The Curious Case of Ashley X

In October 2006, Gunther and Diekema [1] published the article “Attenuating growth in children with profound developmental disability” in the Archives of Pediatric and Adolescent Medicine, which set off a firestorm of controversy. The article hinged upon a case report of Ashley X, a young girl with a diagnosis of static encephalopathy, the etiology of which is unknown. She is dependent in all activities of daily living, immobile, nonverbal, and receives all of her nutrition via a feeding tube. She also is described as responding to others in her environment via smiles and vocalizations. Her developmental stage is described as that of a 3-month-old infant and is not likely to change per her physicians. When Ashley X was 6-1/2 years of age and beginning to show signs of early puberty, her parents took her to a pediatric endocrinologist. In the 6 months before the evaluation, she had advanced from the 50th to the 75th percentile for length and her parents “particularly feared that continued growth eventually would make it untenable for them to care for their daughter at home, despite their strong desire to do so. … After extensive consultation between parents and physician, a plan was devised to attenuate growth by using high-dose estrogen” [1]. To avoid complications from high doses of estrogen (with growth of breasts and early onset of menstruation), they also had Ashley undergo a hysterectomy and breast bud removal. It is this combination of interventions that the parents later refer to in their blog (http://ashleytreatment.spaces.live.com/) as the “Ashley Treatment” [2]. In short, the primary goal of the treatment was to improve Ashley's quality of life by prematurely closing her growth plates and by keeping her small enough that her parents thought she could be cared for in the home by family. Secondary goals according to the parents' blog included the prevention of sexual abuse, prevention of discomfort from large or fibrocystic breasts, prevention of pain and hygiene issues with menstruation, prevention of skin breakdown, infections, ease in bathing, and ease in fitting with a wheelchair harness. The parents also note on their blog that “given Ashley's mental age a nine and a half year old body is more appropriate and dignified than a fully grown female body” [2]. On behalf of Ashley, Disability Rights Washington (formerly the Washington Protection and Advocacy System) investigated the case, in particular to discern whether there had been a violation of Ashley X's rights under state law. In the settlement agreement with Seattle Children's Hospital, they affirmed that the hospital had erred in not seeking a court order before performing the hysterectomy: “The Washington Supreme Court has held that a court order is required when parents seek to sterilize their minor or adult children with developmental disabilities” [3]. They furthermore recommended that before performing other aspects of the “Ashley Treatment” on anyone with a developmental disability—for example, surgical breast bud removal and hormone treatments—that independent court evaluation and sanction should be required. Finally, the court also recommended that the hospital ethics committee include a disability rights advocate and bring in other appropriate experts in developmental disability for future case deliberations [3]. If you are not familiar with the details of the Ashley X case, please do take some time to read the primary source materials footnoted in the aforementioned introduction. As always, the editors welcome your thoughts and responses to this column. In October 2006, a colleague and I published an article discussing the case of a young girl with profound developmental delay named Ashley, whose parents had requested medical intervention to attenuate her growth [1]. Shortly thereafter, the Washington Public Advocacy System (WPAS) responded by investigating the case. Their final agreement with Seattle Children's Hospital required that 2 conditions be met before any future growth-limiting medical intervention or sterilizing procedure in a person with a developmental disability: a court order and placement of a disability advocate on the ethics committee. Because nothing in the WPAS report suggests that the innovative nature of the interventions were a relevant factor, presumably WPAS would have responded similarly to any nonemergent intervention that caused significant irreversible alteration to the body of a child with developmental disability. For historical reasons, most states require court review of procedures being performed for the purpose of sterilization. However, beyond this single exception, there is no precedent to require a court order for interventions that alter the body of a child in invasive, irreversible, or innovative ways if there is reason to believe the child might benefit from the intervention. Many commonly accepted procedures, including gastrostomy tubes, tracheostomy tubes, osteotomies, and tendon releases are body altering, invasive, in some cases irreversible, and frequently performed to improve quality of life. To require court involvement for these and other invasive or irreversible procedures would overwhelm the courts and place judges in the position of making decisions that they are ill-equipped to make. Parents make complex, serious, life-altering treatment decisions on behalf of children every day. In general, state intervention has been restricted to cases in which a child is placed at substantial risk of serious harm by parents who refuse standard-of-care treatment recommended by the child's physician. Even decisions to withhold or withdraw life-sustaining interventions generally are made by parents and physicians without external review. In cases of disagreement, an institutional ethics committee provides sufficient oversight. A requirement for a court order reflects a cynical view of parents, undermines their authority to represent the interests of their children, and fails to respect their devotion and desire to provide a good life to their profoundly disabled children. It involves expense and burden that will preclude many families from obtaining interventions they believe will benefit their child. Although a requirement for court review works well as a nuisance strategy, it is not clear that it offers better protection than parental decisions and ethics committee oversight. WPAS also required that the ethics committee appoint “one or more individuals who can advocate for individuals with developmental disabilities.” Diverse perspectives are important to any ethics committee, and committees should comprise members who are thoughtful and open-minded. The perspective of a member with a disability or a parent who cares for a child with a disability would be an important addition to any ethics committee. Although diverse perspectives are important to ethics committees, I do not believe ethics committees should include members whose role is to advocate for specific groups. Most groups (eg, religious, cultural, political) encompass a wide range of beliefs and opinions, and no group “advocate” can truly know how to advocate for each individual identified as belonging to that group. An advocate, even one with a disability, can only speculate about what a child with profound developmental delay would want, and is likely to replace true knowledge with a pre-determined agenda or ideology. What kinds of protections do I think are necessary before performing innovative, irreversible, and body-altering interventions on a child with profound developmental disability? First, the presence of profound and permanent developmental delay was an essential requirement for the 3 procedures that Ashley underwent. Although children with lesser degrees of disability might experience benefits from the same interventions, the potential for harm also increases. A child who might someday value height or develop the capacity to participate intentionally and consensually in a decision to have a child should not be considered for a procedure that might later cause regret. Second, prognosis should approach near certainty, with agreement on multiple evaluations by different providers with expertise in evaluating developmentally disabled children. Third, any novel intervention with the potential to permanently alter a child with developmental disabilities should undergo review by an institutional ethics committee whose membership includes someone with a disability perspective. Fourth, informed consent should be robust and thorough, including a fair and balanced presentation of risks and harms that might be experienced by the child as he or she ages, an honest rendering of the inherent uncertainties involved, a discussion of reversible or less-invasive alternatives, and a fair presentation of the differing perspectives in the disabilities community regarding the interventions being proposed. Finally, novel or innovative interventions should include a careful review of outcomes. Ideally, this would occur through a formal clinical trial or national registry, but should at least involve careful follow-up of individual patients over time with reporting of late adverse events to others who may be considering or performing similar interventions. There is an old and very familiar adage about not judging another person until you have walked a mile in their shoes. We do not purport to have walked a mile in the shoes of Ashley's parents; we have not faced their particular set of challenges, and we therefore compose this essay not in the spirit of judgment or condemnation, but in the spirit of observation and with the goal of furthering dialog about this most difficult issue. Caring for a person with a severe disability can be a challenging task; whether that individual is an aged parent or grandparent who has survived a stroke or is living with advanced Alzheimer's disease, whether that individual is a loved one who has survived a traumatic brain injury in the context of war, or whether that individual is a child, like Ashley, born with “static encephalopathy of unknown etiology,” or an insult of unknown origin to the brain. Caregiving under such circumstances is a task that requires and calls upon, to use a phrase coined by Lincoln and recently re-used by our new President Obama, all of the “angels of our better nature”: patience, courage, dedication, and sacrifice. To undertake such a task is reflective of the kind of heroism exhibited every day by ordinary people around the country and around the world. Certainly, we wish upon such heroes all of the tools necessary to assist them in this labor of love and commitment, and we wish to see their unique, perceived burden eased. But, to what extent does the caregiver have a right to physically—or mentally—alter an individual with a severe disability, for the sake of that ease? This is the question that is raised by the Ashley case, and strongly denied by the parents in their blog (http://ashleytreatment.spaces.live.com/). In recent months, the new United Nations Convention on the Rights of Persons of Disabilities entered into force. The Convention, which is the first legally binding international treaty to exclusively address the issue of disability, squarely recognizes disability as a human rights issue. Among other things, the Convention seeks to address attitudes toward persons with disabilities and reflects and articulates an understanding of the basic regard and human dignity to which all people are entitled, regardless of their station in life, their economic stature, their cultural background, or their status as a person with a disability or with a profound or severe disability. The Ashley case stirs our passions—our instinctive moral discomfort—because it represents an assault upon that basic human the of Ashley's through invasive, surgical as a violation of the of her to which she is regardless of her disability or mental There are who would that is not a difficult does one the difficult between and for a caregiver and rights of the care no how how or how We that this can and be at very at the of the violation of the human or as which were an of of mental were by and to be an of with difficult is the alteration of a human being an of with the that the individual any child, including a child with a disability, is a and a and will upon our of courage, patience, and We will be We will make we will and in of these for they are difficult and to in should these there including or Although these may be difficult and by their set of we that in no case should the of a child or an adult be considered the for with disability. As we is by the new Convention, the time for such treatment of people with and for the upon basic human rights for the sake of is I have care of many children like Ashley and have many parents of such children to what is they are to whether to withhold life-sustaining other the decision is about a gastrostomy or or Many such parents are by their child's by by care and by their to make their child's life However, I have had a parent for a hysterectomy in a child or for breast bud removal. 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In her example, a medical was to off challenges, as it has in other cases [1]. the from Ashley's was for example, by the of her parents with of care is a issue in and for children with an well in or is not from state and to for with disabilities requires a of from to can be and In the of disability and of care is even more as adult as an for In the is at every and at The is When the first were in at the beginning of the their goal was to children from the of on hospital how to with the care of children with disabilities they beyond the of the was a had many of the of children, in of their on developmental these and a of a toward individual and human rights the in the United and The Disability Rights a place in that the between and of the has a in a recent in on the like and others have and to the As has disabilities the very of our about the [2]. I have been in a fair of and to the Ashley case it was first made have from the to the on the For the issues raised by Ashley X are not and as some The parents' love for their daughter is not in question for Ashley's and also care about the and cultural for her is the of we a in which she can be cared for and On the one when her parents can no care for her at home, are there and or in which she can that the parents not for her of the and risk of in her On the other as the of all children with disabilities should be from care by to of the more home have we made and to them at even as the committee an does such a position not have significant in to Ashley's For the individual case of Ashley and her is an individual case. 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