Not Fade Away: A Memoir of Senses Lost and Found

by Rebecca Alexander

  After that I volunteered for a shelter for battered women and children. This became the ongoing theme in my life: The more I put myself out there, helping other people, and the less time I spent thinking about myself—my disabilities, my weight, whatever else I thought was wrong with me—the better I felt.

  24

  There have been times when I have stopped and thought about how unlikely it is to be born with Usher syndrome. Just the odds. So minuscule. So, when the winds of fate (and my amazing mother) brought together something that seemed almost as unlikely, but wonderful, it felt miraculous.

  Since the moment that my mother learned of my diagnosis of RP, she has done everything in her power to connect me to the right resources and the support I have needed, and she has helped me prepare, emotionally and otherwise, for what lies ahead. She is an incredible force of nature.

  After I was first diagnosed my mom began working as a volunteer for the Foundation Fighting Blindness, eventually leaving her job to work full-time for the organization as their western regional executive director, tirelessly raising funds for research, but well aware that, because my disorder was so rare, it was unlikely that it would be a top priority for researchers. We had no way of knowing then that on the other side of the globe an effort was already under way to identify the genetic mutation causing Usher syndrome type III, nor could we have imagined that our family would end up playing a key role in this process.

  Working for the Foundation, my mom had access to some of the country’s best doctors, several of whom were doing some of the most cutting-edge eye research out there. FFB was funding research at the School of Optometry at UC Berkeley, and my mother wanted to meet with the doctor spearheading the effort, Dr. John Flannery. When she did, in the fall of 2000, it happened that he had just returned from a trip to Finland, where, it turned out, he had been invited to meet with an ophthalmologist/geneticist at Helsinki University about collaborating on research that she was doing on Usher syndrome type III. Apparently, an enclave of affected individuals had been living for decades in a small town in southern Finland, and the Finnish government was funding some of that country’s top vision and hearing researchers, who had been working to clone and characterize the Usher III gene. The goal was to find the offending mutation in the transport of the gene’s protein within the cell so that a treatment for Usher III could be developed.

  My mom was stunned to learn that targeted research on a disorder as rare as mine was already under way, and even more astonished that it was happening in her own backyard, with Dr. Flannery doing work on the disorder at UC Berkeley. Until then, we had always been told that nobody knew for sure if there actually was a separate Usher III gene. Dr. Flannery was equally amazed when my mom told him that doctors believed that I was likely affected with Usher III. He confided that, despite all of their work to date, the Finnish scientific team was finding it particularly complicated to characterize this particular gene.

  Always proactive, my mother asked whether the research in Finland might benefit from receiving DNA samples from our family, though we were Ashkenazi Jews, not of Finnish descent. Was there any way that our DNA might be able to help the researchers “crack the code”? Dr. Flannery put my mother in touch with the lead Finnish researcher, Dr. Eeva-Marja Sankila, at Helsinki University.

  Dr. Sankila was thrilled to hear from an affected American family and welcomed our participation in her research. She promptly sent blood-draw kits to the three generations of my immediate relatives—my grandparents, parents, and brothers. At the time, we were spread out across the United States, and Daniel was studying in France, but everyone immediately drew their samples and sent them back in, fingers crossed. What followed for my mother were many months of watchful waiting. For me, it still seemed so distant and remote, light-years away from anything that could ever possibly help me, that I put it out of my mind, focusing on graduating from college and trying to figure out what to do next.

  My mother got the chance, a few months later, to meet with Dr. Sankila, a brilliant woman who exuberantly told her about the work she was doing. She explained that while her team had cloned the gene and isolated the Finnish mutation, it did not match ours, though in her tracing of our family’s DNA, she had discovered that it would be through my father’s mother and my mother’s father that I had inherited the disorder. A small part of the gene had yet to be decoded, and Dr. Sankila and her team were still looking for possible additional mutations. She had just met with an Israeli researcher who had access to additional DNA samples from Ashkenazi Jews in Israel. She was eager to provide Dr. Sankila’s team with more samples in the hope that this would help the team discover the Ashkenazi mutation.

  I got the call from my mother in December of 2002. I could tell as soon as I answered the phone that she had been crying, but before I had the chance to worry, the words flew out of her mouth. “They found you! The Finnish researchers found the genetic mutation that’s causing your Usher III!”

  I matched her enthusiasm immediately, jumping out of my chair and yelling, “They did?!” Then I stood there for a moment, stunned, and finally asked, “What does that mean?” My mother understood so much more than I did about the science of what was happening to me. While I dealt with the day-to-day challenges, she was actually focusing on the bigger picture, something I had yet to really grasp.

  “It means that they now know what they are dealing with and can begin to figure out a way to stop it,” she explained, and I could hear the love and happiness in her voice. My mother had spent the last ten years working toward this, struggling to find out anything and everything she could, and she knew that any treatment or cure relied on this critical information. Now, finally, someone could take the first steps toward looking for a treatment. This was actually a real, tangible step, something that could give us at least a glimmer of hope. A first step, but a huge one.

  “Oh, Mom!” I cried, and I held the phone against my ear and wept. She did the same, and I felt so close to her, and so proud of her, and so, so lucky to have a mother who loved me this much.

  “There are going to be many more bright days ahead for you,” she told me.

  “Do you really think so?” I asked. I spent so much time trying not to get my hopes up and just focusing on what I had to tackle in the present, but at this moment, I let myself hope, just a little. And her answer was, “Yes, I’m sure of it!”

  And she was right. There isn’t a cure yet, though there are glimmers on the horizon. My days are filled with obstacles, but so many of them are also bright, because I have people in my life, like my mother, who are always there for me and help to keep me filled with hope for the future.

  25

  After college Daniel and I moved to Santa Monica and shared an apartment with our friend Jason. Dan had started law school, striding ahead of me as usual, at the top of his class, while I was answering phones at an international photography syndication agency in the heart of LA, a ridiculous job for someone with limited hearing and a terrible one for someone with body issues. People with all kinds of accents and long, complicated names I usually didn’t catch would call, and I was constantly asking them to repeat themselves, still not getting it right, so that when I had to connect them with the person they’d called for, it generally went something like this:

  Me:Cherie, there’s a woman on the line for you from Vogue España.

  Cherie: Did you ask her name?

  Me: Yep, twice.

  Cherie: (sigh) Fine, put it through.

  Me: Sorry, Cherie, I’ll try harder next time.

  As though that would help.

  I have no idea why they kept me on; though I was overqualified, even I wouldn’t have hired me.

  The people calling wanted photos of celebrities and models for their magazines, and before sending them off, our editing department would digitally manipulate the pictures, erasing any blemishes or body imperfections and making the alr
eady beautiful women flawless. Though of course I already knew that pictures in magazines had been airbrushed and Photoshopped, I had never before watched the “before and after” process. It was shocking to see that the most famous and gorgeous women on earth actually had imperfections like the rest of us—funky tan lines, bad acne, and cellulite—all of which would be turned into glowing skin and the sleekest of figures. Instead of feeling comforted by this—stars are just like us!—I was reminded every day that being anything less than perfect was unacceptable, and I was so very far from perfect. I was still struggling with my eating disorder and my issues with body image. Of course, the real imperfections, the things about me that could not be fixed, were the ones that I tried not to think about.

  It made me angry, though, working in this ridiculous perfection factory. I wanted to rebel, to say “fuck you” and eat whatever I wanted. Of course, I also wanted to be thin and perfect.

  The answer, I knew, was to get out of there. Out of the job and out of LA. So I spent much of my time at work researching graduate schools. I spent hours looking online for the types of jobs I was interested in. I had known for a long time now that I wanted to be in a helping profession. I really loved the idea of working in a developing community overseas, but, given my disabilities, I knew that probably wouldn’t be a wise decision.

  In the meantime I kept answering phones and trying to sneak out of work early so I could drive home while it was still light out. I was never supposed to drive at night, but sometimes I did it anyway, because it was the only way to get anywhere, and I had convinced myself that it was okay. I found out too late that it wasn’t remotely okay.

  I was driving home one evening, long past dark, on Wilshire Boulevard. I saw the barest flash of a figure before there was a dull thud, and my heart stopped. I was dialing 911 even as I jumped out of my car, swallowing down vomit as I saw the man sitting propped against the front of my car, right where I had hit him. People rushed over, appearing in front of me suddenly, because, of course, I had lost much of my peripheral vision, and it was especially bad at night. Which is why I hadn’t seen this man, and why I had absolutely no right to be behind the wheel.

  Someone bent down to help him and he got unsteadily to his feet. I started to sob, babbling apologies, and was quickly and quietly assured by an onlooker that the man was drunk. He and his wife had seen the whole thing, and the man had stumbled out from between two parked cars, right in front of my car. It wasn’t my fault, they assured me, but I knew better. I hadn’t seen him because I had a huge blind spot, and if I had been driving faster I could have killed him. He could have been sober, or a child, or anyone, and I still wouldn’t have been able to stop in time; my eyes gave me too little warning. Though the EMTs assured me that he was basically uninjured, and extremely drunk, the scene replayed itself again and again in my head over the next few months, and I felt like I was choking every time I thought about it, knowing how it could have turned out.

  • • • •

  There are times when I have sensed a sharp acceleration in my hearing or vision loss, usually one or the other. This time—between the driving and my clear inability to hear what I was doing at my job—I felt both, and it made me want to move faster and work harder at the things that I really wanted, quick, before it was too late, before there were more things that I couldn’t do. I knew a lot of people who were bumming around after college, feeling aimless, working shitty jobs, and feeling like they had all the time in the world to figure out what it was they wanted. That wasn’t who I wanted to be, and I knew very well that I didn’t have all the time in the world. One thing I could always hear clearly was the clock ticking.

  I had never been one to simply accept feeling miserable, and I hated feeling like I was doing nothing to better my own life or anybody else’s. All the jobs that interested me required a master’s in either social work or public health. So I started studying for the GRE and applying to graduate schools, setting my sights on Columbia, hoping that New York would give me the independence that I craved and the life that I was ready to begin. I pored over the tiny font in the study guides, squinting to see things in focus until the throbbing behind my eyes became so intense that I had to take a break.

  I knew that I also had to do something to make the next several months fun, something that wasn’t just studying and working at a dull job that I was terrible at and then waiting to hear back from schools. So I started training for the AIDS/LifeCycle ride, a weeklong, six-hundred-mile ride from San Francisco to LA, knowing that a physical challenge was something that I could rise to, something that would make me feel proud and strong, and just as able-bodied as everybody else. I hoped, too, that it would help me vanquish the monster inside me who obsessed constantly about food. I had been attending Overeaters Anonymous meetings, but, though I knew it was important to talk about my feelings, what I really wanted was to do something, to kick my ass into high gear and feel good about myself again.

  I finally started to feel normal again, knowing that I was working to move forward in my professional life, push my body hard, and do something that would help others. I was beginning to feel less stuck. I bought a bike and had six months to train and raise money. I couldn’t wait. The only thing that gave me pause was that I had never ridden a bike more than a few miles at a time before. Most of my time on two wheels had been spent riding around my neighborhood with my friends, my training wheels remaining on long after everyone else had taken theirs off. A bike was a bike, though, so I figured I’d be fine.

  I joined a group of other people who were training, and we would head up into the hills together. At first it was complete, utter torture. But I threw myself into it, the pain in my legs burning every other thought out of my mind. I would fall asleep the instant my head hit the pillow at night, and in the morning when I got out of bed I would walk widely, as though I’d just dismounted a horse, feeling every muscle below my waist, sure that I couldn’t possibly handle another day. I quickly grew to love it, though, falling again into what felt like a Stockholm syndrome with the intense, wonderful pain of pushing my body to its limits. It was just the two of us, my bike and me, and I knew, just as I had at Skylake, that I could work through the pain. Was it dangerous for a girl with little eyesight or hearing to be biking over steep, bumpy hills? Probably, but the exhilaration more than made up for it. I couldn’t be scared to do things that were a little harder—maybe more than a little—because I couldn’t see or hear as well. This was the best that my eyes were ever going to be, and someday I wouldn’t be able to bike alone at all. I was going to do it now. I think that in so many ways it was my accident that made me brave. I had fallen as far as you could fall, and I had risen back up again. It made me adaptable and determined, and that has stood me in good stead since.

  Three months into my training, I found out that I’d been accepted to Columbia, my first choice, as well as several other schools. UCLA had offered me a free ride, and several people encouraged me to take it, because it would keep me closer to home, and I would be left without the burden of student loans. I thought back on the accident, though, and the terrible sound of my car hitting that man, and knew that there was no way I could stay in LA. Polly encouraged me to take the leap and choose Columbia. She knew that I craved independence and felt trapped by my disabilities in LA. It was scary, the idea of taking on that much debt and living so far from my family, but she intuitively understood that this was what would be best for me, and that the freedom and adventure I wanted lay elsewhere, and helped give me the courage to make my choice. I would be sad to leave Daniel and my family behind but was overjoyed at the idea of stepping out on my own. There had already been so many roadblocks and stops and starts in my life, and I knew there would be many more challenges along the way, but I felt as though I was finally setting off down the path that would be best for me. I was only twenty-four years old, but I knew that I didn’t have any time to waste.

  Thrilled that what
felt like my “real life” was finally going to begin, I threw myself into training for the ride, feeling more motivated than ever. I joyfully quit my job two weeks before the ride, and, as I arrived at the start with thousands of other bikers, I felt a sense of camaraderie and excitement, and I couldn’t wait to begin my journey.

  As we started biking, I felt anxious and excited but confident I could handle it. It would end up being one of the most physically challenging days of my life. Our training group had built up to twenty-mile rides, and then forty, with the longest being fifty-five. On the first day of the ride, the longest, we doubled that, riding one hundred and ten endless, often agonizing, miles.

  The things that saved me were the rest stops that were set up for the riders every thirty miles, each one with its own special theme. One stop had a spa theme, with the “roadies” wearing bathrobes, towels twisted on top of their heads, spa slippers, and facial masks. For another stop we rode around and down a steep hill until we hit a huge sign that said CIRQUE DU SO-GAY followed by roadies dressed up as clowns and circus entertainers. There was no better relief from a long stretch of riding than comedy, eating snacks so good that I probably gained weight, and most importantly, butt butter. Each pit stop had “medic tables” where riders could get all types of first aid care—and on each table sat rows upon rows of paper towels with a dollop of thick ointment. The real name of it was chamois butter, but on the road, it went by the appropriate name of “butt butter,” and there was nothing we riders looked forward to more than lubing up with it whenever we could. Not only because it was soothing on our incredibly sore backsides, but because of the comedic factor of having to stand in broad daylight with absolutely no privacy, taking the glop of butt butter in one hand, pulling your riding shorts open at the waist, and applying the creamy substance directly between your legs, getting your hand as far back as you could go in order to lubricate your entire undercarriage. We would all glance over at each other and burst out laughing at how ridiculous we looked. No one cared, though, because it felt so damn good and soothing. We were all riding hard to help fight a plague that had taken so many precious lives, and it just felt so wonderful to stand there and laugh together.