On January 3 of this year the parents of a girl with static encephalopathy, a disorder that leaves her unable to move and with the cognitive capacity of an infant, announced on a blog that they had been using hormones to stunt the growth of their daughter for medical and quality-of-life reasons. [More details are available via the original news report of the story.] The resulting, and very public, debate--much of it carried out in the comment thread of the original blog--has ranged from support for the parents to accusations of eugenics and worse.
In order to cut through the noise, we asked three bioethicists--doctors not unlike those who, as members of a medical ethics board, authorized the treatment in the first place--to relate their professional opinion of the case.
All three bioethicists came down firmly on the side of the parents and the decision of the original ethics board--but with a few reservations. Their discussion ranged from issues of privacy raised by the media frenzy surrounding this case to the question of whether or not this intervention is a technological fix for a social problem.
What follows is an unedited transcript of an e-mail dialogue that took place Friday, January 5, between the following participants:
Joel E. Frader, MD, is division head of general academic pediatrics at the Children's Memorial Hospital, Chicago, as well as a professor of pediatrics/medical humanities and bioethics at Northwestern University's Feinberg School of Medicine.
Norman Fost, MD, MPH, is a professor of pediatrics and bioethics as well as director of the program in bioethics and vice chair of the Department of Medical History and Bioethics at the University of Wisconsin Medical School.
Benjamin Wilfond, MD, is director of the Treuman Katz Center for Pediatric Bioethics at Seattle Children's Hospital and a professor at and chief of the Division of Bioethics, Department of Pediatrics, University of Wisconsin Medicine.
Joel Frader, 9:03 AM EST January 5, 2007Drs. Gunther and Diekema in Seattle reasonably defend the decision to support, and act on, the parents' request to limit growth of the six and a half year old girl with severe cognitive impairment by administering drugs to close the growth plates of her bones. (cf. Attenuating Growth in Children With Profound Developmental Disability Gunther DF, Diekema DS.)
Parents and other care providers for entirely dependent patients with such serious disabilities do face physical challenges when the patients grow large. One encounters difficulties lifting and turning the patients for routine care--including bathing, prevention of skin breakdown, exercises to preserve joint mobility, and so on. I find it difficult to argue that this child and similar patients benefit merely from reaching their genetic growth potential.
In addition, reducing the demands and stress on caregivers serves the interests of the patients. I have more difficulty with measures such as hysterectomy--an intervention with more risk and more discomfort to the patient--in order to avoid having to deal with monthly periods once, or if, menstrual cycles begin. Absent excessive bleeding uncontrollable by medical means, once menstruation begins, hysterectomy seems hard to justify. Also, some family members and health care professionals seem to believe that hysterectomy limits the potential for sexual abuse by others, though one can find no empirical evidence to substantiate that fear.
In the editorial response to Gunther and Diekema, Brosco and Feudtner correctly note that measures such as the one applied to Gunther's patient in some sense miss the point. That is, our society generally provides insufficient support for persons with disabilities and those who devote time, effort, and resources to caring for the patients. A more generous society would make sure caregivers have assistance devices and other help necessary so that the physical, social, and emotional work of providing care does not become overwhelming. I agree with that. However, in the absence of an adequate system, the measures taken to limit the Seattle patient's growth seem reasonable and well within the scope of caring parents' authority to undertake.
Norman Fost, 11:08 AM ESTI'd like to start with the privacy issues, since I am generally uncomfortable when ethicists or the general public criticize individuals who are experiencing a medical/ethical crisis. Part of this is simply a concern for privacy. Part of it is because the press accounts of the medical facts are often incomplete or inaccurate. And part of it is because I wouldn't want my judgments about medical care in my family subject to national or international scrutiny, privacy aside.
These concerns are mitigated in this case because the father has chosen to put his family's story in the public domain, with a website and a link to a blog that includes harsh criticism as well as support. He has been careful to not reveal his name. He does include a family photo with the faces partially blocked, leaving them identifiable at least in their local community. He says the reason for creating the website was to "share our thoughts and experience for two purposes: first, to help families who might bring similar benefits to their bedridden "Pillow Angels"; second, to address some misconceptions about the treatment and our motives for undertaking it." He has been spectacularly successful, as the website has received over one million hits in 3 days, and thousands of messages have been sent by email or to the blog site.
I find his reasons, and those of Gunther and Diekema, for pursuing the treatment plan they did very persuasive. There are compelling arguments that limiting Ashley's growth is likely to be in her interests, as well as the parents' interests, with low risks.
Joel Frader, agreeing with Brosco and Feudtner, says that the measures used to limit her growth "miss the point," which is the lack of social support which might allow the family to care for her at home even if she grew to normal adult size; e.g., by providing mechanical devices to assist in lifting her. I must day I think this misses the point, which is to try to maximize Ashley's interests, or more precisely, to maximize the ratio of benefit to burden.
I think the father makes a compelling argument that her interests will be better served by limiting her size, regardless of whether devices or supportive services are available. No matter how many people or machines are moved into the home, she will be easier to hold and move if she is smaller. She is more likely to be brought on trips and to have more pleasurable experiences. She is less likely to have complications, such as bedsores.
I also agree with the father's observation that having her size be more appropriate to her developmental level will make her less of a "freak" (my word, not his). I have long thought that part of the discomfort we feel in looking at profoundly retarded adults is the aesthetic disconnect between their developmental status and their bodies. There is nothing repulsive about a 2 month old infant, despite its limited cognitive, motor, and social skills. But when the 2 month baby is put into a 20 year old body, the disconnect is jarring.
It reminds of the scandal some years ago when it was discovered that some Cadillacs had Chevrolet engines. The owners hadn't noticed the problem until it was brought it to their attention. If children like Ashley could magically retain the appearance of an infant, they would not only be easier to care for in the physical sense, but the emotional reaction to them would probably be more favorable. This, of course, may be more of a problem for strangers than the parents, who seem wonderfully attached to her, with little difficulty seeing her as a beautiful, radiant, contributing member of their family.
Benjamin Wilfond, 11:43 AM ESTThere is no question that, as Joel suggests, an important priority should be to improve the services and support for children profound disabilities and their families. However, the issue of the impact of size on encouraging "mobility" would generally be true whether the care is provided by family members or by other care givers. So even having better services (i.e. more people) would fully address the issue.
Several other thoughts. First, we may want to distinguish between the objectives and the means. Joel's concern about the hysterectomy is primarily related to the surgical risks. However, an alternitative approach to attenuating growth is to adjust the caloric intake. In fact, since such children are provided nutrition through a feeding tube, the family and physicians must make a conscious decision about their goals for the size of the child. Limiting calories to reduce growth velocity will result in a shorter height. Alternatively, while large amounts of calories will only have some impact on height, it can increase weight significantly. Would we find this case less startling if parents were requesting recommendations for the amount of calories to limit her growth? Further, regardless of what providers recommend, the parents are the ones feeding the child, so they could do as they please in their home. What distinguishes the current case is the request for the involvement of health care providers to helping the family reach their goal.
Second, the while the parents are doing their best to anticipate how difficult it might be to manage their child as she got larger, their prediction is speculative. It is possible even if she were larger; they would still find a way to maintain her mobility and family engagement. While it is impossible to predict how she would be care for as she got larger. Some issues that seem insurmountable when looking forward become more manageable when the situation is at hand. But it doesn't always go that way. I do think that after sufficient discussion and exploration by the family, that a decision to limit growth because of this concern could be reasonable. I do think that we should respect parents' wishes to make such decisions, particularly after such prolonged engagement and discussion.
Third, like Dr Fader, I am most concerned about the surgical risks. But that may reflect me not being a surgeon. However, there are a range of surgical procedures that are often performed on children with profound disabilities to improve their care. These may include some relatively simple surgeries, i.e. gastrostomy placement (feeding tube), tonsillectomy (to reduce airway obstruction), tracheotomy (to facilitate airway suctioning), as well as more significant interventions; i.e. fundoplication (to reduce reflex), and spinal fusion (to prevent further scoliosis-which can affect positioning). Some families choose not do such surgeries because they believe that they can achieve the goals through simpler measures, which others families decide to use such approaches. I think that parents who might be considering the issue of hysterectomy would benefit from studies that describe caregivers and children's experiences with menstruation to provide more guidance about the its impact on quality of life. But it is not uncommon to balance surgical risks for children with disabilities in order to improve their quality of care.
Joel Frader, 12:36 PM ESTThe paper by Gunther and Diekema notes two reasons for hysterectomy besides "complications of menses." First, elimination of the need to give progesterone along with the high dose estrogen to reduce clotting risks. Second, eliminating the risk of "future uterine and cervical cancer." While understandable, one wonders at what point one hesitates to remove additional organs in order to prevent future disease. It would help to know the incremental risk of cancer associated with the estrogen therapy.
Joel Frader, 12:40 PM ESTJust to make sure that Dr. Fost, with whom I agree, and everyone else understand my point: I support the decision made by the family with Drs. Gunther and Diekema. Nevertheless, we do need to address the separate issue of the society's inadequate support for caring for those with severe disabilities.
Norman Fost, 1:54PM EST** This is in reply to Joel's comments about the hysterectomy.
It's helpful to note the father's primary reason for removing her uterus: she doesn't need it. Since it's only purpose is procreation, which will clearly not be in her interest, it presents her with risks and burdens and no benefits. The most likely of these is menstruation, which should not be trivialized. Ashley may be just competent enough to experience the difficulties that menstruation often bring to severely retarded women, include being terrified on a recurring basis. There is also the small risk of pregnancy, which has occurred in such individuals, particularly if she needs to be institutionalized later in life. The risks of thrombosis (from progesterone), and cancer (from estrogen) may be low, but cumulatively these risks should be weighed against the low risk and transient discomfort of surgery. Different people may reach a different weighing than the parents did, but I do not think their conclusion can be described as unreasonable. Notice also her ovaries were not removed, because of concerns about aggravating her already high risk for osteoporosis.
One other point about the hysterectomy. Some of the critics on the weblog have raised the familiar cry of eugenic sterilization. This criticism is singularly misplaced. The appropriate concerns about eugenic sterilization, in the US and elsewhere, were based on coercive governmental programs, for the purpose of preventing procreation by individuals who were believed (often falsely) to be at risk for producing retarded offspring who would be a burden to society. None of these issues are involved in Ashley's case. She is extremely unlikely to procreate, unless she is raped, and has no interest in procreation. The motive has nothing to do with societal burden. And the state is not involved. This was a private decision, made by her parents in consultation with expert, caring physicians, and clearly motivated to make her life easier.
There is another organ she probably doesn't need, with some risk for discomfort; namely, her appendix. This too, was removed, as is commonly done in patients undergoing laparotomy for another reason.
Joel Frader, 2:42 PM ESTIn reply to Norm:
Generally, I agree. However, it would also have been reasonable to see if menstruation represented either psychological or hygienic difficulties for Ashley. If either or both developed, one could respond medically to minimize the frequency and amount of bleeding or decide on hysterectomy after puberty. I raised the issue not because of specific concern in Ashley's case but to alert readers about less-than-careful thinking that has occurred in other cases.
Norman Fost, 3:59 PM ESTI'd like to raise another point in response to some comments on the weblog suggesting that this is an example of parents and doctors going off the deep end and treating a handicapped child in a way that is not her interests. Part of this criticism reflects a belief that the parents have asked for these interventions for their own convenience, to minimize their burdens.
There was a time when parents and doctors commonly conspired to deprive handicapped children of standard medical care, often with the explicit goal of hastening death. The "Baby Doe" controversy in the 1970's and 1980's was largely about a well documented pattern, over many years, of withholding standard medical treatment from infants with excellent prospects for long happy lives. The paradigm cases involved children with Down Syndrome and easily fixable defects, such as duodenal atresia.
That problem has largely been resolved in the US. It is, for example, unheard of for an infant to have standard treatment withheld simply because the child has Down Syndrome, or spina bifida, another common birth defect which resulted in inappropriate discrimination on the basis of handicap. The way in which it was resolved produced an over-correction in many people's minds: i.e., excessive use of technology to rescue infants with little or no prospect for long or meaningful existence. Disagreements now are more commonly about very gray cases, babies with uncertain futures and insufficient facts to predict likely longterm outcomes.
One of the major changes from then to now has been the widespread use of hospital ethics committees, as occurred in this case. While these consultations obviously can not eliminate the possibility that of a decision that, on reflection, is difficult to defend, they greatly reduce the likelihood that decisions will be based on bad information, or sloppy thinking, as was common in the Baby Doe cases.
The detailed account of the medical basis for the decisions in Ashley's case, as well as the careful ethical reasoning that they relied on, are well documented in the article by Gunther and Diekema, and the father's extraordinary detailed account of how and why they came to their conclusions. Whatever disagreements critics may have, it is not possible to say this decision was made casually or quickly, without careful consideration of the relevant facts and arguments.
This is not to say they considered everything. Joel Frader's question, for example, of why not delay the hysterectomy until there is evidence she is suffering from menstruation, was not explicitly addressed in the available materials. So it is always possible to do it better. But the process they used is dramatically different from the approach that characterized decisions 20-30 years ago. Such a process increases the likelihood that a decision will withstand scrutiny, reduces the likelihood that the participants will later realize they left out some important consideration, and should reassure the public, including advocates for handicapped children, that the child's best interest is the focus of decisions.