Psychological Aspects of the Treatment of Patients With Disorders of Sex Development

David E. Sandberg, Ph.D.; Melissa Gardner, M.A.; Peggy T. Cohen-Kettenis, Ph.D.

Disclosures

Semin Reprod Med. 2012;30(5):443-452. 

In This Article

Abstract and Introduction

Abstract

Research on the psychological development of persons with Disorders of Sex Development (DSD) has focused on understanding the influence of atypical sex hormone exposure during steroid-sensitive periods of prenatal brain development on the process of psychosexual differentiation (i.e., gender identity, gender role, and sexual orientation). In contrast, analysis of clinical management strategies has focused on gender assignment and the desirability and timing of genital surgery.
This review focuses on the psychological issues that confront clinicians managing the care of persons born with DSD and their families. Particular attention is paid to processes and factors that potentially mediate or moderate psychosocial and psychosexual outcomes within and across developmental stages.

Introduction

As in other chronic pediatric conditions, accurate diagnosis and delivering appropriate medical and surgical treatment are central aspects of best practices in the clinical management of disorders of sex development (DSD); nevertheless, and potentially more than in other congenital conditions, the affected person's health-related quality of life (HRQoL) and adaptation of the family will also depend on the extent to which healthcare providers attend to psychosocial aspects of the condition. Psychology, particularly research in developmental psychology, guides our approach.

A commonly adopted model for understanding HRQoL outcomes in DSD is one in which assigning the "right" diagnosis and providing the "best" medical or surgical treatment(s) are viewed as the exclusive predictors of positive HRQoL (Fig. 1: I). Ultimately, a linear model in which outcomes are hypothesized to be directly determined by biological factors and/or medical/surgical interventions proves to be an oversimplification that leaves substantial variability in endpoints unaccounted for. Moreover, an exclusive focus on biological and medical/surgical aspects of DSD limits theory development and impedes innovation in clinical management strategies. Instead, and consistent with commonly adopted paradigms in developmental psychology, psychopathology and theory in gender development,[1,2] we propose adopting conceptual models that consider the potential influences of moderating (Fig. 1: II) and mediating variables (Fig. 1: III). In addition to this approach more accurately reflecting the complexity of psychological development, it offers the benefit of studying DSD within the mainstream of clinical research and theory development in pediatric psychology.

Figure 1.

Conceptual models for direct, mediated and moderated developmental effects. I. Simple or direct effect model: single predictor (A) directly related to a single outcome (C) II. When the relationship between predictor (A) and outcome (C) is thought to be either buffered or intensified by another factor (C), then a suitable model is one that tests for moderation. III. A mediation model posits that the predictor (A) influences another variable (B) which, in turn, is more directly responsible for the outcome (C).

In cases in which gender assignment is in question, or gender reassignment is under consideration, decision making needs to be informed by both developmental and clinical psychology. The necessity of psychological counseling also emerges in the context of decisions about the timing of interventions, education of the patient and others about medical history (i.e., disclosure), management of potential psychosocial or educational problems that emerge for the child, or when parents need support in understanding the etiology of the child's condition and its implications. The importance of these aspects of care is reflected by the increasing participation of mental health professionals in multidisciplinary DSD teams.[3–5]

This paper focuses on the psychological issues that confront clinicians managing the care of persons born with DSD and their families. Particular attention is paid to processes and factors that potentially mediate or moderate outcomes (broadly defined) within and across developmental stages.

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