An increasing number of parents consider option a medical miracle — others see it as a violation of disabled people’s human rights
By Kristen Gelineau
Associated Press
BALI, Indonesia (AP) — Whether Charley Hooper has any concept of the space she occupies in the world is an enigma.
She is so disabled that her mother considers her “unabled.” At 10, her brain is believed to function at the level of a newborn’s. She cannot speak, cannot walk, cannot see anything beyond light and dark and perhaps the shadowy shape of a face held inches away.
As her body grew bigger, her parents feared her world would grow smaller. How would they lift her or get her out of the house? They saw for Charley a grim future of hoists and machinery, of isolation and loneliness, of days spent trapped in bed, staring up at a ceiling she couldn’t even see.
So Jenn and Mark Hooper came up with a radical solution. They gave their daughter hormones to stop her growth. Then they had doctors remove her womb to spare her the pain of menstruation. Charley is now around 1.3 meters tall (4 foot 3) and 24 kilograms (53 pounds), and will remain so for the rest of her life.
The Hoopers’ fight to get the hormone treatment — known as growth attenuation — was grueling. Although an increasing number of parents across the U.S., Europe and New Zealand consider it a medical miracle, others see the very idea of stunting and sterilizing the disabled as a violation of human rights.
A doctor once told Jenn he would never do such a thing to his child. Well, aren’t you lucky, Jenn replied, that you never had to make that choice.
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To hold Charley is to experience the sensation of dead weight. She is a jumble of uncontrolled limbs with a floppy head that needs supporting. Her knees are often locked and her legs held stick-straight. Her wide brown eyes roam around, not focusing but occasionally moving toward the source of an interesting noise.
Her parents try to interpret what she feels by the pitch and volume of her moans, and whether her freckled face is slack, smiling or contorted in a gaping yawn when she suffers through intense muscle contractions. Here in Bali, their annual retreat from the bleak winters back home in New Zealand, the warm sun illuminating her olive skin can trigger what looks like a smile.
Everything about Charley is a guess. Mark and Jenn dress her in purple because they imagine she would like it. At Christmas, they have no idea what to give her, but buy her the same number of toys as her little brother Zak to avoid his questions about Santa’s fairness.
They had wanted a baby so badly for so long. The former high school sweethearts had always assumed they’d have children. They married in 1991 and began trying to get pregnant right away. But when years passed and no babies came, they headed overseas in search of other adventures. For years, they lived in the UK, working in pubs and driving forklifts to fund their travels through Europe and Africa.
After returning home to New Zealand’s North Island, they began trying again for a baby and were on the verge of attempting in vitro fertilization when Jenn took a pregnancy test on Christmas Day, 2004. It was positive.
Charley came into the world floppy and not breathing. Midwives bungled their attempts to resuscitate her, and by the time specialists arrived, she had been deprived of adequate oxygen for nearly an hour. Her brain was left irreversibly damaged.
The first few months were misery. Charley screamed around the clock.
They waited for that moment parents rhapsodize about — when your baby first looks into your eyes. But Charley’s eyes rolled aimlessly in their sockets.
After five months, doctors delivered the diagnosis: Infantile spasms, a severe form of epilepsy. The chances of her ever walking or talking were virtually nil. And she was blind.
Charley suffered constant seizures, and vomited everything up. Cerebral palsy weakened her throat muscles, making every swallow potentially lethal. Jenn and Mark barely slept, checking her throughout the night to ensure she hadn’t choked to death. There were dark moments when they wondered whether it might be better for her if she did die.
Valium helped quiet her screams. And they found other ways to soothe her: bouncing her on their knees, giving her warm baths. But eventually, they knew she would be too big to hold or lift from the tub. In a life of so few joys, what then?
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Jenn found an answer to their dilemma in a newspaper article about a severely disabled Seattle girl called Ashley.
This first case of growth attenuation on a disabled child popped up in a medical journal in 2006. Ashley’s parents wanted her to remain small enough to participate in the family’s activities as she grew up. So doctors began giving her high doses of estrogen via skin patches, pushing her body into early puberty, accelerating the fusion of her bones and curtailing her height.
The most controversial part of the “Ashley Treatment” was the removal of her uterus and breast buds. Her parents wanted to eliminate the pain of periods and the risk of pregnancy if she was ever abused, along with the discomfort of breasts since she spent so much time lying down and strapped into wheelchairs.
Growth attenuation, which refers only to the hormone treatment, is in itself not new. In the 1950s and ‘60s, doctors occasionally gave hormones to girls who were expected to grow very tall. And the debate over Ashley’s hysterectomy and breast bud removal was a familiar one, given the early 20th century era of eugenics that saw tens of thousands of disabled people forcibly sterilized to prevent them from reproducing. In 2013, the United Nations dubbed the sterilization of the disabled without their consent “an increasingly global problem,” and last year the World Health Organization demanded an end to it.
What is newer is the idea of stunting disabled children to ostensibly improve their lives.
More and more doctors have received requests for the treatment. A recent survey of the Pediatric Endocrine Society, most of whose members are in the U.S., showed that 32 of 284 respondents have prescribed growth-stunting hormones to at least one disabled child.
“As more people do it, it’s less weird, it’s less freaky,” says Norman Fost, a bioethicist who supports the practice. “There’s comfort in numbers.”
But it is by no means widely accepted. Many doctors have refused to prescribe the treatment, and those who do tend to do so quietly.
Gary Butler, chairman of the European Society for Pediatric Endocrinology’s clinical practice committee, feels there is no need to stunt the growth of severely disabled children because they often enter puberty early, which naturally leaves them smaller.
“We would never, ever resort to these invasive treatments,” Butler says. “It’s completely unnecessary and I think it’s important that one upholds the dignity and the rights of young people with a learning disability and physical disability.”
The public, too, often reacts to the treatment with everything from unease to revulsion. After all, in many families, growth is about so much more than size. It is the bittersweet marker of the passage of childhood, measured in the timeworn practice of pencil markings on a wall, celebrated and mourned by visiting aunts and uncles who cry, “Look how big you’ve gotten!” Growth is considered a given.
“People are really entitled to grow and to become the people they were meant to be,” says Margaret Nygren, CEO of the American Association on Intellectual and Developmental Disabilities. “Would you ever want this kind of treatment done to you without your consent or knowledge? And if the answer is no, then why would one want to do that to someone else?”
Yet for Charley’s parents, that question is moot, because they have never been able to ask for her consent on anything. They have always had to imagine the person who exists inside her broken body, and what that person would want.
Like most parents, they believe their daughter would want an extraordinary life. So in many ways, it comes down to this: What makes a life worth living, and what is a life worth?
To Jenn, a person’s life is a collection of experiences, stories and memories. Charley can’t make those for herself, so her parents wanted to make them for her — from taking her sledding to traveling the world. But that meant keeping her small enough to go along for the ride.
The choice they made for Charley, they say, was a choice made out of love.
“We haven’t stopped her doing anything. Growing would have stopped her doing things,” Jenn says. “We didn’t take away any choices that weren’t already taken from her.”
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Growth attenuation had never been performed on a disabled child in New Zealand, but the potential for a fight hardly dissuaded Jenn. Tough, self-assured and admittedly stubborn, she does not respond well to “no.”
There is a fury behind Jenn’s drive. Her words are often brittle, sometimes brutal, when she talks about the fate that befell her daughter. She acknowledges she will likely be angry for the rest of her life. But anger, she says, can be one hell of a motivator.
They approached Paul Hofman, a pediatric endocrinologist at Starship Children’s Hospital in Auckland, New Zealand. At first, he didn’t like the idea.
“The whole process of making a child permanently into an infant and a small child seemed wrong to me,” he remembers today.
It took just one meeting to change his mind. He could see the Hoopers wanted to give their daughter a good life, and in the end he felt growth attenuation would help her.
“What I thought was better for her was to have a functioning family that worked well,” he says. “I accepted (Jenn’s) argument — very, very logical — that being smaller was more sensible.”
Hofman explained the possible risks: High blood pressure, blood clots, stroke. Then he sought an opinion from an Auckland ethics panel.
They dismissed the treatment as unnecessary.
“There are well developed ways to manage severely disabled people in New Zealand and to assist family members,” the panel wrote. “To consider alternatives raises the concern that these are less than ideal.”
Jenn was livid. Those “well-developed ways” were hardly ideal. Few public places have equipment to move disabled people in and out of wheelchairs. They already had to change Charley’s diapers in the car, or across the seats in a plane. What would they do when she was fully grown?
So she proposed a compromise: What if she got the estrogen patches from a doctor overseas and started the treatment outside New Zealand? Could a local doctor continue the treatment once they returned home?
The panel agreed. If Charley was already receiving a medication, local doctors should continue to treat her, for safety reasons.
It was the break the Hoopers had been waiting for. Soon, Jenn, Mark, Zak and 4-year-old Charley were on a plane to South Korea, where Jenn had found a doctor willing to give them the hormones.
Within days, the Hoopers say, they noticed changes: Her seizures stopped. She began smiling. Her stiff limbs became more pliable. Hofman says that may be because estrogen changes neurological activity and can relax muscles.
After six months, her breast tissue began growing, then flattened.
At 6, she began having breakthrough bleeding, similar to what a woman can experience on birth control pills. Concerned that Charley would have severe period pain like Jenn, the Hoopers discussed a hysterectomy with her gynecologist. She would never be able to consent to sex, they reasoned, let alone to pregnancy.
The ethics board approved the hysterectomy. Charley was 7 when doctors removed her uterus.
“I knew it was the right thing to do, but it was hard,” Jenn says. “There was sort of a finality to it.”
It took nearly four years before Charley stopped growing.
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Jenn sits next to her daughter, whose body lies immobile near the pool. She tickles Charley’s nose with one of her braids and brushes the bangs from her eyes.
Jenn has spent years searching those eyes for some flicker of recognition. A few times, Charley has locked gazes for a fraction of a second — a precious connection, but one Jenn doubts her daughter noticed.
She has never bonded with Charley the way Mark has. To Mark, Charley is his daughter. To Jenn, she is more like a patient. That’s largely because she has spent so much time focused on Charley’s survival. She loves her, but in a slightly detached sense, the way an aunt might love a niece.
Some mornings, in the hazy seconds after waking, Jenn hears a child’s footsteps outside her room. It is always Zak, but in her half-conscious state, there’s a magical moment when she wonders if it’s Charley.
She and Mark still get up throughout the night to ensure Charley is breathing. Mark eventually quit his job as an architectural draftsman to help Jenn with the kids. They get by on a government-funded insurance program that supports at-home caregivers, and sales of a torso support wrap Jenn designed for disabled children.
Jenn has concluded that Charley has no idea she is her mother. Mark believes she recognizes them — at least, he likes to think she does. “Don’t you?” he murmurs, pressing his nose against hers.
This is perhaps the biggest issue brought up by those who oppose growth attenuation: How does anyone know what goes on in a profoundly disabled person’s mind?
Sue Swenson raised a son who was quadriplegic, autistic, and couldn’t speak. He grew to 1.8 meters (6 feet) tall and 86 kilograms (190 pounds) before dying at age 30. He had a good life, she says, and still took part in family activities when fully grown.
Swenson made medical decisions for her son all the time. But to her, growth attenuation is not just another treatment — it is a mutilation. She wanted her son to grow up and be the same size as his peers. She wanted him to fit in.
“Even if they can’t speak, how do we know what their experience is?” Swenson asks. “What if they’re aware that they’ve been made different?”
The question of Charley’s mind is the one that pierces through Jenn’s wall of anger to reveal the anguish underneath. Because while she is certain she is right — that Charley is unaware of what has happened to her, and that she will never improve — she so wants to be wrong.
“I would be the happiest person in the world to have her come to me,” Jenn says, the words catching in her throat as tears begin to run down her face. “If I’m totally wrong and she can walk tomorrow, I haven’t changed her potential.”
And she does see potential in her daughter, even if her daughter can’t see it in herself. Even if her daughter can’t see at all.
Jenn sees people gain perspective just by meeting Charley. She sees other disabled children benefit from the support wrap she designed for Charley. She sees women helped by the advocacy group she launched to improve maternity care after Charley’s botched birth.
“Life isn’t about being successful, it’s about being significant. And she’s certainly significant,” Jenn says. “So many others aren’t, because they’re never seen. We make sure she is.”
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In the first weeks of Charley’s life, Jenn found herself wanting an “out.” So she made a deal with Mark: She would give Charley her best two years as a mother. But if their daughter hadn’t improved by then, they would consider giving her up.
It never came up again.
Today, she can’t imagine their world without Charley. When the family gathers to watch “Survivor” at night, Charley sits with them. When Mark, Jenn and Zak splash around in the pool, Charley lies a few feet away. When Jenn rocks her infant son, Cody, she murmurs in his ear, “See your sister?”
When Charley was 5, she almost died from a chest infection. Jenn began to wonder what they would say at her funeral; they couldn’t talk about her laughter or her favorite games. So they wrote up a bucket list for Charley with experiences they imagined a little girl would enjoy, and spend their days trying to accomplish as many as they can. Still to do: Go down a water slide, be a flower girl, meet the Queen.
Would Charley actually like those things? They don’t know. But they believe she’d prefer checking items off the list to being stuck at home.
Though Mark and Jenn don’t believe she has any concept of her mortality, they try not to talk about it in front of her, out of respect.
“We don’t expect her to live forever. We don’t want her to live forever. Who wants this life forever?” Jenn says. “So we give her the best life we can while we’ve got her.”
From the radio attached to Charley’s stroller, Kesha begins to belt out “Die Young.” Mark lifts his daughter’s arm in the air and waves it back and forth to the beat.
He hums along to the chorus: “So while you’re here in my arms, let’s make the most of the night, like we’re gonna die young.”
Charley lets out a small moan.
Her father lets go of her arm. It drops back into place at her side, unmoving.
