Tuesday, May 24, 2016

Growth attenuation

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…

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.”…

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…

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.”…

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.”…

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…

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….

“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…

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.’ ”…

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.

Courtesy of my mother.


  1. 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.”...

    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.”...

    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.”


  2. Gunther DF, Diekema DS. Attenuating growth in children with profound developmental disability: a new approach to an old dilemma. Arch Pediatr Adolesc Med. 2006 Oct;160(10):1013-7.

    Caring for children with profound developmental disabilities can be difficult and demanding. For nonambulatory children with severe, combined neurologic and cognitive impairment, all the necessities of life must be provided by caregivers, usually parents, and these tasks become more difficult as the child grows to adolescence and adulthood. Many parents would like to continue caring for their child with special needs at home but find it difficult to do so as the child increases in size. If growth could be permanently arrested while the child was still small, both child and parent would likely benefit because this would facilitate the option of continued care in the home. Treatment of the child with high-dose estrogen, initiated at an early age, could provide this option. High-dose estrogen both inhibits growth and rapidly advances maturation of the epiphyseal growth plates, bringing about permanent attenuation in size after a relatively short period of treatment. We present a case report and discuss the medical and ethical considerations of such an intervention strategy. We suggest that after proper screening and informed consent, growth-attenuation therapy should be a therapeutic option available to these children should their parents request it.

  3. 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...

    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.”


  4. Eva Fetter Kittay. Forever small: The strange case of Ashley X.


  5. The parents' decision has drawn criticism and even outrage from some doctors and caregivers, who say such treatment is a violation of a person's dignity. Some say it's also a violation of the medical oath: First do no harm.

    But Ashley's parents say the move was a humane one, allowing her to receive more care, more interaction with her younger brother and sister, and more of the loving touch of parents and others who can carry her.

    As a result, they say in a written account posted on the Web this week, "we will continue to delight in holding her in our arms and Ashley will be moved and taken on trips more frequently and will have more exposure to activities and social gatherings (for example, in the family room, backyard, swing, walks, bathtub, etc.) instead of lying down in her bed staring at TV (or the ceiling) all day long."

    The treatment has involved a hysterectomy, surgery to prevent breast growth and subsequent high doses of estrogen.

    Doctors say that the treatment's major risks come from the surgeries, but that it also carries potential benefits such as eliminating the risk of ovarian and breast cancers.

    High-dose estrogen to inhibit growth was used occasionally in the 1950s and 1960s, mostly on teenage girls whose parents were concerned about the social stigma of being too tall. The drugs could stop a 5-foot-9 girl from becoming, say, 6 feet tall.

    As that stigma has gone out of fashion, so has the treatment, medical ethicists say.

    But Ashley's case involves an entirely separate ethical realm, that of whether a severely disabled person might be better off having his or her growth impaired.

    Publication of the case in a national pediatric journal in October set off criticism of the parents' decision; "eugenics," "slippery slope," "despicable" and "Frankenstein-esque" are some of the printable contributions to websites on the topic.

    But it wasn't until 11 p.m. on New Year's Day that Ashley's parents told their side of the story.

    That was when Ashley's father hit the send button on an e-mail to doctors, reporters and others, and began entering chat rooms with a link to their story --http://ashleytreatment.spaces.live.com/blog -- and to photos of the girl: http://ashleytreatment.spaces.live.com/photos.


  6. That we have to hire help or rely on equipment to do what parents’ hands and arms can do is a loss; but although the ability to care for Ashley without such assistance may be lost, the ability to care for Ashley, and even to provide that care at home, is not. Learning what services and resources are available, advocating for more services, and adjusting to new people and equipment all take time and energy. And, surely, raising a child with severe disabilities is demanding enough: I know, I have been there. GA (growth attenuation) is a shortcut through these difficulties. But, as I will argue below, if these procedures would not be done but for the disability when these do nothing to cure or mitigate the disability, and if we believe that no reason justifies restricting the height of a person who otherwise would be within normal range to the height of a six-year-old, when that person does not have severe cognitive disabilities, then we should not avail ourselves of this procedure in the case of this disability…

    Imagine parents requesting GA for their talented prepubescent son who passionately wants to be a champion gymnast (or jockey or coxswain, where small size is necessary), and is willing to trade height for the realization of a dream. Surely the request would be denied. To accede to it, some may argue, would be to close the boy’s future options; but note that to refuse him, we foreclose an option for which he has demonstrated passion and talent. Instead, I venture, our moral intuition is that when we regard his body as merely instrumental to a specified ambition or goal, we also treat him as a mere instrument: our bodies are ourselves—what is done to our bodies is done to us…(continued)

  7. (continued)As the childishness in her face fades, Sesha’s body has taken the form of a woman. I don’t know if Sesha can rejoice in her breasts, if she notices them at all, if she would miss them, or if she compares herself to other girls. What I do know is only that I don’t know. Nothing about Sesha—with all her profound incapacities—tells me that Sesha is incapable of these feelings. I can say that I, as her mother, delight in her womanliness. It is very much a part of Sesha, as she is now…

    To have the mind of a baby and the capacities of a baby are not the same thing, for the disabled person may well have an understanding and a set of emotional responses that far exceed her capacity to act. The brain may be impaired, but it is not frozen. Synapses continue to be formed as they do in all brains. As the Board of the American Association for Intellectual and Developmental Disabilities writes: “The abundant evidence [is] that all children are able to learn and that the cognitive capabilities of children with severe motor impairments can be grossly underestimated . . .”(AAIDD 2007)…

    A young woman with Rett’s Syndrome with whom I am acquainted has no more capacities than a very young child, perhaps a baby of three months. But this young woman, when told that her father was dying, would be found by the caregivers sitting quietly shedding tears for weeks. This is not within the understanding of a baby. A dear caregiver joked in front of my daughter that the secret of her youthful appearance was that she had no worries, like paying taxes and bills. I countered by saying that Sesha has a lot to worry about, like not being able to scratch an itch, move out of an uncomfortable position, tell us what she wants, and so on. My daughter turned to face me, gave me an intense look, smiled broadly and reached out to hug me. I was startled, as were all who witnessed this response. Maybe she only grasped the tone, but it is no less possible, indeed plausible, that she understood the words. She has, after all, been listening to human speech for forty years…

    I deeply appreciate the worry and the fear that such a young woman might be exploited or abused, but removing breast buds and preventing breasts will not guarantee or even reduce these possibilities. If one is perverse enough to sexually abuse such a girl, might he or she even be attracted by the strange history of a child who parents removed her breast buds? Who can say? Only careful screening, supervision, and respect for these girls and women can offer the needed protection.


  8. Children with disabilities are subject to many in¬vasive procedures that are limited mostly to people with disabilities. Diekema and Fost cite “tracheotomies to improve airway patency and enable suctioning, tonsillectomy to reduce airway obstruction, fundoplication to reduce gastroesophogeal reflux, gastrostomy tubes to aid feeding, spinal fusions to prevent advancement of scoliosis, and tendon releases to reduce the effects of spasticity”(Diekema and Fost 2010, 36).

    But all these are also carried out on children not otherwise disabled or they are directed to a specific medical disorder, not a class of persons per se. Tracheotomies are used for whoever needs this assistance for breathing; tonsillectomy and spinal fusions are carried out regardless of a person’s cognitive or physical capabilities; gastrostomy tubes may be more frequently administered to old and young whose cognitive function is impaired, but only because such impairment is coupled with conditions that impact swallowing and food intake. Severe cognitive disability is not an indicator for any of these or any other procedure save AT[Ashley treatment], for which it is the sole and necessary indicator.

    AT is intended for ease in handling the person and preemptively treating sources of pain or discomfort. The disanalogy with all the other cases is that AT alone is not considered as a treatment for any other people whose size, or potential size, can pose equivalent difficulties in care or exclusion from family life—for example, an autistic boy who is likely to turn into a bulky six-footer and have violent tantrums that will require restraints.

    The main reason for limiting the eligible population in this way is the supposition that these people alone will never know the difference—even if we cannot be sure this is true. But the treatment is not directed at their cognitive disability. It is directed at the difficulty of caring for them—a condition shared by many other populations. Then what else might we be able to do to this population on the supposition that they will never know the difference?


  9. The long, cruel, and gruesome history of people with cognitive disabilities, especially when the disabilities are severe, are all justified on the supposition that these people don’t know the difference or can’t feel the indignity—that they won’t know the difference if a part of their brain is lobotomized, if they are deprived of clothing, if they are showered communally by being hosed down. The horrid shame of it all is made that much worse when some who are included are totally cognizant of their mistreatment. Yet we also have learned that once we stop supposing that “they don’t know the difference anyway,”we find out that they were entirely capable of knowing, understanding, or at the least experiencing the treatment as mistreatment.

    People with severe cognitive disabilities are often not told when a close family member dies because “they just wouldn’t understand.”Yet the young women with Rett’s understood all too well when told. So did another young man, diagnosed as severely cognitively disabled, whom I know. I myself witnessed his howling pain when told of his father’s death. The uncertainty of us not knowing what they know is a great risk indeed. And the risk of further stigmatizing this group and discriminating against them—doing something to them that would not be done to anyone else—is also far too grave a risk to permit AT or even just GA[growth attenuation] to be routinely offered to parents of these children. We reopen a Pandora’s box of well-intended salves that turn into nightmares when we allow severe cognitive disability to be the lone and sole indicator for a certain treatment.

    In medical care as well as parental care we do balance risks and benefits. Caring is often a matter of making these difficult choices, but if we fail in our care when we do more damage than good, an ethic of care would suggest that some risks are not worth taking, no matter the benefits. The psychological pain expressed in some other cases where parents have intervened in their child’s development while their child was very young and where there was not a medical necessity indicates that AT is not a risk worth taking, especially since the difficulty is ultimately less with the child’s body and more with the social re¬sponse, or rather lack of social response, to the needs of families of a SCDN child.


  10. As children such as Ricky become bigger and heavier, it is more difficult for their parents to care for them, said Dr. Michael Kappy, who prescribed the treatment, called growth attenuation.

    A smaller child can be moved more easily, allowing families to continue caring for them at home when they are older, Kappy said.

    “These are kids who are total-care,” said Dr. Philip Zeitler, chairman of the department of endocrinology at Children’s. “They need to be transferred from wheelchairs into beds, beds into bathtubs. If the child becomes 6-foot-3 and 250 pounds, the child may need to go into residential treatment. The family will no longer be able to care for them. Keeping them in their home is better for them.”

    Before the therapy, Ricky’s parents knew that at some point the boy would become too heavy to lift and move easily, narrowing the opportunities he would have to be with his 12-year-old brother, Max, parents and others.

    “I’m not a big person, and neither is my husband,” said Cindy Preslar, 46, Ricky’s mother. “Knowing that my husband and I would be taking care of Ricky for the rest of his life, I didn’t want him to be in a group home or institution. We will be able to care for him longer because he is small,” she said.

    She and her husband, Matt, 43, who live in Cloudcroft, N.M., spend time on the road with Ricky and Max, traveling in a motor home.

    Ricky’s small size makes it possible for him to get out more and spend more time with the family, Cindy said.

    With Ricky at 43 pounds, they can still carry him, even up the stairs and through the motor home’s narrow door.

    In July and October, family members will push Ricky in a jogging stroller as they participate in two marathons.

    “If he was fully grown, we would probably have to have a hoist to lift him into his wheelchair, and I would push him in his wheelchair. He would be in the wheelchair most of the time,” Cindy said.

    But for some, interfering in the growth of a child who has no say in the matter is an outrage.

    “It is what it is saying about valuing, or devaluing, people with disabilities,” said Curt Decker, executive director of the National Disability Rights Network. “Granted, they need an incredible amount of care, and some grow up to be big and difficult to manage. But I don’t think it is right to mutilate them.”

    Doctors believe that Ricky’s cognitive functioning will not grow much beyond its present level, but there are many families who were told their child would not escape a vegetative state, only to see their cognitive ability grow, said Julie Reiskin, executive director of the Colorado Cross Disability Coalition.

    “This has the effect of making someone something they are not, and that is deeply concerning,” she said.

    When The New York Times ran an article about attenuated growth recently, Zeitler was surprised by the number of objections.

    Children such as Ricky have devastating central nervous system abnormalities and little ability to interact with their environment, he said. They may recognize and smile at a family member, but their ability to function is very low, he said.

    “Most families wrestle with this, and we need to recognize that all we are doing is making them smaller,” Zeitler said. “It is offensive that people think that height is associated with any other benefit other than height.”


  11. Kerruish N. Growth Attenuation Therapy. Camb Q Healthc Ethics. 2016

    The "Ashley treatment" has provoked much debate and remains ethically controversial. Given that more children are being referred for such treatment, there remains a need to provide advice to clinicians and ethics committees regarding how to respond to such requests. This article contends that there is one particularly important gap in the existing literature about growth attenuation therapy (GAT) (one aspect of the Ashley treatment): the views of parents of children with profound cognitive impairment (PCI) remain significantly underrepresented. The article attempts to redress this balance by analyzing published accounts both from parents of children who have received GAT and from parents who oppose treatment. Using these accounts, important points are illuminated regarding how parents characterize benefits and harms, and their responsibilities as surrogate decisionmakers. This analysis could contribute to decisionmaking about future requests for GAT and might also have wider relevance to healthcare decisionmaking for children with PCI.