Family Stunt Daughter Charley Hooper's Growth With Hormones To Make Disability 'Manageable', Anger Campaigners

Family Stunt Daughter's Growth With Hormones To Make Disability 'Manageable '
|

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.

Open Image Modal

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 gruelling.

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 sterilising 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.

Open Image Modal

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 fertilisation 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 rhapsodise 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?

Open Image Modal

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 oestrogen 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 sterilised to prevent them from reproducing. In 2013, the United Nations dubbed the sterilisation 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.

Open Image Modal

More and more doctors have received requests for the treatment. A recent survey of the Paediatric 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 Paediatric 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."

Open Image Modal

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 paediatric 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 oestrogen 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 oestrogen 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 gynaecologist. 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.

Open Image Modal

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 recognises 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."

Open Image Modal

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 favourite 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.

21 Rare Diseases
Stiff Person Syndrome(01 of21)
Open Image Modal
People who suffer from this acquired neurological disorder experience repeated, often painful, muscle spasms as well as muscular rigidity and stiffness. According to the National Organization of Rare Diseases, spasms can occur at random or they can be caused by something as seemingly benign as light physical contact or an unexpected noise. The cause of Stiff Person Syndrome isn't yet known, but symptoms can be stabilized with medication. Left untreated, however, a person can lose the ability to walk.
Gigantism(02 of21)
Open Image Modal
This disease, which according to the NIH is most often caused by a begnin tumor in the pituitary gland, results in an excess of GH, or growth hormone. This causes sufferers to grow abnormally large, not just in terms of height, weight but also organ size. It results in complications like delayed puberty, increased sweating, and secretion of breast milk.
Pica(03 of21)
Open Image Modal
Characterized as an eating disorder, Pica causes people to eat what the National Organization for Rare Disorders describes as "non-nutritive" things. That umbrella term can include (but isn't limited to) dirt, clay, paper, and paint. Interestingly, it's not unusual for young kids to experience transient pica as a kind of phase, and pregnant women are also known to develop temporary pica cravings. The cause? Unknown. But in order to be diagnosed with full-blown Pica, a person's symptoms must last for more than a month.
Maple Syrup Urine Disease(04 of21)
Open Image Modal
MSUD, which is passed down through families, is a life-threatening metabolic disorder passed down through families that stems from the body's inability to process certain amino acids, leading to a build-up of them in the body. According to the NIH, symptoms usually surface in early infancy and can include vomiting, lack of energy, seizures, and developmental delays. MSUD takes its name from another symptom -- the urine in affected infants smells like caramel or maple syrup.
Situs Inversus(05 of21)
Open Image Modal
Situs Inversus is a congential condition in which internal organs of stomach and chest lie in mirror image of their normal body position -- something many sufferers aren't aware of until they seek medical help for an unrelated problem. People with Situs Inversus typically wear some form of identification to help doctors in the case of a medical emergency.
Trigger Thumb(06 of21)
Open Image Modal
Trigger thumb, or trigger finger as it's sometimes known, causes a person's finger or thumb to get caught in a locked position. According to the Mayo Clinic, it can then stay stuck or straighten with a painful "snap." The cause? It depends. Trigger Thumb is the result of a narrowing of the sheath around the tendon in the problem figure, but that can be caused by a lot of things, including any activity that requires people to grip things frequently. Trigger Thumb is also more frequent in women.
Scurvy(07 of21)
Open Image Modal
Once the disease of sailors and pirates, scurvy does still exist in the United States, though predominantly in older, malnourished adults. It usually stems from a Vitamin C deficiency, which can result in gum disease, skin leisons, and swelling of the joints. (credit:Getty )
Wilson's Disease(08 of21)
Open Image Modal
This progressive genetic disorder causes sufferers to store excess copper in tissues, including the brain and liver. Though the Mayo Clinic says that the body depends on copper in order to use iron and sugar, too much of it in the body can have real consequences. Which is why sufferers of Wilson's Disease are prone to liver failure.
Foreign Accent Syndrome(09 of21)
Open Image Modal
According to researchers at the University of Texas at Dallas, this speech disorder causes people to experience a sudden shift in their accents and though it is known to be caused by things like brain trauma, conversion disorder, or multiple sclerosis, an exact reason behind the syndrome is unknown. Sufferers of FAS dramatically shift their speech in terms of timing and intonation, which often causes them to sound foreign, but they remain totally comprehensible. Documented accent shifts include from American English to British and from British to French.
Carcinoid Syndrome(10 of21)
Open Image Modal
According to the National Organization of Rare Diseases, about 10 percent of people with carcinoid tumors -- which the Mayo Clinic says are slow growing tumors that produce excess serotonin and usually appear in the gastrointestinal tract or lungs -- get this syndrome. It occurs only in patients whose tumors have metastasized to the liver. The symptoms? Wheezing, hotness, and extreme facial blushing.
Cyclic Vomiting Syndrome(11 of21)
Open Image Modal
Children and adults afflicted with CVS experience recurrent episodes of severe vomiting, which can last for days, followed by sudden periods of no vomiting. While kids are likely to experience more frequent attacks, adults's often last longer. To date, the cause of CVS is unknown.
Peeling Skin Syndrome(12 of21)
Open Image Modal
This obscure, genetic skin disorder does exactly what its name implies: causes sufferers to experience constant shedding of their skin. (In some patients, peeling is limited to the feet and hands.) Along with that, sufferers often feel itching and redness -- symptoms that can appear from birth or develop later in life. Although the exact cause is unknown, a mutation in the TGM5 gene has been identified in many sufferers.
Platelet Storage Pool Deficiency(13 of21)
Open Image Modal
This is a blanket designation for several rare platelet abnormalities, most of which lead to mild or moderate bleeding disorders. According to the NIH, the problem stems having limited granules -- the parts of platelets that, among other things, store ADP -- the energy released when a molecular bond is broken. That reduction in storage space inhibits the platelets' ability to secrete ADP in a speedy fashion, which is thought to be what causes the bleeding. Classic symptoms of a Platelet Storage Pool Deficiency are nosebleeds, excessive bruising, and profuse bleeding in surgery.·
ACDC(14 of21)
Open Image Modal
Only nine people in the United States are known to have this disease, which was just given a name in a recent study in the New England Journal of Medicine. ACDC, or arterial calcification due to CD73 deficiency, results in calcium build-ups in the arteries below the waist of sufferers and in the joints of their hands and feet. According to the NIH, the breakthrough discovery found that the disease is related to a variant in the NT5E gene.3
Conversion Disorder(15 of21)
Open Image Modal
This condition, which typically occurs after an extremely stressful emotional event, causes a person to experience sudden blindness or paralysis that can't be otherwise explained. People with mental illlnesses are at particularly high risk and psychological treatment can help lessen the symptoms.·
Ochronosis(16 of21)
Open Image Modal
Ochronosis results in black or blue external tissues, often the ear cartilage or eye, though it can occur throughout the body. It affects people who suffer from certain metabolic disorders, but it can also be caused by exposure, though scientists aren't exactly sure to what. Not just a cosmetic issue, the affected areas can become brittle and degenerate over time.
VLCAD Deficiency(17 of21)
Open Image Modal
This condition, which is caused by genetic mutations in the so-called ACADVL gene, keeps people from converting certain fats to energy. According to the NIH, the deficiency often presents during infancy in symptoms including low blood sugar, weakness, and lethargy.
Hairy Tongue(18 of21)
Open Image Modal
As the name suggests, Hairy Tongue is a condition in which the tongue develops a black, hairy texture. According to the Mayo Clinic, the disease is "harmless" and is caused by an overgrowth of bacteria in the mouth, which can be treated with antibiotics. The NIH reports that hairy Tongue can also present as brown, yellow, or green discoloration. ·
Ochoa (Urofacial) Syndrome, or, Peculiar Facial Expression(19 of21)
Open Image Modal
This obscure, inherited disorder presents at birth and causes infants to grimace when, in fact, they are attempting to smile. The disorder also includes an extreme urinary abnormality: an obstruction that interrupts the connection between nerve signals in the spinal cord and bladder, leading to incontinence. Though treatment does exist in the form of antibiotics and bladder re-education, some patients can develop renal failure in their teens and 20's, which can be life-threatening.
Eagle Syndrome(20 of21)
Open Image Modal
This syndrome, caused by a particular calcified ligament or elongated bone, frequently causes sufferers to feel that something is stuck in their throat. (Scientists don't know the reason behind said elongation/calcification.) Other symptoms can include ear and throat pain as well as difficulty swallowing. According to the NIH, it can be treated surgically or non-surgically with steroid injections and other anti-inflammatories.
Progeria(21 of21)
Open Image Modal
According to the Mayo Clinic, only around 130 cases of this devastating genetic disorder have been documented since it was discovered in 1886. Affected babies normally appear normal at birth, but within 12 months begin to have symptoms like hair loss and wrinkles. According to the Mayo Clinic, progeria is caused by a genetic mutation, but not one that's passed down through families; it's a chance event that affects only one egg or sperm. The average life expectancy for sufferers is 13.