Growth attenuation: health outcomes and social services.
In examining the working group's argument, it is important to note the particular way it has constructed the issue. The group focused on growth attenuation rather than on the broader "Ashley Treatment," which also included a hysterectomy and breast bud removal. This choice brackets the potentially more inflammatory aspects of the original case. The working group also asserts, "The primary benefits sought by short stature resulting from growth attenuation relate to facilitating increased involvement in a family's social and recreational activities that relate to mobility." This contrasts with Daniel Gunther and Douglas Diekema's original claim that the intervention would permit families to care for these children at home for a longer period of time, and Ashley's parents' contention that it would provide greater physical comfort and an appearance more congruent with her development. It is not clear whether the working group believes that the reason they offer is the most defensible or the most common among families seeking growth attenuation.
The claim that growth attenuation will increase involvement in social and recreational activities is unproven. (The alternative justification that growth attenuation and decreased size will delay out-of-home placement is not supported by the literature on parental reasons for placement.) As the working group acknowledges, because many children with profound disabilities have conditions that limit their growth, the incremental effect of administering high-dose estrogen is unclear. The critique of the biomedical model of disability emphasizes that disablement does not solely inhere in the individual's body; it is also constructed by the person's relationship with his or her physical and social environment, including social attitudes. Even if growth attenuation produces a statistically significant decrease in length or weight, this will not necessarily result in increased involvement in activities. Characterizing the disability critique as a claim about growth attenuation's effect on third parties misses this important point.
While the authors correctly state that "support for growth attenuation and support for improved social service funding are not clearly at odds, nor are the two mutually exclusive," a fundamental issue is where one devotes one's attention. The working group, for example, reiterates the call to establish a registry of patients undergoing growth attenuation. Such research is essential to test the hypothesis that "treatment" with high-dose estrogen increases participation in family activities. In spite of calls for research since 2006, http://www.clinicaltrials.gov does not list any growth attenuation trials. I am not sanguine that further prolonging this ethical debate will contribute to the broader goal of increasing services and resources for individuals with profound disabilities and their families.
Armand H. Matheny Antommaria
University of Utah
School of Medicine
To the Editor: Arguments about the ethics of attempted growth attenuation interventions--administering high-dose estrogen to children with profound disabilities who have not yet entered puberty--are about many things, but one aspect that needs to be underscored is that they are about different conjectures regarding the potential benefits or harms of this intervention.
The purported benefits of growth attenuation intervention are predicated on the following chain of events unfolding: high-dose estrogen causes bones to reach their final length quickly, shortening their final length and thus causing children so treated to be of shorter stature. This, in turn, will supposedly make them easier to carry and care for, enable them to participate in more activities and to remain in the home setting for longer, enhance the quality and duration of their life, and thereby justify the intervention.
What is the probability of this chain of events unfolding just like this? Is the expectation realistic, or rather speculative benefit bravado? Is the alternative position, which sees no conceivable possibility of benefit, more than haranguing?
In the history of medical care, several interventions have been proposed because they offer "clear benefit"--and only later was it discovered that instead of benefits, the interventions caused harm (recall sodium channel blockers used to "prevent" cardiac arrhythmia after heart attacks, or hormone replacement therapy for menopausal women). Conversely, other interventions have been roundly criticized as being ethically inappropriate until improvements in the intervention or better data demonstrated a compelling benefit (consider cardiac transplantation for heart failure, or temporal lobectomy for otherwise intractable seizures).
Shorter individuals may weigh less and be more mobile. Or they could weigh the same and be harder to lift safely or position comfortably than if they were longer. Shorter stature might ease the development of contracture deformities, or it might worsen them. And who knows what this intervention will do to bone density and how it will affect the risk of osteoporosis and pathologic fractures? Early fusion of vertebral body growth plates could help prevent progressive scoliosis of the spine and offer substantial physical benefits, preventing respiratory compromise and obviating the need for spinal fusion surgery. Or the growth attenuation could result in a shorter, scoliosis-deformed thorax, making matters worse. Likewise for the abdomen and feeding tolerance or difficulties, and for the challenges of providing total care at home, which may or may not be lessened, and the time lived at home, which may or may not be lengthened. The list of what we don't know about the pros and cons of this intervention goes on.
In this argument, earnest ardor to protect children with profound disabilities has diverged into opposing directions and hardened into adamancy. These stances are premature at best and pernicious at worst. One of the first duties of philosophy and ethics is to point out what we presume but don't actually know. To ballyhoo the benefit of an unproven intervention is unbefitting of serious ethical debate, and so, too, is to deny out of hand the possibility of such benefits. Indeed, if any aspect of this debate highlights the vulnerable position of these children in our society, it is this subtle but substantial disregard for whether this intervention will really work as advertised.
The Children's Hospital of Philadelphia
To the Editor: The twenty-person Seattle Growth Attenuation and Ethics Working Group set out to accomplish an impossible though admirable goal--"a moral compromise rather than consensus" concerning growth attenuation. This long-awaited paper surely rekindles a debate that has raged since growth attenuation was first reported by Gunther and Diekema in 2006. Prior to the working group's effort, scholarship fit squarely into one of two entrenched camps--those for growth attenuation, and those against it. Thus, it is no surprise some members of the working group are "dissatisfied, even distressed, by the paper produced." The entire debate surrounding growth attenuation has been unusually heated, and I have contributed to that heat ("Protest From a Bad Cripple: The Ashley Treatment and the Making of a Pillow Angel," Counter Punch, January 18, 2007; "Ashley and Me," Bioethics Forum, June 22, 2010).
Let me be very clear: I was and remain staunchly opposed to growth attenuation. I disagree with the premise of the working group--specifically, that growth attenuation is a viable course of treatment. However, this does not mean growth attenuation should never be performed. In some exceedingly rare cases, growth attenuation may be medically necessary. But growth attenuation is an extreme treatment and must be subject to thorough review. At minimum, that review would entail internal and external review, as well as independent legal representation of the child (guardian ad litem) whose growth is attenuated. I am by no means a proponent of court intervention, but given the extreme and irreversible nature of growth attenuation, extreme measures of protection must be put in place. In short, an odious burden must be used to justify this treatment.
Since I first read about growth attenuation, as a person with a disability, I have gone from stunned and angry to deeply concerned. I continually ask myself how loving parents and doctors could implement such a radical intervention. The answer is as simple as it is complex. As the working group states, families "need greater social support. To date, there has been insufficient public discussion about how to provide that support and improve the lives of people with profound disabilities. Further, the issues facing this population have not been the primary focus for many health care professionals." This is a discussion we continue to avoid, in spite of the fact that Americans have enacted legislation designed to empower people with disabilities for the last forty years. As a nation, we have also become less reliant on institutional care. Despite solid progress for people with disabilities, they still struggle daily against social isolation and despair. The answer to this disenfranchisement will not be found in attenuating the growth of children with disabilities.
A deep chasm remains between those with and those without a disability. The divisive literature arguing for and against growth attenuation merely highlights this. Thus, I commend the working group for making me realize the us-versus-them mentality that has dominated the discussion surrounding growth attenuation is a false dichotomy. We do have common ground. I am sure the working group would agree that what is desperately needed is not radical medical intervention, but rather social and practical supports that make growth attenuation untenable.
William J. Peace
Katonah, New York
To the Editor: As a parent of a child with profound physical and cognitive impairments, and one who is opposed to growth attenuation, I cannot hide my deep disappointment at the Hasting Center Reports article on growth attenuation. Though the article asserts its concern that "many people and institutions in society do not positively value people with profound developmental disabilities," by supporting growth attenuation, it perpetuates the continued devaluation of these people. Our severely disabled children are marginalized on the basis of their cognitive abilities only, treated "for their own good" by a society that arrogantly asserts it has a scientifically solid and unbiased understanding of what constitutes "awareness." "In trying to understand how people with profound intellectual disabilities experience their world, we sometimes extrapolate from our own lives," the group writes, and this is precisely the problem.
The working group's enthusiastic support of choice in parental decision-making begs the question, choice between what? Growth attenuation and lack of support? "We agree that investing in improvements in medical and social services (medical equipment, human assistance, respite care, lift-equipped vans, and the like) is a priority," the group states. Certainly, the article makes it clear that "the like" is most certainly not a priority, as they are first and foremost looking for ways to make growth attenuation more readily available by attempting to provide guidelines for its use. Parents are left with little hope for improvements in societal attitude but are considered wholly supported with a perceived medical "solution" to their "problems." Though the supposed benefits of growth attenuation are touted, none have as yet been scientifically proven. How many parents are provided with this salient detail when they are faced with a supposed "choice"?
Finally, all talk of social benefits for the children ring hollow in this parent's ears. Parents who support growth attenuation where a child is deemed so compromised that he or she won't notice or care about his or her altered bodily appearance argue the exact opposite, saying "it promotes the child's dignity by helping her to flourish and to foster social connections in her particular familial context."
The truth about growth attenuation is very clear to me: it is a procedure that discriminates based on cognition and that supports society's view that care of the profoundly disabled is a burden that requires mitigation. It sanitizes for public consumption the realities that parents face daily in the care and socialization of their profoundly disabled children. It provides a "solution" to woeful societal attitudes by placing the onus of change on the disabled individual. It appears to give parents an easy, onetime "fix" to the "problems" of caring for their children, yet fails to empower them to create full and lasting supports that will benefit them and their children long after the parents cease to be able to be primary caregivers.
London, Ontario, Canada
To the Editor: I concur with Eva Kittay's disagreement with the group's "compromise" for exactly the reasons she articulates.
The glaring flaw in the Seattle Growth Attenuation and Ethics Working Group's recommendation is the disconnect between the indication for growth attenuation and the criterion for who is eligible for it. The indication for growth attenuation is extensive immobility, such that growing to typical adult size will prevent the patient from taking part in as wide a range of activities as would be possible if she stayed smaller and lighter. But the decision of the working group is not that growth attenuation should be offered to all children who experience extensive immobility, although all children who are immobile face the same restriction of activity as they grow larger and heavier. Rather, the working group decided that only those children who are immobile and profoundly intellectually impaired should be offered growth attenuation.
The working group claims that this decision does not discriminate against profoundly intellectually impaired children because "children with vastly different medical and social needs may require different options." But the difference between immobile children without intellectual impairment and immobile children with intellectual impairment is not the difference that growth attenuation treats. Intellectual impairment in and of itself is not an indication for growth attenuation, and growth attenuation does not remedy intellectual impairment. When the working group states that "If an individual may develop greater self-awareness and interactional ability, we would be less confident about proceeding with an irreversible alteration to the person's body, as he might later wish it had not been done," they are acknowledging that growth attenuation is a suspect procedure that they are only comfortable authorizing for children who supposedly will not know what has been done to them.
The wording of the decision reinforces the dangerous belief that we can ever be sure that someone is profoundly intellectually impaired and lacking self-awareness in the absence of access to a means of communication. As any undergraduate who takes a critical thinking course learns, absence of proof is not proof of absence. Anne McDonald (http://www.deal.org.au/rowingupstream-0) was an Australian woman who was physically similar to Ashley X and was believed to be profoundly intellectually impaired until the age of sixteen, when she gained access to communication through typing with support to stabilize her posture and arm position. Her intellectual competence was validated by the Supreme Court of Victoria in 1979.
I provide similar support to people without speech. One is a man in his thirties who only just started working with me. His body is drawn up tight from cerebral palsy, and he has used a wheelchair and a feeding tube all his life. But with appropriate physical support and an iPad that requires only a light touch, he is able to communicate and demonstrate his intellectual competence. Another, a young man of thirteen with cerebral palsy who began communicating two years ago, previously scored below twenty-five on IQ tests because he cannot talk and could not point reliably without support. He was recently retested and scored over 130. The people I support had access to years of speech and language therapy. But what was tried with them was not meeting their needs. It is erroneous to assume, at any point, that professionals have done everything possible.
If growth attenuation is a good choice for immobile people, then immobile young people who are self-aware and able to communicate would choose it for themselves. They would see that the benefit of accessing a wider range of activities is worth a slight decrease in their stature. That the working group presumes that self-aware, immobile young people would either not make this choice or might regret making it is all we need in order to understand that growth attenuation is not acceptable for any immobile child.
Benjamin S. Wilfond replies:
These five letters to the editor focus on three themes: the lack of evidence of benefit, the potential for distraction from addressing the broader social issues facing people with disabilities, and the problem of singling out children with profound cognitive disabilities.
Armand Antommaria notes that the working group's justification for growth attenuation differed from that in other papers. Our group found the promotion of family interactions to be the most compelling--but not the only--justification. I agree with him and with Chris Feudtner that the impact of estrogen on height and the impact of height (and weight) on social interactions have not been clearly established, and that further research is necessary. But I am willing to consider parents' requests to promote short stature in some circumstances since reasonable clinical decisions are often made without sufficient data.
What resonates most in these letters is the suggestion that perhaps we are focusing too much on growth attenuation. William Peace and Claire Roy pick up this theme; they are concerned that growth attenuation takes the place of adequate social services. Promoting services is vital, but I am less concerned that growth attenuation is likely to have adverse affects. An underlying critique from these letters is whether our academic analysis and advocacy should focus on something as mundane as growth attenuation when people with disabilities face greater problems.
I had multiple motivations in working on this project. I wanted the opportunity to inform the public on this issue, to develop relationships and collaborations with my new colleagues in Seattle, and to engage other scholars whom I respected in a complex debate. Looking back, the personal impact was much deeper than I could have imagined. I gained a new colleague and friend in Paul Miller, the coleader of the project, who passed away in October of 2010. His guidance and wisdom shaped every step. If our group had little consensus, we do all agree on how much we miss Paul. I learned from the working group about the range of styles in negotiating disagreements and the intensity of commitment to process. As the essays that accompanied the paper illustrated, no one member agreed with the entire paper, and some still hold views similar to the ones expressed in these letters. In particular, the parents of children with profound disabilities and others with long histories of disability advocacy in the working group taught me how experience can make the same issues look so different that they can come to disparate views with equal sincerity.
I also learned from Ashley's family. I met Ashley's father while working on this project. We both enjoy bicycling. As a relative newcomer to Seattle, I did not know the roads, so an offer to join him and his friends was welcome. Although our routes varied, we almost always came to a spot on a low bridge across a river that afforded a view of the mountains and the birds flying overhead. He would insist that we stop to appreciate the setting. I imagine that Ashley's parents also pause every day to appreciate Ashley and what she brings to their lives. At the end of these rides, I would see Ashley at home. I saw that she was more than the recipient of a controversial intervention; she was simply part of the family.
I am confident that Ashley's family, our working group members, and the authors of these letters share a commitment to people with profound cognitive disabilities. I appreciate Anna Stubblefield's thoughtful letter, which highlights a distinction between the use of estrogen for short stature and its primary application to children with profound cognitive disability. She points out that our conclusion to limit growth attenuation to profoundly disabled children because of worry that self-aware individuals might not choose this option could be problematic. We did not mean to imply that self-aware individuals would not desire growth attenuation, only that there may be fewer concerns for individuals who lack self-awareness. In fact, we do not know how self-aware immobile children, such as those with spinal muscular atrophy, would respond if their parents had made clinical decisions to promote short stature.
Stubblefield also reminds us that people with severe athetoid cerebral palsy were mistakenly labeled as having profound intellectual impairment. Anne McDonald's case occurred over thirty years ago, and it is unlikely she would appear "physically similar" to Ashley if evaluated today by the physicians caring for Ashley. But the concern lingers: children who have profound intellectual impairments may be more self-aware than we appreciate, and pursuing growth attenuation might distress them. It is impossible to fully appreciate the thoughts of those have profound cognitive impairments, but families and providers still have to make decisions about what they believe is best for them.
In our paper, we disaggregated the issue of self-awareness by considering children with conditions such as Leigh's disease, Tay-Sachs disease, and Trisomy 13. Parents of these children are generally supported in deciding to forego life-prolonging or sustaining interventions such as tracheostomy and gastrostomy tubes. We suggested that in these conditions, where meaningful self-awareness is not questioned, surely growth attenuation is less problematic. But a more serious ethical issue related to these conditions dwarfs growth attenuation. Many clinicians refuse to provide life-prolonging or sustaining care when parents request it for children with "lethal" conditions that include profound disabilities and a shortened life span. I hope that all of the authors of these letters will join me, our working group members, and many others to support these parents.
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|Author:||Antommaria, Armand H. Matheny; Feudtner, Chris; Peace, William J.; Roy, Claire; Stubblefield, Anna|
|Publication:||The Hastings Center Report|
|Article Type:||Letter to the editor|
|Date:||Sep 1, 2011|
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