Wiesemann C, Ude-koeller S, Sinnecker GH, Thyen U. Eur J Pediatr. 2010 Jun; 169(6):671-9

DOI: 10.1007/s00431-009-1086-x

Abstract 

The medical management of differences of sex development (DSD)/intersex in early childhood has been criticized by patients’ advocates as well as bioethicists from an ethical point of view. Some call for a moratorium of any feminizing or masculinizing operations before the age of consent except for medical emergencies. No exhaustive ethical guidelines have been published until now. In particular, the role of the parents as legal representatives of the child is controversial. In the article, we develop, discuss, and present ethical principles and recommendations for the medical management of intersex/DSD in children and adolescents. We specify three basic ethical principles that have to be respected and substantiate them. The article includes a critical discussion of the best interest of the child and of family privacy. The argumentation draws upon recommendations by the working group “Bioethics and Intersex” within the German Network DSD/Intersex, which are presented in detail. Unlike other recommendations with regard to intersex, these guidelines represent a comprehensive view of the perspectives of clinicians, patients, and their families. CONCLUSION: The working group identified three leading ethical principles that apply to DSD management: (1) to foster the well-being of the child and the future adult, (2) to uphold the rights of children and adolescents to participate in and/or self-determine decisions that affect them now or later, and (3) to respect the family and parent-child relationships. Nine recommendations for the management of DSD indicate how these ethical principles can spelled out and balanced against each other in the clinical setting.

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Summary  

The clinical management of disorders of sex development (DSD) is contentiously debated. This paper delineates nine principles and recommendations for interdisciplinary healthcare teams delivering care to affected newborns or young children and their parents. Because outcome data in DSD are so limited and best practices remain elusive, the paper’s process-oriented recommendations are timely and may serve as a stimulus for discussions about quality of care within existing interdisciplinary DSD teams and inform the development of guiding principles among nascent teams.

In 2005, under the auspices of the Lawson Wilkins Pediatric Endocrine Society (LWPES) and the European Society for Paediatric Endocrinology (ESPE), 50 international experts convened to develop a consensus statement on the management of intersex conditions. A new diagnostic nomenclature was proposed and “disorders of sex development” (DSD) introduced as the umbrella term {1}. DSD is defined as “congenital conditions in which development of chromosomal, gonadal, or anatomic sex is atypical” and was chosen to replace terms such as “intersex” and “hermaphroditism”, which are vague, confusing and can be experienced as stigmatizing. The DSD consensus statement emphasized functional outcome rather than appearance in decision making related to genital surgery. Participants noted that evidence is lacking that elective/cosmetic surgery performed in the first year of life relieves parental distress and improves parent-child attachment. The importance of the principle of patient-/family-centered care for DSD was described with some specific recommendations provided. As a significant sign of convergence in thought among healthcare providers and patient advocates, the consensus statement made reference to the “Clinical Guidelines for the Management of Disorders of Sex Development in Childhood” in the context of a discussion of “optimal clinical management of DSD” {2}. The guidelines are disseminated by the Accord Alliance, a non-profit organization promoting comprehensive and integrated care by fostering collaboration among all stakeholders. Despite the enormous value of the consensus document, it did not provide adequate details to serve as practice guidelines. The paper by Wiesemann and colleagues fleshes out details that, in some cases, were only alluded to in the consensus statement. The DSD bioethics working group that produced this paper was formed out of a DSD research network supported by the German government for studies of rare diseases. Wiesemann et al. substitute the term “disorders” in DSD for “differences” in sex development to acknowledge the criticism by some advocates that “disorders” is pathologizing and does not take into account natural variability existing in sex development and genital phenotype. Critics of irreversible surgical procedures performed on newborns note that the substitution of informed consent with proxy permission violates the child’s autonomy when there is no immediate threat to the child’s health. According to this position, surgery should be postponed until the affected person is old enough to actively participate in informed decision making. In contrast, this bioethics working group interpreted the “best interests” of the child in a broader developmental context that posits a potential conflict between the needs of a young child and the adult that they will become. For example, anatomic differences might be a source of a distorted body image of a child wanting to blend in with his/her peers whereas, for adults, these same differences may be cherished. The authors recognize that what is in the best interest of the child is inherently subjective. In part for this reason, parents and families are afforded substantial latitude in making decisions on behalf of the child. The authors cite the United Nations Declaration of Human Rights, which states “The family is the natural and fundamental group unit of society and is entitled to protection by society and the State.” {3}.

Reinforcing a central recommendation in the DSD consensus statement {1}, the authors indicate that ethical care of those affected by DSD and their families requires that family-centered care be delivered by interdisciplinary healthcare teams. The nine principles and recommendations for interdisciplinary DSD teams regarding genital surgery decision making will serve as an excellent starting point for discussions within such teams.

References

1.  Consensus statement on management of intersex disorders. Hughes IA, Houk C, Ahmed SF, Lee PA, Lawson Wilkins Pediatric Endocrine Society/European Society for Paediatric Endocrinology Consensus Group J Pediatr Urol 2006 Jun; 3(2):148-62 PMID: 18947601

2.  https://www.accordalliance.org

3.  http://www.un.org/Overview/rights.html     

Recommendation Citation:

Sandberg D and Dore-Stites D: F1000Prime Recommendation of [Wiesemann C et al., Eur J Pediatr 2010,169(6):671-9]. In F1000Prime, 21 Dec 2009; DOI: 10.3410/f.1282965.750067. F1000Prime.com/1282965#eval750067