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

Thursday, October 12, 2017

My voice in the Jewish Journal

I have always drawn a clear line between my personal life and my professional life. I pride myself on being a “boundaried” therapist. I don’t typically disclose details of my personal life (e.g. whether or not I have kids) and I don’t insert my feelings or opinions into the room. I welcome questions of any nature, but rather than offer answers, I use those moments as opportunities to explore what may be coming up on behalf of the patient. I have always subscribed to the theory that the more patients know about their therapists, the less authentic they may be; they may be more likely to censor themselves out of fear of being judged or in an attempt to please the therapist or there may be other subconscious processes that could potentially contaminate the work.

It is tricky to maintain privacy as a therapist in the digital age. For the last 10 years, I have been fiercely protective of mine. I don’t have any identifying features on my blog and I have asked people to not attach my last name to any speeches or events they may be publicizing for the NDF.

But as the voice in my head grows louder around my own acceptance of this disease, so does my willingness to invest in patient advocacy and disability awareness. I feel ready to use my voice.

A couple of weeks ago I was asked by the editor of the Los Angeles Jewish Journal, David Suissa, if I would contribute a column. In the past, I’ve always responded “no“ to such requests. This time, I said “yes.”

Here is the link below:

Growing From the Depths of Pain

Wednesday, October 4, 2017

The In-Between

Let everything happen to you
Beauty and terror
Just keep going
No feeling is final

–Rainer Maria Rilke

I’ve had some hard moments in these last few weeks. I’ve had to navigate some unexpectedly tricky terrain on middle school tours while trying to keep up with the rest of the pack. I’ve had to face the fact that I can no longer walk across the grassy area where all the families with young kids traditionally congregate after high holiday services. I had to stand up in front of all the parents at my younger son’s class social to talk about my role as the fundraising rep while simultaneously realizing that I was having trouble simply standing. And then I had to ask a mom whom I barely know to link arms with me to help get me up 4 stairs.

Rewind to last month when sign-ups went out asking for  parent volunteers to chaperone the various 5th field trips for the year. As someone who loves an opportunity to get an in-vivo glimpse of my kids out in the world among their school peers, I always hope that some of the field trips will fall on Wednesdays and Fridays, the days I’m not in my office.

I saw on the list that there was a full day trip to the California Science Centeron Friday, October 20th. It’s one of my favorite LA kid places. I’ve been on field trips there before, but that was when they were in preschool, before I was even wearing leg braces.

When I received the email, I was in a good place. I knew my son would be thrilled if I joined, and so in a very rational and non-emotional way, I decided it would be a perfect opportunity for me to use my TravelScoot. I had just used it all weekend in Washington DC. It’s not my speedy bright yellow standup scooter, but rather my sit-down, slower- moving, below-eye level mobility scooter. I’ve never used it in my “real life” – as in, never in my day-to-day, get around LA life.  I cannot lift the scooter out of my trunk, but I figured I would just ask another adult to help me. I signed up and said I could seat five kids in my car.

Then a few days ago, it occurred to me that when I put my fully assembled scooter in my trunk, it takes up the whole back row. That leaves seats for only two kids. This awareness coincided with me being in a more raw and vulnerable place about where I’m at in my disease right now.

Each of my micro losses, or micro traumas, slowly chip away at whatever self protective defense mechanisms I have left in place. They form cracks in my resolve to keep showing up and participating. They make the path of avoidance appear so much more appealing than the one of exposure to the discomfort.

I emailed the teacher explaining that somebody else should probably drive because with my scooter in the car, I could only fit two. I felt relief. I had started to let myself think about what it would actually look like: me having to find the elevator when everyone else was taking the escalator, potentially having to field questions or comments from the kids in the 3 classes going, confronting the reality that I am the only physically disabled parent among all the able- bodied parents in our group (as far as I know).

And that’s when it started. That’s when I knew I had to start feeling and seeing the truths hiding behind all of those hard moments in the past few weeks. It is a sickening feeling. It is fear and panic. It is dread. It is knowing what is coming and knowing what is no longer. It is the loosening of the grip on what I so desperately wish could remain my reality. It is the excruciatingly painful part of the process that occurs right before I am about to make another adaptation. And it is always, always, exponentially harder than the time before, because that is the reality of living with a progressively debilitating disease. It doesn’t get better. It doesn’t stay the same. It only gets worse. It is the feeling that it will be impossible to adapt yet again. And knowing that it will have to be possible, because there is no other way. It is desperately not wanting to be here, where I am.  And then finding myself trapped, right here. It is knowing that it is been a long 11 years. And at the same time, it has only been 11 years.

The exceptions to the rule have now become the rules. The TravelScoot was to be used just for traveling. It was to be used when I had long distances to traverse or new terrain to navigate to make vacations easier. That’s how I tolerated the emotional discomfort that came with purchasing it. The story I told myself was that it would only be used on certain occasions, on my terms. But this disease doesn’t operate on my terms.

I have learned by now that I do most of my crying and grieving before the actual adaptation takes place. After I’ve used up most of my strength trying to hold on as tightly and as fiercely as I can to what I wish were the truth,  I use the last bit of strength I have to just let go.  I let go and float from the scary In-Between unknown to the sad but grounding known.

This particular In-Between is the one I’ve been dreading most of all.  It’s in between standing and sitting down.  It’s not happening all at once, of course, but here is the part where I begin to loosen my grip.  I begin to decide how to plan my first exposures to this new reality. The In-Between is this cruel, exhausting space and I know I’ll be spending some time in it.

Will the class field trip be the first time I decide that participation in my “real life” means sitting in my scooter and tolerating reactions (especially my own)? Yes, this is what I go through when I get an email sign-up sheet. It’s a lot.

As I move through the terror, I desperately hold onto what I’ve learned from the pain along the way, which is that yes, I will keep going and no feeling is final.

Monday, September 18, 2017

What’s the plan?

This weekend we had the honor and pleasure of attending my husband’s cousin Julie’s wedding in Washington D.C. Julie is one of those shiny bright sparks in the world. She is the definition of exuberance. (Don’t take my word for it, just ask one of her eight best friends/ bridesmaids.)

Not only were we excited to celebrate her marriage to the equally fabulous Justin, but we were thrilled to take the boys to D.C. for the first time, even if just for a few days.  We were going to take in as many sites as possible and our dear friends were coming in from New York to spend a day with us.

I felt confident the trip would be a success. I had traveled with my cool new TravelScoot once before and it had been a game changer. I was ready for the adventure and in a great mood when we left. So much so that when I struggled for the first time to turn on the airplane overhead fan because of my worsening upper body weakness, I didn’t let it get me down. I registered it but chose not to react emotionally to it and I chose not to think about what it meant for the future. I am always assessing whether it’s worth expending emotional energy on a loss versus reserving it for later. I let it go.

The true joy and happiness I derived from being in D.C with my family, extended family and good friends raised my threshold for tolerating most of the frustrating and disappointing moments I encountered while there.

There was the first restaurant we stopped into for breakfast on day one that didn’t seem very interested in finding a space to store my scooter. (We left, of course.) There was the second breakfast place that was happy to accommodate my scooter, but though the bakery was downstairs, the actual restaurant was upstairs. (I climbed the stairs and my husband carried the scooter up.) Then there was the shocking discovery that the bus tour we had planned to take, the double-decker Hop On Hop Off bus, wouldn’t let me/wasn’t equipped to bring the scooter aboard. Even if we broke it down and stored it in the carrying bag, they wouldn’t allow it. They said that though they did have some accessible buses, they ran much less frequently. (This, in our nation’s capital, mind you.) No matter, we went with the Old Town Trolley and had ourselves a wonderful time.

We stayed at the Four Seasons Hotel in Georgetown where the wedding was being held, courtesy of the bride’s incredibly generous parents. (Thank you again, Aunt Max and Uncle Alex.) Though I had lived in D.C. for a summer after college, I had never spent much time in Georgetown. For those who don’t know, the sidewalks consist mostly of cobblestones and bricks. Uneven walking surfaces are a foot drop patient’s worst nightmare, with or without leg braces. When I did take a break from scooting to walk (one day I’ll write about how physically uncomfortable it feels to move around while sitting all day), I had to hold on to my husband for dear life at all times. Suboptimal for me and for him, especially when he needed to tend to one of our boys. But again, the charm of the city, the indulgent treat of the fancy hotel, and the positive vibes of the weekend outweighed it all.

Even when at the end of the beautiful wedding ceremony, I stood up and tried to stabilize myself on a chair before pivoting towards the door and subsequently took down a handful of chairs with me as I fell, only to be caught by my husband, I still didn’t let it get me down. I shifted right into easing-worry-and-discomfort mode for the concerned guests who were gathered around me. I cracked a joke about feeling relieved I didn’t knock over one of the aisle candles and light the room on fire. (I internally felt extremely grateful that I didn’t have a mishap during the actual ceremony.)

The cocktail hour came and went. I’m used to not being able to serve myself food at a buffet anymore. (If you’re holding a cane in one hand and a plate in the other hand, all the while trying to maintain balance, there’s no third hand to serve yourself food.) But that’s where the willingness to be needy and ask for help, sometimes from total strangers, really comes in handy.

Finally, we entered the ballroom for the reception. The band was on fire. Most everyone went right to the dance floor. I watched from my seat as the bride and groom were lifted up in their chairs and guests danced around them. I felt pure joy seeing their love playout on the dance floor. I smiled thinking about my own wedding more than 14 years ago as I watched the bride rock out with her friends and family. I remembered how blissfully happy and carefree I felt getting down on the dance floor at my own wedding, surrounded by my most favorite people in the world. It was a magical feeling. Back then, I had no idea I had this disease.

I considered getting up to try to join in on the fun, but in my sandals, my balance is worse than usual and with such a packed dance floor, I felt almost certain I might get knocked down. I didn’t need to make another spectacle of myself. Lucky for me, the people (I won’t name names) at my table weren’t really the type to jump up onto the dance floor. But as I looked around, most tables were empty. And then, little by little, my own table cleared out to join in.  Of course, not completely. My husband, who was beyond content to stay seated, was sitting next to me and my father-in-law was a few seats away. But that was it. Just the three of us.

And just like that, without warning, I started to cry. My joy suddenly morphed into pain. I felt ashamed and embarrassed. I told myself to pull it together. This was such a joyous occasion. I bent down to pick up a napkin and stayed there to wipe my tears. I was desperate and determined to not let anyone see me. I was unravelling at such an inopportune time.  I tried to talk myself down. I told myself to get over it. I told myself I had a good 30 years of it, some people don’t get any of it. I tried to use the power of gratitude to overcome the sadness. It didn’t work. My husband looked at me for a second too long and I broke again. He pulled me in.  I tried to quickly get it all out onto his shoulder. I tried to do that thing where I plaster a fake smile across my face in the hopes that my tears might get  confused and dry up. But they didn’t buy it. I found myself wishing my pre-disease baseline was of a totally different temperament. Maybe then I wouldn’t envy the revelers on the dance floor, so free in their bodies. I fantasized how much easier this aspect of my life would be with this disease if I didn’t have to say so many good-byes. But of course, I know that’s foolish because then I wouldn’t have accumulated all the joy and wonderful memories that have made me who I am.

Everything we love can be taken from us. That’s simply a fact. It’s the risk we consent to when we truly let go and give in to the joy.

In moments of pain, I often find myself making futile attempts to come up with a “plan.” What’s my plan from here on out? How will I keep this up?  If I had a plan, I think to myself, maybe it would feel easier. I start wondering, am I going to keep going to parties and weddings and bar-mitzvahs? Am I slowly going to retreat? What do other patients do? Am I going to limit my exposure to the emotional discomfort? Is that selfish?  A few years ago, my less progressed self would have adamantly responded, hell no.  But now, as my limitations become more pronounced, it feels harder to just say yes. What’s the right answer? As someone who likes to pride herself on not living life in avoidance of emotional discomfort, it feels like a betrayal. But how much can I take? Why is it so hard to say good-bye to this particular part of my identity? I’ve already somewhat successfully said goodbye to so much else. How will I ever stop missing the old me?

I wish I could tell you that I came up with a solid plan for how to move forward with this disease. But of course, I didn’t. What I can tell you is that I stayed as long as I could at the reception, but eventually gave in to my husband’s blessing that it was okay to go back upstairs before they cut the cake. As per my usual, I continued to cry all the way up to my room and then in my bed while everyone else was asleep.

But here’s the thing- the next morning, I woke up after little sleep and though my eyes were puffy, the rest of me was at peace. There were no more tears. The waves that had been crashing the night before had receded and settled down. We went down to the farewell brunch and I was fully present and engaged.  I wasn’t bitter or angry. I enjoyed mingling with other guests, debriefing about the wedding and watching how adorably my two boys were eating together at their own little table. And I especially loved hearing about how at the end of the night, the bride and groom rode the elevator with Stevie Wonder who was staying at our hotel. He even sang to them. True story.

Wednesday, September 6, 2017

My Podcast interview for “Last Cut Conversations” with Samantha Paige

Two weeks ago I attended the Neuromuscular Disease Foundation’s 4th annual patient symposium. Though I thought I’d be ready to blog about it by now, it turns out my mind is still busy unpacking all of my thoughts and feelings around it. There was the collective grieving of the failed clinical trial, the experience of meeting patients I had only ever corresponded with on Facebook, the painful realization that I am by far no longer the least progressed patient in the room and finally, the sobering news that the most promising treatment-gene therapy-will require a staggering 3.5 million dollars in funding.

In the meantime, I want to invite you to listen to an interview I recently did with Samantha Paige for her podcast, “Last Cut Conversations.” Last Cut is a multi-media documentary project that Samantha is working on about “those big life decisions (last cuts) made to bring us closer to living a life that feels like our own.” You may have seen Sam proudly and boldly baring her mastectomy scars in Equinox’s “Commit to Something” ad campaign or more recently, featured in Allure magazine’s video series,  “Dispelling Beauty Myths.” Sam has made it her mission to live life with honesty and authenticity and she is inspiring others to do the same.

I was honored to spend time with Samantha and her incredibly talented collaborator, photographer Lisa Field.  I talked about what it’s like to live with a debilitating disease, what I believe is crucial to emotional survival (love), how I think we can do better in our conversations around disability and loss, and what true freedom looks and feels like for me. I also touched upon the cultural stigma of disease in my Persian community and offered some suggestions on how to truly show up for someone who is suffering. Oh, and I also somehow managed to share the fact that people called me “Slash” from Guns and Roses in 8th grade because of my big hair. (Photo provided upon request.)

Just when I thought I couldn’t feel any more vulnerable, I learned that speaking spontaneously into a live microphone about deeply personal experiences feels a whole lot more exposed than sitting in the comfort of my own home, editing carefully chosen typed words in a blog post.  But particularly since we are living in such difficult times in which we all need more kindness and humanity, I feel it is important to put my voice out there, even when it’s a bit uncomfortable.  Thank you for listening.

Tuesday, August 22, 2017

I feel

I feel sad. I feel deflated. I feel disheartened. I feel angry. I feel betrayed. I feel frustrated. I feel helpless. I feel overwhelmed. I feel rage. I feel tired. I feel drained. I feel exhausted. I feel scared. I feel terrified. I feel desperate.

While innocently scrolling through Facebook during my older son’s High Holiday choir practice just minutes ago, I was ambushed by an article announcing that Ultragenyx Pharmaceutical is terminating the development of the drug ACE-ER because phase 3 clinical trials failed to demonstrate significant improvement in muscle strength. They’ve been at it for years. Ultragenyx’s mission is to develop drugs for rare and ultra-rare diseases.

So many patients world-wide invested so many hopes in this trial. This was going to be the first drug to come to market for us. This was supposed to work. The science was there. The commitment and resources were there. The dedication to the cause was there. And yet, we are here. I am here. In this ugly moment.

“We are disappointed by these results, as we had hoped that Ace-ER would offer a new option for GNEM patients. We would like to thank the patients, caregivers, and investigators involved in the Ace-ER development program,” said Emil D. Kakkis, M.D., Ph.D., Chief Executive Officer and President of Ultragenyx.”

I am well aware that this is how it goes. This is the process. Sometimes it works, sometimes it doesn’t.

I am grateful to Dr. Kakkis and his team for investing in us. And even for the conference room named after me. That part was neat.

I wish it could have worked. I wish I weren’t sitting here in the back of the sanctuary quietly crying. I wish I didn’t have to mourn yet another loss.

Friday, July 14, 2017

Permeable Membranes

I have been a feeler of feelings for as long as I can remember. When I was young, I used to write long, sentimental birthday and Mother’s and Father’s Day cards.  My sister used to tease me (still does), about my overly expressive, touchy-feely “treatises.” On Thanksgiving, I was always the one (still am) to make people go around and share what they were thankful for in the past year. Or on Shabbat, what they were grateful for in the past week. I have about 15 diaries from my youth, sitting in my garage, all filled with pages and pages of feelings.

I never realized what a tricky business feeling feelings was until I got a bit older. Though I was never one to cry at the drop of a hat or while watching a sappy commercial, I definitely learned early on that I was quite sensitive, more so than others. Whereas some people would feel sad at the end of a movie, I would be crying in the theater long after the credits rolled.

I started to become envious of people who had thicker skins. People whose membranes weren’t so permeable. When I went off to college, I struggled. I couldn’t find “my people.” I felt lonely and lost and overwhelmed academically. I called home countless times, crying.  I felt weak. I longed to be more resilient in the face of life stressors. What felt like a blessing at a younger age, started to feel like a curse in early adulthood.

Fast forward 10 years and at age 31, I was diagnosed with HIBM. Of course, in an instant, every life stressor I had ever experienced shifted way down on my newly recalibrated scale. I had no defense against the sadness, fear and emotional trauma. I cried and cried and cried. I felt the enormity of it in a way that people around me didn’t seem to. I couldn’t “look on the bright side” nor see anything positive in it.  I kept telling my husband that I wasn’t cut out for this sort of thing. That I wasn’t wired this way. That I wasn’t equipped with the proper tools to cope with something of this magnitude. I worried that the weight of it would crush me.

Five years into my diagnosis, I mustered up the courage to write my first blog post. I started publicly sharing my scariest, most painful and darkest moments. The really depressing ones. The really uncomfortable ones. The really emotionally intense ones. Just as I’m not so good at defending against what feelings come in, I’m also not so good at defending against what comes out. That’s not to say that it’s easy for me to open myself up. After publishing many a blog post, I have immediately regretted it. Did I share too much? Go too far? Turn people off? Bring people down?  I try to hold that discomfort just long enough to remind myself that the alternative discomfort-the one that comes with keeping all the pain locked up inside, the one that feels so overwhelmingly lonely- that one is far worse. And more destructive.

People often fear being “pitied” if they were to let others in on their struggles.  I’ve never quite understood that fear. If the basic definition of pity is “the feeling of sorrow and compassion caused by the suffering and misfortunes of others,” then why is it such a bad thing? I actually rely on other people’s compassion, both on a practical and an emotional level. Maybe I wouldn’t need to so much if I didn’t have this disease. But we are all vulnerable and in need of compassion, disease or no disease. If you are afraid of being “pitied,” then perhaps you’re afraid of people holding up a mirror to your pain and seeing what they see. Only when you can get comfortable with your own suffering,  can you accept other people’s compassion for it. And as a result, life can feel a lot less scary and a lot less lonely. At least, that’s been my experience.

For a long time, I was surprised and even confused by people’s comments on the blog. Words like “strong” and “inspirational” and “brave” were being used describe me. Me, strong? Just for feeling my feelings?  It was so contrary to the perception of myself I had carried around for so long.

Then one day, I understood the true definition of strength. Strength is not about being able to “see the positive” or “look on the bright side” or push past the pain or bury it deep and move on. Strength is having a non-distorted perception of how bad things really are and accepting it; locking eyes with the beast of loss and pain directly without turning away. Strength is being able to let in the heaviness of the sadness, fully and wholly, and still, keep moving.

Sometimes I wish I didn’t feel my feelings so deeply.  I wonder if my life would feel easier. But then I wonder, if I didn’t make room to feel the depths of my pain, how then could I make room to experience the height of my joy? If I didn’t grieve for what I don’t have, how could I hold the extreme gratitude for what I do have? If I can’t have compassion for myself and make room for all the feelings, then how can I make room for anyone else’s?

And finally, if I can’t let loss all the way in, how then can I possibly let love all the way in? True, pure, unbridled love?  What good is a life without love, really?

A few weeks ago, I woke up in the morning of my birthday to this card written by my husband. I can’t quite come up with the words to adequately describe the experience of what it felt like to read it. But as I let all the feelings come rushing in, one thing was for certain: I’m not planning to stop feeling my feelings anytime soon.

Friday, July 7, 2017

Muted Screams of Summer

Summer used to be one of my favorite seasons. Not just because it is kicked off by my summer solstice birthday, but because there has (historically) always been a carefreeness built into my summers- wearing sundresses and flip-flops, frolicking around at the pool or beach, traveling on summer vacations (my personal favorite). Throw in hours of sunshine and long lazy summer nights and there’s not much to not love.

Except when you live with HIBM. Because just as with pretty much everything else in life, HIBM has found a way to contaminate my love for summer.  All of the things I used to love about summer have now become triggers for me. The sources of those fun, easy, carefree feelings have been transformed into triggers for my own sadness, anxiety, fear, and anger.

This summer we have been lucky enough to spend a few weekends away. For the first one, my husband and I went to Palm Springs for our anniversary, motivated by my desire to make it out to the famed integratron. I brought my sit down electric scooter because by now, there’s really no other way to travel without it, a fact I’ve mostly come to accept. Upon hotel check-in, we were delighted to hear they had upgraded us to a private room with a private balcony, so as to avoid being right off the noisy, boisterous family pool. (When we travel without our kids, we want to be without all kids.) I felt excited and relieved. But because I am still not used to traveling with my increasingly disabled body, I didn’t ask until she was about to give us our keys if it was on the second floor. She excitedly said yes! Then I asked if there was an elevator. She said no. And that’s when I had to start investing the all too familiar emotional energy in evaluating the pros and cons of whether or not I could handle it. I really wanted that private room. I really wanted this to be a weekend truly away for us. I decided I could do it if there were railings. (Yes, my husband would have to schlep the scooter up and down the stairs, but it would be worth it.)  So I asked her. She went to inquire and came back with a no. That settled that. I told myself it wasn’t a big deal. I reminded myself how lucky I was to be in a nice hotel, blah blah blah. It was annoying, but I knew we’d get over it. And anyway, the main event was the following day.

The next day, we drove through the Mojave Desert to get to the Integratron. I was so excited. As my disease continues to limit me physically, I find myself seeking out more and more novel experiences, especially in the spiritual realm. This was one of them. I felt giddy when we arrived, staring out onto the giant geometric dome. But as we got closer, I saw that access to the dome was actually on the second floor.  And not via a staircase with a railing, but rather a steeply-angled ladder. Damn it. The anguish set in- a combination of foolishness for not having researched the accessibility of it and anger that I couldn’t seem to have just one non-HIBM adulterated experience. I was determined to make it up there. One of the owners took me over to the entrance ahead of the rest of the group to give me an opportunity to try to climb the ladder. She was gentle and patient. I finally made it up. And it was worth it. But I was acutely aware that given my current rate of progression, had I waited any longer to check it out, I wouldn’t have been able to make it up into that experience. I held the gratitude for having been able to do it when I did, alongside the pain of knowing I probably wouldn’t  be able to do it again.  The constant process of having to hold both is emotionally exhausting, on top of an already physically exhausting reality.

This past July 4th weekend, we took the boys to Legoland for three days. It’s their happy place. Rather than renting an electric scooter like I did last summer, I brought my own, which at least alleviated some of the bitterness of having to spend an extra 50 bucks a day. But there’s a different kind of bitterness (and underneath that, sadness and frankly sheer terror) that sets in when going to the same place exactly one year later when living with a progressively debilitating disease. It strips me of any shred of denial that I am, in fact, getting weaker. It is a nasty reminder that no matter how many supplements I take, no matter how healthfully I eat, no matter how much physical therapy I do, I am getting worse. I feel powerless against it. I am trapped in this disease and traveling through it in one direction. The destination, which once seemed so far away I that could barely see it, is slowly coming clearer into view.

Last year I got to enjoy the wave pool (among others activities) with my boys. I got to bob up-and-down with them and join in on their gleeful anticipation of the next wave. This year, I sat on my scooter in the scorching sun and watched them from a distance. The effort that would’ve been required of me to join in felt too much. I did my best to experience joy through theirs, though they were so far out, I could barely see them and my husband.  This year, I took my older son who’s obsessed with roller coasters up to the front of the line using my disability pass, and then watched him climb into a car by himself while I stayed back. If you’re someone who doesn’t enjoy roller coasters, maybe that sounds ideal to you. But for me, roller coasters were always one of my favorite thrills. I no longer have the neck strength to hold my head up safely on those jerky turns. I smiled at his squeals coming down, but inside I yearned for nothing more than to share the experience alongside him.

One of the highlights of our hotel was the waterslide. As you might imagine, I love water slides. On the second day, I couldn’t stand it anymore, so I decided to go for it. My boys were thrilled. (A wonderful feeling and yet a painful reminder that my inability to participate physically in my boys’ lives likely takes more of a toll on them than I care to recognize.) I slowly and arduously climbed up each step to the top. I did it.  But the thrill of coming down was tainted by the fact that my head slammed into the slide from the G-force. I decided to sit the rest out. I looked around the pool and tried to convince myself that I could be happy doing what so many other moms were doing: sitting on a lounge chair,  reading a book, sipping a drink, maybe not even knowing where my kids were. But that’s not who I am. What may seem like a luxurious choice to someone else is for me an upsetting reminder that I no longer get to do what I want to do, when I want to do it.

I am sick and tired of not having choices. I’m sick and tired of being forced to suppress the most basic, fundamental nature of who I am. I’m tired of being trapped in this body.  Have you ever had that horrible stress dream where you’re being threatened by something or someone and you try to scream, but your voice is muted? That’s how it feels,  as if my body’s scream is muted.

My spirit, my energy level, my excitability, my desire for adventure and play and spontaneity, even down to the sneakers I have to wear- none of these align easily with the body I have become forced to live inside. I can barely remember what it felt like to not have my body be in conflict with who I am. I want to believe that it must have felt so carefree, but in actuality, it probably wasn’t something I was ever even conscious of.  How liberating it must feel, to not be constantly emotionally triggered by the awareness of what you used to be able to do, what you currently aren’t able to do, and what you know you will likely never be to do.

I look at Facebook photos of people’s summer vacations- mothers taking sons on solo trips abroad, families hiking to the tops of mountains in national parks, couples indulging in exotic trips to hot springs in Iceland or exploring narrow, cobblestone alleys of small towns in Italy.  All things I always thought I would do with my own family, all things I  wish I could do, but can’t.

Don’t get me wrong, I’m not saying people with disabilities can’t travel, of course. I’m saying there are endless restrictions on where we can go and what we get to experience. And between the logistics involved (figuring out what’s accessible and what’s not), the physical effort required, and the subsequent emotional sequelae, it takes a lot of the fun out of it. At least for me.

I miss easygoing weekends away. I miss my pre-disease summers. Though I am grateful for having had 30 years of them, I wish I could have more. I’d even settle for just one day.

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.

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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