Far From the Tree

by Solomon, Andrew

  ELL is both a cosmetic and a functional intervention, though many who have chosen it avoid discussing the cosmetic piece. Skeptics contend that ELL is complicated, painful, and has many troubling side effects, and that the procedure is unwarranted given that little people can function quite well in society without it. Like those who militate against cochlear implants, ELL opponents object to the surgery’s stigmatizing implication that their condition needs to be corrected.

  It is often arduous to distinguish the political position from the medical one. People who have had ELL tend to speak well of it, and studies show that the procedure boosts self-esteem. “Looking up all the time is hard,” one LP who had had ELL explained. “Not just hard on the neck, but hard on the spirit.” The response has an aura of the self-fulfilling prophecy about it. People who have opted for the procedure presumably needed a boost in self-esteem before they began and would find it difficult to belittle a process to which they had devoted many years of life. Nonetheless, people who have experienced complications are among the procedure’s most vehement opponents.

  The tension around this issue within LPA was reified in its decision to invite Dror Paley, the leading surgeon for ELL in the United States, to the 2002 national convention, and then to uninvite him when members objected. Gillian Mueller, who underwent limb-lengthening as a child and who has become an outspoken proponent for it, has said, “The most important thing any new parent can do is accept their child and teach him to accept himself. No child should grow up believing he has a condition that his parents are going to ‘fix’ when he’s older.” However, she states that the procedure can help people to live without the disadvantages of short stature. She is thrilled that she had it. One LPA executive has said, “We need to wait to an age where you can have a real discussion with the individual who’s going to have it done, and have it really, truly be their decision. We’d recommend that they meet with a psychologist, that there be a very open and extended dialogue about it before a decision is reached.” But like the arguments for delaying cochlear implants, this one is deeply flawed. This process works only during the time of natural growth: late childhood and early adolescence. It’s later than the period of language acquisition, but much earlier than full maturity.

  Some doctors have claimed that limb-lengthening may help to prevent the spinal and other orthopedic problems associated with dwarfism, and this is a topic of urgent debate. Dan Kennedy, who has not pursued ELL for his daughter, candidly writes, “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?’” Every case of ELL is different, so the risks and rewards cannot be generalized, and because ELL is fairly new, the long-term outcome is unclear. The rate of complications—ranging from mild and transient to severe and permanent—is higher for ELL than for any other orthopedic surgery. The target population for the procedure faces many orthopedic problems even without surgery, which further muddies the swampy waters.

  Some children seem to move easily toward a celebration of their difference. For others, difference is almost insufferable. Likewise, some parents can tolerate having a child who is different, and some can’t. At nine, I’d have given anything not to be gay and would have gone through a procedure like this had there been one for my condition; now that I’m forty-eight, I’m glad that I didn’t compromise my body. The trick is knowing which prejudices of a nine-year-old are nine-year-old prejudices that will change with time, and which ones are true readings of the heart that will last into adulthood. The attitude of parents frequently shapes the mind-set of children, and surgeons must try to penetrate that film so they can clearly see the interests of the person on whom such a procedure would be performed. “My daughter hated being a dwarf,” one mother said to me. “She would point to the dwarfs we introduced into the house, lovely people, and say, ‘I’d rather be dead than be like those people. Those people are freaks. I hate them.’ She didn’t want to be a part of their world. We tried so hard to make it nice for her.” The daughter insisted on and is glad to have had the procedure. Writing about elective surgery on children, medical ethicist Arthur Frank observes in the Hastings Center Report, “The possibility of fixing renders inescapable the question of whether or not to fix.”

  Surgery originated as a process of excision; the augmentative model of surgery is a modern intervention. Though descriptions of orthopedic procedures date back to ancient Greece, the use of such procedures in a recognizable way came from the eighteenth-century French physician Nicholas Andry. Michel Foucault famously used an image from Andry’s Orthopaedia: or, the Art of Correcting and Preventing Deformities in Children (1743) to open his own Discipline and Punish, where it was intended as a model of persecution. The image shows, simply, a bent tree tied to a straight stake. Foucault would have regarded ELL as a form of torture brought about by a society that insists on conformity. Yet, while it may be a high calling to make the world more welcoming of dwarfs, it is easier in any given case to make dwarfs fit the world. The question is whether dwarfs who accommodate the world facilitate the continuance of social injustice, whether there is a moral imperative for them to refuse such procedures to keep the pressure on for the world to accommodate other dwarfs. This may be a great deal to demand of an LP who is trying to live a life of some personal satisfaction.

  While human growth hormone (HGH) does not confer greater height on people with skeletal dysplasias, its use has long been approved for people with pituitary dwarfism. In recent years, HGH has increasingly been used aesthetically for young people in the general population who are not tall and wish to be so, or whose parents seek to protect them from the social disadvantages of shortness. Like ELL, such hormone therapy must be undertaken during the growth years, usually in the early teens. Whether it is effective in people with adequately functioning pituitary systems is debatable, but some studies indicate that it can add up to four inches of additional height. The FDA recently approved Humatrope for “unexplained shortness”—that is, for men with a final height under five foot three and for women with a final height of under four foot eleven. Of course, it is impossible to know what someone’s final height will be until he reaches it, at which point it’s too late for Humatrope, so this whole process is based on statistics and guesswork. The cost of treating people with Humatrope through those critical growth years is between $12,000 and $40,000. Some wealthy parents have sought HGH for children of average height because they believe that making their children really tall is a favor to them.

  The advantages of height have been broadly established. Tall people garner more votes in elections, and recent studies show that men over six feet earn on average a salary 12 percent higher than shorter men. Tall people are icons of beauty in films, in advertisements, and on fashion runways. Proportionality has been praised as the essence of beauty since ancient times. Vitruvius, writing in the time of Christ, said that the Greek sculptors had understood this perfectly and had expressed a universal ideal: “For the human body is so designed by nature that the face, from the chin to the top of the forehead and the lowest roots of the hair, is a tenth part of the whole height,” he begins, proposing a very undwarflike body type. Our language is full of praising expressions such as stand tall and proud, and of disparaging terms such as fall short of, comes up short, paltry, and puny. The use of dwarf as a verb—a disparaging one in most instances—does not help matters. William Safire once wrote in the New York Times of how Pluto had been reclassified as “a new category called dwarf planet, and all textbooks in all languages are ordered to refer to it with that adjectival derogation.” The journalist John Richardson, who has investigated the lives of LPs, wrote, “Dwarfs will never assimilate. As long as movie stars have full lips and oval faces, as long as women dream of ‘tall, dark, and handsome,’ dwarfs are the difference that stays different.”

  • • •

  Kiki Peck was born with Kniest dysplasia, a ran
dom mutation that leads to a rare variant of dwarfism characterized by lack of type II collagen, which occurs in cartilage and in the clear gel that fills the eyeball. It results not only in diminutive stature, but also in enlarged joints, a flat nose, severe myopia, hearing loss, and distortions of all other areas of the body where cartilage plays an important role. Kiki has “Swiss cheese cartilage,” which results in arthritis-like symptoms and joint stiffness, a barrel-shaped trunk, large hands and wide feet, hips described by one of her doctors as “like melting ice,” and bones that are unnaturally thin, with ends that are unnaturally wide. Her illness was not observed at birth, but when her mother, Crissy Trapani, brought her to the doctor for her one-month visit, she had lost weight. The doctor told Crissy to stop breast-feeding and use bottles so she could keep a rigorous account of Kiki’s feeding habits. The weeks that followed were frightening; Kiki was diagnosed with “failure to thrive,” and her life hung in the balance. She was taken to the University of Michigan Hospital, within driving distance of the Pecks’ house, and even though none of her doctors had ever seen a case of Kniest—at the time, there were only two hundred known cases worldwide—they arrived at a correct diagnosis from X-ray images of her unusually shaped bones.

  The months that followed were occupied with visits to geneticists and other specialists. “I just wanted to talk to somebody who knew what it would be like for her,” Crissy said. “There was nobody.” Kiki was found to be severely myopic and was fitted for spectacles at two months. “I went to four different places to find glasses that would fit her tiny face,” Crissy recalled. “The woman was adjusting the glasses and Kiki was screaming, screaming, screaming. All of a sudden she stopped and she just stared. You could tell from the look on her face that she was like, ‘I can see!’” Because cartilage is a part of the inner structure of the ear, Kiki also had significant hearing deficits, and she was fitted for hearing aids at six months. “That was a whole other adventure,” Crissy said. “Try keeping hearing aids on a six-month-old. We lost a lot, and they’re not cheap things to lose.” By that time, however, Kiki had started to grow, and while she didn’t grow as much as an unaffected child, she was keeping pace for her condition.

  Crissy’s parents had been upset by the diagnosis. “When I told my dad, my mom said he went right out to the golfing range and just slammed a whole basketful of balls. Then he came inside and started researching, and he found a Kniest group in Minnesota.” The whole family flew out to meet them. “I remember preparing myself for the shock of meeting an adult who was affected,” Crissy said. “Then I met her. She was a great person, super-friendly and super-accommodating, with all kinds of answers. So it was really good for me and my parents.”

  Building a life was extremely challenging, and Crissy found an outlet in poetry; the form entails control and was a good one to pursue in a powerless situation. “We didn’t know if Kiki would survive,” Crissy said. “We didn’t know what kind of surgeries she might need. We didn’t know what would happen to her spine; disks are cartilage. She didn’t begin to walk until she was two, and even when she was just learning to stand up, she’d look arthritic, like she’s eighty.” Crissy said people with Kniest are also sure of themselves and strong-willed. “And quite intelligent,” she added, “perhaps because they have had to do problem-solving since day one. Even in preschool, Kiki’s teachers said that she always knows what she wants, and she’s always had a great self-image.”

  When I met Kiki, she was in fifth grade, almost eleven. She had crutches next to her in the living room where we sat to talk and had recently been fitted with a brace to keep her back straight. Crissy and I were in jeans, but Kiki was wearing a party dress and big boots for our meeting; something in her was incorrigibly festive. “When I wake up, I’m stiff,” she said. “I can’t make a fist, and when I go to school, my fingers aren’t ready to write yet.” She uses a tricycle to get around the building. She announced to me that she was planning to be a veterinarian and a rock star when she grew up. Crissy said, “I’m sure she will one day, if that’s what she really wants to do.” Kiki had asked for a pet Chihuahua because she thought they could be little together. Money being tight, she got a hamster.

  While I was at the house, Kiki and her older brother, Josh, had an argument because Kiki had kicked something and it had hit Josh. “I needed to move it,” she said. “Why couldn’t you just lean over and do it?” he asked. Kiki said, “I don’t want to, because then it would be hard to get up.” Josh was righteously indignant, but Kiki had a faraway look I have come to recognize in disabled children who don’t know to what extent they are exploiting their difference for their own benefit. “Sometimes my brother thinks I have too much attention, and I try to tell him that it’s not my fault,” she said. “Yeah, it is,” Josh said. “Sometimes, we actually say we hate each other,” Kiki said to me pointedly. She paused and crossed her arms, then said very definitively, “And the truth is that we really love each other.”

  Crissy divorced Kiki’s father, Caleb, when Kiki was in second grade. “Her father thought that she needed less medical care than I thought she needed,” Crissy said. “When she had her surgeries, Caleb didn’t come to the hospital; I think he was scared. For the last ten years, I’ve been just barely above water, gasping for air. All my vacation time was spent at University of Michigan Hospital.” Crissy described the relentless rounds: the orthopedist four to six times a year, the ophthalmologist one or two times a year, the audiologist and otolaryngologist each two times a year at least, the rheumatologist regularly. Kiki has constant physical therapy, and Crissy does stretching exercises with her daily. “So many decisions,” Crissy said. “She’s in pain and replacing her hips might help, but if we replace them too soon, that could disrupt other growth, so when do we want to do it? Because it’s so rare, there isn’t much information, and that’s the worst part, really.” Crissy sighed. “I used to run marathons, and someone told me once that if you smile the whole way, you won’t feel the pain. It worked. So that’s what I do with this, too.”

  Mothering Kiki has been nearly revelatory for Crissy. “I grew up painfully shy. I was a teenager who worried about whether I was a few pounds overweight, or if my hair and makeup were right. Then when she came into my life, it was just like, ‘How can I say I have to be a certain way, when I know she’ll never be that certain way?’ Why was I ever obsessed about that? Even when we’ve had our tantrums and I’m at the edge, I realize her strength. I was always really shy and self-conscious about the way I looked or I didn’t feel so good about myself. Here I have this child who is the epitome of self-esteem under the most extreme circumstances. It’s just a source of wonder to me.” Crissy later wrote, “I think of the word brave, how I have to say it as a mantra, one syllable, one beat. She is more brave than I.”

  A few years after Crissy and Caleb divorced, and a few months after Kiki had major leg surgery, Crissy was diagnosed with breast cancer, requiring surgery, chemotherapy, and radiation. “There was a point where Kiki and I were joking with each other about who goes to see doctors more,” Crissy said. “Having had Kiki for so long made the cancer easier. Because I’m like, ‘This is just another thing to deal with and overcome. Just keep moving.’ I didn’t hide it from the kids. Josh was more frightened by it. Kiki was just head-on, like she’s always been. Her response was ‘My mom is always taking me to the doctor and now I’m taking my mom.’ When I went through my lumpectomy and I was lying on the couch recovering, she put a wet towel on my head and cut up oranges and fed them to me.”

  When Kiki learned that her mother needed to shave her head before starting chemo, she offered to do it for her. When they were finished, Kiki announced that she would shave her own head, too. Crissy tried to stop her, but she was absolutely adamant. “My mom got so involved with my surgery,” she said. “I hope it didn’t give her cancer. Since I’ve spent so much time feeling different, I know how hard it is. So I wanted my mother to have someone else and not be different all by herself.”<
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  IV

  Down Syndrome

  Anyone involved in any way with disability has come across “Welcome to Holland,” a modern fable written by Emily Perl Kingsley in 1987. In fact, any such person has come across it repeatedly: several hundred people have forwarded it to me since I started writing this book. Google shows more than five thousand postings of it, in connection with everything from leukemia to cranial abnormalities. Dear Abby runs it every October. It is standard issue from doctors to parents of disabled newborns. It has been set to music as a folk song and as a cantata. It serves as a theme for conferences and has been published in one of the Chicken Soup for the Soul books. People have even named their disabled children after it: Holland Abigail, for example. It is as iconic to disability as “How do I love thee?” is to romance. Many told me that it gave them the hope and strength to be good parents; others told me that it was too rosy and set up false expectations; and yet others said that it didn’t adequately acknowledge the special joy of special-needs children. Here is the piece in its entirety: