Far From the Tree

by Solomon, Andrew

  Cheryl said she thinks often about whether she would have wanted things to go another way. “When he was born, one of the nurses started crying and said, ‘Oh, I feel so terrible. Why you? You’re such nice people.’ I said, ‘Why not us?’ Would I trade it? I would never trade it now.” Clinton Sr. agreed, “I have to work with new, young guys on the job, and when they’re lazy or say they can’t do certain things, I don’t tell ’em it’s my son, but I mention that I know someone that it takes half an hour to get dressed in the morning, just to get outside and breathe fresh air. ‘You guys have two hands, two arms, and a head. You’ve got every God-given tool you could have, and you’re wasting it.’” He paused. “And you know what? I used to waste it, too. I learned that lesson from Clinton myself.”

  Both Cheryl and Clinton Sr. are somewhat in awe of their son—his courage, his academic and professional achievements, his big heart. “I don’t think we did anything to make him into him,” Cheryl said. “What did I do? I loved him. That’s all. The other day these people, much higher up than us socially, much more educated, called me up and said they couldn’t handle this. They were in Texas politics and thought the stigma would be harmful to them, and they gave their baby up for adoption. That’s just what they were going to do, and it’s the opposite of what I was going to do right from the beginning. The other day Clinton came home, and he goes, ‘Ma, I saw a blind man today with a stick, in Manhattan. There were people rushing back and forth, and he was all alone. I just felt like crying, I felt so sorry for him, so I offered to bring him to where he needed to go.’ Clinton just always had that light in him, and we were lucky enough to be the first to see it there.”

  • • •

  There are many infrequent kinds of short stature for which genes have yet to be found, but for the primary forms the genes are now located, and many turn out to be closely related. Achondroplasia, for example, is in most cases based on a dominant mutation on fibroblast growth factor receptor 3 (FGFR3). A different mutation of FGFR3 causes hypochondroplasia, a milder form of dwarfism; another mutation in the same spot causes thanatophoric dysplasia, which is a lethal skeletal dysplasia. Because achondroplasia is dominant, if two achondroplastic dwarfs conceive a child, they have a 50 percent chance of having a dwarf child, a 25 percent chance of having a full-height child, and a 25 percent chance of having a double-dominant child; double-dominant children die in infancy. Numerous other skeletal dysplasias lead to death at or shortly after birth. Finding the gene for achondroplasia offered a deeper understanding of the mechanisms of the condition and allowed prenatal diagnosis of double dominants, giving parents the option to terminate pregnancies that were certain to end in tragedy. The process also allows people to select against healthy achondroplastic children.

  The gene was identified by John Wasmuth in 1994; since then, genes have been found for SED, pseudoachondroplasia, and diastrophic dwarfism. Wasmuth was concerned about potential uses of his discovery. At the press conference to announce it, he was accompanied by officers of LPA. Leslye Sneider, who was on the podium with Wasmuth that day, recalled that he “understood the implications, and he wanted the world to see us—happy, thriving, well—standing there on the stage with him at the same moment that they learned that news.” He opined that the test should be used only to identify double dominants. Because dwarfism is infrequent, it is not screened for in standard genetic testing. It is, however, possible to request a review for achondroplasia, either preimplantation for people using IVF or in amniocentesis or CVS (chorionic villus sampling). In many cases, the condition will be picked up in ultrasound later in the pregnancy. A quarter of respondents in a recent survey would choose abortion if they found out they were expecting a dwarf. Even more strikingly, more than 50 percent of medical professionals surveyed would make that choice.

  The question of testing has since been hotly debated among little people, with some couples expressing the desire to screen out average-size fetuses and ensure a dwarf child. Dr. Darshak Sanghavi at the University of Massachusetts supports the right of dwarfs to make this choice, writing, “Many parents share a touching faith that having children similar to them will strengthen family and social bonds.” As chairs of the LPA Advocacy Committee, Betty Adelson and Joe Stramondo wrote in a letter to the New York Times that physicians who refuse such requests “are actively practicing coercive eugenics.” One LP couple described going in for preimplantation genetic testing solely with the purpose of avoiding a double dominant, only to be told by multiple clinics that they supported “healthy” pregnancies and would implant only nondwarf embryos. Carol Gibson, who has achondroplasia, as does her husband, said, “You cannot tell me that I cannot have a child who’s going to look like me. It’s just unbelievably presumptuous.” Many little people, worn out by all this, choose to adopt short-statured children, who are routinely rejected by their birth families, especially in the developing world.

  Ginny Foos and her husband have two children with achondroplasia, one biological and one adopted. “My nightmare is that my biological son’s going to say to me, ‘It’s your fault,’” Ginny said. “My husband and I couldn’t say that to our parents because it was a fluke for them. But he could very well say, ‘You knew the genetics, and you went ahead and made me a dwarf.’” When Ginny and her husband decided to adopt their second dwarf child, it was because, she said, “I think dwarfism shapes the soul as well as the body. There’s an immediate bond between two LPs, be it same-sex friends, lifelong partners, or any other variant. When I met my husband, there was something that we had in common that was more than a physical attribute; it was a life experience. My husband grew up in Beirut—during the civil war!—while I grew up in Boston, so our histories are very different. Yet, simply because we’re dwarfs, we’re similar.”

  Many dwarfs live full, rich lives, and often dwarfism seems more of an inconvenience than a disability. On the other hand, the medical challenges can be daunting. Observers of trends in prenatal diagnosis have expressed concern that wealthier parents will opt for expensive testing and that poorer ones will be consigned to bring dwarfs into the world, a troubling demographic shift. Achondroplastic disability activist Tom Shakespeare addressed these issues in a BBC radio interview, saying, “I’m ambivalent about impairment. I don’t think it’s a tragedy—that’s the traditional view. But neither do I think that it’s irrelevant—which is, in a way, the radical disability view. I think it’s a predicament.” He identified problems with both seeking and avoiding such pregnancies. The advantage to knowing early that you are going to bear a dwarf is that you can adjust to the idea and either finish the grieving in advance if grief is part of what you feel, or terminate the pregnancy. The advantage to not knowing is that you don’t carry the burden of choice, which can be terrifying and overwhelming for expectant parents.

  LPA has responded to the issue of genetic testing with a statement that reads in part, “We as short-statured individuals are productive members of society who must inform the world that, though we face challenges, most of them are environmental (as with people with other disabilities), and we value the opportunity to contribute a unique perspective to the diversity of our society. For LPA members there is a common feeling of self-acceptance, pride, community and culture.” Ericka Peasley, a dwarf and a genetic counselor who has worked on the LPA position papers, emphasized the hope that genetic information will not be used to eliminate human variety. “Giving families the opportunity to make early decisions about lethal conditions, rather than having to go through an entire pregnancy for a baby that we know is essentially going to die, is great,” she said. “But we feel that people with achondroplasia or other viable skeletal dysplasias are able to have healthy, productive lives, and while we don’t question anyone’s right to terminate a pregnancy, we want to create an awareness that this may not be a good reason to do so.” For now, genetic testing is usually employed for diagnosis, allowing families to know what to expect and what to do. A child with Morquio syndrome, for exampl
e, will need to be monitored for degeneration of eyesight and hearing; such children sometimes have cervical instabilities, and fusing the upper vertebrae can prevent significant damage to the spinal cord. Some researchers are studying how to turn off the gene that is prematurely activated in achondroplasia and stops bones from growing. Their work would not eliminate the gene, but would alter its activity and could eradicate the phenotype.

  Writing in the New York Times, Virginia Heffernan described dwarfism “as a cherished inheritance—a trait, like deafness, that is simultaneously a stigma, a handicap, a source of pride and a prerequisite for membership in a complex, charismatic and highly exclusive culture.” Ericka Peasley said, “I didn’t feel when I was growing up that I didn’t want to be this way; I just couldn’t understand why people needed to see me the way they did, and I kept on being hurt by that. As I’ve grown older, I’ve had neck issues that cause me chronic pain. We have information now that life span is decreased for people with achondroplasia. You try to figure out if adding this unique LP perspective to the world outweighs the real and true disability and pain that can go along with having a condition like this. Some of us might say if you take away the surgeries and the pain, but leave the shortness behind, we’d go for that—but it’s all or nothing.”

  • • •

  Monique Duras, a Frenchwoman living in New York, went with her Russian partner, Oleg Prigov, for a fifth-month sonogram, expecting everything to be fine, and assuming it would take five minutes. “We were waiting to find out if it was a boy or a girl, and we asked what was happening, and they said, ‘You’ll see in the doctor’s report,’” Monique recalled. “When we finally saw the doctor, he mentioned that there was a disproportion between our child’s limbs and the size of his head. But it was not a big warning.” Monique’s obstetrician suggested an additional ultrasound at a specialist lab, where the doctor confirmed that the fetus had a big head, but observed that Oleg, too, had a big head and encouraged them to go enjoy their summer holiday.

  By the time they returned, Monique was in her seventh month. Her gynecologist suggested another ultrasound, which was performed by yet another doctor. That doctor referred them to a genetic counselor, who said there was a risk of skeletal dysplasia. “I found it a little bit cold, and too distant, the fact that she used the medical term,” Monique said. “I suddenly felt a heavy load of worries on top of me.” The genetic counselor said, “The bad news is that there is a problem, and the good news is that we know exactly what it is. Achondroplasia is the most common form of dwarfism, and it has fewer complications than other dwarfing conditions do. But there is a risk of hydrocephalus, cervicomedullary compression, spinal stenosis, restrictive and obstructive lung disease, otitis media, and tibial bowing.” Monique almost fainted. “I didn’t want to face this,” she explained to me. “I was close to eight months at this point. I thought, ‘I hate all these studies. I wish we didn’t know anything.’ I also thought, ‘I wish we’d known sooner.’ My ob-gyn didn’t want to give any kind of advice, which meant she didn’t give me any support. She told me to look up Little People of America. That was all she had to say about it.”

  Monique talked to doctors she knew in France. “They all said that you don’t take on problems or issues or differences you can avoid. They all thought that we should abort.” The genetic counselor in New York referred them to a psychologist with a background in genetics. “The psychologist was saying that either way, there’s always going to be a moment when you regret your decision. That had a very strong negative impact on me. I thought, ‘I don’t want to make a decision I’m going to regret.’ It’s very simple and very basic.”

  Of the possibility of aborting, Oleg said, “For my family, it was out of the question. They converted from Russian Orthodox to Catholicism and they believe deeply. My mother sent me a fax from Moscow asking us to rethink it. But I didn’t tell Monique; it was not for my mother to decide this.” Monique said, “Oleg doesn’t want to know what people are thinking. I like to know everybody’s opinion. I go all over the place, and then I choose. But that’s the way I do everything. So we finally decided to abort. In France you can abort at any stage of the pregnancy. I needed to be away from New York and closer to my family. They were against my having this child, and I wanted their support.”

  So Oleg and Monique went to France and visited a clinic in Monique’s hometown, Lyon. They secured an appointment with the senior doctor who reviews all the complex prenatal genetic cases for east-central France. “She has vast experience, and the people she sees almost always abort,” Monique said. “We sat down and started to speak to her, and this assistant comes in with all the paperwork to start the process. I thought, ‘What am I doing here?’ I was shaking. The doctor said, ‘If you don’t want to do it, don’t do it.’ I was terrified. Oleg said, ‘If you keep this baby, it’s going to be okay.’ I needed to go to the point of almost doing it, to just see what I wanted to do. And suddenly it was clear that I would have this baby.” Monique told me this story with tears running down her face, and at the end of it she began to smile. “It was suddenly very clear,” she repeated.

  Monique and Oleg headed home to New York. “Then it was a race against time to learn everything we could about achondroplasia.” They met with Lisa Hedley, who was a friend of a friend, and her daughter, Rose. “Now that we were going into it deliberately, it wasn’t frightening, even when we learned difficult things about the orthopedics and other complications,” Monique said. “The psychologist was wrong; I never regretted it. I wished I didn’t have the choice at that time. But now I’m so happy that I did—that it was my positive decision to have this baby, not just something that happened to me.”

  When I first met Monique and Oleg, Anatole was four. “We wanted to give Anatole a brother or a sister who is dwarf as well,” she said. “We can’t do that; his condition is happenstance. Somehow, we have to ensure that he doesn’t feel alone, like the strange one. We’ll organize some interaction with LPA, and if there’s a connection, we’ll continue.” Monique is an ardent patriot for French medicine, but equally so for an American social environment. She and Oleg take Anatole to Michael Ain, the dwarf orthopedist at Johns Hopkins. “I think it’s good for Anatole to see him as a role model,” Monique said. She also likes that Ain’s patients are almost exclusively little people, and that he is well versed in the surgeries they might require. She seeks opportunities for Anatole to mix with both disabled and nondisabled peers.

  “I’m into cultivating difference,” Monique said. “So that’s what I will communicate to him. ‘Okay, you’re different. How can you benefit from that?’ I’m starting to love Anatole’s proportions, the way he’s very compact. He said, ‘I want to be tall and strong, like Spider-Man.’ I said, ‘Anatole, you’re not going to be tall like Spider-Man and Mommy and Daddy. But you can be very strong and very small.’ He said, ‘I don’t want to be different!’ I thought, ‘Okay, so now it begins.’” In much of Europe, valued identities are still collective and conforming: Catholic, French, white. Difference is avoided as much as possible; it’s striking that limb-lengthening has been particularly popular in southern Europe. “I was looking at the literature from an excellent school, and they mention at the end, ‘We welcome children with disabilities,’” Monique said. “You’d never see that in a French school. New York is definitely the best place to live and now is the best time, too. I wouldn’t want to be dealing with this in my grandparents’ generation.”

  Monique’s relationship with family in France has remained layered. “Aesthetics are so much more important there,” Monique said. “My mother still thinks in terms of ‘poor Anatole,’ and I know she loves him, but my whole life is so strange to her. She respects my choices, but she cannot understand them, and so the family I’ve created has pulled me away from the one that created me.”

  I talked with Anatole about life as an LP a few years later, when he was almost seven. By that time, he had a younger brother who had just grown tall
er than him. I wondered whether he was having a hard time with that. He thought about it, then said, “No—I’m glad he’ll be able to reach things for me.” But he showed me with considerable pride that he had the upper bunk in the room they shared and explained how much further ahead he was at school. “Anatole figures out how to do things, and he’s pretty independent,” Monique said. “The kids are more nice than you would think, though there is teasing.” She laughed. “But he’s a nice person, and, you see, he brings out niceness in other people, so maybe his life won’t be too hard after all.”

  • • •

  For dwarfs, function follows form. The shape of their bodies determines their physical capabilities. Dwarfs decry two issues: the problem of how they look to other people, and the problem of how the world is not set up for people of their dimensions. Nowhere are the two more confused than in the debate around extended limb-lengthening, or ELL. Treatment commences at the growth-spurt age, usually around eight or nine. The child is sedated, and metal screws are inserted into the lower leg bones at one-and-a-half-inch intervals, so that they stick out through the flesh of the leg. Each leg is then broken in about ten places. Because there is no longer a functional bone in the lower leg, a large brace is affixed to the outside of the leg and attached to the projecting screws. In a month or so, the bone begins to heal—the fragments, in effect, reaching toward one another. When they are nearly connected, the brace is adjusted to pull them apart again and stretch out the leg, maintaining the breaks in the bone. This is repeated regularly for about two years, with the bone kept perpetually broken, perpetually healing, the ligaments and muscles and nerves all constantly stretched. When the lower legs have fully healed, the process is repeated on the lower arms, then the upper legs, then the upper arms. Limb-lengthening surgery means spending the end of childhood and most of adolescence in considerable pain, the fiber of the body shattered. It means spending those years with enormous metal braces covering your body and metal screws projecting from your arms and legs. But it does work. It can add fourteen inches to a person’s height—making the difference between being three foot eleven and being five foot one, which can be the difference between being seen as freakish and being seen as normal. The intervention runs between $80,000 and $130,000.