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Should Parents of Children With Severe Disabilities Be Allowed to Stop Their Growth?

the Preslar family
Sicky gazed up toward the pine trees as his mother, Cindy Preslar, pushed him along the village road in an orange jogging stroller. She was marking the route for the Summer 2014 Run Through the Clouds 10K, a fund-raiser for the public schools in Cloudcroft, N.M. “You’ll run with Dad and Max tomorrow,” she said. “Right, Ricky?” She ruffled his fine blond hair. By “run” she meant “ride” — Ricky was 7, but his legs were unable to bear his full weight. As a result of a complication during pregnancy, Cindy says, he was born with a form of cerebral palsy known as spastic quadriplegia with static encephalopathy, which meant permanent brain damage and severely limited eyesight because of cortical vision impairment.

Ricky’s problems were not recognized immediately. He was a fussy eater but an otherwise genial baby; the Preslars’ friends commented on the twinkle in his eyes. Then, at about 3 months, he began to jolt awake at night, the back of his pajamas soaked with sweat. One afternoon, when Cindy laid him on his changing table, he arched and crossed his arms, and his eyes rolled back in his head as if he were in the throes of a seizure. A CT scan taken soon after that revealed a scarred, atypically small, or microcephalic, brain. The Preslars don’t know how much Ricky understands, but based on medical assessments, he is thought to have the developmental age of a 6-month-old infant.

When I visited Cloudcroft two summers ago, Ricky was small for his age — just 42 inches tall and 37 pounds, about the size of an average 4-year-old boy. In part this was because of genetics and his various health problems, but it was also partly a result of his having been through a controversial medical intervention known as growth-attenuation therapy. From the time he was 4 until just shy of his 7th birthday, he received doses of estrogen high enough to stimulate the premature closing of the epiphyseal or “growth” plates, the thin wedges of cartilage found at the end of the long bones in children and adolescents. (Long bones include the femurs, tibias and fibulas of the legs and the humeri, radii and ulnas of the arms.) The younger the child when treatment begins — usually between 3 and 6 years of age — the greater the reduction in height.

Physicians began prescribing estrogen to treat children with acromegaly, or excessive-growth disorder, in the 1940s. Later, in the 1950s through the 1970s, healthy preteen and teenage girls whose tall stature was merely deemed unattractive were given estrogen to reduce their predicted height by several inches. But as greater height in girls became increasingly acceptable, even desirable, growth attenuation fell into disfavor. By the turn of the century, the practice was all but obsolete.

Then, in 2006, Douglas Diekema, the director of education at the Treuman Katz Center for Pediatric Bioethics at Seattle Children’s Hospital, and Daniel Gunther, a pediatric endocrinologist, announced in the Archives of Pediatrics and Adolescent Medicine that they had resurrected the treatment once known as estrogen therapy for a different population: what Diekema and Gunther described as “nonambulatory children with severe, combined neurologic and cognitive impairment.” In other words, children like Ricky Preslar, expected to rely on caregivers for every basic need for the rest of their lives. When such dependents enter adolescence and adulthood, the doctors posited, the simple tasks of caring for them — dressing, toileting, bathing, holding and carrying — can become prohibitively difficult for parents. Arresting a child’s growth could benefit both child and parent. A smaller person who required no hoisting apparatuses, Diekema and Gunther reasoned, would in all likelihood benefit from more attention and greater inclusion in family activities. And he or she would be more likely than someone fully grown and similarly impaired to be cared for in the home, rather than an institution. “We propose,” the authors concluded, “that in situations in which parents request such an intervention, it is both medically feasible and ethically defensible.”

Diekema and Gunther’s patient was a 6-year-old girl who could not walk, sit or hold her head up without assistance. She was fed exclusively through a gastrostomy tube. Although she couldn’t speak, she often smiled and made noises and expressed pleasure in the company of her siblings. Her parents — worried that their daughter’s continued growth would restrict her ability to join family trips, swing in the backyard, take baths or cuddle in their arms — formed a plan with Gunther to limit her adult stature. After Diekema and a hospital ethics committee gave their approval, Gunther administered the estrogen estradiol, which effectively reduced her future predicted height by approximately 13 inches.

Additionally, the child — unidentified in the original paper but soon known as Ashley, her first name, or “Ashley X” — underwent a hysterectomy to preclude the discomfort of painful menses as well as the uterine bleeding that can accompany estrogen in high doses, and the removal of her breast nodules to forestall the growth of breasts. For the treatment they called “growth-attenuation therapy” to be most effective — resulting in a shorter and lighter child — a careful monitoring of calories was also required.

The “Ashley Treatment,” as the combined procedures were soon referred to, sparked controversy. Nearly 60 percent of the respondents to one MSNBC poll seemed to believe that the extreme measures were justifiable, if shocking. Some protested bitterly, however. The advocacy group Feminist Response in Disability Activism picketed the American Medical Association and printed Gunther’s and Diekema’s office addresses and phone numbers on their website, calling them “our targets.” According to the disability activists’ criticism, growth-attenuation therapy and its associated procedures violated Ashley X’s rights to privacy and freedom from unnecessary bodily manipulation, which are the sorts of things the Americans With Disabilities Act, the Rehabilitation Act and even the 14th Amendment were supposed to protect people from. Her care did not constitute a “treatment” or “therapy” at all, they said, because it did not treat an illness. A statement issued by the American Association on Intellectual and Developmental Disabilities claimed that “this practice, if judged acceptable, will open a doorway leading to great tragedy.”

In response to the outcry, the legal-advocacy group Disability Rights Washington investigated Seattle Children’s Hospital and concluded that it had broken state law by sterilizing a disabled minor without a court order. No charges were brought against Seattle Children’s, but the hospital acknowledged having erred and said in a statement: “We are instituting safeguards so that something like this never happens again.” Since then it has not performed growth-attenuation therapy on a child with a developmental disability.

Still, the treatments were now a known option for the families of children with profound disabilities. They congregated on Pillowangel.org, a website and private message board maintained by Ashley’s parents, who have never made their names public, identifying themselves only as “Ashley’s Mom” and “Ashley’s Dad,” or AM and AD. (Their site’s name comes from the endearment given to Ashley and other children like her who spend most of their hours propped up by pillows in bed.) The carefully screened message boards became a supportive oasis in an online environment where, one Washington State mother told me, “parents looking for information about this are made to feel like monsters.”

For years following the controversy at Seattle Children’s, Diekema was unsure how many children had been through growth-attenuation therapy, because no registries track the practice. But then last summer, the Pediatric Endocrine Society published a survey of its members in The Archives of Diseases in Childhood indicating that at least 65 children have received the therapy. (The survey did not break down participants by sex or note whether they had hysterectomies or breast-bud removal.) And by many doctors’ accounts, the demand for it is increasing even as doctors prefer not to talk about the practice publicly. “You might wonder if we’re getting underreporting,” says David Allen, a co-author of the survey and head of the endocrinology and diabetes department at University of Wisconsin American Family Children’s Hospital. “The survey response rate was only 30 percent. We have no good numbers at this point.”

To begin to understand why a parent would choose to disrupt the growth of a child, it helps to know what goes into caring for someone with severe disability. Sandy Walker was a member of the Seattle Growth Attenuation and Ethics Working Group, a panel of concerned parents, medical experts, scholars and bioethicists who developed ethical and policy guidelines for health professionals. In her 50s and physically fit, Sandy has a 20-year-old daughter, Jessica, who has quadriplegia, is nonverbal and needs assistance with all daily activities. In good overall health, Jessica is 5-foot-3 and weighs 95 pounds. She requires either a two-person transfer between bed and wheelchair or a Hoyer lift, a sling-like hanging chair on wheels that moves people in and out of beds, chairs and bathtubs. But the Hoyer is not meant for use in the shower, where Jessica is propped on a stool, so Sandy and her husband, James Walker, rely on two-person lifts that are very difficult when their daughter is wet. “There are many things we can no longer do,” Sandy says, “or perhaps we could do them, but as she gets older, we also get older, and we choose not to.” This means no more hikes in the mountains or swimming at the public pool and the beach.

About nine years ago, the family went to Disneyland. It was still relatively easy to fly with Jessica then, but upon landing she vomited on herself and Sandy. Sandy made a mat from clothes in her suitcase, lay her daughter down in a handicapped stall at the airport and wiped off both of them. “But then I realized the terrible truth,” Sandy says. “I had to lift her, by myself, from the floor to the chair — no easy feat. If ever I wished for a shorter girl, it was then.” Walker believes that if growth-attenuation therapy had been available when Jessica was a small child, she and James would have considered it. “I’ve been shocked by how the disabled community has reacted to it,” she says. “These people speak of the ‘perspective of the disability community’ as though we are not part of it. It makes us feel disenfranchised by the very organizations that were put in place to protect Jessica and our family.”

Cindy Preslar did not want to end up in a similar situation. “I knew we would be his caregivers forever, no matter what,” she told me. “But you think about: My goodness, when my kid’s 15 years old, how am I going to hold and move him?” When she first learned about growth attenuation in a magazine article, shortly after Ricky’s diagnoses, Cindy felt a wave of relief: The idea that Ricky could get the therapy one day was comforting. But she mentioned it to no one. “We’d go to his doctor appointments, then just go on,” she says. She finally brought it up with her husband, Matt, when Ricky was about 2. As it turned out, Matt had seen a reference to the Ashley case on “Law and Order: SVU” and had been waiting for the right moment to bring up the subject himself. They readily decided the therapy was the right thing for Ricky.

The Preslars searched for months before they found a willing doctor, Michael Kappy at Children’s Hospital of Colorado, in Aurora. Kappy had never administered growth-attenuation therapy, but he was an author of a paper justifying and laying out guidelines for its practice. The Preslars flew with Ricky to Colorado twice, first to consult privately with Kappy, then to meet an ethics committee convened by the hospital to approve Ricky’s treatment. Cindy found the ethics-committee members, especially one mother who had had a daughter with disabilities, to be immediately sympathetic. “The meeting lasted an hour or two,” she says. “And Dr. Kappy said: ‘I’ll call it in. What pharmacy do you use?’ And that was it.”

Kappy initially prescribed 2 milligrams daily of estradiol, but on the private message boards at Pillowangel.org, Cindy learned that there were no regulations on how much estrogen physicians could prescribe off-label; other children were getting much higher dosages than Ricky, and their bone age (measured by the degree to which the growth plates are closed) was increasing more rapidly, which would lead to an overall greater reduction in height. She persuaded Kappy to increase the dosage incrementally over the next three years to 8 milligrams daily. Kappy told me that he closely monitored Ricky “to be sure we weren’t going to do him harm.” The higher doses, he said, were “quite effective in speeding up the rate of skeletal maturation.” Within six months, Ricky’s bone age jumped three years, up to that of a 6-year-old. When he began to develop breast tissue, a side effect of the estrogen, Kappy told Cindy that Ricky could have his breast buds removed for cosmetic purposes.
The Preslars declined the surgery as unnecessary. Ricky was weaned off the estrogen when his bone age reached about 15 years, instead of the recommended 16, because his toes were turning purple. This was a symptom of thrombosis, a side effect of estrogen treatment. Multiyear studies on those lanky teenage girls showed an increased risk of mild hypertension, ovarian cysts, amenorrhea and hyperprolactinemia, an excess of prolactin in the blood. But because growth-attenuation therapy for very young children is so new and unstudied, its prolonged effects on their bodies remain unknown.

For parents seeking to restrict the growth of a child, the prospect of being able to provide care at home for many years is a strong motivating factor. Yet despite the fact that three out of four people with intellectual and developmental disabilities live with a family member, the assumption that smaller people actually get better care at home — or in any setting, for that matter — is not supported by empirical evidence; no studies have correlated body size with quality of life. There are only the arguments of parents in opposing camps.

The pediatric bioethicist Nikki Kerruish, a senior lecturer at the University of Otago in New Zealand, has analyzed the perspectives of parents who support the therapy and those who oppose it. In the journal Cambridge Quarterly of Healthcare Ethics, she explains that the very definition of “quality of life” is disputed between the groups. Parents who object to curbing growth prioritize their child’s pleasure and comfort but never at the cost of higher-order concerns like bodily integrity and self-determination. They equate “growing” with “thriving.” No amount of cognitive impairment justifies nonessential medical treatment; growth attenuation is always inappropriate. Conversely, parents who back growth attenuation tend to believe that maximizing pleasurable experiences and minimizing unpleasant ones is the best way to serve a child with extreme disabilities. If this can be achieved by caring for that child as if he or she were a young infant, then that is entirely appropriate.

“My daughter is trapped in a wheelchair all her life, and she’s more comfortable” being smaller, says Nancy, who believes her daughter, Nykkole, may have been the second girl ever to undergo the three interventions that make up the Ashley Treatment. (She asked for their last name not to be published to avoid criticism.) Nancy and her husband began caring for Nykkole as foster parents in her infancy, shortly after she received a diagnosis of brain damage resulting from shaken-baby syndrome and was removed from her teenage parents’ home. Nykkole, now in her late teens, is thought to have a developmental age of 4 to 6 months. “She’s going to be a baby all her life in her brain,” Nancy says. “She smiles and laughs and loves music and lights, but she can’t talk, she can’t tell us what’s hurting her: whether her hips are torqued a little one way, whether a mosquito’s biting her. I didn’t want to add to that PMS and cramps and all that stuff.”

In 2007, after her adult son heard a story on the radio about the Ashley Treatment, Nancy decided to pursue it for Nykkole. “I was [also] thinking about people taking care of her for her whole life, and I just didn’t want to think about how vulnerable she is,” Nancy told me. Like Ashley’s parents, she believed that if her daughter had no breasts, she would be less likely to be a target of sexual abuse. Before undergoing growth-attenuation therapy, Nykkole was given both a hysterectomy and a bilateral mastectomy at the University of Minnesota Masonic Children’s Hospital. When the family then met with the hospital’s ethics committee to discuss Nykkole’s growth attenuation, Nancy recalls, “We brought her out of her chair and into our laps, and said: ‘This is why we want to do this. She needs to be in our laps for our whole life.’ ”

This is the sort of reasoning that frustrates Eva Kittay, a professor of philosophy at Stony Brook University who has written widely about the Ashley Treatment, disability ethics and her experience raising Sesha, a daughter now in her mid-40s, who has multiple physical and cognitive disabilities. Kittay remembers the anxiety she felt in Sesha’s infancy that life with her daughter would only get harder with the passing years. In fact, she says, they have achieved a rhythm in their lives, with Sesha dividing her time between the family home on the weekends and a residence and school for people with disabilities on weekdays. Kittay says that she hopes new parents considering growth-attenuation therapy will not rush into an intractable decision while still coming to terms with a child’s diagnosis of intellectual and developmental disability.

“You cannot halt things, keep them children,” Kittay told me. “You have to think about your family changing. You will have other needs. They will have other needs.”

Philip Zeitler is chairman of the Department of Endocrinology at Children’s Hospital, in Aurora, which has overseen growth-attenuation therapy for about 20 children. He considers the treatment to be as ethical as the practice in his field of withholding medicines that suppress precocious puberty, a medical condition that causes maturation to start too soon and leads to reduced stature. Allowing precocious puberty, which happens to be common in children with disabilities, to proceed without intervention is like letting a kind of natural growth attenuation take place. Zeitler would like to see the therapy come “out of the shadows” by becoming the focus of empirical studies or at least an informal national registry where doctors could report cases and compare outcomes. “Given the controversies here,” he asks, “is it unethical not to be studying it?” That is, if parents are free to seek growth-attenuation therapy and some physicians are accommodating them, shouldn’t we understand more about its efficacy and long-term effects?

Yet greater scrutiny also brings the risk of regulation. “It’s probably not high on the F.D.A.’s radar, and to be frank, I would think that would be a terrible thing if it were, because it would create new barriers for these families,” Zeitler says. “The F.D.A. is going to say, ‘You shouldn’t be doing this unless there’s outcome studies.’ It would be ideal if we had outcomes, but they take 10 years. And meanwhile, what about all these families who are earnestly looking for help?”

Families already face difficulty finding hospitals and doctors sympathetic to growth-attenuation therapy. Because of his early involvement with Ashley X’s care at Seattle Children’s Hospital, Diekema occasionally gets calls from parents. He advises some of them — like one parent who wondered if the therapy might make an autistic child’s violent outbursts more manageable — against pursuing treatment. Diekema usually refers appropriate candidates to the network of families on Pillowangel.org. Physicians and hospital ethicists also call him in search of guidance. He estimates that as many as 10 pediatric hospitals have at least presented cases to ethics committees, but he doesn’t know those outcomes. “They don’t really want anybody knowing what they’ve decided,” he told me, “because they don’t want it publicized.”

Part of that reluctance, Diekema believes, stems from doctors’ unwillingness to be identified as supporters of growth-attenuation therapy. A year after Diekema and Gunther published their paper, Gunther committed suicide in his home. A flurry of speculation ensued: Had the criticism been too great? Had he regretted his actions? Diekema and Gunther’s family believe his death was brought on by depression, not by the negative attention from the Ashley X case. All the same, Diekema says, he thinks many physicians decided that prescribing growth-attenuation therapy “wasn’t going to be worth it.”

For those endocrinologists willing to administer the therapy, Diekema has some advice: Do so only in consultation with a developmental specialist who has evaluated the child. Because the treatment is recommended only for children with the lowest cognitive function, Diekema says that “anyone doing this has to think hard about ‘How sure of the prognosis are we?’ Not everybody is very good at parsing out what’s a communication problem and what’s actually going on in the brain. And I do think you need a fair amount of certainty there.”

Can such certainty ever be guaranteed? A significant body of research shows that the intellects of people with severe motor impairments can be grossly underestimated. What’s more, the minds and abilities of many people with cognitive disabilities evolve over their life spans, especially when they are given means to communicate. Karrie Shogren, co-director of the Kansas University Center on Developmental Disabilities, believes that growth-attenuation therapy is too often administered without consulting developmental specialists. “One of my major concerns,” she says, “is that places where this is being done might tend to be places that don’t have strong disability affiliations or support.” She also questions the use of outdated evaluation criteria, like mental or developmental ages, in assessing candidates for treatment. These measuring sticks can misrepresent a person’s capacity for understanding, especially someone who lacks speech and is severely restricted in movement. But whatever the level of impairment, Shogren says, “there’s an inherent conflict between the Ashley Treatment and the current way of thinking about disability,” which is expressed by the modern mantra of her field: Presume competence. In that light, medical treatment can never be justified on the grounds that a child “does not have the potential to take on adult roles.”

At its core, the battle over growth attenuation is a battle between old and new ways of thinking about disability: the old “medical model,” which regards disabilities as a problem to be fixed, and the new, “social model,” which frames disability as a natural facet of the human experience. The social model promotes self-determination for those who have even the most complex disabilities; society should adapt to them, not the other way around.

Ricky Preslar is now 9. Two years after completing growth-attenuation therapy, his height has stabilized around 43 inches. He is still nonverbal (unless you count his easy laugh), but in the past year, Cindy says, he has shown signs of purposeful movement, an important developmental milestone, by using a head-motion-activated assistive-communication device to stop and start videos at his school for the visually impaired. He can move around the kitchen in a gait trainer and goes to hippotherapy (a kind of physical therapy on horseback) once a week. Cindy’s goal this year is to run a marathon while she and her husband push him the whole way. She says that Ricky, who is now 43 pounds, is beginning to seem conspicuously smaller and lighter than many of his peers at school. The Preslars have never regretted their decision to attenuate Ricky’s growth. For Cindy, there are times — like when she recently strained her back — when she is particularly grateful that her boy is, and always will be, small.

A couple of years ago at a Parents Reaching Out conference at Ricky’s school, Cindy and Ricky’s caregiver, Jessica Covak, were chatting with the parents of a boy in Ricky’s class when the father expressed sadness at the prospect of not being able to pick up his child when he got too big. Cindy felt tears welling in her eyes, and the words just came out.

“You guys might think this is weird, but Ricky’s done growing, pretty much,” she said. She explained about the effect that high-dose estrogen has on growth and offered to give them more information, if they were interested. The couple stared at Cindy, jaws dropped, then found an excuse to wander away.
“Did I freak them out?” Cindy asked Jessica.

“Yeah, I think you did,” Jessica answered.

Cindy didn’t mind too much. “Every child is different,” she says. “Every family does the best they can.”

By GENEVIEVE FIELD

Source : http://www.nytimes.com/2016/03/27/magazine/should-parents-of-severely-disabled-children-be-allowed-to-stop-their-growth.html?rref=collection%2Ftimestopic%2FDisabilities&;action=click&contentCollection=health&region=stream&module=stream_unit&version=latest&contentPlacement=10&pgtype=collection

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