Should Parents of Children
With Severe Disabilities Be
Allowed to Stop Their Growth?
Caring for people with severe mental and physical limitations
becomes vastly harder as they get older. Some parents believe
medically stunting them is the answer — but is it ethical?
By GENEVIEVE FIELD
MARCH 22, 2016
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Ricky 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-yearold 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.”
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Recent Comments
Mary D March 28, 2016
My heart aches for these families. My daughter and son in law were
faced with a horrible situation when their baby was DX with a very...
Polly Meulenberg March 27, 2016
I fully understand the issues here and do not disapprove. My child
sufferedextreme brain damage at Sybley Hospital here in MD. Lack of...
gm March 27, 2016
Having recently retired from a state agency that serves the
developmentally disabled I have witnessed the anguish of parents who
no longer... See All Comments
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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, growthattenuation 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 breastbud 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 growthattenuation 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.
Photo
The Preslar family at home this month. Credit Sasha Rudensky for The
New York Times 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.
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“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 rega...

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