Ethical Issues in Decision-Making for Infants with ...

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Author

A. A. Kon

Affiliation

Naval Medical Center San Diego and the University of California San Diego, San Diego, USA

Key words ▶ ethics ● ▶ decision-making ● ▶ children ●

Abstract

received 23.09.2014 accepted 19.02.2015 Bibliography DOI http://dx.doi.org/ 10.1055/s-0035-1547269 Horm Metab Res 2015; 47: 340–343 © Georg Thieme Verlag KG Stuttgart · New York ISSN 0018-5043 Correspondence A. A. Kon, MD, FAAP, FCCM Naval Medical Center San Diego 34800 Bob Wilson Drive San Diego CA 92134 USA Tel.:  + 1/530/9022 133 Fax:  + 1/858/7557 444 [email protected]

Medical decisions for infants and children should generally be based on the best interests of the child. When there is legitimate controversy over the child’s best interests, the right of the child to an open future should generally determine the course of treatment. In the case of infants born with disorders of sex development (DSD), early cosmetic genitoplasty was long believed to be in the child’s best interest and was therefore the standard of care. New data suggest that early genitoplasty may be more harmful than helpful, therefore the best interest standard is no longer

determinative in such cases. Because children born with DSD have a right to an open future, and because the openness of their future is clearly enhanced by delaying cosmetic genitoplasty until they themselves can participate meaningfully in decision-making, early genitoplasty is ethically supportable only when medically indicated (e. g., when the child is unable to urinate without surgical intervention). Further research is needed to clarify the benefits and burdens of early and delayed genitoplasty. In parallel with further research, efforts should focus on educating society broadly to decrease stigmatization of persons with DSD.

Deciding whether an infant born with a disorder of sex development (DSD) should undergo surgery in infancy and/or early childhood is complex and raises significant ethical concerns. This is particularly true when considering surgery to make the child’s external genitalia appear more “normal” when compared with male or female children born without DSD. In this brief essay, I will describe the best interest standard, and explain why this standard is not dispositive when considering early genital surgery for infants with DSD. I will then explain the child’s right to an open future, and discuss why this right necessarily leads to delay of cosmetic surgery that is not medically necessary for affected infants. Finally, I will present recommendations for future research as well as how best to support these children given our current understanding of DSD. The best interest standard generally guides decision-making in the care of infants and children [1, 2]. Under this standard, “decision makers should assess the incompetent or incapacitated person’s immediate and long-term interests and select the option (or from among those options) that maximizes the person’s overall good and minimizes his/her overall risks or harms” [3].

When there is a decision to be made on behalf of an infant, and one option is clearly in the infant’s best interest, then choosing that option is the only ethically supportable course of action. In the case of an infant born with DSD, the best interest standard should therefore apply. When surgery is necessary to correct significant physiological problems (e. g., an infant who, without surgery, would be unable to urinate), then surgery would generally be in the infant’s best interest. In such cases, there may be little ethical debate regarding the appropriate course of treatment. While surgery to correct imminent threats to health clearly falls within the best interest standard, cosmetic procedures (genitoplasty to create more “normal appearing” genitalia) raises greater ethical concerns. Historically, an infant born with DSD would undergo genitoplasty in order to give the infant a more typical genital appearance to match the gender of rearing [4–6]. More recently, however, adults who underwent such procedures in infancy and early childhood have raised serious concerns about early cosmetic interventions. Many of these patients report substantial pain due to repeated procedures in childhood and appear to be at significant risk of long-term



Kon AA. Decision-Making in DSD …  Horm Metab Res 2015; 47: 340–343

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Ethical Issues in Decision-Making for Infants with Disorders of Sex Development

decreased sexual enjoyment and potential inability to experience an orgasm. Further, it is well established that gender identity (the gender with which the person self-identifies, regardless of his/her biological sex) is not determined merely by genetics nor by physical characteristics [7, 8]. Indeed, recent work in the care of transgender children indicates that some children born genetically and phenotypically as one gender clearly self-identify as the other gender even from a very young age [9]. Similarly, patients with DSD may develop a gender identity that does not match the gender assigned by the surgeon, which would likely cause significant distress and long-term psychological and emotional stress for the child. Such gender “misassignment” would likely represent a significant harm to the child. For these reasons, the Accord Alliance recommends deferring cosmetic and/or “gender normalization” surgery until the child himself/ herself can actively participate in decision-making [10]. Of note, however, there may be some meaningful harm to the child if “gender normalization” surgery is not performed early. Society uses dichotomous gender assignment even at very young ages. Babies’ names, hair styles, and clothing are often guided by gender. Preschool children often use dichotomous gender-based bathrooms, line up in the “girl’s line” or the “boy’s line”, wear their hair in gender-specific ways, and wear clothing with styles based on their dichotomous gender assignments. As such, even without “gender normalization” surgery, most children with DSD are assigned (or provisionally-assigned) a gender of rearing by parents. While in the ideal situation, family members, friends, and peers would be open and nonjudgmental of children with DSD, in reality many communities lack such open and accepting norms. When extended (or in some cases even immediate) family members, friends, or peers learn of the child’s DSD, the child may become subject to bullying, marginalization, and other stigmatization. Concealing the child’s condition may be complicated due to use of shared bathrooms or changing rooms (e. g., for gym class or at the community swimming pool), family and friends assisting in diaper changing, etc. As such, in many families and communities, raising a child with ambiguous genitalia may pose a meaningful risk to the child’s long-term psychological and emotional well-being. Because cosmetic surgery for infants born with DSD used to be considered clearly in the infant’s best interest, such surgery was considered ethically appropriate (if not ethically obligatory). With myriad patients raising objections to early cosmetic surgery [10], and the lack of data demonstrating improved outcomes with early cosmetic surgery [11, 12], the best interest standard is called into question. Because there is now legitimate disagreement about which option (early cosmetic surgery vs. delayed cosmetic surgery until the child himself/herself can actively participate in decision-making) is in the child’s best interest, the best interest standard can no longer be determinative in guiding the course of treatment. Without a clear “best interest” choice, clinicians and parents must turn to a different principle in pediatric ethics, the child’s right to an open future [13, 14]. As first articulated by Feinberg, there are some rights that only adults have (e. g., the right to vote), others that adults and children both have (e. g., the right to food and clothing), and some that only children have (e. g., the right to be loved and cared for). The children-only rights can be further subdivided into 2 broad types. First, there are rights, which children have by virtue of the dependency on others. For example, because children cannot obtain sufficient income to pay rent, purchase food, pay for healthcare, pay for their educa-

tion, etc., children have a right to have such things provided to them. Consequently, adults have a corollary obligation to provide those things to which children have a right (in general this obligation falls to parents, however, when parents fail to meet this obligation the state assumes this obligation). Second, there are what Feinberg termed “rights-in-trust”, which he also equated with the child’s right to an open future. These are rights that the child herself has, but which the parent holds in trust on her behalf. The parent is therefore obligated to safeguard such rights until the child is mature enough that she may exercise these rights on her own behalf. In other words, children have a right to make major decisions for themselves once they are able, and therefore parents have the corollary obligation to make choices for the child that allows the child an “open future” in which she herself can make major life choices. To illustrate, one of the most widely recognized examples of a child’s right to an open future is the case of a child whose parents are Jehovah’s Witnesses. When such a child presents with a life-threatening need for a blood transfusion, the parents may prefer that their child not be transfused due to their religious beliefs. Because the child herself, however, is too immature to choose her own religion, she has a right to receive life-saving interventions (even over parental objection) so that she can mature sufficiently to make her own religious decisions. As such, the child’s right to an open future trumps the parents’ right to raise their child how they wish. In contrast, there are some decisions that parents are allowed to make that may seem to limit the child’s open future. One common example is that of circumcision. On the one hand, it can be argued that preserving the child’s open future would require a moratorium on circumcision so that boys (or men) can choose for themselves whether they prefer to be circumcised. Because, however, circumcision is widely accepted and generally deemed a benign procedure (long-term), the practice is generally considered ethically permissible. Applying the right to an open future can be nuanced and complex. For example, because the long-term outcomes of patients who receive a cochlear implant is related to the age at which the patient undergoes surgery, the compelling interest to maximize benefit to the child may outweigh the child’s right to an open future in which she herself decides if she will hear. As such, delaying implantation of a cochlear implant may not increase the openness of the child’s future because the benefit of such a device will be significantly less if surgery is postponed until the child can choose for herself. When determining how to apply the child’s right to an open future, parents and clinicians must balance the short-term and long-term benefits and risks of all options. If one option clearly enhances the openness of the child’s future, then adults have an obligation to choose that option on behalf of the child (unless a different option is clearly in the child’s best interest). Then, when the child becomes sufficiently mature, she may herself choose from among the various options open to her. With an understanding of the best interest standard and the child’s right to an open future, we turn to the case of an infant born with DSD. Prior belief was that without “gender normalization” surgery, the child would suffer greatly due to societal norms of dichotomous gender assignment and risk to the child if the external genitalia do not match what society considers “normal”. Therefore, there was a longstanding belief that “gender normalization” surgery was in the child’s best interest. As such, surgery was routinely performed long before the child could Kon AA. Decision-Making in DSD …  Horm Metab Res 2015; 47: 340–343

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participate in decision-making. Because we now understand that early genitoplasty often leads to significant long-term problems and therefore may not be in the child’s best interest, we must turn to the child’s right to an open future to assist in determining the best course of action. The question, therefore, is which option facilitates more openness in the child’s future: early genitoplasty or delayed genitoplasty. Early genitoplasty may be seen as facilitating the openness of the child’s future by allowing the child to have a “more normal” childhood. That is, by performing surgery to make the child’s genitals look like those of peers, the child might be more likely to have a more normal childhood, have more stable relationships, and be better positioned to then choose options as an adult. To date, however, there are insufficient scientific data to support these claims. In contrast, delayed genitoplasty significantly decreases the risk of gender misassignment, long-term sexual dysfunction, and significant pain and suffering during childhood. As such, on balance, it would seem that delaying genitoplasty until the child can self-assign his/her gender and can choose whether genitoplasty is preferable to maintaining the status quo best facilitates the openness of the child’s future. Under this analysis, therefore, children born with DSD have a right to not be subjected to “gender normalization” surgery (Note: when surgery is necessary due to imminent threat to the baby’s health, then such surgery is clearly in the infant’s best interest and should be performed). Parents (and the state) have an obligation to foster the child’s open future and therefore are obligated to decline any “gender normalization” surgery that is not medically necessary. Further, pediatricians and others caring for children have long recognized that we have a special obligation to our patients, often beyond that of parents and the state, and must advocate for children and protect our patients (even from well-meaning parents) [15]. As such, the infant’s right to an open future leads to an obligation on the part of physicians and surgeons to avoid genitoplasty that is not medically indicated. Ideally, surgery should be delayed until the child can actively participate in decision-making. The American Academy of Pediatrics supports the involvement of children in decision-making when doing so is appropriate [15]. Further, the American Academy of Pediatrics, American Medical Association, and other professional organizations support sharing the responsibility and burden of decision-making between patients and providers (and parents in the case of children) [15–17]. Shared decision-making can best be conceptualized as a continuum ranging from a patient-driven (or parent-driven) decision-making model to an equal partner model to a physician-driven model [18], and key communication components have been identified [19]. The model employed, however, should be tailored to the patient’s preferences. In the case of infants born with DSD, the shared decision-making team should include the child (when the child is old enough to actively participate in decision-making), the parents, the providers, and other members of the multidisciplinary care team [20]. Future research must focus both on clarifying the open future question and illuminating the best interests of the child. Specifically, data regarding outcomes for patients who underwent genitoplasty in infancy must be obtained. Selection bias in such studies must be carefully attended to so that the sample is not overly populated with individuals who are satisfied with the treatment they received nor with individuals who are vocal opponents of early genitoplasty. Such studies must also consider Kon AA. Decision-Making in DSD …  Horm Metab Res 2015; 47: 340–343

the surgical innovations in this field that continue, since outcomes for patients operated on today are likely quite different than outcomes for those who underwent surgery in past decades. Further, data regarding outcomes for children born with DSD who do not undergo early genitoplasty must be obtained, again attending to selection bias as well as to social factors that may have significant impact (region where patient lives, general openness of family and friends, etc.). With such data, we will be better positioned to accurately judge the benefits and burdens of early genitoplasty vs. delayed surgery. If such studies suggest that early genitoplasty improves long-term outcome (as judged by the patients themselves), then resuming early surgery may be indicated. At this time, however, without evidence to support early genitoplasty, and with compelling reasons to delay such interventions, genitoplasty in infancy should be restricted to only those cases in which such interventions are medically necessary for physiological (not social) reasons. Perhaps most important for the patient are ongoing efforts to normalize DSD within the broader society. Although there are significant differences between children born with DSD, individuals with disabilities, and the lesbian-gay-bisexual-transgender-queer (LGBTQ) community, we may find important lessons in the experiences of these other groups. For example, over the past decades significant strides have been made in the social treatment of persons with disabilities. Historically, such individuals were labeled as “defective”; however, through advocacy, education, and legislation there has been a significant shift in our understanding. We now see barriers to equal participation as a society-based problem, not as a person-based problem, and we understand that breaking down such barriers is best accomplished by changing society rather than by changing those with disabilities [21]. Similarly, the LGBTQ community has been successfully advocating for LGBTQ rights and educating the general public. For example, in the past, homosexuality and transgenderism were considered disorders [and indeed diagnosed as disorders in earlier versions of the American Psychiatric Association’s Diagnostic and Statistical Manual of Mental Disorders (DSM)]. Recent strides include increased acceptance of same-sex marriage in many jurisdictions, and the prohibition of mental health provider attempts to change the sexual orientation of minors1. Further, the World Professional Association for Transgender Health urged de-psychopathologization of gender nonconformity [9], and subsequently the American Psychiatric Association introduced the term Gender Dysphoria in the DSM-5, replacing the previously used terminology of Gender Identity Disorder in order to clarify the these individuals are not “disordered” [23]. Indeed, the American Medical Association now officially endorses appropriate access to treatment for transgender patients [24]. The experiences of the disability and LGBTQ communities suggests that rather than attempting to “fix” children with DSD, society should accept these individuals for who they are and allow those with DSD to choose for themselves whether genitoplasty is indicated. While such efforts remain in the fledgling stage, there are signs that there is growing awareness of DSD in the general public. For example, the M-TV series “Faking It” fea1

 In 2012, California enacted SB-1172, and in 2013, New Jersey enacted AB3371. Both statutes prohibit mental health providers from attempting to change the sexual orientation of minors, and both have been upheld by appellate courts. Several other states have also introduced similar legislation [22].

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tures a main character born with DSD, and several celebrities have openly disclosed that they have DSD. Further, since November 2013, Germany has officially included 3 gender options on birth certificates: male, female, and undetermined [25]. In summary, although early genitoplasty was previously considered to be in the child’s best interest, patient testimonials and the lack of data supporting such surgery raise serious doubts as to the benefits of such treatment. Because the best interest standard is not determinative in these cases, the child’s right to an open future should guide medical decision-making. Based on the above analysis, delaying cosmetic genitoplasty until the patient himself/herself can participate meaningfully in decisionmaking allows for a significantly greater openness of the child’s future than does early genitoplasty, therefore unless genitoplasty is medically indicated (to allow urination, etc.), such surgery should be deferred until the child can choose for himself/ herself. Further research is needed to illuminate how best to manage these difficult cases, and further efforts should also focus on normalization of DSD within the broader social context.

Statement



The views expressed in this publication are those of the author and do not necessarily reflect the official policy or position of the Department of the Navy, Department of Defense, National Institutes of Health, Eunice Kennedy Shriver National Institute of Child Health and Human Development, or the U.S. Government.

Conflict of Interest



The authors declare no conflict of interest.

References

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