C lin ica l G u id e lin e s

1 Clinical Guidelinesfor the Management ofDisorders of Sex Developmentin ChildhoodCONSORTIUM ON THE MANAGEMENT OF disorders OF SEX DEVELOPMENTC opyright 2006 Intersex Society of North AmericaFunded by The California Endowment and Arcus FoundationClinical Guidelinesfor the Management ofDisorders of Sex Developmentin ChildhoodCONSORTIUM ON THE MANAGEMENT OF disorders OF SEX DEVELOPMENTC opyright 2006 Intersex Society of North AmericaFunded by The California Endowment and Arcus Foundationclinical_cover_letter 3/3/06 8:53 AM Page 2 CLINICALGUIDELINESFOR THE MANAGEMENTOF DISORDERSOF SEXDEVELOPMENTIN CHILDHOODC onsortium on the Management of disorders of Sex DevelopmentPublished August 16, 2006 First EditionCopyright 2006 Intersex Society of North AmericaISBN: 0-9773201-1-1 Published by Intersex Society of North America (a 501(c)3 tax-exempt corporation)979 Golf Course Drive #282 Rohnert Park CA 94928 Website: handbook is the result of an extraordinary collaboration among clinicians who care for people with disorders of sexdevelopment (DSDs), parents of children with DSDs, adults with DSDs, and other patient advocates.

2 We are truly gratefulfor the assistance and support that has been provided to us in this work, and we hope that readers will use the associatedwebsite ( ) to give us feedback about are grateful for the participation of David Cameron, Peter Trinkl, and Esther Morris Leidolf in this project. However,they would like to make it known that they do not support the term disorders of Sex development . We would like to thank Yvonne Day ( ) for design assistance and Bob Stayton ( ) fortechnology assistance with document has been made possible by generous grants from The California Endowment ( ) and theArcus Foundation ( ) provided to the Intersex Society of North America ( ).Web-browseable and downloadable versions (both with clickable links) of Clinical Guidelines for the Management of Disordersof Sex development in Childhood and Handbook for Parents are available at no cost from AnthonyCARES Foundation, Millburn, NJCassandra L.

3 Aspinall MSW, LICSWC raniofacial Center, Seattle Children s Hospital;University of Washington, School of SocialWork, Seattle, WAArlene B. Baratz, MDMedical Advisor, Androgen InsensitivitySyndrome Support Group, Pittsburgh, PACharlotte Boney, MDPediatric Endocrinology and Metabolism, RhodeIsland Hospital, Providence, RIDavid R. Brown, MD, FACEP ediatric Endocrinology and Metabolism; StaffPhysician, Children s Hospitals and Clinics ofMinnesota, Minneapolis, MNWilliam Byne, MDPsychiatry, Mount Sinai Medical Center, NewYork, NYDavid CameronBoard of Directors, Intersex Society of NorthAmerica, San Francisco, CAMonica Casper, PhDMedical Sociology, Vanderbilt University,Nashville, TNAnita J. Catlin, DSNc, FNP, FAANN ursing and Ethics, Sonoma State University,Sonoma, CACheryl ChaseFounder and Executive Director, Intersex Societyof North America, Rohnert Park, CAKimberly Chu, LCSW, DCSWD epartment of Child & Adolescent Psychiatry,Mount Sinai Medical Center, New York, NYSarah Creighton, MD, FRCOGG ynecology, University College LondonHospitals, London, UKJorge J.

4 Daaboul, MDPediatric Endocrinology, The NemoursChildren s Clinic, Orlando, FLAlice Domurat Dreger, PhD (ProjectCoordinator and Editor)Medical Humanities and Bioethics, FeinbergSchool of Medicine, Northwestern University,Chicago, ILChristine Feick, MSWAnn Arbor, MIKaye Fichman, MDPediatric Endocrinology, Kaiser PermanenteMedical Group, San Rafael, CASallie Foley, MSWC ertified Sex Therapist, AASECT; Dept. SocialWork/Sexual Health, University of MichiganHealth Systems, Ann Arbor, MIJoel Frader, MD, MAGeneral Academic Pediatrics, Children sMemorial Hospital; Dept. Pediatrics andProgram in Medical Humanities & Bioethics,Feinberg School of Medicine, NorthwesternUniversity, Chicago, ILiiiJane GotoBoard of Directors, Intersex Society of NorthAmerica; Board of Directors, AndrogenInsensitivity Syndrome Support Group USA,Seattle, WAJanet GreenCo-Founder, Bodies Like Ours; Board ofDirectors, CARES Foundation; Board ofOverseers, Beth Israel Hospital; Board ofTrustees, Continuum Healthcare, New York,NYPhilip A.

5 Gruppuso, MDAssociate Dean of Medical Education, BrownUniversity; Pediatric Endocrinology, RhodeIsland Hospital, Providence, RIWilliam G. Hanley, BPSM emphis, TNDebora Rode HartmanBoard of Directors, Intersex Society of NorthAmerica, Swedesboro, NJGinny Hayes, CMAA ndrogen Insensitivity Syndrome SupportGroup, Hampton, VAApril Herndon, PhDIntersex Society of North America; GustavusAdolphus College, Saint Peter, MNKatrina Karkazis, PhD, MPHM edical Anthropology and Biomedical Ethics,Stanford University, Palo Alto, CADixie Korlutis, RNCrouse Hospital, Syracuse, NYEsther Morris LeidolfFounder and President, MRKH Organization;The Fenway Institute; Fenway CommunityHealth, Boston, MAKelly LeightFounder and Executive Director, CARESF oundation, Millburn, NJAngela Moreno LippertChair, Intersex Society of North America.

6 Androgen Insensitivity Syndrome SupportGroup, Coupeville, WALynnell Stephani LongChicago, ILBev Mill, RTR (retired)Androgen Insensitivity Syndrome SupportGroups of Western Canada and Australia,Nelson, BC, CanadaBarbara Neilson, MSW, RSWS ocial Work, Hospital for Sick Children,Toronto, ON, CanadaJohn M. Park, MDPediatric Urology, University of MichiganMedical School, Ann Arbor, MISharon Preves, PhDMedical Sociology, Hamline University, St. Paul,MNCharmian A. Quigley, MBBSS enior Clinical Research Physician, LillyResearch Laboratories, Indianapolis, INWilliam G. Reiner, MDDept. Urology/Sect. Pediatric Urology, Child & Adolescent Psychiatry,University of Oklahoma Health Sciences Center,Oklahoma City, OKivDavid E.

7 Sandberg, PhDPsychiatry/Pediatrics, University at Buffalo; TheWomen and Children s Hospital of Buffalo,Buffalo, NYJustine M. Schober, MDPediatric Urology, Hamot Medical Center, Erie,PAAron C. Sousa, MDGeneral Internal Medicine and Associate Deanfor Academic Affairs, College of HumanMedicine, Michigan State University, EastLansing, MIHerberta Smith, RN, PNP (retired)Kissimmee, FLEric Vilain, MD, PhDPediatrics/Urology/Human Genetics, DavidGeffen School of Medicine at UCLA, LosAngeles, CAMichael Walker, PhDFounder, Hypospadias & Epispadias Association,Auckland, New Zealand and Point Reyes, CANina Williams, PsyDRobert Wood Johnson Medical School, (Dept.,Psychiatry); Institute of Psychotherapy andPsychoanalysis of New Jersey, Highland Park,NJBruce Wilson, MDPediatric Endocrinology, DeVos Children sHospital, Grand Rapids, of this of of Patient-Centered Care for Team Objectives.

8 12 Team Leader, Team Coordinator, and Team Protocols for the Management of Newborns with Protocols for the Management of Children Diagnosed with DSDs after the and Support ..27 Timing of of Hormonal Stigma Related to Medical Care ..31 Telling the Importance of Sexual for Talking with References and and Journal Issues (Devoted to DSD Management)..42 Key Group 1 IntroductionPURPOSEOF THIS DOCUMENTThe purpose of these guidelines is to assist health care professionals in the provision of diagnosis,treatment, education, and support to children born with disorders of sex development (DSDs) and totheir families. While debates about the best way to care for patients with DSDs continue,1-7 thishandbook offers individuals and institutions a model aimed at minimizing the potential for harm topatients and their families.

9 It is therefore also designed to reduce the potential for liability and toimprove patient guidelines begin with the commonly-held assumption that the goal of DSD treatment is thelong-term physical, psychological, and sexual well-being of the approach is thereforetermed patient-centered. What follows is largely an ideal treatment scenario involving a well-organized, well-trained, andinstitutionally-supported multidisciplinary health care team. The authors of this document hope thatit will be used to refine, to foster, and even to create such ideal scenarios, but also recognize that thereality for many health care professionals may be is critical, therefore, to emphasize that it is more important to focus on providing patient-centeredcare than to create and maintain a multidisciplinary team precisely as described in the dedicated multidisciplinary teams which focus on patients psychosocial well-being may bemore likely than alternatives to provide optimal care.

10 A dedicated multidisciplinary team is neithera guarantor of nor a necessity of patient-centered care for intended audience for this handbook is health care professionals who provide care to pediatricpatients with DSDs. Nonetheless, we expect that this handbook will also be read by health professionalstudents, educators, parents of children with DSDs, and adults with DSDs. For this reason, thishandbook occasionally repeats points which would be obvious to the specialist clinician. The authors1do not intend to be condescending to the specialist, but rather to be helpful to those readers less familiarwith the specialist s DSDsDisorders of sex development (DSDs) are defined as conditions involving the following elements.