I got away to Portland for the weekend. It was so great, except for the sad realization, once and for all, that I can’t really explore a new city on foot the way I used to, the way I wish I could.  Even stepping up onto a curb has proven to be a challenge. While we were there, on that Saturday night, we waited in the hour-long line in the drizzling rain at a famous ice cream shop. We were having so much fun, except for when I walked out of line to go to the bathroom, slipped and fell, had a crowd gather around me, heard a bystander so sympathetically say, “oh no, she has a cane” and somehow ended up getting helped up by a police officer.

A couple of weeks ago, I attended an annual psychopharmacology conference at the Skirball. It’s always a wonderful opportunity to see old friends from residency, but this time, it was also an unwelcome opportunity to see how much I’ve progressed since the last time I was there. I can handle the more “minor” challenges like difficulty clipping my name tag onto my lapel or trying to keep up with everyone while heading to the luncheon area. But sitting down for lunch and watching everyone at my table so effortlessly cut into their food, while I sat their acutely aware of how awkwardly I now hold my utensils and struggle to cut my own food, did not feel like something I could handle. It took all of me to not flash forward to a time where I might have to sit down to a meal and ask the person next to me if he or she wouldn’t mind cutting my chicken for me.



If you’re still here, if you’re still reading, if you are still with me, I am grateful for you.
I have tried to hold these experiences quietly within myself. But I can’t. I can’t hold them alone. They are too heavy. They will crush me.

Thank you for holding them with me. Thank you for not running away out of discomfort or fear. Thank you for receiving my invitation and for choosing to attend.

I am grateful for you and I am grateful for the one thing this disease has forced upon me- the strength to share and be vulnerable. Because if I didn’t have it, if I couldn’t do what I’m doing right now, I would be so lonely. And the loneliness of carrying this by myself would disable me in a way that, unlike all the rest, I wouldn’t be able to adapt to.

Sunday, November 1, 2015

Kitchen dreams (and nightmares)

As I sat down to write this post, I realized how often the topic of my home has come up on the blog. Or at least, that’s how it feels. Probably because I’m so intimately acquainted with it on a such a micro-physical level. It plays an integral role in my daily life and overall feeling of well-being, as I’m sure many homes do for many people, but in different ways and for different reasons.

We recently embarked on a kitchen remodel. We wanted a new kitchen not only because we love cooking and being in the kitchen (especially with Noah’s recent into foray baking bread), but because as I slowly get worse, I am becoming increasingly impatient with my limitations. I am continuing to have to increase my tolerance for frustration and, quite frankly, I’m over it. I love cooking, I love coming home from work and making dinner for my family, but as the only adult home during that time, I can’t function as efficiently as I used to.  As my calf muscles melt away, it’s becoming harder to stand on my tippy toes to reach the dishes on the second shelf of the cabinet. Or as balance becomes trickier, it’s getting harder for me to walk over to the trashcan holding food waste and then press the pedal that lifts open the lid. And it’s been a while since I’ve been able to fill up a big pot with water and lift it up onto the stove by myself.

I initially justified the expense to myself by saying I needed my kitchen to be accessible and adaptive to my needs. Naturally, right? Makes perfect sense. Then once we decided to move ahead, I quickly forgot that part.  I had told myself that my job in this process would be to be mindful not only of my physical needs now, but also to try to anticipate my future needs with this disease. But who wants to do that?  That’s no fun. The fun part is going to appliance warehouses to pick out cooktops or staying up late to tag photos on design sites. It is the two of us embarking on this journey together, for the first time ever. Creating something together. Making something completely our own. Customizing our physical space. I now fully appreciate why people who have the funds often go that route, building an entire home. It’s such a luxury- building your vision, watching your dreams transform into reality.

In the first couple of weeks, when questions came up from the contractor about what would work for me physically, I told myself in a very quiet but very strong voice, “Oh come on, you don’t really need to worry about that. You won’t really need to check the counter height to see if it would be suitable for a wheelchair, because you’re never going to get there.” I tried to believe it.  Was that my defense mechanism kicking in and trying to prevent me from confronting the truth? Buying me some time? Or was it a rational voice that genuinely believes that the scientific community will figure something out before I get to that point? Of course, there is no one, not one person on this planet, who can answer that question. It’s a scary place to be. I would think it feels lonely, but it’s surprisingly, reassuringly not. Because I am not alone. I have people next to me who hold it with me, no matter how heavy. And wherever I go, they follow along right beside me, still carrying it. Of course, they didn’t just appear alongside me, ready to work. I had to invite them to come along. I had to let them in. I had to expose my vulnerabilities. [See previous blog post on the benefits of vulnerability and love being everything,]

Although I have people to share the load with me, that doesn’t mean I am always willing to hand it over. Sometimes, just sometimes, I want to let the people around me relax. Take a break and just take the load off. Sometimes I just don’t want it to be about my disease. Not because they need that, but because I need that. Sometimes I just want to pretend. Try on what it would feel like to not have this disease. I don’t want to have to mention to my husband in an attempted nonchalant way that we may indeed have to choose our flooring based on whether or not it will leave an uneven surface between the dining room and kitchen which will potentially make it difficult for me to walk with my future gait.

I want to be able to choose things because I like them, not because my disease and my progressively weakened body requires me to need to have them.

And yet, that simply isn’t an option for me.

So I will feel sad about it for a little while. I will feel that vague bummed out feeling.  That feeling you aren’t always conscious of  until you’re reminded of it and realize, “Oh yeah, that is what’s been feeling hard lately. ”

And then I will presumably get back to my happy, grateful, I’m-so-lucky-I-even-have-the-opportunity-to-do-this-kitchen-remodel baseline.

Yes, I know I can do it. But that doesn’t feel as satisfying as it used to,  just knowing I can do it. Sure, I know I will move through this. I just wish I didn’t have to.

And yet.

Wednesday, October 7, 2015

The million-dollar question

Exactly a month ago, I was walking over to say goodbye to my younger son and our nanny at an indoor play area and I tripped on a toy and fell. I went down hard and my left hand, the much weaker one, bore the brunt of it. A bunch of people saw and rushed over to me, but per my usual, I played it cool. I told them I was fine. I can’t stand that kind of attention, probably because it’s on top of what is now a new baseline of daily unwanted attention. Luckily, my son didn’t see and I decided to skip the kiss goodbye and instead wave from afar. I needed to get out of there as quickly as possible. I came outside and saw blood leaking out from under my rings on my ring finger and discoloration on my skin. It hurt like hell. I walked to my car, not just feeling the physical pain but the emotional angst of realizing that I probably wasn’t going to be able to go exercise that morning. (Exercise is no longer an optional luxury but rather a means of muscle preservation and survival.) Within minutes, my ring finger started to blow up. Luckily I had the sense to take my rings off right away. I started driving. I didn’t know where to go. All I wanted to do was drive to the gym. But two blocks in, it was getting so bad that I had to pull over at a random Taqueria and ask for a cup of ice.

I really did not feel like spending the morning at the urgent care. So I started driving towards my physical therapist’s office. Maybe she would tell me it didn’t look that bad and I could go about my day. But of course, she was worried it was fractured and told me I needed an x-ray. Of course I did. I knew that I did. So I cried to her and she gave me a hug in that most tender, loving compassionate way that she always does when she sees first-hand how unforgiving this disease is and I headed to the urgent care at Kaiser.

It’s amazing when you enter a hospital setting and see so many others using a cane. And then it’s even more amazing to see that the majority of people using a cane are all elderly or seem very infirmed.

I sat in the waiting room, wondering how many more times I would find myself there. In the next year. Or the next five years. Would it be for a for laceration on my forehead like another HIBM patient I know? A broken arm or a leg? Not catastrophic fears. Real outcomes in real patients. The hardest part of that urgent care visit was when I had difficulty arranging my already weak fingers in the correct positioning for the various views needed for the x-ray. The reminders never stop. There’s never a pause in the awareness that I have this disease. Or that I will always have this disease.

For all intents and purposes, I had fractured my finger. Though he couldn’t detect a break on that initial x-ray, he said it’s common for a fracture to show up later on and based on how it was presenting, we would need to treat it as such. By that time, the entire finger and parts of the surrounding ones had turned purple and black and my finger was swollen in a way that was rather fascinating to me. He put my finger in a splint and that was that.

That was a month ago. Though the swelling has reduced somewhat and the discoloration has started to fade, it remains incredibly painful and I have almost no range of motion in that finger. On the one hand, my joke has been that it’s such a weak finger anyway, it doesn’t really matter what happens to it. On the other hand, because it is already so weak, I need to try to preserve strength as much as possible. To be quite honest, the most frustrating part is that I haven’t been able to wear my wedding and engagement ring.

So a few days ago, I decided I should probably go in for a follow up and figure out what’s going on. I had the appointment this morning. The doctor was a lovely middle aged Persian man with kind eyes. He looked at me then looked at my cane and I quickly explained that the cane had nothing to do with why I was there. Well, not really. He asked me what the cane was about. I put on my doctor hat and told him about HIBM. He was shocked. He’d never heard of it. He wanted to write it down. He asked questions. He asked if I had seen the neurologist at Kaiser. I said there was no point in being followed by a doctor. He said that’s true, but he was actually thinking about for other patients as there are a fair share of Persian patients at Kaiser. Of course, I said. Then he started to examine my hands. It’d been a while since I’ve had that kind of exam. He asked me to bend the tips of my fingers on my left hand. To resist against his. I told him and showed him that I couldn’t. He looked up at me and when his eyes met mine, I saw in them a mixture of disbelief and confusion. Then he asked me if I could bend my fingers at my knuckles. Of course, I couldn’t. I could see him trying to take it in. Not understanding how this mysterious disease worked. I saw him compare my two hands and feel where the atrophy has melted away the muscle between my thumbs and forefingers. The area my eyes are always checking in on but my mind is constantly trying to ignore.

I felt my doctor hat slipping off and my patient hat coming on. I tried to resist. I told myself not to cry. I repeated it to myself like a mantra. Don’t cry. Don’t cry. I swallowed it back down. We talked about how there isn’t a whole lot to do. We can “buddy tape” it, but the fractured finger doesn’t have a healthy ‘buddy”
to move around with it. We could put on a night splint, but unlike a normal finger, my fingers don’t really bend much anyway so it’s not really indicated.

He said he would refer me for hand therapy and we left it at that. Lastly, I asked him when he thought I might be able to wear my rings again. Or if my finger would just remain this deformed permanently. He said that was the million-dollar question. My least favorite answer.

My rings. My beloved rings. I’ve been so hung up on the fact that I haven’t been able to wear them. Why? Why has that been such a big deal? It’s so minor in the grand scheme of things. But then I let myself unpeel that piece. And underneath was hiding the realization that when I leave the house with those rings on, they serve as my shield out in the world. They protect me. They reassure me. They scream out that I am loved. That I don’t just have this thing going on. This unsightly gait. (Please allow me to hate it.) I wear my ring and I am reminded I have have a whole life that balances this. This what you see before you. They represent the innocence and carefreeness I was so blessed to live in during my pre-disease life. They are my security blankets. Without them I feel vulnerable. I feel insecure.

I wish i didn’t find myself in a position where this is something that I care about. Something I find myself relying on. And yet, here I am.


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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