011-Disclosures of Disorders of Sex Development to Children: When and How Do We Tell Kids About Their Diagnosis?

Abstract

Background: Children can be diagnosed with a disorder of sex development (DSD) at birth with ambiguous genitalia, or later in childhood after investigations of other symptoms. In all cases, the diagnosis comes as a surprise, and there is initial parental distress and uncertainty about their child's future. The diagnosis of DSD can have a significant impact on the psychosocial development and wellbeing of the child, with challenges around diagnosis complexity, sex of rearing, uncertain outcomes, treatment options and psychological stressors. Purpose: Whilst disclosure in practice often occurs in a gradual manner when working within a multidisciplinary team, we propose to formalise a guidance system describing a developmentally staged approach. The process involves sequential information about body parts, differences between male and female, the wide potential for gender roles and later the potential for different individual development of sex, gender, sexuality and fertility. Description of Topic: The development of gender identity begins after the first year of life, with children generally identifying with one sex by age three. Self-awareness regarding identity and gender role evolves during childhood, with sexuality developing through adolescents, along with physical secondary sex characteristics. Critical psychological development also starts to occur, including the capacity to understand concepts such as future fertility potential. Therapeutic events such as genital surgery or requirement for sex hormone therapy can affect this process. Information provided to a child about their diagnosis therefore needs to be age appropriate, and tailored to the individual's developmental needs. Criticism of past DSD management has been poor or late disclosure of diagnosis to the young person, and although advice now supports earlier disclosure, there is limited literature guiding a method for this. Clinical Implications: The goal of treatment is to ensure that the child's psychological, physical and sexual well-being are supported in a respectful and appropriate manner. This can be achieved via a multidisciplinary team working with the parents to support the child, communicating honestly and respectfully to maintain trust, and providing guided appropriate information through defined developmental stages from infancy to adolescence and adulthood. The paediatric endocrine nurse plays a critical role in this process as both child's advocate and family educator.