Promoting Shared Decision Making in Disorders of Sex Development (DSD): Decision Aids and Support Tools

Abstract

Specific complaints and grievances from adult patients with disorders of sex development (DSD), and their advocates center around the lack of information or misinformation they were given about their condition and feeling stigmatized and shamed by the secrecy surrounding their condition and its management. Many also attribute poor sexual function to damaging genital surgery and/or repeated, insensitive genital examinations. These reports suggest the need to reconsider the decision-making process for the treatment of children born with DSD. This paper proposes that shared decision making, an important concept in adult health care, be operationalized for the major decisions commonly encountered in DSD care and facilitated through the utilization of decision aids and support tools. This approach may help patients and their families make informed decisions that are better aligned with their personal values and goals. It may also lead to greater confidence in decision making with greater satisfaction and less regret. A brief review of the past and current approach to DSD decision making is provided, along with a review of shared decision making and decision aids and support tools. A case study explores the need and potential utility of this suggested new approach.

Key words

disorders of sex development - shared decision making - decision aids - decisional regret - genital surgery - gender assignment - disclosure

• References

• 1 Lee PA, Houk CP, Ahmed SF, Hughes IA. in collaboration with the participants in the International Consensus Conference on Intersex. Consensus statement on management of intersex disorders Pediatrics. 2006; 118: e488-e500
• 2 Sandberg DE, Mazur T. A noncategorical approach to the psychosocial care of persons with dsd and their families. In: Kreukels BPC, Steensma TD, de Vries ALC. (eds.) Gender dysphoria and disorders of sex development. New York: Springer; 2014: 93-114
• 3 Zucker KJ. Intersexuality and gender identity differentiation. Ann Rev Sex Res 1999; 10: 1-69
• 4 Money J, Hampson JG, Hampson JL. Imprinting and the establishment of gender role. AMA Arch Neurol Psychiatr 1957; 77: 333-336
• 5 Creighton S, Minto C. Managing intersex. Most vaginal surgery in childhood should be deferred. Br Med J 2001; 323: 1264-1265
• 6 Section on Urology, American Academy of Pediatrics . Timing of elective surgery on the genitalia of male children with particular reference to the risks, benefits, and psychological effects of surgery and anesthesia. Pediatrics 1996; 97: 590-594
• 7 Creighton S, Chernausek SD, Romao R, Ransley P, Salle JP. Timing and nature of reconstructive surgery for disorders of sex development – introduction. J Pediatr Urol 2012; 8: 602-610
• 8 Mouriquand P, Caldamone A, Malone P, Frank JD, Hoebeke P. The ESPU/SPU standpoint on the surgical management of disorders of sex development (DSD). J Pediatr Urol 2014; 10: 8-10
• 9 Stein MT, Sandberg DE, Mazur T, Eugster E, Daaboul J. A newborn infant with a disorder of sexual differentiation. J Develop Behav Pediatr 2003; 24: 115-119
• 10 Karkazis K, Tamar-Mattis A, Kon AA. Genital surgery for disorders of sex development: Implementing a shared decision-making approach. J Pediatr Endocrinol Metab 2010; 23: 789-805
• 11 Frader J, Alderson P, Asch A, Aspinall C, Davis D, Dreger A, Edwards J, Feder EK, Frank A, Hedley LA, Kittay E, Marsh J, Miller PS, Mouradian W, Nelson H, Parens E. Health care professionals and intersex conditions. Arch Pediatr Adolesc Med 2004; 158: 426-428
• 12 Diamond M, Sigmundson HK. Management of intersexuality. Guidelines for dealing with persons with ambiguous genitalia. Arch Pediatr Adolesc Med 1997; 151: 1046-1050
• 13 Diamond M, Sigmundson HK. Sex reassignment at birth. Long-term review and clinical implications. Arch Pediatr Adolesc Med 1997; 151: 298-304
• 14 Reiner WG, Gearhart JP. Discordant sexual identity in some genetic males with cloacal exstrophy assigned to female sex at birth. N Engl J Med 2004; 350: 333-341
• 15 Dessens AB, Slijper FM, Drop SL. Gender dysphoria and gender change in chromosomal females with congenital adrenal hyperplasia. Arch Sex Behav 2005; 34: 389-397
• 16 Cohen-Kettenis PT. Gender change in 46,XY persons with 5a-reductase-2 deficiency and 17b-hydroxysteroid dehydrogenase-3 deficiency. Arch Sex Behav 2005; 34: 399-410
• 17 Meyer-Bahlburg HFL. Gender identity outcome in female-raised 46,XY persons with penile agenesis, cloacal exstrophy of the bladder, or penile ablation. Arch Sex Behav 2005; 34: 423-438
• 18 Dreger AD. Hermaphrodites and the medical invention of sex. Cambridge MA: Harvard University Press; 1998
• 19 Chase C. What is the agenda of the intersex patient advocacy movement?. Endocrinologist 2003; 13: 240-242
• 20 Crouch NS, Liao LM, Woodhouse CRJ, Conway GS, Creighton SM. Sexual function and genital sensitivity following feminizing genitoplasty for congenital adrenal hyperplasia. J Urol 2008; 179: 634-638
• 21 Crouch NS, Minto CL, Laio L-M, Woodhouse CRJ, Creighton SM. Genital sensation after feminizing genitoplasty for congenital adrenal hyperplasia: A pilot study. BJU Int 2004; 93: 135-138
• 22 Money J, Lamacz M. Genital examination and exposure experienced as nosocomial sexual abuse in childhood. J Nervous Mental Dis 1987; 175: 713-721
• 23 Creighton S, Alderson J, Brown S, Minto CL. Medical photography: Ethics, consent and the intersex patient. BJU Int 2002; 89: 67-71
• 24 Pasterski V, Prentice P, Hughes IA. Consequences of the chicago consensus on disorders of sex development (DSD): Current practices in europe. Arch Dis Child 2010; 95: 618-623
• 25 Moran ME, Karkazis K. Developing a multidisciplinary team for disorders of sex development: Planning, implementation, and operation tools for care providers. Adv Urol 2012; 2012: 604135
• 26 Charles C, Gafni A, Whelan T. Decision-making in the physician-patient encounter: Revisiting the shared treatment decision-making model. Soc Sci Med 1999; 49: 651-661
• 27 Charles C, Gafni A, Whelan T. Shared decision-making in the medical encounter: What does it mean? (or it takes at least two to tango). Soc Sci Med 1997; 44: 681-692
• 28 Wisniewski AB, Sandberg DE. Parenting children with DSD: A developmental perspective beyond gender. [published online ahead of print February 5, 2015] Horm Metab Res 2015; DOI: doi: 10.1055/s-0034-1398561.
• 29 Pyke-Grimm KA, Degner L, Small A, Mueller B. Preferences for participation in treatment decision making and information needs of parents of children with cancer: A pilot study. J Pediatr Oncol Nurs 1999; 16: 13-24
• 30 Miller VA, Nelson RM. Factors related to voluntary parental decision-making in pediatric oncology. Pediatrics 2012; 129: 903-909
• 31 Laventhal NT, Shuchman M, Sandberg DE. Warning about warnings: Weighing risk and benefit when information is in a state of flux. Horm Res Pædiatr 2013; 79: 4-8
• 32 O’Connor AM, Rostom A, Fiset V, Tetroe J, Entwistle V, Llewellyn-Thomas H, Holmes-Rovner M, Barry M, Jones J. Decision aids for patients facing health treatment or screening decisions: Systematic review. BMJ 1999; 319: 731-734
• 33 Whelan TJ, O’Brien MA, Villasis-Keever M, Robinson P, Skye A, Gafni A, Brouwers M, Charles C, Baldassarre F, Gauld M. Impact of cancer-related decision aids. Evidence reports/technology assessments. no. 46 Rockville, MD: Agency for Healthcare Research and Quality; 2002
• 34 Holmes-Rovner M, Wills CE. Improving informed consent insights from behavioral decision research. Med Care 2002; 40: V30-V38
• 35 Siminoff LA, Step MM. A communication model of shared decision making. Accounting for cancer treatment decisions. Health Psychol 2005; 24: S106-S110
• 36 Feldman-Stewart D, Tong C, Siemens R, Alibhai S, Pickles T, Robinson J, Brundage MD. The impact of explicit values clarification exercises in a patient decision aid emerges after the decision is actually made. Med Decis Making 2012; 32: 616-626
• 37 Boyse KL, Gardner M, Marvicsin DJ, Sandberg DE. “It was an overwhelming thing”: Parents’ needs after infant diagnosis with congenital adrenal hyperplasia. J Pediatr Nurs 2014; 29: 436-441
• 38 Duguid A, Morrison S, Robertson A, Chalmers J, Youngson G, Ahmed SF. The Scottish Genital Anomaly Network . The psychological impact of genital anomalies on the parents of affected children. Acta Pædiatr 2007; 96: 348-352
• 39 Crissman H, Warner L, Gardner M, Carr M, Schast A, Quittner A, Kogan B, Sandberg D. Children with disorders of sex development: A qualitative study of early parental experience. Int J Pediatr Endocrinol 2011; 2011 10 pages http://www.ijpeonline.com/content/2011/2011/2010
• 40 Streuli JC, Vayena E, Cavicchia-Balmer Y, Huber J. Shaping parents: Impact of contrasting professional counseling on parents’ decision making for children with disorders of sex development. J Sex Med 2013; 10: 1953-1960
• 41 Sanders C, Carter B, Goodacre L. Parents’ narratives about their experiences of their child’s reconstructive genital surgeries for ambiguous genitalia. J Clin Nurs 2008; 17: 3187-3195
• 42 Sanders C, Carter B, Goodacre L. Searching for harmony: Parents’ narratives about their child’s genital ambiguity and reconstructive genital surgeries in childhood. J Adv Nurs 2011; 67: 2220-2230
• 43 Hiort O, Thyen U, Holterhus PM. The basis of gender assignment in disorders of somatosexual differentiation. Horm Res 2005; 64: 18-22
• 44 Lorenzo AJ, Pippi Salle JL, Zlateska B, Koyle MA, Bagli DJ, Braga LH. Decisional regret after distal hypospadias repair: Single institution prospective analysis of factors associated with subsequent parental remorse or distress. J Urol 2014; 191: 1558-1563
• 45 Lorenzo AJ, Braga LHP, Zlateska B, Leslie B, Farhat WA, Bägli DJ, Pippi Salle JL. Analysis of decisional conflict among parents who consent to hypospadias repair: Single institution prospective study of 100 couples. J Urol 2012; 188: 571-575
• 46 Sheehan J, Sherman KA. Computerised decision aids: A systematic review of their effectiveness in facilitating high-quality decision-making in various health-related contexts. Patient Educat Counsel 2012; 88: 69-86
• 47 Stacey D, Légaré F, Col N, Bennett C, Barry M, Eden K, Holmes-Rovner M, Llewellyn-Thomas H, Lyddiatt A, Thomson R, Trevena L, Wu J. Decision aids for people facing health treatment or screening decisions. Cochrane Database of Systematic Reviews 2014; Art. No.: CD001431 DOI: 10.1002/14651858.CD001431.pub4.
• 48 Wroe AL, Turner N, Owens RG. Evaluation of a decision-making aid for parents regarding childhood immunizations. Health Psychol 2005; 24: 539-547
• 49 Wallace C, Leask J, Trevena LJ. Effects of a web based decision aid on parental attitudes to mmr vaccination: A before and after study. BMJ 2006; 332: 146-149
• 50 Miller VA, Ittenbach RF, Harris D, Reynolds WW, Beauchamp TL, Luce MF, Nelson RM. The decision making control instrument to assess voluntary consent. Med Decis Making 2011; 31: 730-741
• 51 Wyatt K, Prutsky Lopez G, Domecq Garces J, Erwin P, Brinkman W, Montori V, LeBlanc A. Study protocol: A systematic review of pediatric shared decision making. System Rev 2013; 2: 48
• 52 International Patient Decision Aid Standards (IPDAS) Collaboration. http://ipdas.ohri.ca/ Accessed September 23, 2014
• 53 Elwyn G, O’Connor A, Stacey D, Volk R, Edwards A, Coulter A, Thomson R, Barratt A, Barry M, Bernstein S, Butow P, Clarke A, Entwistle V, Feldman-Stewart D, Holmes-Rovner M, Llewellyn-Thomas H, Moumjid N, Mulley A, Ruland C, Sepucha K, Sykes A, Whelan T. Developing a quality criteria framework for patient decision aids: Online international delphi consensus process. BMJ 2006; 333: 417