Talking to Families About Disorders of Sex Development

1. Talking to Families About Disorders of Sex Development Jennifer L. Rehm, MD American Family Children’s Hospital Division of Pediatric Endocrinology University of Wisconsin - Madison

2. Outline • Terminology • Nature vs Nurture – history of DSD • Multi-Disciplinary Team Approach and Patient Centered Care • Gender Assignment • Resources • Discussion

3. Changing Terminology Disorder of Sex Development • Intersex Hermaphrodite Sex Reversal Pseudohermaphroditism

4. Changing Terminology

5. Thanks to David E. Sandberg, PhD, Division of Child Behavioral Health, U of M

6. More TerminologyPsychosexual development • Gender Identity • Sex is biology and physiology • Gender is a social and culture construct • Gender Role • “sex-typical behaviors” e.g. toy preference • Sexual Orientation • sexual behaviors and attractions

7. Past MistakesFuture Directions • Nature vs Nurture • John Money • David and Brian Reimer • Early assumptions became the dominant viewpoint • How do we do things differently now? • Patient and Parent input • Long-term f/u – careful not to jump to conclusions • Consider additional outcome measures

10. Gender Assignment • “It is impossible to predict with complete confidence what gender any child will eventually come to identify with. Like all other children, children with DSDs are given an initial gender assignment as boys are girls.” • “But team members should be aware – and advise parents in relevant instances-that children with certain DSDs are more likely then the general population to feel that the gender assignment given to them at birth was incorrect.”

11. Gender Assignment • Factors to consider • This is stressful for families!!! • Help guide parents with information on • Underlying diagnosis • Genital appearance • Potential for fertility • Views of families • sometimes, cultural practices

12. Generalities on Gender Assignment and Adult Gender Identity • CAH 46XX and Complete AIS • >90% as female, less than 10% self-reassign • 5-alpha reductase deficiency • ~60% assigned female in infancy and virilizing at puberty live as males • Others: Partial AIS, androgen biosynthetic defects, & incomplete gonadal dysgenesis • Dissatisfaction with sex of rearing in ~ 25% whether raised as males or females

15. In Summary • Put the Patient First • Parents and children are our patients • New Guidelines Show Promise • It’s a work in progress • Tailor to your patient • Long-term Follow-Up is still needed • There’s a lot we don’t know!

16. Resources • Accord Alliance - http://www.accordalliance.org/home.html • University of Michigan DSD - http://www.med.umich.edu/yourchild/topics/dsd.htm#what • Helpful Animations “How the Body Works” - http://www.aboutkidshealth.ca/En/HowTheBodyWorks/SexDevelopmentAnOverview/Pages/default.aspx • From “Intersex” to “DSD” - http://www.intersexinitiative.org/articles/intersextodsd.html

17. Recommended Reading • Clinical guidelines for the Management of Disorders of Sexual Development in Childhood • PA Lee et al., Consensus Statement on Management of Intersex Disorders, Pediatrics, 118:2, August 2006 • PT Cohen-Kettensis, Psychosocial and psychosexual aspects of disorders of sex development, Best Practice & Research Clinical Endocrinology and Metabolism 24 (2010) 325-334 • Colapinto, J (2001) As Nature Made Him: The Boy Who Was Raised as a Girl • Eugenides, Jeffrey (2002) Middlesex

18. Thoughts & Discussion