An Unexpected Journey

From almost the moment we were told that our beautiful new daughter was a dwarf, I’ve been on something of a quest — a quest to find meaning and purpose in Becky’s having a life-altering genetic difference. I don’t want to suggest that I’ve been looking for meaning in her life. That’s a given. I’ve always believed that she has the same potential, the same opportunities, the same chance to succeed or fail, to be happy or sad, as anyone else. But I wanted more. I wanted to know why she was different — or, to be more accurate, why her particular difference was so much more obvious than those that affect most of us. And I wanted the answer to that question to be positive, uplifting, life-affirming.

Maybe it’s a function of fatherhood, of the ever-so-slight distancing from family life that is imposed on men by biology and social conditioning. For my wife, Barbara, who’s more focused on Becky’s immediate needs, such questions of meaning are a waste of time and energy. She is who she is. But for me, forever part participant, part observer, the search for meaning is central.

My compass is the idea of diversity, of the belief that human variation is in and of itself a positive good, and that, though dwarfism will surely cause Becky problems, there are beneficial aspects to it as well. Greater empathy. Strength of character. Even service to humanity: she could be carrying genes whose purpose is unclear at the moment but that someday might prove beneficial. But is that really an honest way of looking at Becky’s dwarfism? Or am I just kidding myself, rationalizing, trying to create a comforting fantasy out of a complicated reality?

By now you’d think I would know better. After all, Becky is my only daughter, someone I’ve looked at, admired, and worried over every day of her life. You’d think it wouldn’t require the puzzled reaction of strangers, of the outside world, of a culture that simultaneously celebrates and fears diversity, to pull me out of my reverie and force me to focus on the facts of the matter: That at the age of ten she is no taller than an average five-year-old. That her head is already much bigger than her older brother’s, with a heavy, prominent forehead. That her arms and legs are impossibly short, that her butt sticks out, and that she sways back and forth, waddling, when she walks. That she is a dwarf not a poster child for some abstract ideal of diversity.

Not that I ever lose sight of this, exactly. In some ways, the realities of Becky’s dwarfism are always with me. Every day I think about the dwarfism-related breathing complications that nearly killed her when she was a baby and that held sway over her first three years. And I know that dwarfism continues to alter her life in ways both large and small — from the slight hearing loss that makes it difficult for her to learn, to the miniature clarinet that she plays because she can’t reach the bottom keys on a standard-size instrument. But these are side effects, consequences of her dwarfism, not dwarfism itself. To strangers, she is — as a physician’s article on dwarfism that I once read so charmingly put it — a funny-looking kid. To us, though, her dwarfism has little to do with how she looks and everything to do with the ways it has changed — not diminished — her life, and ours.

Her learning disability makes it hard for her to understand what she reads, but she is brimming with empathy for others. Her tastes in television shows, movies, and computer games lean toward those of a much younger child, yet she has such a sense of responsibility that she will do her chores and her homework without having to be told. Her size often relegates her to playing with kids half her age, yet she is so outgoing with adults that she thinks nothing of picking up the phone and pitching Girl Scout cookies to friends of ours whom she’s only met a few times. These are tangible signs of who she is, of a personality and an identity shaped partly by her dwarfism, mostly because no two kids are alike. The labeling — the idea that she is a dwarf, and that’s all she is; that her individuality is subservient to how differently she happens to be put together — well, that’s what I often lose sight of. Except on those occasions when others remind me.

On Labor Day weekend in 2002, right after Becky’s tenth birthday, I took her to her favorite amusement park, a place in New Hampshire called Story Land, near Mount Washington. It was a rare father-daughter day; my son, Tim, had gone camping with friends; Barbara stayed home. As with the TV shows and movies that Becky watches, Story Land is geared mainly for children several years younger than she. But the reason she likes Story Land more than, say, Six Flags is strictly practical: she is just tall enough to be allowed on every ride.

I resolved to pay attention on this cool, sunny Sunday — to stand aside as much as I could and take in the scene. What I saw was a revelation — not that I should have been surprised. People were staring. There was a little pointing — not much — and some long, incredulous looks. Shuffling through the slow-moving lines for the more popular rides, I noticed people looking at Becky, whispering, wondering.

As I was waiting to order lunch, a woman came up to me and asked how old Becky was. Now, I’ve gotten those questions before, and normally I suggest that the person ask Becky herself. Many adult dwarfs say there’s a common assumption that because they’re short, they must be mentally disabled as well. To counter that, I like to nudge people into engaging with Becky, to experience her outgoing personality and her wit. This time, though, Becky had found a couple of kids to play with, and they were running in and out of a mock jail with rubber bars, shouting and laughing. So I told the woman that Becky was ten, and waited for the look of confusion, followed by enlightenment, that invariably follows. She then asked me the politically correct term for — well, you know. I should have replied “Becky,” but my tongue ran ahead of my brain. “‘Dwarf’ is fine,” I said. She seemed satisfied and walked away.

Later I thought, What an odd encounter. Not unpleasant, mind you. The woman had been friendly and inquisitive, and there was nothing about her demeanor to suggest that she thought Becky was to be pitied or looked down upon. Still, her boldness in coming up to a perfect stranger and asking why his daughter was — well, a funny-looking kid — communicated the unspoken message that Becky is public property, and that her parents are obliged to explain her to the world. And I realized that obligation would eventually have to be taken on by Becky herself.

I’ve heard dwarf adults say that, from childhood on, they’ve been treated as if they’re always on display, never anonymous, never able to blend into the crowd. I once heard a woman — an attractive thirty-nine-old mother of four who happens to be four-foot-two — lament that she can’t even get out of the mall without fielding questions about what it’s like to be a dwarf.

Whenever Becky and I go about our business near our home in Danvers, a suburb on Boston’s North Shore, people —  kids and adults alike — are always stopping, rolling down their car windows, waving, and saying, “Hi, Becky!” Because of her hearing problems, compounded by a certain congenital obliviousness, I sometimes have to give her a nudge before she’ll wave back. And at least half the time she can’t tell me the names of the people who’ve just greeted her. They know her; she doesn’t know them. They’re anonymous; she’s not. They can choose whether to wave or not, to say hello or not, to drive on by or not. But she’s expected to be polite and friendly at all times, to acknowledge the presence of others because they have chosen to acknowledge hers.

I have come to understand that Becky must learn to be an ambassador — a visiting dignitary from the Land of Dwarfism, always upbeat, always polite, always on. It may not be fair. It may not even be possible. But it’s there, all the time, tied up in our cultural fascination with difference, with otherness. Dwarfism, in effect, functions as a metaphor for that fascination, inspiring laughter, fear, revulsion, condescension — but never the sort of half-conscious non-reaction we normally experience when encountering ordinary people going about their ordinary lives. Dwarfs, too, are ordinary people leading, for the most part, ordinary lives. Yet they invariably provoke an extraordinary reaction.

This book tells Becky’s story. But it also tells my story, a father’s story — and that of a people, a race even, whose members are no different from anyone else except for the way they look, and yet whose difference has cast them in the role of perpetual outsiders. Above all, it is the story of my quest to find meaning in that difference.

***

Rebecca Elizabeth Kennedy was born on August 29, 1992. My education about dwarfism — and about our culture’s conflicted attitudes toward people who are visibly different — began a week and a half later, in a nondescript office in downtown Boston. I can’t say there was any real sense of drama in that moment. Barbara and I had already been told that Becky had achondroplasia, which is the most common form of dwarfism. We knew there was little likelihood of that diagnosis being wrong. Still, when the pediatrician had suggested we take Becky to see a geneticist, we readily agreed, figuring we’d learn — well, who knows?

Maybe somewhere in the backs of our minds — or, to be more honest, in the back of my mind — I held out the possibility that there really was nothing wrong with Becky. That the doctors who had examined her in the local community hospital couldn’t tell the difference between normal and abnormal. Becky was a fairly typical-looking newborn, and at that point I found it impossible to imagine — emotionally, if not intellectually — that her genetic programming would stop her from growing when she reached the four-foot mark. How, I wondered, could they be so sure?

That last sliver of psychic defiance evaporated as soon as we were called into the geneticist’s office. The doctor — a well-manicured, middle-aged woman in a business suit, not a white coat or stethoscope in sight — put us at ease with her direct, friendly manner. She explained that x-rays of the bones in Becky’s arms and legs had confirmed her achondroplasia. She examined Becky and showed us several subtle characteristics that proved she had achondroplasia: a slight separation between the third and fourth fingers of each hand — sort of a permanent Mr. Spock greeting —  and fatty deposits on the small of her back. There were less subtle signs, too, such as the soft spot on the top of her head, which wasn’t really a spot at all but rather encompassed pretty much her entire skull from the ears up.

There was more. Following this brief examination, we reconvened in the geneticist’s office, whereupon her voice took on a portentous tone.

“I want you to know,” she told us, looking grave, “that there really wasn’t anything you could have done about this.”

A slight pause ensued before I responded, “What do you mean?”

She explained that achondroplasia couldn’t have been detected by amniocentesis or any other tests given early in pregnancy to determine whether the fetus has some sort of genetic disorder. (This is no longer true, but, for now at least, it might as well be: even though the genetic mutations that cause achondroplasia and several other forms of dwarfism have been discovered in recent years, they are not yet included in routine genetic screening — as, say, Down syndrome is for women who are in their mid-thirties or older.)

For a moment, I was dumbfounded in the literal sense of that word, at a loss as to what she was driving at. Then, one of us — I’m no longer sure whether it was Barbara or me — figured it out, and asked her if she meant there was no way we could have found out in time to terminate the pregnancy. I’m pretty sure that’s how we put it, too — “terminate the pregnancy.” There was, after all, a living, breathing baby in the room, wiggling in Barbara’s arms, and we were talking about her. Somehow it would have seemed obscene to say “abortion” out loud, even though Becky was many months away from understanding that or any other word.

“Uh, yes,” the geneticist responded, looking distinctly uncomfortable. Given the choice, she added, many expectant parents — maybe most — would choose to terminate. Well, we wouldn’t, we replied with some defiance.

And with that, our appointment began to draw to a close. The doctor recovered enough of her composure to congratulate us on having such a beautiful baby — no small thing for parents who’ve just been told their child has a major genetic condition —  and gave us some literature from Little People of America, an organization about which we would learn much in the months and years to come. She also told us that, in all likelihood, Becky would enjoy normal health, normal intelligence, and a normal life span. As we were leaving, Barbara and I admired Becky anew, and asked ourselves, with a mixture of smugness and incredulity, how could anyone talk about terminating this?

But is it really so simple? Our convictions, after all, couldn’t be separated from the healthy baby who was with us right at that moment. What about months earlier — say, during the first trimester of pregnancy, when, at least according to legal and cultural norms, the stakes were lower? What if we had learned Becky would be born not with dwarfism but, rather, a fatal genetic defect, or one of those horrible degenerative conditions that seem to crop up regularly on the front page of the local paper, with photos of a sick-looking kid and his stoical parents beneath headlines such as “Benefit Walk Scheduled for Courageous Youngster”? What if it were something more difficult and challenging and heartbreaking than mere short stature — like mental retardation or blindness or deafness or some profound disfigurement that would frighten children, disturb adults, and require years of chancy surgery?

For that matter, what if we had known that within five months of being born, Becky would nearly die when a common virus invaded her respiratory system, overwhelming airways that, because of her dwarfism, were smaller than a normal baby’s and twisted in such a way that her oxygen supply was nearly cut off. That she would have to undergo a tracheostomy and spend two difficult years breathing through a tube in her throat. That our home would be transformed into a miniature medical center, filled with air tanks and oximeters and beeping alarms and nurses who watched Becky at night so that Barbara and I, exhausted from tending to her needs during the day, could get something like a normal night’s sleep. What would we have said if we’d known all this when Barbara was ten weeks pregnant?

I’m framing this as though the issue were abortion, but it isn’t. Rather, this is about a considerably more profound issue: the differences between human beings, whether we fear those differences, whether we value them, whether we would stamp them out if given the opportunity. The geneticist had brought up another matter as well. She told us there was an experimental operation, pioneered in Russia, to lengthen a dwarf’s arms and legs so much that they appeared almost normal. (I’m using the word “normal” advisedly; I would later learn that those in the dwarf community prefer to use the term “average size” to describe folks who are not dwarfs. As with race, gender, and sexual orientation, there is a whole vocabulary of politically correct language preferred by many dwarfs and their families that is violated at one’s peril.) The surgery, she explained, was extremely painful and sometimes resulted in life-threatening side effects such as infections and nerve damage. She was — to her credit — adamantly opposed to putting a child’s life at risk simply to make her appear more like everyone else. Yet she added that she had had parents in her office requesting, demanding information about the surgery, insisting that their child needed longer arms and legs in order to enjoy a normal —  that word again — life.

Now, the case against such surgery isn’t airtight. Dwarfs must move about in a human-made world designed for people between five feet and six feet tall. If you’re four feet tall, you get stared at. But it goes well beyond that. You also can’t reach ATMs (not all of them, anyway), drive a car without special adapters, adjust a showerhead, or get the cereal off the top shelf without a step stool. A dwarf’s short arms make personal hygiene difficult as well. Some dwarfs who’ve undergone limb-lengthening surgery are quite happy with the results, and they say that reports of pain and infections have been exaggerated. But limb-lengthening is, at root, a statement of deep cultural discomfort with difference. It’s sadly ironic that it is most successful when performed on adolescents, who desperately want to look like their peers. By the time a dwarf is in her twenties, limb-lengthening is much more difficult. Of course, by then, her maturity and self-image may well have developed to the point that she’d no longer want the surgery in the first place.

We live in a culture of entitlement, and one of those supposed entitlements is the right to have perfect children — that is, children who are conventionally attractive, intelligent, even-tempered, and well-behaved. We all expect our children to be like those who populate Garrison Keillor’s Lake Wobegon, where everyone is above average. With adults embracing the ethic that bigger breasts or thinner thighs or a longer penis are just a surgeon’s scalpel away, it’s not surprising that they would transfer these attitudes to their offspring as well. Some kids with Down syndrome undergo surgery to make their eyes look less “mongoloid,” and studies show that a substantial minority of people —  perhaps 10 percent or so — would choose abortion if they learned that their fetus would have a genetic predisposition to obesity. Obesity!

These attitudes are juxtaposed against a backdrop of incredible scientific change. The decoding of the human genome will eventually make it possible to diagnose in utero all sorts of conditions and predispositions and characteristics. Within a few years, or a few decades, it may be possible to screen routinely for, say, the hundred most common genetic conditions, a category that would include achondroplasia and several other types of dwarfism. Will it become just as routine for doctors to recommend termination? What if insurance companies withdraw coverage from would-be parents who refuse to abort a fetus with a genetic condition?

What happens a few decades or a few centuries from now, when we are well past the primitive abort-or-don’t-abort conundrum and instead routinely alter the genes of embryos to eliminate a predisposition for stupidity or fatness or manic depression or homosexuality or a mean streak or a tendency to watch holographic sitcoms while talking to your friends on the videophone instead of doing your homework right now, young lady? Will we all be the same? Will we all be above average?

***

There are thought to be somewhere between thirty thousand and fifty thousand people in the United States with some type of dwarfism, of which there are about two hundred or more different varieties. The definition of dwarfism itself is difficult to pin down. Little People of America defines it as a medical or genetic condition that usually results in an adult height of four-foot-ten or less, with no distinction made between men and women. Medical definitions vary. I saw one that attempted to define dwarfism in statistical terms: someone whose height is in the lowest 0.25 percent of the general population. By this measure of dwarfism, the adult height limit for men would be five feet and one-half inches, and for women, four feet eight inches.

But if you go to a Little People of America gathering, you’ll quickly see that there’s no need to pull out a tape measure: the vast majority of dwarfs in LPA are about four feet tall, with a substantial minority who are quite a bit shorter than that. In part, this may be because people who are close to the limit — taller dwarfs, if you will — see no need for LPA. Mainly, though, it’s because the most common types of dwarfism result in an adult height of somewhere between three feet and a little more than four feet tall.

By far the most common type of dwarfism is achondroplasia, responsible for perhaps two-thirds or more of all cases. Studies show that about one in every twenty-six thousand to forty thousand babies is born with achondroplasia. The vast majority of the parents — around 85 percent — are not themselves dwarfs; that is, they are “average size.”

Because achondroplasia is a genetic condition — a genetic defect, really, though some in the dwarf community don’t like such harsh terminology — it is present at the moment of conception. It makes no sense for me to wish Becky didn’t have achondroplasia because without it, she wouldn’t be Becky. An achondroplasia-free child would have required a different sperm (the mutation is believed to originate with the father) or a different egg, and thus a person with a completely different genetic endowment. Some damaged bit of genetic material was present at the creation during our lovemaking, and unlike most such occurrences — which are incompatible with life, and which result in miscarriages, many of them so early that the woman doesn’t even realize it’s happened — this one did not prevent a fetus from taking hold in the womb.

Until the genetic basis for achondroplasia was discovered, it was thought that it somehow involved a failure of the cartilage to develop into normal bone. In fact, the word achondroplasia, which is derived from Greek, means “without cartilage formation.” Now scientists understand that achondroplasia is actually caused by a gene mutation that affects the body’s ability to regulate how much bones should grow and when they should stop growing.

Achondroplasia belongs to a broader group of dwarfing conditions known as skeletal dysplasias, all of which are genetic disorders that affect bone development. There is essentially no treatment; giving growth-hormone shots does little or no good, although such shots can help with a rare condition known as growthhormonedeficient (GHD) dwarfism, which is caused by a hormonal imbalance rather than a bone disorder. (GHD dwarfs, whose proportions are the same as an average-size person’s, used to be called “midgets,” though that is now regarded as an offensive term. “Dwarf” and “little person” are generally acceptable in describing a person with any type of dwarfism.)

The genetic mutation that causes achondroplasia is dominant, a term you may remember from high-school biology classes about Gregor Mendel and peas. Each of our inherited characteristics comprises two genes, one from our mother and one from our father. When a characteristic is dominant, only one of the two genes needs to be affected. An average-size person cannot be a carrier of achondroplasia, since if you inherit just one mutated gene, you’re a dwarf. Thus, average-size parents who give birth to an achondroplastic child might as well have gotten a personal visit from Ed McMahon bearing a check with a dollar sign followed by lots of zeroes: it is the result of a random genetic mutation, and has nothing to do with their own family history. (As far as Barbara and I know, there have been no dwarfs in either of our families.)

Many other genetic conditions, including some types of dwarfism, are recessive, meaning that you have to inherit mutated genes from both parents in order to have the condition; if you inherit just one mutated gene, you are unaffected, though you are, indeed, a carrier. Conceive a child with another carrier and that child has a one-in-four chance of inheriting the genetic mutation from both parents, and, therefore, of having the condition.

When someone with achondroplasia has a child with a person who is average-size or who has a different type of dwarfism, the likelihood of passing on achondroplasia increases to 50 percent. In fact, such mixed couples are just as likely to have a healthy achondroplastic child as are couples in which both partners have achondroplasia. That is because of an immutable fact of genetics known as double dominance.

Here’s how it works. The child of two achondroplastic parents has a 25 percent chance of not inheriting the mutation from either parent, and would thus be average-size (an unaffected gene from each parent); a 50 percent chance of being a dwarf (an affected gene from one parent, and an unaffected gene from the other); and a 25 percent chance of inheriting the mutation from both parents, the aforementioned double-dominant condition, which inevitably leads to death shortly after birth.

Such heartbreaks could not be prevented until 1994, when a research team led by the late John Wasmuth, of the University of California at Irvine, identified the genetic mutation that causes achondroplasia. Concerned that his discovery might be misused, he invited officers of Little People of America to the news conference at which he announced his findings, and expressed the hope that prenatal testing for achondroplasia would only be used by dwarf couples seeking to learn whether their child would inherit the fatal double-dominant condition. Still, Wasmuth clearly knew his discovery would eventually make it more difficult for achondroplastic children to come into the world. As that geneticist in Boston had told us in 1992, two years before Wasmuth’s announcement, There really wasn’t anything you could have done. But that was then. When Barbara was pregnant with Becky, we opted not to have her undergo amniocentesis. But if accurate pre-natal screening that was not potentially harmful to the fetus had been offered, and if such screening included achondroplasia —  well, as I said earlier, who knows? I’m reasonably sure we wouldn’t have been congratulated and given literature about Little People of America; instead, I imagine, we would have been pressured, subtly or otherwise, to choose abortion. All under the additional pressure of knowing that with each passing week our decision would become that much more difficult.

***

In those first months after Becky was born, I’d often find myself walking around with my right hand extended in front of me, level, palm down, squinting, measuring, adjusting. I was commuting by train and subway that fall, so I saw hundreds of people every morning. I’d look for the short ones, the women in particular, and try to figure out their height, where the tops of their heads would be if they were standing right next to me, how that compared with the unexpected new reality of our lives. I’d squint again, move my hand up, then down. Here? Maybe a little lower? Look at that woman over there. She couldn’t be much more than four feet. She doesn’t look too bad. We could live with that.

Later, when my focus had shifted from the train station to the hospital, from How tall will she be? to Will she come out of this okay?, I understood that I’d had it all wrong. I’d been holding my hand out in front of my chest; I should have moved it down to my waist. The short women I’d been looking at were just that — short — and nothing more. They weren’t burdened by anatomical anomalies that could cause life-threatening medical problems, complications that could develop over a period of years or, as we were to learn, at a moment’s notice. They didn’t get stared at (except by me), pointed to, called “Midget!” behind their backs — or right to their faces. They didn’t need to ask for help in pressing an elevator button. They didn’t drag themselves up the curb as though they were working out on a singularly diabolical exercise machine.

In other words, they weren’t dwarfs. Becky, I realized, was the first dwarf I’d ever seen, not counting television or maybe once or twice in the flesh, in a crowd, off in the distance, somewhere, maybe. As an adult, Becky would be four feet tall, give or take a couple of inches — and yes, her head would be larger than most people’s, and yes, her arms and legs would be tiny, and yes, she’d waddle when she walked.

We could live with that. I mean, what choice did we have?

I’ve long since put my hand back in my pocket. Height is not an issue in our home. When I was growing up, my father would measure me every so often, his pencil marks clearly visible on the doorjamb next to the bathroom for years after I’d reached my full six-foot height. Not so in our house, not even for Tim, who’s well on his way to being a six-footer himself. From the time she was old enough to understand, we’ve been telling Becky that size doesn’t matter, that she would grow up just like everyone else, even though she wasn’t going to grow up. She struggled with this, told us she didn’t want to be a “dorf” because she didn’t want to be a kid forever. She kept this up even though she’d seen dwarf adults, even though one friend, in particular, had been to our house on several occasions with her own adopted son, who’s also a dwarf, and had gone out of her way to show our daughter the pedal extensions she uses so that she can reach the gas and the brake in her car — the ability to drive, of course, being sure proof of adulthood.

Having worked so hard to convince Becky that height didn’t matter, it would have been hypocritical or even harmful for us to cluck approvingly over each quarter-inch she’d managed to gain, to mark it on the wall as if it were some sort of accomplishment. The motto of Little People of America is “Think Big.” It’s a harmless, benign message from the organization’s early days in the late 1950s and early 1960s, an artifact of a less contentious, less politically charged era. But I suspect that if LPA members today were to take a second look at their motto, they’d come up with something different.

Len Sawisch, a psychologist and disability-rights activist from Michigan who’s also a dwarf, says that telling dwarfs to “think big” makes about as much sense as telling African-Americans to “think white.” Think big? Hey, short is beautiful, baby.

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