The Bone Machine
No one would think that Gillian Mueller is a dwarf. Twenty-seven years old at the time that we met, at a Thai restaurant near her condo in Washington, D.C., Mueller stands just a shade under five-foot-two. She tilts forward slightly and sways back and forth as she walks, but those idiosyncrasies appear to be no more than a trivial impediment — the lingering effects of a childhood accident, perhaps, or the result of a mild congenital condition.
In fact, Mueller has achondroplasia or hypochondroplasia — she’s never received a definitive diagnosis — and was probably destined for a maximum height of about four-foot-two. Her extra eleven or twelve inches of height are the result of the surgeon’s art: a massive, painful, and experimental undertaking in which her upper and lower leg bones were broken, external cylindrical frames known as fixators were screwed into her bones, and her legs were stretched, about a millimeter a day, to allow new bone tissue to form in the gaps. For good measure, her upper arms were stretched, too.
Mueller was the first dwarf in the United States to undergo a full course of what’s called extended limb-lengthening, or ELL, which is currently the only treatment available for short-limbed dwarfism. The surgery is generally performed only on people who have achondroplasia, hypochondroplasia, and a rare type called cartilage-hair hypoplasia; most other forms of dwarfism are considered too orthopedically complicated for such drastic treatment. The day before our meeting, Mueller had helped show me around the International Center for Limb Lengthening, at Sinai Hospital of Baltimore, where Dr. Dror Paley — who operated on Mueller, and who is the best-known limb-lengthening surgeon in the country — has been based since 2001.
I had known about Mueller for a long time. After Becky’s birth, one of the first articles about dwarfism that I dug up was a People magazine story from early 1992 on Mueller and her surgery. Titled, inevitably, “Long on Courage,” the article included a picture of her with nasty-looking metal fixators surrounding and piercing her legs. People described the pain she went through as “often excruciating” — and yet showed an “after” photo of her lying on her back, smiling and kicking her long, normal-looking legs over her head, forcing Barbara and me to ask ourselves, What if? The geneticist’s admonition about the horrors of limb-lengthening was still fresh. Yet here was this happy, healthy-looking teenager who was still a dwarf but who, by all outward appearances, was not a dwarf. Maybe this wasn’t such a bad idea after all. If Becky wanted this when she was older, who were we to say no?
Two developments brought an end to our flirtation with limb-lengthening surgery. The first was Becky’s two-year medical ordeal, which served to remind us that hospitals and surgery were things to avoid, not seek out, and that a healthy child was to be treasured no matter what her height. The second was our increasing involvement in Little People of America, where we met plenty of well-adjusted dwarf kids and adults who were obviously getting along in the world just fine despite their short stature.
Yet I never really forgot about Gillian Mueller. In the mid-1990s she started speaking out on the Dwarfism List, an Internet forum that I help run, defending limb-lengthening — and Dror Paley — against their numerous critics. After I posted the LPA medical-advisory board’s decidedly negative statement about limb-lengthening on the organization’s Web site, LPA Online, warning of such possible complications as damage to blood vessels and nerves, paralysis, arthritis, and other debilitating consequences, I agreed to post an essay she had written in defense of the procedure. And, now, here she was.
Her mother, she told me quite frankly (and thus confirming the gist of the People article), had pushed her into it. “My mother explained to me the differences this could make in my life,” she said. “I was thirteen, I was happy. In no way was I feeling ‘I hate life, I can’t go around being short.’ I was perfectly happy. But my mother explained to me what I would be able to do and the difficulties that were facing me in the future — driving a car, buying clothes, reaching the top shelf in a supermarket, anything like that. And I understood the functional benefits that this would have for me, and I decided it was something I should do.”
Her first round of surgery, on her lower-leg bones (the tibias and fibulas), took place in the summer of 1988, right after school had gotten out, which gave her a chance to get back on her feet by September. “There was pain,” she said, “but the first couple of weeks were the worst.” She gained four inches in six weeks — too much — and Paley slowed her down, which reduced the pain to a manageable level. “It was never intolerable,” she said. “I was never saying, ‘I shouldn’t have done this, get them off me.’ Those thoughts never entered my mind.” She took a year off from surgery, had her upper arms done in the summer of 1990, and her femurs — that is, her thighs — in the summer of 1991. By any measure, her surgery has been a success. Even her gait, though slightly off, is smoother than that of most dwarfs.
“It makes life easier,” she told me. “I didn’t do this for looks. I didn’t care about my appearance. I mean, I care about my appearance, being neat and all that, but I didn’t do this to blend in with society or to make myself appear normal when I’m not. I don’t hide from the fact that I’m a little person. I did this to function.”
She even runs, an activity that is at the top of any list of don’ts for dwarfs. In the past she’s run as much as two to five miles a day. She admitted that Paley had advised her against it, and that her insistence on doing it anyway had already led to an operation on her right knee. Still, I often find myself thinking about the things that I love that Becky will never do: running, hiking, backpacking in the mountains. Tim and I have been heading up to the White Mountains every summer since he was nine, and I have often wished that Becky could come, too. I’m not sure it would be a good idea for Mueller to strap a twenty-pound backpack across her dwarfism-narrowed spine and head up the trail. But she certainly looked like she could.
To Barbara and me, major surgery with lots of pain, long periods of incapacity, and the possibility of dangerous complications is not the answer. Yet I couldn’t look at Gillian without thinking of that question we had asked ourselves ten years earlier.
Gillian Mueller’s recollection of how she felt about her dwarfism when she was thirteen years old struck a chord. Becky, at ten, has just begun to think about what it means to be a dwarf — to see into the future, to understand the difficulties she’ll face in everything from reaching the upper shelves at the grocery store to persuading a reluctant employer that she can do the job just as well as, if not better than, anyone else. Partly this is because Barbara and I have protected her from this knowledge. Why fill her head with foreboding before she needs to face these obstacles in her daily life? Mostly it’s because the realities of dwarfism, the negative aspects, are simply not a part of her life at ten the way they will be at, say, sixteen, or twenty-five, or forty.
Ten-year-olds aren’t big on abstract reasoning, and Becky is no exception. We have almost never asked her to tell us her feelings about dwarfism, figuring it would be unproductive and could lead her to worry that there was something wrong with her. Recently, though, I tried. And yes, she told me that she wished she were taller, but her reasons were specific, narrow, of the moment.
I asked her if she thought there was a reason why she was a dwarf and most other people weren’t.
“Because all people are different,” she replied, then added: “What were they thinking when they invented dwarfs? What a dopey idea.”
Why, I asked, was it a dopey idea?
“I already told you ten million times,” she said, a rather overwrought commentary on the fact that this was the third time I had tried to ask the same question.
Tell me again.
“Make it ten million one times. One, we don’t reach things without a stool or a chair. And two, we can’t go on rides that we like. That’s the two reasons. I told you ten million one times.”
Yet it was clear, too, that she was just beginning to develop a sense of self-consciousness. Some months earlier, when she was in the third grade, New England Cable News had done a feature story on her and her “misunderstood” condition. She’d enjoyed it, recalling that she showed the reporter how she used a ruler to flip the light switch in her room. But she had refused to let the camera crew visit her school when it was in session, instead giving a guided tour after classes had gotten out for the day.
I asked her why.
“Because everyone will say, ‘Becky, who’s that? Becky, who’s that?’” she replied. And when I asked her what would have been wrong with that, she answered, “Well, if they shoot me writing a paragraph, everyone will do this.” She leaned forward as if mugging for the camera before adding, “Yeah, everyone will want to be on TV. So I did it after school.”
A few days after the piece aired, the school principal asked if he could show a tape of it during a weekly assembly known as “family meeting.” Sure, we told him, after Becky let us know that it was fine with her. Yet, all these months later, she remembered it as a moment of intense discomfort. Why? “Because forty kids and all the teachers watched it. And it was really embarrassing. I was scared at first — you know, showing the video and all that. But it felt good,” she said.
You felt good?
“Well, a little. Everyone wasn’t laughing at me.”
But it turned out that the most intense source of embarrassment for her was a chair — a special chair that Barbara had learned about through another LPA mother. It was made of blond wood and was adjustable, with a footrest, so that her legs wouldn’t dangle and fall asleep. She’d been using it since the first grade. Recently, though, she’d stopped — something I wasn’t aware of until she told me during our talk.
“I got rid of my old chair,” she said, quite proud of herself. “I have a regular chair like all the others, and put a stool under my desk for my feet.”
You’re not using that special chair anymore?
“Yeah. Good thing I have brains.”
Why did you do that?
“I thought it would be brilliant. And it is brilliant.”
Yes it is. And so is she. It won’t be too many years, though, before she understands far better than she does now that it’s not just her chair that looks wrong to her peers — it’s she who looks wrong. Becky is brimming with self-esteem, but until now that has never been challenged in any significant way. We’ve all seen happy, energetic kids turn into sullen teenagers. They have entered the culture of adolescence, in which each person seeks to demonstrate his individuality by being just like everyone else. We have no doubt that it will be the most difficult challenge Becky has faced — more difficult, in a way, than the medical problems she had as a baby.
Becky can’t be like everybody else. She already knows that, and soon she’ll feel it in ways that she — and we — can’t imagine. Gillian Mueller’s family chose one answer to that dilemma. Our answer — one we hope Becky will eventually embrace herself — is to see her difference as a positive, as something good and distinctive, despite the pain and prejudice that she will sometimes experience.
“Because,” as she said, “all people are different.”
America today is a multicultural society. This multiculturalism is a tenuous thing, always in danger of being riven by those who wish to return to 1950s conformity on the one hand and by those who attack any deviation from militant political correctness on the other. But for the most part it works. It’s never been easier to be different, and to embrace a non-mainstream identity, than it is in the United States, right here, right now.
But easier isn’t the same as easy. And there’s a countervailing force at work, too. Just as there’s never been a better time or place to embrace one’s identity, there’s also never been a better time or place to change one’s identity. Identity has become fluid, malleable, whatever you want it to be. Women can have bigger breasts. Men can have longer penises. Michael Jackson has transformed himself from a black man into someone who looks a white woman, albeit one from another planet.
These examples may seem trivial, but that’s only because they represent the limits of what is now possible — or was, until recently. Already the unimaginable is becoming routine. For more than a century, deaf people struggled to form a community. They largely succeeded. One Sunday morning, our church “heard” a moving presentation by a deaf woman. Her family had given her a topflight education so that she could learn to speak, which had been the goal of schools for the deaf since the late nineteenth century. But as she grew increasingly aware politically, she came to reject that mentality — and refused to talk, using sign language exclusively. Indeed, she delivered her Sunday-morning sermon in American Sign Language, with her hearing fiancé — now her husband — interpreting.
Today, though, deaf culture is threatened by cochlear implants, surgery that can allow some deaf people to hear. The most famous recipient is the radio talk-show host Rush Limbaugh, who had gone deaf in a matter of months because of a rare auto-immune-system disorder. It is far more common, though, to perform cochlear-implant surgery on young children. This has led some deaf-community activists to protest that their culture is being eliminated — even to go so far as to label it “genocide.” I disagree. If we had a child who could be helped by a cochlear implant, I have no doubt that Barbara and I would choose it. And the parallels between cochlear implants and limb-lengthening are pretty obvious. I can make a defensible argument that cochlear-implant surgery is less risky and painful, and has a more positive effect on a person’s quality of life, than limb-lengthening. But that’s strictly a mechanistic argument: make limb-lengthening less risky and less painful, and my reasoning melts away.
What was once stigma is now identity. The disabled are perhaps the last group to embrace its difference as a source of empowerment, even pride, following in the footsteps of African-Americans, women (the majority, after all), and lesbians and gay men. And dwarfs are among the last groups of people with disabilities to accept the idea of disability.
Certainly there is something illogical in taking pride in one’s deafness or paralysis or dwarfism. After all, it just is, and on one level it makes no sense to take pride in something over which one has no control. On another level, though, it makes eminent good sense to take pride in one’s ability to function in a world made for people without disabilities, just as it makes sense for African-Americans to take pride in their ability to succeed in a society designed for the psychic comfort of those whose ancestors were European.
But advances in genetic science have led, and will continue to lead, to a redefinition of what it means to be disabled. Those advances will create many more options in choosing identity and will thus call into question its very meaning. Already, genetic screening is used to weed out fetuses with Down syndrome and many other kinds of genetic conditions. In the future, doctors may routinely edit and enhance embryonic genomes, resulting in a healthier, happier, less diverse, less interesting human race.
Oh, and did I say taller, too?
After all, among the most enduring forms of discrimination is that of the tall against the short. Paying deference to those who loom above us is among our most primal behaviors, caught up, possibly, in such instinctual, precivilized matters as which male gets to breed with the most desirable female, who leads the hunt, who is dominant, who is subservient. The journalist Ralph Keyes once recounted a conversation he had with the late Harvard paleontologist Stephen Jay Gould, who, at five-foot-eight, was right around average. Gould noted that if he were just two inches taller he would actually be considered to be on the “tallish” side. “As he said this,” Keyes writes, “Gould spread his thumb and forefinger slightly apart. ‘It’s that much. It’s nothing. It’s eye level that matters — whether you’re looking up or looking down. So I wonder if the angle of the sight line may not function as a cue to inferred rank.’”
Study after study has shown that taller people earn more money, that their life-insurance policies are worth more, that tall politicians are able to use their height to intimidate their shorter colleagues. (Contrary to popular belief, except in presidential politics, there is little evidence that tall candidates win more elections than short ones.) Studies have even shown that people are more likely to invade the personal space surrounding a short person than a tall person. Short men wear lifts in their shoes; short women, high heels. Influential public figures go to great lengths not to appear short. Keyes notes, for example, that Jimmy Carter, who is perhaps just slightly below average, is a master at appearing taller than he really is: he moves slowly, talks softly, and slouches a bit, all attributes of someone several inches taller than he.
What is true of the merely short appears to be true for people with dwarfism as well. A recent study by a group of scientists at the National Institutes of Health and the Johns Hopkins University Biostatistics Center found that there are considerable psychic detriments to being an achondroplastic dwarf. The team interviewed 189 people with achondroplasia and 136 close relatives of people with achondroplasia. It found that people with achondroplasia have significantly lower self-esteem than average-size family members, as well as lower income, less education, and a lower rate of marriage.
The authors note that achondroplastic dwarfs whose income was higher, who were more educated, and who attended religious services regularly had more self-esteem and a better quality of life — yet this was the flip side of the finding that the dwarfs who were surveyed were less likely to have a high income and a good education in the first place. The authors observe that achondroplastic women had better self-esteem than men, an indication of the importance that the culture places on male height. They also warned that they had no way of knowing how much of the low self-esteem that they observed was due to being four feet tall, and how much was a result of other aspects of achondroplasia, mainly chronic pain and a disproportionate appearance.
But if, as Stephen Jay Gould noted, being five-foot-eight can have the effect of making that person subservient to someone who is five-foot-ten, then that would suggest that being three-foot-ten, or four-foot-two, can make someone not just subservient, but invisible, unless she can somehow show that she’s worthy in the eyes of the average-size world. For dwarfs, then, basic human dignity is not something that is automatically conferred; rather, it must be earned.
It’s no wonder that some dwarfs will do just about anything to be taller — to negate their identities, to become someone new. To start over. It is an enduring part of the American mythology that each of us can be anyone we choose to be. For some dwarfs, that choice starts with a long, surgical nail being hammered into their legs, up against the bone, tap-tap-tap, until it breaks cleanly in two.
Erica Gunnels was sitting up in her bed at Sinai Hospital when Dr. Dror Paley stopped by to check on her. A fourteen-year-old girl with achondroplasia from Mountain Grove, Missouri, Erica had huge metal fixators around each leg, with ugly-looking metal spikes running right through the surface of her skin down to the bone beneath. She was also facing additional surgery: one of the nerves in her legs had become compressed as a side effect of the limb-lengthening process, and Paley needed to get in there to relieve the pressure. It’s a common complication of limb-lengthening, Paley explained, comparing it to surgery for carpal-tunnel syndrome.
Paley doesn’t deny that limb-lengthening is serious and complicated and that unanticipated problems can crop up. But he stresses that cases such as Erica’s are why the surgery should only be done by experienced practitioners. He said that Dr. Gavril Abramovich Ilizarov, a Russian orthopedist who virtually invented limb-lengthening in the 1950s and 1960s, and for whom the most commonly used fixator is named, once told him that as many as 10 percent of his patients suffered permanent nerve damage from the type of compression that Erica had. By performing decompression surgery as soon as symptoms appear, Paley told me, he’s been able to prevent any of his patients from incurring such damage.
I asked Erica and her mother, Gina Gunnels, why they had sought out surgery rather than simply accepting her dwarfism as a fact of life. Erica responded with a bracing dose of sarcasm. “It would be kind of nice to drive a car with pedals, and when you sit down your feet touch the ground. And to be able to reach the shelves in stores,” she told me. Like, duh.
She said she eventually hoped to add seven to ten inches to her three-foot-ten-and-a-half-inch height. Her mother added: “Everybody in town loves her. She’s been on the prayer list of every church in town.”
Dwarfs comprise only about 5 percent of the caseload at Sinai’s International Center for Limb Lengthening. Over the past fifteen years or so, Paley and his co-director and longtime surgical partner, Dr. John Herzenberg, have operated on more than five thousand patients, around two hundred of whom were dwarfs. The vast majority of their patients come in for entirely different reasons — because one leg is longer than the other, or because they have joints that need to be rebuilt, or congenital deformities that need to be corrected. Often, Paley and Herzenberg are a parent’s last hope of avoiding the amputation of one of their child’s congenitally malformed limbs.
But it’s dwarfs who have brought Paley the most attention, from high-toned magazines such as The New Republic to mass-audience television shows such as ABC’s 20/20. And it’s made him a controversial figure as well.
Forty-six years old at the time we met, tall, trim, and athletic-looking, Paley moved rapidly through a long day of seeing patients in his clinic and assisting with surgery in the operating room. It was late August, just a couple of weeks before the first day of school, and many families were there to wrap up a summer of treatment. Indeed, he was so busy during this traditional downtime for physicians that a reporter and photographer for the Baltimore Sun were on hand to document his long day. And though most of his patients were there for various types of limb deformities, several dwarfs were seeking treatment as well.
Among Paley’s dwarf patients was Kevin, a twelve-year-old from Massachusetts, who’d already had four and a half inches of lengthening and leg-straightening, and who now stood four-foot-six. His father, Jim, told me that they might stop right there, explaining, “I think by going out further than that you run into more complications.” (“Kevin” and “Jim” are their real first names, but they asked that I not use their last names, or identify what community they’re from.) When I related that conversation to Paley later, he laughed and said, “That’s what they say now. Kevin’s going to be five-foot-four, I’ll bet you anything. Because they won’t stop halfway. They had such a positive experience.”
I watched as a teenage boy with dwarfism had the fixators removed from his legs while under general anesthesia. Paley stuck a pair of surgical scissors into each hole where the huge pins had come out and cut away at the flesh underneath. The result was a bloody mess — but Paley explained that the crude-looking procedure virtually eliminated the indented scars that many of his earlier patients have. I accompanied him as he consulted with a hypochondroplastic teenage girl and her mother; she’d had leg-lengthening done elsewhere, and had suffered nerve damage as a result. I visited with a mother and her achondroplastic son as he awaited the surgery that would begin his last round of lengthening. He was four-foot-eight — six inches taller than his anticipated natural height of four-foot-two — and she was expecting him to end up at around five-foot-four. Why do it? “To make life easy,” she said. “Just like anything else in life, we take some risks to make things easy.”
The vague disrepute that surrounds limb-lengthening had obviously had its effect on the families whom I met. They knew they were violating our culture’s supposed embrace of diversity, and they knew their decision would be unpopular with many people — even those who might, in a similar position, do exactly the same thing. Not one of the families I talked with had ever been involved in Little People of America. Erica and Gina Gunnels were the only folks I met that day who allowed me to identify them fully. And Jim, Kevin’s father, started to become angry when he perceived that my line of questioning betrayed my disapproval of the surgery.
Jim was right. To me, the surgery has too many risks, is too painful, and is too incapacitating for too long a time period. At best, it takes three summers out of a child’s life — summers he will never get back. But, still, there’s no question that it’s a lot easier to be five feet tall than four feet. There’s also no question that these parents only wanted what was best for their children — and that they had sought out one of the best doctors in the country to perform the surgery. Yes, I disapprove, but I don’t condemn. Who knows? Maybe they’re right and I’m wrong.
Yet there’s also no question that limb-lengthening raises troublesome ethical questions, none more so than the fact that it is most effective when performed on kids in their early teens, or even younger. Paley said he’s learned in the past few years that he gets the best results if he does one round of limb-lengthening between the ages of six and ten, and then does subsequent rounds when the kids reach their teens. The mother I talked with who told me she wanted to “make life easy” for her son said this when I asked her about the notion of informed consent: “I just don’t think they’re old enough to make this kind of big decision. This is a very high-risk operation.”
By the time a child reaches her late teens, the surgery simply doesn’t work as well. Thus limb-lengthening is irretrievably intertwined with the perverse reality that it is best done at an age when a child is most influenced by peer pressure, is most concerned about body image, and is obsessed with looking like everyone else. A child who might willingly — eagerly — embrace limb-lengthening at, say, twelve could have a completely different opinion by the time he is eighteen, or twenty-one, or thirty. I have talked with dozens of dwarf adults over the past ten years. Without exception, every one of them has told me that adolescence was the most difficult time for them, that they wished they weren’t dwarfs, that being excluded from dating and the high-school social scene was excruciating. Yet virtually every one of them say that with age came wisdom — that, as adults, they came to realize that their dwarfism gave them a perspective and outlook they would not have had otherwise, and that they no longer had any interest in being average-size.
Then, too, a dwarf who has undergone limb-lengthening is still a dwarf. Someone with achondroplasia who marries an average-size partner still has a fifty percent chance of giving birth to a child with achondroplasia. Their spinal openings are still too small — although Paley says that, because his method of limb-lengthening also straightens the back, there is less pinching and, theoretically at least, a somewhat reduced risk of spinal-cord compression. Their hands are still tiny, their heads are still big, their faces still shine with achondroplastic features. (Gillian Mueller, who has few dwarf features, is a rare exception to this truth.) And they’ll be short — not four feet tall, of course, but in many cases six or more inches shorter than average-size people. Rather than curing dwarfism, limb-lengthening can sentence those who’ve had it to a kind of neither-fish-nor-fowl limbo.
Dr. Michael Ain, the Johns Hopkins orthopedic surgeon who’s also an achondroplastic dwarf, actually considered limb-lengthening when he was a kid. Yet he is unstinting in his criticism of the procedure and especially of the notion that parents should make such an important decision for their children. During my day with him in Baltimore, he told me he had just seen a fifteen-year-old boy who was considering the surgery. “He really had a good grasp on it, and he was old enough to make it,” Ain said. “His parents weren’t making it for him. I think that’s a terrible thing. That’s what I fight. I don’t want some parent who’s guilty or who has some kind of different agenda or hang-up make the decision for the child. That’s a horrible thing. He or she should understand all the risks and complications of it, from significant arthritis possibly, stiffness, nerve problems, paralysis, death — all these things. I’m not being melodramatic.”
Ain added: “The complication rate is incredibly high. They’ve gotten better, but it’s still amazingly high. John Herzenberg and Dror Paley are two of the best people. If my daughter needed limb-lengthening, I would without a doubt go to them, because I think they’re fantastic in doing it. But I’m not rushing out to bang on their door and make an appointment, either.”
Michael Ain’s invocation of the D-word — as in “death” — drives Dror Paley up a wall. Ain didn’t just use it with me; he used it when 20/20 interviewed him, when CBS interviewed him, and who knows on how many other occasions as well. “I got very upset with Michael when he was interviewed on television and they had him saying, ‘You could die from this,’” Paley told me. “Yeah, so big deal, Michael, you could die from this. Tell me one operation you do where you couldn’t die from it. Have any of my patients died from it? No. So why are you getting on TV and doing this sensationalism?”
Paley’s right, of course, but he’s wrong, too. It depends on your perspective. Death in the course of a twelve-hour spinal decompression might be a risk worth taking if the alternative is to spend the rest of your life in pain, in a wheelchair, incontinent, impotent, depressed. But is it worth it to be eight, ten, twelve inches taller? To undergo what is regarded by many people as cosmetic surgery? Paley’s response: “Yeah, it’s great, it’s wonderful to accept yourself as a dwarf, but you know what? It’s great to be a Michael Ain, but not everybody’s a Michael Ain. It’s a lot easier for a lot of them who aren’t going to be Michael Ains to be taller. It solves a lot of problems.”
During my day at Sinai, I had a chance to interview Paley’s partner, John Herzenberg, as well. And he made what I thought was an important point: that a dwarf gains considerable benefit from limb-lengthening simply because his upper arms are made longer. “What is the most important thing you can think of other than being able to wipe yourself?” he asked. I don’t disagree. Becky has a terrible time trying to clean up after going to the bathroom. Recently we bought her a “magic wand” — a reacher for personal hygiene that Danny Black sells — but she’s having a tough time figuring out how to use it. On a less intimate note, Becky started playing the clarinet in the fourth grade — and nearly had to give it up because she couldn’t reach the keys at the bottom of the instrument. Fortunately a sympathetic teacher located an E-flat soprano clarinet, which is smaller and easier for her to handle. But it was just another indication of the little ways in which life can be more difficult for a kid with dwarfism. And her problems will only get worse as she grows older and changes from a double-jointed ten-year-old to a prematurely stiff adult.
Dr. Michael Goldberg, an orthopedist at New England Medical Center, in Boston, who sees a number of dwarf patients (including Becky), has told me that he thinks it might actually make sense for dwarfs to undergo limb-lengthening on their arms, although he remains opposed to leg-lengthening on the grounds that it is too painful and dangerous, with too limited a payoff. Goldberg said that surgeons at his institution are adding maybe an inch to the upper arms of achondroplastic kids and fixing their elbows so they can straighten their arms fully. But when I asked Dr. Herzenberg whether anyone had ever come to him seeking just arm-lengthening, his reaction appeared to be one of genuine puzzlement, like the thought had never occurred to him. Obviously by the time people find their way to Paley’s and Herzenberg’s door, they’re not interested in any half-measures. They want to be tall. Or, to be more accurate, taller.
Paley and I finally had a chance to sit down around ten o’clock at night, at a Middle Eastern restaurant in a strip mall near his home in the Baltimore suburbs. He was candid, persuasive, eloquent. An immigrant from Israel by way of Canada, he bantered with the waiter over how hummus is pronounced in Hebrew and Arabic. And he impressed upon me that he is absolutely sure that he is doing the right thing.
Paley studied limb-lengthening in Italy and Russia in 1986, beginning his work at the University of Maryland the following year. Herzenberg became his surgical partner in 1991, and they ran the Maryland Center for Limb Lengthening at the university until 2001, when Sinai made them the proverbial offer they couldn’t refuse. They may well be the most experienced limb-lengthening surgeons in the country, and Paley said they got that way by taking all comers, not just dwarfs. He said dwarfs shouldn’t even consider going to limb-lengthening surgeons whose only area of expertise is dwarfism, since they lack the wide range of experiences necessary to solve problems as they come up.
The politics of limb-lengthening within Little People of America is fraught with emotion. But it is safe to say that Paley is unpopular in LPA circles — not just because he does limb-lengthening, but because he’s perceived as arrogant, a showman, a salesman. Some years ago the organization decided to recommend that those seeking the surgery have it done at Cedar-Sinai Medical Center, in Los Angeles, which was following an experimental protocol and which mandated psychological counseling for all of its prospective limb-lengthening patients. Paley expressed disdain, telling me that his method is better than the one used at Cedar-Sinai, and that he’d long ago stopped offering counseling for dwarfs. “I decided that doing psychological evaluations of teenagers had no value whatsoever,” he said, laughing slightly.
But he grew more serious when he talked about what he sees as LPA’s unwillingness to let him meet with members and discuss his work. Paley had actually been invited to make a presentation to the medical-advisory board at the 2002 LPA conference in Salt Lake. It never happened: the invitation was rescinded by the LPA executive committee. LPA’s president, Matt Roloff, told me that the committee disinvited Paley because his presentation was scheduled to be open to the general membership, not just the medical professionals. “The official reason why he was disinvited was that they feel he is overly zealous in the marketing of ELL,” Roloff said. “They were scared to death that Dr. Paley would be off in a corner recruiting people.” Roloff added that he would like to see LPA become more open to the whole subject of limb-lengthening — but that he and other officers would rather hear from someone they consider to be more low-key than Paley — such as, ironically, Herzenberg.
Paley, though, labeled the withdrawal of the invitation as “narrow-minded,” and disagreed with those who criticize him as a self-promoter. He doesn’t seek out the media, he said; rather, they come to him. If nothing else, he added, LPA should be willing to help members find limb-lengthening surgeons with a good track record. “There were at least ten patients in clinic today who had been screwed up by other doctors,” he said. “They just don’t know what they’re doing.”
We discussed one case — briefly celebrated by the media in the 1980s — involving a young patient who had been turned away by Paley because he was simply too short and who later ran into trouble with another surgeon. “They end up getting half-assed treatments, bad treatments. That gives what I do a bad name. I don’t have a single patient like that,” Paley said. (I later learned that the boy, now a young adult, was involved in a $1.85 million legal case against his surgeon. I wrote to him, seeking an interview, and received a call from someone identifying himself as his brother, who told me that he was not allowed to discuss the case. The law firm that represented him did not respond to my inquiry, either.)
Yet Paley’s track record is not perfect, as he himself willingly revealed. In just the past few years, he and Herzenberg have learned that dwarfs with a certain type of spinal deformity are vulnerable to spinal-cord injury when undergoing some of the procedures involved in limb-lengthening. The most extreme case involved a young woman who eventually was sent to Dr. Ain for decompression surgery and who now is partially paralyzed and walks with a cane. “To me, she’s a terrible result,” Paley said. “I actually don’t know how she rates herself. But to me, she would have been better off if she had never had the lengthening.”
So why do it? Why put dwarfs through major surgery when there’s a strong case to be made that there’s nothing wrong with them in the first place? Why should insurance companies lay out as much as $230,000 to pay for a full course of treatment?
“Our society is designed for easier accessibility around the height of about five feet tall, maybe even taller than that. If you’re five feet tall, the height of the chair, the distance to the gas pedal in a car, the coat hanger right there — I mean, that coat hanger right there is almost inaccessible to most dwarfs,” Paley replied, pointing to one behind my chair. “But who designed it to be that way? If we were all three feet tall, it wouldn’t be that high, would it? The position of that handle on the door is not designed for a dwarf. It will hit him in the forehead. So he has to reach up here to hold that handle. The door knob, the light switches — it’s a fact of life.
“Okay, fine. You can live as a short-statured person, but you are more challenged. And if you could safely make someone fit into the more normal height range, their life would be easier. And I think if somebody has the resources to do that — insurance, or personal funds, because it’s not emergency surgery — and is willing to go through the pain and suffering and inconvenience and everything else to go through it, and you’ve got a caretaker who can provide it in a reasonable fashion where the risks are acceptable, then it’s a reasonable thing to do. And that’s my philosophy on it.”
As is so often the case in such conversations, Paley was talking about dwarfism as a socially constructed disability, an inability to fit into the human-built environment. But is limb-lengthening the answer to that dilemma? And are those who undergo it really happier? Michael Goldberg told me that he opposes limb-lengthening not because it is unnecessary in all cases, but because, in his view, it is unnecessary in this culture. Several years ago, for instance, he helped conduct a study of how achondroplastic adults view their own physical and mental health. He found that their self-perception of physical health began to decline in comparison to that of the average-size majority once they reached their forties — the result, he suspects, of age-related orthopedic problems. Yet he found no difference in mental health between achondroplastic and average-size adults.
In contrast to the situation that prevails in the United States, Goldberg recalled meeting a teenager from Verona, Italy, who’d undergone limb-lengthening at the hands of Italian doctors. It had not gone terribly well; she’d suffered some sciatic-nerve damage, needed a leg brace, and had had to give up sports. Yet without the surgery, she would not have been allowed to drive a car or to attend a regular school for “normal” kids. For her, at least, Goldberg considered the operation a success, adding: “She was happy as can be.”
In other words, our culture is better at accommodating disability than some others are. Doesn’t it make more sense to keep changing the culture and making it even better?
After I turned my tape recorder off, as we were walking outside to our cars, Paley said something else. I wasn’t taking notes, but it went pretty much like this: Now that you’ve seen limb-lengthening up close, now that you know it can work and improve people’s lives, don’t you have an obligation to tell your daughter about it? Shouldn’t she at least know that such a procedure exists? Maybe some day she’ll want it for herself.
I made a small joke about his trying to lay some guilt on me, but I did think about it. It struck me as contradictory coming from someone who, just twelve hours earlier, had told me that psychological evaluations of dwarf teenagers were worthless, that of course it’s the parents who must take the lead in making the decision. Not that Paley would advocate limb-lengthening against a child’s will — after all, given the pain and commitment and months of physical therapy involved, the results would be disastrous unless the patient were thoroughly on board. But if a parent can push a child into having limb-lengthening surgery, why can’t he do the opposite as well: simply keep knowledge of the procedure to himself, to withhold that option?
Still, Paley had a point. Much as Barbara and I oppose limb-lengthening for Becky, if she somehow learned about it and insisted on having it done, we’re not sure what we’d do. If we couldn’t talk her out of it, and if her overall health made her a good candidate, I doubt that we’d refuse.
Just before writing this, I showed Becky a videotape of a twelve-and-a-half-minute feature story that CBS News had broadcast on a recent Sunday morning. It included Paley and Ain making the case for and against limb-lengthening (yes, Ain used the D-word again); the dwarf actor Danny Woodburn, who was adamantly opposed to the surgery; and several of Paley’s young patients, who were shown both walking around and lying in the operating room, getting huge steel screws inserted into their legs.
I didn’t say much. I told Becky that I had met both Dr. Paley and Dr. Ain, and that Ain had once examined her in a hotel room at an LPA meeting. Her response: That looks like pain. Getting tubes put in my ears and getting shots is bad enough. I assured her that she was fine just the way she was, that we would never push her to have such surgery, and that, in fact, we hoped she wouldn’t want it.
And we do hope she won’t want it, notwithstanding the possibility that we might talk with her about getting her arms lengthened when she’s a little older. But the truth is that I have no idea of what seed I might have planted in Becky’s mind by showing her that videotape. Within days, she was telling me that she wanted to show it to her class. (Her teacher talked her out of it.)
So far, being a dwarf hasn’t been much of an impediment for Becky. She’s too young to drive, she’s too young to date, and if she really, really needs to reach something, she can just ask Mom or Dad to grab it for her. In a few years, though, she no doubt will have developed a full-blown critique of dwarfism, and it’s not likely to be a favorable one.
We wouldn’t have chosen dwarfism for her. If there was a magic pill that would “cure” her of achondroplasia, we’d give it to her. But there isn’t a magic pill. Limb-lengthening is an imperfect solution to a problem that is more the culture’s than hers.
Besides, there is value to Becky’s dwarfism — as there is to diversity, to individuality, to identity. Breaking her bones and stretching them out would call that identity into question. She would be a tall dwarf, genetically programmed to have children who are dwarfs. She would still be destined to have the same back and joint and respiratory problems that she would have had if she had only grown to be four feet tall. But now those problems would be complicated by the uncertain long-term health of surgically lengthened bones. She would be stuck between two worlds, no longer quite accepted by the dwarf community but not really part of the average-size majority, either.
As F. Scott Fitzgerald once wrote, “The test of a first-rate intelligence is the ability to hold two opposed ideas in the mind at the same time, and still retain the ability to function.” Here are the two contradictory thoughts that I struggle with: Becky’s genome is imperfect, and thus her life will be imperfect in ways different from — but not worse than — those of her average-size peers. And Becky is perfect just the way she is.
It’s a message that she not only needs to hear, but understand and absorb as well.