For five months everything appeared to go well. Then Becky contracted a respiratory virus that overwhelmed her delicate system. She ended up in the intensive care unit for more than a month and was given a tracheotomy. For two years, she had to have supplemental oxygen, and the Kennedys lived with a parade of nurses. By the time Becky was two and a half, her airways were developed enough so that the tracheotomy could be closed, and she has since been a reasonably healthy child. “The dwarfism was not that big a deal, but all this other stuff was a big deal,” Dan recalled. “We’ve always wondered what effect those two years—with the tracheotomy, the night nurse—had on the subsequent development of her personality, and I don’t think we know yet.”
When Becky fell sick, Dan found LPA, and he was put in touch with Ruth Ricker. “Ruth was employed in a good job, turned out to have gone to the same college that we did, and was a smart and funny person, and I would have been happy to see Becky turn out like her,” he said. Through Ruth, the family started to attend regional LPA events. Dan and Ruth developed the LPA website in the early days of the Internet, and Dan continued to manage and edit it for many years.
Becky has had some learning issues, which Dan attributes to hearing loss, a not uncommon complication among people with achondroplasia. When I interviewed Dan, Becky was ten and a half, and her father was anticipating the difficult waters of adolescence. “Becky looks in the mirror and she likes what she sees,” Dan said. “But I don’t kid myself. I assume that her most bitter critique of dwarfism is yet to come. Every adult dwarf I’ve ever talked to, almost without exception, says by the time they hit their twenties, they’re proud of who they are and wouldn’t change anything. But their teen years were hell. She doesn’t have many friends now, and it’s only going to become more difficult.”
Dan began writing Little People: Learning to See the World Through My Daughter’s Eyes. “I look at dwarfism as a metaphor for difference,” he said. “Whether we value it; whether we fear it; whether we would stamp it out if given the opportunity.” The research he did gave Dan insights that have helped Becky. He got a handicapped placard for his car because he realized that walking long distances was bad for anyone with a compressed spine. “Lee Kitchens, former president of LPA, said to me, ‘Better a handicapped placard now than a scooter when she’s thirty,’” Dan said. In his book, Dan complains that the freedom people felt in approaching him with questions about his daughter communicated “the unspoken message that Becky is public property, and that her parents are obliged to explain her to the world.” Whether they like it or not, parents of dwarf children often feel they must display their families as emblems of diversity. “I’d like to think grappling with this has made me a better person,” Dan said, “but I still don’t think I’m very patient. Frankly, your life is in the hands of outside forces, and you just have to go with it. This definitely made me better at that.”
• • •
More than two hundred genetic conditions lead to exceptionally short stature. Approximately 70 percent of dwarfs have achondroplasia; other dwarfing conditions include pseudoachondroplasia, spondyloepiphyseal dysplasia congenita (or SED), and diastrophic dysplasia. Little People of America designates as a dwarf anyone who is four foot ten or under as the result of a medical condition. This description does not officially include people with dwarfing conditions who grow taller than four feet ten inches, nor would it apply to children with no genetic anomalies whose dwarfism results from malnutrition or parental abuse and neglect. Nonetheless, such people are in general welcomed at LPA. The average height of a female achondroplastic dwarf is four feet, and of a male, four foot three. There are more than two hundred thousand people of short stature in the United States, and Victor McKusick, a geneticist specializing in diseases of connective tissue, has estimated that there are several million worldwide. The distance such people must travel to find expert help can be considerable; medical costs can be staggering; insurance often covers only a fraction of the liability a family faces. More than two dozen physicians serve on LPA’s Medical Advisory Board, and conferences allow dwarfs to avail themselves of expert advice.
The mechanism of achondroplasia is an overactive gene, the same one that causes the bones of average people to stop growing at the end of adolescence. This process is kicked into gear prematurely by a variation of a single nucleotide. Achons (slang for people with achondroplasia) have short limbs in proportion to a fairly average trunk, and a large head with a protuberant forehead. People with SED, a more disabling condition, tend to be shorter than those with achondroplasia; they often have clubfeet, cleft palate, wide-set eyes, a small mouth, and a barrel chest that develops when their ribs grow faster than their spine. Diastrophic dysplasia is distinguished by clubfeet and cleft palate; “hitchhiker’s thumb,” which is low on the hand and has little flexibility; and a “cauliflower ear” similar to the calcified-ear deformities that many professional boxers develop. Diastrophic dwarfs often become so bent that they are unable to walk. The condition results from a recessive gene, so both parents must be carriers—and are usually unaware of it. Though the numbers vary, it would appear that achondroplasia occurs in about one in twenty thousand births, while one in ten thousand has a dwarfing condition, some of them fatal.
Since newborns always have short limbs in relation to their heads and torsos, the revelation, as with deafness, may come immediately or may be gradual. Most dwarfs are diagnosed by the age of two. Because their chests are small, their airways may be dangerously narrow, leading to rapid breathing, obstructions, and sleep disturbances. Infants with achondroplasia are also at increased risk for life-threatening brain-stem compression, in which pressure on the lower brain impedes its function. A study of mortality in achondroplasia determined that the risk of dying within the first four years of life was more than one in fifty. The chance of dying in childhood, adolescence, or young adulthood is also vastly increased. The body temperature of newborn dwarfs is somewhat higher than that of average infants, and carbon dioxide retention causes them to sweat more. Hydrocephalus and recurrent, damaging ear infections caused by variations in craniofacial shape may also complicate matters. Several other, low-incidence conditions are associated with mental retardation, including dwarfism caused by inadequate iodine, intrauterine growth restrictions, or psychosocial deprivation. Although cognitive and intellectual development generally proceeds apace, little people may still be challenged at school because of early oxygen deprivation resulting from an underdeveloped pulmonary system; because of damage to their hearing, from the repeated ear infections to which they are prone; or because of the need to focus energy on compensating for social stigma.
Early diagnosis is critical; many serious complications can be avoided through appropriate prophylactic treatment. Children with achondroplasia should have X-rays and scans to monitor their neurological and skeletal development. They may need complex dental work if their jaw is too small for their teeth. Some children have spinal columns too thin for their nerves to fit in without being pinched. This can lead to weakness, numbness, and pain. Small airways make the risks of anesthesia higher for dwarfs. If a curvature of the spine is not corrected early on, a dwarf child may develop a hunchback. An infant with a skeletal dysplasia should not be left sitting up, as his head is too heavy for his spine to support. Additionally, he should not be placed in any seat that curves the back; car seats should be padded to prevent him from resting his chin on his chest.
Because their heads are too heavy for their necks, many young children with achondroplasia cannot hold them up when they are crouching; only a fifth of them learn to crawl. Snowplowing and reverse snowplowing entail resting the head on the floor as a balancing point while using the legs for propulsion; spider crawling, log rolling, army crawling, and seat scooting involve exactly the sorts of motion that their vivid names imply. When children with achondroplasia are ready to walk, they often stand by jackknifing, keeping their head on the floor while straightening their legs, then lifting the
upper body to achieve a full upright posture; muscle tone may be low, and joints may be unusually stiff or loose. Short-statured children perform these and many other acts in a unique way or at a later developmental stage, and LPs are supposed to avoid gymnastics, high diving, acrobatics, and collision sports because of possible joint and skeletal issues. They are encouraged to do swimming, golf, and other lower-impact sports. Because LP children should properly eat only about half as much as their average counterparts, many struggle with weight, a problem that LPA attempts to address in educational materials and panel discussions.
In adulthood, LPs may suffer chronic back problems, allergies, sinus problems, arthritis, rheumatism, hearing impairments, spine deformities, sleeping difficulties, chronic neck pain, or paralysis or weakness of the upper or lower limbs; they are far more likely than their average counterparts to undergo surgeries throughout life. The defining issues for most adult dwarfs are skeletal. The dysplasias are often associated with spinal stenosis, joint deformity and degeneration, and disk problems. In adults with achondroplasia, the narrowed spine often needs to be decompressed surgically to alleviate symptoms such as shooting pains down the legs, weakness, numbness, tingling, and pins and needles. Curvature of the spine may result in mechanical and neurological complications that affect the heart and the lungs, as well as mobility. Surgeries often performed on dwarfs include lumbar surgery for spinal stenosis to prevent paralysis and pain, cervical-spine surgery that ameliorates limb weakness, surgical division or sectioning of bone for bowleggedness, the insertion of shunts for hydrocephalus, and interventions to resolve obstructive apnea.
• • •
Leslie Parks’s parents were not pleased when she began hanging out with Chris Kelly during her senior year in high school in Huntsville, Alabama. The future they had imagined for their daughter had not included a romantic entanglement with a dwarf, even one who was a local celebrity, a DJ with his own radio show. “I was your typical middle kid, nothing special about me,” Leslie said. “So I sort of fell into it with him. I was in student government, and he would DJ parties. From the beginning, my parents were like, ‘Nip it in the bud. He’s divorced, he’s got kids, he’s a dwarf, he’s a DJ, and he’s no good.’” Leslie felt she was dating a star, but her parents didn’t see it that way, and they threw her out of the house her senior year. Within a few months, Leslie and Chris were married.
When Chris was young, his parents had tried every new “treatment” on the market, including injections of growth hormone made from the pituitary glands of monkeys. Because of or in spite of the shots, Chris had made it to four feet ten inches, which is tall for a person with achondroplasia, and firmly refused to regard his dwarfism as a medical condition to be cured. “He got into being a DJ and a stand-up comic because he needed the approval of the masses to feel good about himself,” Leslie said. “What he didn’t particularly need was a one-on-one relationship.” Chris’s two children from his previous relationship were both of average height. When Leslie became pregnant, a few months after marrying Chris, she had no thought that she might be carrying a dwarf. At seven months, she went for an ultrasound. “They said, ‘His head is too big for seven months. But his femur is much too short for seven months. What’s going on here?’” Leslie knew exactly what was going on. “I was devastated. I’m glad I found out ahead of time, because I had time to get the mourning over with by the time he was born.” Leslie could not talk to her husband about her despair at the prospect of a child who resembled him.
A tomboy who went through precocious puberty, Leslie had always had a skewed self-image. “In third grade I was developing already, and people made fun of me. I was always ashamed that my body wasn’t right.” She was overweight when she met Chris, became bigger after they married, and grew enormous and somewhat depressed when she had Jake. “I remember bringing him home from the hospital, thinking, ‘This is the worst babysitting job I’ve ever had. When is his mother coming for him?’” Leslie’s parents were horrified to have a dwarf grandson, but over time their attitude softened. Leslie’s mother was a pediatric nurse, and she sent Leslie to a neurologist at Birmingham Children’s Hospital who had experience with LPs. Leslie’s pediatrician had told her that Jake’s frequent vomiting was normal and that she could straighten him out when he arched his back in his sleep. “Then this specialist said, ‘Does he sleep with his head back and his neck arched? That’s how they achieve the most free, unobstructed breathing. Don’t move his head.’ I didn’t know.”
Chris, like the local doctors, tended to minimize his son’s condition, while Leslie’s parents clearly regarded her life as a calamity. Leslie and Chris became increasingly alienated as they navigated these issues, eventually divorcing when Jake was two. As a child, Jake would sometimes weep, saying, “I don’t want to be little.” Leslie wanted to cry, too. “What would be wrong about letting him know that you hurt for him, too?” she said. “You don’t want your child to perceive that you think his situation is hopeless, but also you don’t want to deny his experience. A few times, I’ve said, ‘Have you talked to your daddy about this?’ ‘No, I’m crying ’cause I don’t want to be like me, which means I don’t want to be like him. That would hurt his feelings.’”
Jake had some learning delays; his focus has been social rather than academic. By the time he finished third grade, Leslie was worried he was falling behind. Private testing revealed that he had a learning disability, so she transferred him to a magnet school for special education. He hated it. “Jake can act,” Leslie said. “He’s done stuff on TV; he’s very outgoing. He can think it, he can speak it; but as far as putting it down on paper, he can’t do that for shit. You are entitled to free occupational therapy for fine motor skills for young dwarfs, but your pediatrician has to refer you. I didn’t know to ask.”
When Jake was a few years old, Chris remarried. Soon his new wife, Donna, was pregnant. Like Leslie, Donna had assumed she was going to have an average child and was astonished when her newborn son was diagnosed with achondroplasia. Donna called Leslie to ask for advice, and Leslie was incensed. “It was like, ‘You bitch. I’ve had to sue him for child support ’cause he’s spending everything running around with you. Now you want me to make the road easy for you?’” But when Leslie actually saw the baby, Andy, she knew she had a role to play. “I started praying, ‘This is the only brother that Jake is going to have, and I need to get past this.’ And I did.” Leslie took Donna under her wing, set her up with the doctor in Birmingham, and warned her about the orthopedic challenges that lay ahead. Leslie told me, “A year ago, Chris and Donna came to me and said, ‘We’re working on our will. Would you take Andy if something happened to the two of us? We would want you to have him.’ I just cried. ‘Oh, my God, yes. Yes, I would.’”
Leslie and Chris have very different takes on parenting. “Dad is the fear side,” Jake told me. “Mom’s like, ‘Hell, yeah, you’re gonna play T-ball; you’re gonna play baseball; you’re just like everybody else.’” Leslie said, “There’s been a lot of clinging. ‘Where you going, Mom?’ ‘I’m just going to the bathroom. In forty-five seconds I’m going to come out.’ But he was almost having panic attacks. I said, ‘Get out of the uterus! You were born! Go away!’ But he needs someone going, ‘It’s okay, you can do it.’” Leslie described how she had traveled to a family event when Jake was twelve, and how everyone reprimanded her for letting him roam the hallways unsupervised. “I said, ‘He’s in seventh grade. You’re not thinking what’s age appropriate; you’re thinking what’s size appropriate.’”
Eventually, the typical problems of adolescence set in. “I don’t look at myself as a little person until somebody brings it to my attention. Normally, people do,” Jake said. Leslie explained, “Everybody loves Jake. He’s very popular. ‘Yeah, I’ll go to the dance with you. We’ll go as friends.’ They all love him and Jake’s the first one to get out and dance. Both of his counselors over the last two years said, ‘I wish all kids had his self-esteem.’ But
I know that we’re coming into that painful stage of wanting girlfriends.”
Leslie decided to take Jake to an LPA convention when he was thirteen. “We didn’t know a soul,” she said. “He had planned, ‘I’m gonna make all these friends; I’m gonna go to the dance; I’m gonna do this, that, and the other.’ Now he was overwhelmed, and I was overwhelmed.” Later Jake said to me, “In regular life, I use my stature to start conversations with people, to make friends. At that first conference, all I had was myself.” Jake befriended only tall people that week, most of them siblings of dwarfs. “You’re too mainstream!” Leslie told him. “Why don’t you make some little friends?” But he wasn’t ready yet. The next year was different. “He became a real teenager,” Leslie said. “I sneaked into the dance and just sort of pasted myself to the wall. I saw, ‘He’s dancing! He’s slow-dancing!’” Leslie also caught her son lying about his age to a much older girl; it can be hard to guess the age of short-statured people, and Jake is relatively tall. “I said, ‘If I have to bust you myself, you’re not eighteen,’” Leslie said. “But at the same time, I was so glad he could carry it off.” Jake adores LPA, but it’s important for Leslie that he’s happy in his own world, as well. As Jake said to me, “It’s not like it’s the only thing about me.”
The permanent question of cure versus acceptance that runs throughout this book had particular resonance for Leslie Parks. When I met her, she had recently undergone gastric bypass surgery. She had already lost thirty pounds and was aspiring to lose another hundred. “Being fat was my cross to bear,” she said. “Being short is Jake’s cross to bear. I’ve had these terrible feelings of guilt that I’m abandoning him. How can I say to my child, ‘You have to learn self-acceptance and be okay with who you are’—but yet, I’m not. It’s not my agenda for him to be tall. But if the work on regulating that gene went to trials, I’d be right there. Feeling so bad about my body, I’m open to what could be done for him. But I don’t want to put my issues onto him. Unfortunately, it’s almost impossible to get both those messages across.”
Far From the Tree Page 19