Parents of children with severe developmental disabilities often face a difficult choice: struggle to care for their growing child at home with limited support or give up their child to the care of others. Now an unprecedented move by parents of one child in the Pacific Northwest has disability advocates debating how far parents should go to keep their child at home.
Faced with the prospect that their daughter - who cannot walk, talk or feed herself - would physically outgrow their capacity to care for her at home, a Seattle couple has taken the extreme measure of reining in her growth.
Doctors at Children's Hospital and Regional Medical Center in Seattle described in a medical journal in October how they gave high doses of estrogen to a 7-year-old girl, effectively stunting her growth, and performed a hysterectomy to pre-empt the expected side effects of the treatment.
While the moves have shocked many disability advocates, parents in the same untenable position have been much more reluctant to judge.
In Bend, Chablis Dafoe, a 30-year-old single mother caring for a daughter with profound disabilities, says she understands the Seattle parents' concerns. She worries that she might one day have to give up her own daughter, Melissa, if she grows too big.
”That's one of my biggest fears,” Dafoe says. ”If she's smaller, I could keep her at home longer.”
Such families are desperate for new options, but many fear what Pandora's box may have been opened by the decision to keep a child from growing.
”To me, this was not much of a moral dilemma. I believe that this is in her interest,” says Dr. Daniel Gunther, the Seattle pediatric endocrinologist who devised the strategy and treated the child. ”I know some people will respond viscerally, but most people, when they consider the pros and cons, the costs and benefits, will conclude that this was a positive thing to do.”
The parents, who declined to be identified or to speak with The Bulletin, came to see Gunther in 2004 when their daughter began showing signs of puberty at age 6, a common occurrence among children with developmental disabilities. Their daughter has been developmentally delayed since her first month of life, and doctors were never able to uncover the cause.
She cannot sit up and must be fed through a gastric tube. She responds to others with infantile sounds, and smiles in response to care and affection. She never developed beyond the cognitive level of a toddler, but her body continued to grow. It was her sudden rapid growth that had her parents, both of tall stature, concerned. She had progressed on growth charts from the 50th percentile for height to the 75th percentile in only six months.
The difficulty of caring for such children - dressing, bathing, diapering, transferring to and from a wheelchair - increases exponentially as they grow to adolescence and adulthood. While parents often prefer to continue caring for their child at home, they eventually find themselves physically unable to do so.
Gunther suggested treatment with high doses of estrogen, which both arrests growth and rapidly advances maturation of the growth plates in the bones, bringing about a permanent end to growth in a relatively short period of time.
”They were immediately struck with the idea,” he says. ”It was a novel proposition. To the best of my knowledge, this had not been done before. We couldn't know with complete assurance what exactly the risk would be or what the result would be in terms of the decrease in final height that we could achieve.”
In the 1950s and 1960s, it was common practice to use high-dose estrogen in teenage girls who were perceived as outgrowing their reasonable chance of attracting a husband. The practice, although infrequent, continues to this day. Nearly a quarter of pediatric endocrinologists surveyed in 1999 reported having treated at least one tall girl with high-dose estrogen within the previous five years.
In those cases, girls were already well past puberty and had already completed most of their growth. Still, the estrogen treatment reduced their final height by one to four inches. And studies showed that the earlier the girls started the treatment, the greater the result.
So it remained to be seen what the result would be for a younger child, such as the girl in Seattle, and what risks might accompany the treatment. Doctors feared the procedure could result in a cascade of other effects, including uterine bleeding and blood clots.
To avoid the risk of uterine bleeding, they suggested the girl also undergo a hysterectomy. That would eliminate the complications of menses altogether and the inconvenience of a lifetime of hormone injections. But that also framed the treatment in the context of forced sterilization of people with mental retardation during the late 19th and early 20th centuries.
The doctors decided the treatment protocol would have to be approved by the hospital's ethics committee. After meeting the parents and their daughter, the committee had to decide two distinct issues: Was it ethical to attenuate the child's growth, and was it acceptable to perform the hysterectomy?
Years ago, involuntarily sterilization was justified with the belief that people with mental retardation were genetically unfit and would pass their ”defects” on to future generations.
”Those were being done because it was perceived to be better for society in some way,” says Dr. Douglas Diekema, director of education at the Treuman Katz Center for Pediatric Bioethics, and one of the doctors involved in the decision. ”That wasn't a consideration here. It was really focused on this little 7-year-old girl who was sitting in that room listening to her father talk. Every decision that was made was made with her in the lens. What is going to make this little girl's life as good as it can possibly be?”
In this case, sterilization was not the intent, only a side effect. Had it been a boy, sterilization would not have entered the discussion, Diekema says.
Menstruation is problematic for many girls with developmental disabilities, who often are frightened by bleeding of any sort. Many are given medications to avoid menses altogether.
”The parents made the argument that in addition to being easier to care for her, she's not having monthly periods,” Die-kema says. ”A uterus is not an advantage to her. Their case was it's only a disadvantage, and it's even more of a disadvantage in light of what we wanted to do in atten- uating her growth.”
The committee also had to consider whe-ther the girl would be harmed by being deliberately kept small. Whether it should or not, height does hold significant value in our society, Diekema says. Tall people earn higher salaries, are more likely to attract romantic partners, and more likely to achieve status in society.
”Not one of those sociologic advantages of height would accrue to this little girl, who was unable to walk, was unable to relate at more than a 2-year-old level, would never hold down a job, would never live independently, would never have a romance,” Diekema says. ”It was just hard to imagine how height would be of any advantage to her.”
Meanwhile, caring for a smaller child would be less burdensome. She would be easier to move, leading to more physical contact, more stimulation and fewer medical complications. The child would be more likely to be included in family activities and outings. ”She stays a family member,” Diekema says.
That connection was visible in the room during the committee's deliberation, and carried significant weight, Diekema says.
”There were several times when she was becoming a little agitated, and you could see that she was not entirely happy,” Diekema recalls. ”And then her dad would say something to the committee. She would hear his voice, and she would immediately stop. You could see her whole affect change. She would focus on him and his voice, and it was very clear that there was a really powerful relationship there between her and her parents.”
The committee decided to allow the parents to decide their child's fate. She began her treatment in August 2004.
Gunther had originally projected that the girl would quickly grow to a height of about 5 foot 5 inches and a weight of about 130 pounds. After two years of treatment, she has nearly completed her growth spurt, and at her last visit, she was 4 foot 2 inches tall, weighing about 65 pounds. Gunther expects she may grow up to another half-inch.
”There's no reason to believe she can't live a full life span,” he says.
As news of the procedure filtered out, reactions were mixed. Diekema heard from other physicians who backed the ethics of the decision. Others were less supportive.
”The idea of involuntarily interfering with the person's growth or the person's biological function for the convenience of others is something that is, at least in my mind, ethically unacceptable,” says Robert Joondeph, executive director of the Oregon Protection and Advocacy Agen-cy, a nonprofit that advocates for the disabled. ”One wonders how far that can go.”
Joondeph argues that while helping the child stay with caring parents is a laudable goal, disability advocates have been fighting for more support to help parents of children with disabilities.
”I don't think, to a disability advocate, that is a way to treat a person with dignity and to honor their identity as a human being,” he says. ”The idea that they could be surgically, genetically, artificially modified for the convenience of others would be anathema.”
In an editorial accompanying the Seattle case study article, Dr. Jeffrey Brosco, from the University of Miami Department of Pediatrics and Dr. Chris Feudener, from the Children's Hospital of Philadelphia, called the procedure ”ill-advised,” arguing that the doctors could not be sure of the outcomes.
However, they also noted that while most people will initially recoil at the notion of artificially modifying height, adults and even teenagers can easily have breast implants or nose jobs for much less noble purposes.
”More distressing is how this solution fails to situate the plight of these parents, struggling to care for their children in the larger context of a society failure to provide adequate social support in this most admirable of undertakings,” they wrote.
The concerns of the parents in Seattle are being replayed in the homes of parents of children with developmental disabilities all over the country. Parents find themselves in a hopeless battle against time, hoping their children - who are totally dependent on them for care - won't grow too big for them to handle.
In Bend, Dafoe has already had two shoulder surgeries, the impact of lifting her now 94-pound daughter. Melissa, 11, suffered an injury as an infant, resulting in epilepsy and serious development disabilities. She is legally blind, incontinent and must eat through a feeding tube.
”It's basically like having an infant,” Dafoe says. ”She can't do anything on her own.”
Melissa also hit puberty early, and Dafoe had to make a tough call on whether to give her daughter medication that would delay puberty and possibly slow her growth.
”It may sound bad, but it does help with being able to take care of them longer,” Dafoe says. ”Because if she's not as big, it's easier, especially myself as a single parent, to have the ability to lift her and do all those things.”
Dafoe ultimately opted against it, unwilling to give her child even more medication than she already has to take. ”She didn't have to have that.”
Dafoe gets some assistance from public programs providing money to hire additional help, but often she can't find anyone qualified and willing to do the work for the wages offered.
The community has rallied around her, helping her get a Habitat for Humanity home specially designed for Melissa. The bathroom has a raised tub to make bath time easier and a mechanical lift to help get Melissa from her bed to her wheelchair. She goes to school every day, working with special-education teachers, allowing Dafoe to hold a part-time job.
The return on that investment is the exposure it gives to the community. Dafoe takes Melissa everywhere: the bowling alley, movies, restaurants, even camping. At the grocery store, children from her school will run up to say ”Hi!” and gleefully proclaim to all who will listen, ”She goes to our school!””One of the greatest things that Melissa has achieved throughout her life and will continue to is the knowledge that she gives other kids and even some adults being around her,” Dafoe says. ”They learn tolerance, that not everybody is the same, but that they're still OK.”
Without that community support and public funding, it's likely Melissa would be in a medical foster care home, possibly not even in Central Oregon. When parents can no longer provide care to such children, their last resort is to rely on the state's developmental disability crisis system, says Kathy Drew, manager of the developmental disabilities program at the Deschutes County Health Department.
”When you get into the crisis system, you lose a lot of your choices,” she says. ”We do our very best there to meet individual needs and meet family needs, but a lot of times, we don't have any choices. We have to take the first available slot and it might be in Astoria.”
While public health officials and disability advocates have worked to provide support for parents to continue to care for their children at home, even the limited dollars that are available often come with strict eligibility guidelines. It may be the one place in America where those financially disadvantaged are better off.
Another Bend girl with severe developmental disabilities, Andrea Zifka, 15, never qualified for Medicaid assistance while she was home because of her family's income. They received about $400 a month from Family Support Services, one of the few programs that looks only at medical need for eligibility. That paid for some help to get Andrea ready to go to school each day. But after 13 years, the emotional and physical toll of providing round-the-clock care for their daughter finally broke her parents.
”We became one of those families that really had no choice,” her mother, Judy Zifka, says. ”Because we weren't going to get any help if we kept her at home.”
Andrea now resides at the Center for Medically Fragile Kids, a pediatric nursing facility at Providence Child Center in Portland. Her mother shuttles back and forth from Bend to Portland three hours each way, splitting time between her two children. It is precisely the worst-case scenario the Seattle parents hope to avoid.
”My heart broke for those parents even having to consider the decision,” Zifka says. ”But it didn't strike me as an odd decision.”
She admits that she hoped Andrea would not grow too quickly, either.
”We don't want her to gain weight. When she's big, she's harder for the caregivers,” she says. ”It's a blessing for everybody when she stays small.”
Had the Zifkas received some additional help, Andrea might still be living at home. Instead, the state is paying more than $10,000 a month for her to stay in the nursing facility.
Other states have secured a waiver to federal Medicaid eligibility rules to enroll children whose parents' income excludes them. But no such allowances exist in Oregon as yet.
”Many of these families face some pretty stark choices: place their child in a nursing home, relinquish custody to foster care, or artificially impoverish themselves so that they can maintain Medicaid eligibility,” says state Rep. Sara Gelser, D-Corvallis. ”We have a system that is biased toward services only when a family gets to crisis and providing services out of home.”
Gelser is working on several legislative measures that would change the financial incentives, making it easier for parents to get the help they need to keep their kids at home.
”If we're giving families the tools to choose to keep their kids at home, not only is that great for kids and families, it's also an economically viable option,” she says. ”The state currently spends about $13 million a year for nursing home services for 60 kids. We could serve about 125 kids on this waiver for about $9 million per year.”
Not all children will be able to come home from nursing homes, but proponents of expanding at-home services argue that already some parents have been given the help to care for kids at home with more complex needs than those being cared for in nursing facilities.
Developmental disability advocates admit a massive restructuring of the support services for adults with developmental disabilities has monopolized limited state and nonprofit resources, pulling attention away from children with similar needs.
”We recognize that the whole system of services to kids has kind of fallen on hard times, that we as advocates have not been as vigilant in making sure those services are adequate,” says Bill Lynch, executive director of the Oregon Council on Developmental Disabilities. ”Not to make excuses, but there's only so much you can do both as advocates and bureaucrats in implementing all this stuff.”
Last year, the Legislature directed the Department of Human Services to address shortcomings in at-home support services for kids with disabilities. Officials and advocates held a series of listening sessions around the state to gauge what services and supports would benefit children with disabilities and their families. The work group is now drafting recommendations for changes to the system.
Those types of changes will give parents additional options, but it's not clear whether the parents in Seattle would have opted for a different path. The father told the hospital's ethics committee he knew he could resort to using a mechanical lift to move his daughter. He wanted to be able to lift his daughter with his own two arms.
Gunther says that two other families had also come forward hoping for the same procedure, but as the initial case unfolded, one of those families moved from the region and another family simply gave up.
The doctors maintain that every new request will have to be dealt with individually to protect against harming children who might be able to benefit by growing to full height.
”This is not something we are trying to push on everyone,” Gunther says. ”The decision is up to the parents, but we think it's an option that should be available to them. It shouldn't be foreclosed by people with different ethical or philosophical views. They don't know the hardships and terrible dilemmas that these parents face.”