Ohad Oren, a 3rd year medical student at Bruce Rappaport Faculty of Medicine, in Haifa, Israel considers an ethical dilemma.

A profoundly disabled child whose annual growth rate is subnormal approaches your care. The parents ask you to test growth hormone function and to consider an active intervention to help their child attain a larger stature. How should you proceed? Increased growth will surely not benefit a severely brain impaired child, with no trace of body image whatsoever. A larger stature, moreover, will impose a higher burden on his caregivers, hampering their continuous care of their child.

This paradoxical scenario - whether to use medical treatments to increase the final stature of a developmentally-disabled child - is not uncommonly encountered in pediatric endocrine clinics. Rejecting the GH hormonal therapy may portray abandoning an effective medical therapy on the grounds of little or no benefit to the patient. But, is offering parents of severely disabled children proactive measures to attenuate growth an ethical thing to do? The Ashley X case, presented in this article, sparked an intensive public discussion concerning the ethical justification of irreversibly altering the body of a profoundly disabled and nonconsenting child.

Ashley X, the core of the ensuing debate, suffers from static encephalopathy. According to her physicians, she would never progress beyond the developmental stage of a 3 month-old infant. (1),(2) At the age of 6, Ashley is completely dependent in all daily activities, being unable to walk, communicate, eat, sit up, or roll over. Her parents were concerned about her growth, which would make it “untenable for them to care for their daughter at home, despite their strong desire to do so”.(1) The parents and the physicians thoroughly deliberated the condition, and devised a treatment program using high-dose estrogen to attenuate final adult height (box). To reduce long-term complications of puberty, removal of her uterus and breast-buds was considered. An ethics committee carefully analysed the family’s reasons for their requests, and concluded that all were ethically appropriate (table 1).

Therapeutic measures to attenuate growthIn the 1940’s, physicians observed that children exposed early to sex hormones (due to precocious puberty) had premature closure of the epiphysial plates and were markedly shorter as adults. On the other hand, children with sex hormones deficiency have a prolonged period of growth.w3 Based on this, in most countries growth attenuation is accomplished by the use of supraphysiologic levels of estrogen. Estrogen’s biphasic effect on the epiphysis is exploited for this therapeutic aim. Children with acromegaly were the first recipients of estrogen therapy, in an attempt to prevent further growth.w4 Treatment is generally safe, but decreased fertility has recently been reported. In Sweden, a different means is used, namely Epiphysiodesis. This is an operative procedure which is mainly performed to equalize leg length, sometimes due to poliomyelitis. Cessation of growth is achieved by disruption of the growth plates, or even by the incorporation of a bone graft to produce fusion of the epiphysis.

Table 1

Potential benefits and harms inherent in each procedure of the Ashley Treatment (5), (6), (7), (8)

Against	For
- Surgical procedure - Ovaries malfunction (due to compromised blood supply), increasing risk of heart disease and osteoporosis	- Adjunct to high-dose estrogen treatment - Eliminates complications of menses - Spares a lifetime of hormone injections - Eliminates need of progesterone (thus reducing thrombosis risk) - Eliminates possibility of future uterine and cervical cancer	Hysterectomy
- Thrombosis (DVT*) - Minimal if any adverse effects: nausea, headaches, weight gain**	- Arrests growth	Growth attenuation (high-dose estrogen treatment)
- Surgical procedure	- Decreases risk of fibrocystic breast disease and breast cancer (known family history) - Better wheelchair fitting - Reduces risk of sexual abuse	Breast-bud removal

* DVT - Deep Vein Thrombosis.

** These adverse effects were demonstrated in constitutionally tall adolescent girls that were given high-dose estrogen, in trials done in the 1950’s. (9)

Risks not fully determined

The case of Ashley X was the first to present the question of whether hormone therapy should be used to make children with profound impairments smaller. We may still gain knowledge and perspective if we think about other medical treatments whose risk-benefit calculation may seem comparable to the case we discuss (fig 1). On the one hand, there are risky treatments that are taken prophylactically, in an attempt to avoid a life-threatening condition (i.e. mastectomy in BRCA carriers). Genetically-unfortunate women can weigh the risks and benefits, and reach a decision based on their values and beliefs. On the other side, prophylactic interventions, like immunization, carry high benefits, with risks that are extremely low. Which category could describe Ashley’s condition? You could argue that the risks involved in Ashley’s treatment are substantial, and not in proportion to the possible gains she might get. Or you could say, as her physician did, that “these risks do not seem to be unreasonable and are not out of line with the risks of other medical interventions these children undergo”.(5)

Figure 1 Common medical procedures that may be comparable to the treatment that Ashley X underwent, when analysing their risk to benefit balance.

How, then, could risks be quantified, or at least estimated, when there were no children like Ashley undergoing growth attenuation therapy combined with hysterectomy and breast-bud removal? The only similar trials involving high-dose estrogen to stunt growth were treatments of constitutionally tall adolescent girls, wishing to minimize any further gain in height. (10) Ashley’s physicians extrapolated data from these 1950’s studies to support the efficacy and safety of the treatment. But Evidence-Based Medicine principles oblige the medical profession to seek conclusive scientific evidence prior to using a presumably efficient therapy. It was not yet shown that children with significant developmental disabilities who are shorter stay at home longer. In fact, we don’t even know whether keeping Ashley at home has any beneficial effect on her social or health status. Observational studies should test these issues, among others, to gain a better understanding of the therapy’s potential risks.

Parental motives

In most cases, parents are in the best position to make medical decisions for their child. It is believed that they understand and attempt to achieve the “best interests” of their child better than anyone else. Therefore, parents are given the legal and moral responsibility to care for their children. (11) Being Ashley’s legal guardians, her parents asked the doctors to perform an intervention that was neither a medical necessity nor a hitherto-recognized therapeutic option.

Now, try scrutinizing the Ashley case appropriateness through less shiny lenses, such as those of a developmental disabilities professional. It has been claimed that what we have here is an invasive, risky treatment, whose goals are essentially quality of parental life, and with the patient “good” defined by someone other than her.

The American College of Obstetrics and Gynecology (ACOG), in their paper “Sterilization of women, including those with mental disabilities” , notes that involuntary and nonvoluntary medical interventions and surgical procedures in general should only be provided when there are clear health benefits.(8) Ashley’s parents wish to take care of her, and stunting her growth, they argue, will make it easier for them to extend the time they can care for her at home, and enhance their child’s mobility, social interaction and physical contact with them (table 2). (5), (13)

This reasoning of the parents led some to claim that their own convenience was the primary rationale for attenuating Ashley’s size. They could have hired people to help them, or go to the gym, or purchase special devices for their home, instead of surgically modifying their child’s height. But, stating Judith Jarvis Thomson, “nobody is morally required to make large sacrifices, of health, and all other interest and concerns… in order to keep another person alive”. (12) In my humble opinion, as long as motives of convenience form only part of the parents’ justifications, with the child’s benefits as the center of gravity (in choosing the requested treatment), they should not be considered as distinct from those of the child. Consider, for example, a parent bringing a child with a toothache to the dentist, hoping that the child will undergo a lengthy procedure, so that he will earn a few hours of sleep. Do these latent motives make the procedure unethical? Of course not. (13) Further emphasized in a child with developmental disabilities is the complete dependency upon his parents for all his daily care. In circumstances such as this, the child’s interests and needs are intertwined with those of the parents. Defragmenting the family to pieces, to isolate and analyze the needs of the disabled child only, is analogous to prescribing medication based upon mere lab results, without seeing the patient.

Table 2

The pros and cons of the “Ashley Treatment” (5), (13)

Cons	Pros
- Adverse effects of therapy (i.e. thrombosis) - Parents’ motive may be their own convenience - Irreversible alteration of the child’s body, without her awareness - Aggressively replaces the societal role in providing social support for people with disabilities	- More stimulation - More social interactions - Fewer medical complications - Better physical contact between parent and child - Extends the time parents can care for their child at home - Reducing potential for abuse

Society’s role

In a perfect world, with unlimited social support resources, Ashley could be nursed and cared for in a normal size. In view of the options open to them, Ashley’s parents sought after the possibility that would enable them to improve their child’s (current and future) medical as well as social state.

If we target a fundamental change in the harrowing predicament that parents of such children face, medication is a possible adjunct, but solving the problem requires more funds for community-based living programs. If the collective community response proscribes the Ashley treatment, society must be prepared to provide the caregivers with enough assistance that they would not have to resort to these means. (13) As long as social support is lacking, however, the medical profession may offer its interventions, in cases like Ashley’s, taking into account her natural and social circumstances.

As I view it, what the Ashley Case showed was that there was much more needed discussion about the value of disabled children in our society. Many disciplines in medicine may help translate such challenging dilemmas into reasoned and balanced solutions. Specialists in pediatric endocrinology, neurology, surgery, development, and ethics contribute their perspectives on highly debatable issues such as Ashley’s. An interdisciplinary group of experts of that type may only further emphasize the pivotal discussion we must engage in regarding social attitudes toward people, in particular children, with neurologic and cognitive impairments.

Acknowledgements: I am deeply grateful for the critical and insightful review provided by Zeev Hochberg, MD PhD (Director, Pediatric Endocrinology Unit, Meyer Children’s Hospital, RAMBAM Health Care Campus, Haifa, Israel) as well as for his fascinating perspectives and ideas with regard to the ethical issue at hand.

Ohad Oren, 3rd year medical student, Bruce Rappaport Faculty of Medicine, Technion - Israel Institute of Technology, Haifa, Israel. ohadoren@gmail.com

References 1-13:

1. The “Ashley Treatment“: Toward a better quality of life for “Pillow Angels”.
2. Coombes R. Ashley X: A difficult moral choice. BMJ 2007;334;72-73.
3. Fancher TK. Some observations on anterior lobe hyperpituitarism. Endocrinology. 1932; 16:611.
4. Kirklin OL, Wilder RM. Follicular hormone administration in acromegaly. Proc Staff Meet Mayo Clin. 1936; 11:121-125.
5. Gunther DF, Diekema DS. Attenuating growth in children with profound developmental disability. Arch Pediatr Adolesc Med 2006: 1013-7.
6. Bladbjerg EM, Skouby SO, Andersen LF et al. Effects of different progestin regimens in hormone replacement therapy on blood coagulation factor VII and tissue pathway inhibitor. Hum Reprod. 2002;17:3235-3241.
7. Girolami A, Spiezia L, Rossi F, Zanon E. Oral contraceptive and venous thromboembolism: which are the safest preparations available? Clin Appl Hemost. 2002;8:157-162.
8. Committe on Ethics, American College of Obstetrics and Gynecology: Sterilization of Women, including those with Mental Disabilities.
9. Lee J, Howell J. Tall Girls; The Social Shaping of a Medical Therapy. Arch Pediatr Adolesc Med. 2006;160:1035-1039.
10. Goldzieher MA. Treatment of excessive growth in the adolescent female. J Clin Endocrinol Metab. 1956; 249-252.
11. Kopelman LM: The best-interests standard as threshold, ideal, and standard of reasonableness. J Med Phil 1997;22:271-289.
12. J. J. Thomson, “A defense of Abortion” in Ethics in practice, ed. by H. Lafollette (Oxford, UK: Blackwell, 1997), 69-78, at 77.
13. S. Matthew Liao, J. Savulescu, M. Sheehan. The Ashley Treatment: Best Interests, Convenience, and Parental Desicion-Making. Hasting Center Report 37, no. 2 (2007): 16-20.

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