Limb-lengthening surgery would make my daughter tall. But I’d rather she accept her dwarfism as an important part of who she is.
BY DAN KENNEDY
NO ONE WOULD think that Gillian Mueller is a dwarf. Twenty-seven years old when we sat down to talk, at a Thai restaurant near her condo in Washington, DC, 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 11 or 12 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 condition called cartilage-hair hypoplasia; most other forms of dwarfism are considered too orthopedically complicated for such drastic treatment. The day before our meeting at the restaurant, 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. Not long after we learned that our daughter, Rebecca, had achondroplasia, 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 my wife, 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 years with a tracheostomy, with its accompaniment of oxygen tanks, beeping monitors, home nurses, and wild rides to Mass General whenever she got a cold. That 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 (www.lpaonline.org), 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 13, 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. My son, 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 20-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 10 years earlier.
GILLIAN MUELLER’S recollection of how she felt about her dwarfism when she was 13 years old struck a chord. Becky, at 10, 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 10 the way they will be at, say, 16 or 25 or 40.
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 10 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 10 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 10 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 40 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."