Not Fade Away: A Memoir of Senses Lost and Found (2 page)

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shkenazi genetic disorders are common. Tay-Sachs, a devastating, progressive disease of the nervous system, is probably the most well-known, but there are many, and it’s estimated that one in four people of Eastern European Jewish ancestry is a carrier for one of them. These disorders come from a recessive genetic mutation, and, since genes come in pairs, if only one is mutated, you don’t get the syndrome, but you’ll carry the gene with you. There are now simple blood tests for many of them, since, if both parents are carriers, there is a 25 percent chance that the child will inherit it. If only one parent is a carrier, it is harmless. Well, “harmless” being a relative term. Maybe “dormant” is a better word.

Usher syndrome is rare enough that most people have never heard of it. It was named for the British ophthalmologist Charles Usher, who in 1914 discovered a common defect among sixty-nine deaf-and-blind people that he’d studied.

Children who are born with Usher syndrome type I are profoundly or completely deaf at birth; suffer severe imbalance due
to vertigo, as their inner ear doesn’t function properly; and usually don’t benefit from the use of hearing aids. By around ten they start to lose their sight and then generally go blind very quickly.

Type II is a little less severe. Children are usually born with some hearing loss but tend to have normal balance and generally keep at least some vision until their teens.

My Usher, type III, is the least severe, which makes me comparatively lucky, if you look at it that way. And why not look at it that way? Any vision or hearing loss I had at birth was undetectable. The onset is slow, and subtle at first, and you are not generally blind and deaf until you are an adult.

There was a time before I was living with the constant reminder that I was going blind and deaf, and for that I feel incredibly grateful. My mother felt guilty about not noticing my symptoms sooner, but since there was nothing that she could have done, it seems better to have had those blissful years of ignorance. It wasn’t even possible, until a long time later, for our family to be tested for the gene, because at that point, nobody could even prove its existence. There were years where my parents didn’t worry, didn’t fear for their daughter, and didn’t feel the heartbreak of knowing the things that I would lose, or might never have.

For me, those were years of not being treated differently or feeling isolated, of having a chance to gain some sense of myself before this became part of who I was and who I would become. I feel so lucky that my disabilities didn’t define me as a child and for what my eyes and ears have had a chance to experience. I have been able to see so much beauty, to read, to look into people’s eyes, and to hear music and laughter and the voices of everyone I love. I have created memories that will stay with me long after my eyes and ears have lost their ability to capture new ones.

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normally sighted person can see one hundred eighty degrees without turning her head. Today, I can see less than ten. The scientific explanation for retinitis pigmentosa, or RP, is that the retina, made up of photoreceptor cells, those rods and cones we learn about in science class, die. But the better understanding of what it’s really like is described by its common nickname, “donut vision.” I have a donut-shaped ring of blindness floating in my field of vision, and the donut gets bigger and the hole gets smaller every day. I have a sliver of vision left on the outside of the donut, at the outermost edge of my peripheral vision, and I can see straight ahead through the hole. But everything else, all of the donut, is gone. I’m left with about a square foot of vision directly in front of me, and every day, a little bit more of the world is taken from me. It sometimes feels like the end of one of those old Warner Bros. cartoons on TV, where Bugs Bunny sits in the center of the screen waving good-bye as the picture becomes an increasingly smaller hole, until it’s finally gone, leaving only blackness.
That’s all, folks.

For a while, when my eyesight first started to deteriorate, if I was somewhere familiar my memory would fill in what I couldn’t see. When I was younger I would move my eyes rapidly from side to side, scanning to be able to piece together a complete picture. I still do this, but as my vision loss has progressed, the donut hole becoming increasingly smaller, I can no longer overcome the blind spots. People now appear in front of me as if out of nowhere. I’m unable to see them approach me from my periphery, and then suddenly there they are, in the tiny center hole of my vision. It’s like a startling and unpleasant magic trick, one that I never get used to.

Often, my brain creates made-up images in an attempt to compensate for the vision I no longer have—for a long time, I kept a frying pan by my desk at home because when I was at the computer, my brain kept projecting a peripheral image of a man walking through my apartment, or sometimes standing right at my shoulder. With a huge gasp, I would jump out of my chair, and then, like in a horror movie, he would be gone.

When I’m sitting across from someone in anything but bright light, I can no longer see their full face. I can’t see their expressions: what’s going on between their eyes and their mouth, how their cheeks are moving or the arch of their eyebrows, the subtle shake or nod of their head. I can see pieces, but never the whole picture. If I’m introduced to someone in a dark, noisy room—like a dimly lit bar or restaurant—not only do I usually miss the name of the person I’m being introduced to, I generally have no idea where they’re standing, and I don’t know where I should be sticking my hand out to greet them, so I just give it my best shot. I usually get it wrong, and sometimes I don’t even know if I’m meeting a man or a woman. Once, in a crowded club full of pounding music, a friend introduced me to a guy she knew and
left me alone with him while she went to get us drinks. I leaned in toward him a few times to comment on the music and the crowd, despite the fact that I could barely see or hear him. I’m not sure when he wandered away, or if he said good-bye, but I kept right on talking, and then reached out to touch his arm at one point and realized that, for at least a few minutes, I had been talking to a large column next to our table. Before I’d even had a drink. Luckily, I couldn’t see enough to note the reactions of the people around me. Though really, I’m so used to not seeing or mishearing people that I’m almost beyond embarrassment. When something like that happens, I have to laugh. What’s the alternative? The doctors who diagnosed me thought I’d be blind by the time I was thirty. I’m thirty-four now, and every day that I wake up and can still see is a gift.

Every day, the cartoon hole closes in on me, and I push back against it with all my
might.

T
he first lie I can remember telling was when I was seven years old. It was late one afternoon, and I heard the faint, magical tune of the ice cream truck. Whenever we started to hear it—and sometimes, unimaginable now, I would even be the first to—my brothers and I would drop everything and perk up our ears, like dogs intently focused on a sound in the distance. We’d stay frozen until the tune became loud enough for us to realize that this was our chance, and then we’d chase each other up the long flight of stairs from the backyard into the house directly to our piggy banks, grab our precious coins, and jump down the stairs two or three at a time, flying out the front door just as the ice cream man was slowly cruising by our house.

On one such afternoon I raced in and opened my piggy bank, and found nothing but a few dark brown, tarnished pennies lying there. I thought quickly, and right after I heard Danny race back down the stairs, I ran into his room to “borrow” some of his change to buy my ice cream. I didn’t know if he’d find out, but I knew that no matter what I would deny it at all cost. Of course
he noticed, and my mother asked me later that evening if I had taken his money. Though the Good Humor strawberry shortcake ice cream bar I had eaten hours before weighed heavily in my stomach, I was sure that if I just kept denying it, it would mean that I wasn’t really the one who had done it, so that’s exactly what I did. I denied it, despite there being no way that anybody else could have taken it, despite the fact that I’m sure they all knew it was me. It didn’t matter, because I knew I couldn’t bear the disappointment in my mother’s voice if I admitted it. I already felt like a huge disappointment to my mom for not being the little girl she wanted me to be.

If you asked my mother, she would say that I was the most beautiful, precious, perfect little girl. And she would mean it. She would say that she couldn’t be more proud of me, that I am extraordinary and wonderful, that I have done anything a fully sighted and hearing person could do and more. And she would believe it with all of her heart. But that was never what I thought. I knew I could never be anything like my mother, who could do anything and everything, and do it all with grace and charm. She was so beautiful, and feminine, and competent, and there was a part of me that thought I wasn’t what she wanted in a daughter. I was sure, though, that I knew exactly what she did want: for me to be just like my best friend, Melissa Neuwelt.

Melissa was perfect. She was little and tidy, with small, slender hands, and she played the piano beautifully. She had lovely features, a tiny nose, and absolutely no freckles. She was very smart, well behaved, and polite to a fault. She was any parent’s dream child. Of course, my mother adored her. And so, of course, I bullied her when she would come over, and when she threatened to tell on me to my mom, I locked her in my bedroom, barring the door. The minute I let her out she ran directly to my mother’s
room and leapt straight into her arms. As I watched my mother comfort her, I felt terrible and guilty. But I also hated her, for being able to be so good and honest all the time.

I, on the other hand, saw myself as clumsy and lumbering, dreamy and messy, too sporty. I tried to be what I thought my mother wanted me to be, but I knew deep down that I wasn’t. For as long as I could remember I had felt like I had to be someone different from who I was. And I was sure, even at seven, that I just wasn’t good enough. I was sneaky, and I lied, and, even if other people didn’t realize it, I knew that I wasn’t a good girl.

Where do we get these ideas about ourselves? In my practice as a psychotherapist, I see people every day who are still trapped in the patterns that they learned when they were young, the things that they believed about themselves as children remaining with them every day. I knew that there were differences between my mother and me, and because I so desperately wanted to be like her, I was sure that every difference was a fault.

When I turned eight, my mom threw me a doll party. This must have been her idea, because I was not one of those girls who carried her dollies everywhere. But once she said it, all I wanted was a Cabbage Patch doll for my birthday, and they were sold out everywhere. That was at the height of the insane Cabbage Patch craze, and they were impossible to find. Of course, Melissa had one of the preemies, the most sought-after Cabbage Patch dolls of all. She brought it to my party, carrying it like a real baby and looking darling in her pretty dress sprinkled with tiny little flowers. There were doll centerpieces, doilies, and Cabbage Patch plates and napkins, all as girly as could be. This was not the party I would have chosen, but I did my best to play the part. My mom came to the table and taught us lullabies to sing to our dolls. The irony was that while the other girls had their little dolls, I had
Montgomery Moose, Daniel’s favorite stuffed animal from the Get Along Gang. My mother sang in her beautiful voice, and all the little girls loved it, except me. There is a home video of this event, which shows Melissa holding her baby in just the right way, gently stroking her perfectly swaddled little preemie as she sings sweetly to her. I am sitting at the head of the table in a big chair that Danny is sharing with me, looking off somewhere, not paying much attention to the goings-on, Montgomery Moose resting listlessly in my arms. Danny had a Western-themed party. I had more fun at his.

I wasn’t a particularly smart child, but what I lacked in intelligence, I made up for with my imagination. I had such an active world in my head. I know now that when my teachers said that I was a daydreamer or had my head in the clouds, some of that must have been because I couldn’t see or hear them as well as I should have. Still, they weren’t wrong. I liked my imaginary world much more, because I could be anyone that I wanted to be. I could take all of the things that I thought were wrong with me and replace them with beautiful ones. I would do this when falling asleep at night, because I was terribly afraid of the dark and suffered from nightmares. For years I slept in Daniel’s room, because I had seen
Friday the 13th
at a friend’s house when I was far too young and refused to sleep alone. Then I taught myself a trick where I would replay the same happy story again and again in my head until I fell asleep. When I was young I would fantasize about dancing as Clara from
The Nutcracker,
and as I got older I would imagine that a boy whom I liked was about to kiss me, and as soon as he leaned in toward me, I’d replay the scene over and over, until I was asleep.

My imagination was not exclusive to my dreams. I spent years of my childhood telling lies that I’d created so vividly in my
head that even I believed them. They were never malicious, and as a young child I mostly told them to avoid getting in trouble, but they came easily.

As I got older, my lies became more elaborate, and, like dominoes set up one after another, each one led to more lies as I tried to cover them up. They were absurd, too, and totally unnecessary, but I always felt an overwhelming need to make myself seem better than I was.

In seventh grade I remember talking on the phone to a guy I liked and was trying to impress by telling him that I was related to Cindy Crawford, thinking somehow that this might make me worthy of him. It sounds absurd now and I can laugh about it, but at the time, I was desperate for approval. I just thought I needed something to make me seem better, cooler, different.

I told another guy whom I was trying to impress that I was going to be doing a photo shoot for
Seventeen
magazine and that I needed to choose a guy to be in it with me. I wrote down a list of questions to ask him about what he’d want to wear and how he’d want to be positioned in the shoot. I sat in the kitchen on the phone, twirling the phone cord as I actually wrote down his answers to these questions, nearly believing the ridiculous things that I was saying. I remember at one point a group of these boys called me and made fun of me for the lies I’d told. And just as I had when I was a little girl, I denied, denied, denied.

Perhaps this is part of why, when we found out that there was something wrong with my eyes, it didn’t surprise me as much as it might have. Instead, it confirmed what I already knew about myself. I wasn’t like other people. I had been right: I was deeply flawed, and I was never going to be perfect.

All of the lying was useful in one sense: It prepared me for the much bigger lies that were to come. I was well practiced by the
time I was hiding my hearing aids, hiding my vision problems, and still doing everything I could to try to be just like everyone else.

Then I started to steal. My friend Jamie and I took lipstick from the drugstore one day, and when we found a cigarette on the ground while walking home that afternoon, we hid under the deck in my backyard, smoking in our bright stolen lipstick. When I was a teenager I started stealing more, from stores like J.Crew and Victoria’s Secret. It was a release, a high, and I felt exhilarated every time I got away with it. There was a part of me that felt like the world owed me something. That was how I justified it as I got older. I was just evening the scales.

Of course, the world doesn’t owe me anything. It doesn’t owe any of us anything. It was me who owed the world, and myself, something: to be better than that. Though that’s a lesson that I couldn’t yet comprehend, one that I had to learn over and over again, until it finally stuck.