Stigma Associated with Classical Congenital Adrenal Hyperplasia in Women’s Sexual Lives

Abstract

The risk of intersex-related stigma often serves as social indication for “corrective” genital surgery, but has not been comprehensively documented. In preparation for the development of an intersex-specific stigma assessment tool, this qualitative project aimed to explore stigma in girls and women with classical congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency. As part of a comprehensive follow-up project, 62 adult women with classical CAH (age range 18–51 years) took part in an open-ended retrospective interview focusing on the impact of CAH and its treatment on various aspects of girls’ and women’s lives. Deductive qualitative content analysis (Patton, 2014) of de-identified transcripts involved categorization of three types of stigma: experienced, anticipated, and internalized. Two-fifths of the participants reported CAH-related stigma in romantic/sexual situations. Stigma enactment by romantic partners occurred in reaction to both genital and non-genital sex-atypical features of CAH and sometimes included explicit questioning of the women’s true gender. Stigma anticipation by the women and their related avoidance of nudity, genital exposure, and romantic involvement altogether were frequent. Internalization of stigma occurred as well. In conclusion, the data suggest that many women with CAH experience, anticipate, and/or internalize intersex-related stigma in the context of their romantic/sexual lives.

This is a preview of subscription content, log in to check access.

References

  1. Binet, A., Lardy, H., Geslin, D., Franҫois-Fiquet, C., & Poli-Merol, M. L. (2016). Should we question early feminizing genitoplasty for patients with congenital adrenal hyperplasia and XX karyotype? Journal of Pediatric Surgery, 51, 465–468. doi:10.1016/j.jpedsurg.2015.10.004.

    Article  PubMed  Google Scholar 

  2. Bougnères, P., Bouvattier, C., Cartigny, M., & Michala, L. (2017). Deferring surgical treatment of ambiguous genitalia into adolescence in girls with 21-hydroxylase deficiency: A feasibility study. International Journal of Pediatric Endocrinology, 2017, 3. doi:10.1186/s13633-016-0040-8.

    Article  PubMed  PubMed Central  Google Scholar 

  3. Cools, M., Simmonds, M., Elford, S., Gorter, J., Ahmed, S. F., D’Alberton, F., … Management Committee of the European Cooperation in Science and Technology Action BM 1303. (2016). Response to the Council of Europe Human Rights Commissioner’s issue paper on human rights and intersex people. European Urology, 70, 407–409. doi:10.1016/j.eururo.2016.05.015.

  4. Davis, G. (Ed.). (2015). Narrative symposium: Intersex. Narrative Inquiry in Bioethics, 5, 87–150.

    Article  PubMed  Google Scholar 

  5. Diamond, M., & Garland, J. (2014). Evidence regarding cosmetic and medically unnecessary surgery on infants. Journal of Pediatric Urology, 10, 2–7. doi:10.1016/j.jpurol.2013.10.021.

    Article  PubMed  Google Scholar 

  6. Dreger, A. D. (Ed.). (1999). Intersex in the age of ethics. Hagerstown, MD: University Publishing Group.

    Google Scholar 

  7. Earnshaw, V. A., & Quinn, D. M. (2011). The impact of stigma in healthcare on people living with chronic illnesses. Journal of Health Psychology, 17, 157–168. doi:10.1177/1359105311414952.

    Article  PubMed  Google Scholar 

  8. Earnshaw, V. A., Quinn, D. M., Kalichman, S. C., & Park, C. L. (2013). Development and psychometric evaluation of the Chronic Illness Anticipated Stigma Scale. Journal of Behavioral Medicine, 36, 270–282. doi:10.1007/s10865-012-9422-4.

    Article  PubMed  Google Scholar 

  9. Feder, E. K. (2006). “In their best interests.” Parents’ experience of atypical genitalia. In E. Parens (Ed.), Surgically shaping children. Technology, ethics, and the pursuit of normality (pp. 189–210). Baltimore, MD: The Johns Hopkins University Press.

    Google Scholar 

  10. Frank, J. D., Mouriquand, P., Caldamone, A., & Malone, P. (2014). [Editorial]. Journal of Pediatric Urology, 10, 405. doi:10.1016/j.jpurol.2014.04.004.

  11. Frisén, L., Nordenström, A., Falhammar, H., Filipsson, H., Holmdahl, G., Janson, P. O., … Nordenskjöld, A. (2009). Gender role behavior, sexuality, and psychosocial adaptation in women with congenital adrenal hyperplasia due to CYP21A2 deficiency. Journal of Clinical Endocrinology and Metabolism, 94, 3432–3439. doi:10.1210/jc.2009-0636.

  12. Goffman, E. (1963). Stigma: Notes on the management of spoiled identity. Englewood Cliffs, NJ: Prentice Hall.

    Google Scholar 

  13. Hatzenbuehler, M. L., Phelan, J. C., & Link, B. G. (2013). Stigma as a fundamental cause of population health inequalities. American Journal of Public Health, 103, 813–821. doi:10.2105/AJPH.2012.301069.

    Article  PubMed  PubMed Central  Google Scholar 

  14. Hollingshead, A. B. (1975). Four-factor index of social status. Unpublished manuscript, Department of Sociology, Yale University, New Haven, CT.

  15. Hughes, I. A., Houk, C., Ahmed, S. F., Lee, P. A., LWPES Consensus Group, & ESPE Consensus Group. (2006). Consensus statement on management of intersex disorders. Archives of Disease in Childhood, 91, 554–563.

    Article  PubMed  PubMed Central  Google Scholar 

  16. Institute of Medicine. (2011). The health of lesbian, gay, bisexual, and transgender people. Washington, DC: The National Academies Press.

    Google Scholar 

  17. Karkazis, K. (2008). Fixing sex: Intersex, medical authority, and lived experience. Durham, NC: Duke University Press.

    Google Scholar 

  18. Lee, P. A., & Houk, C. P. (2014). Commentary to ‘Evidence regarding cosmetic and medically unnecessary surgery on infants’. Journal of Pediatric Urology, 10, 7. doi:10.1016/j.jpurol.2013.11.006.

    Article  PubMed  Google Scholar 

  19. Lewis, V. G., Ehrhardt, A. A., & Money, J. (1970). Genital operations in girls with adrenogenital syndrome. Obstetrics and Gynecology, 36, 11–15.

    PubMed  Google Scholar 

  20. Lindemann, H., Feder, E. K., & Dreger, A. (2010). Fetal cosmetology. http://www.thehastingscenter.org/Bioethicsforum/Post.aspx?id=4470. Accessed Feb 14, 2010.

  21. Major, B., & O’Brien, L. T. (2005). The social psychology of stigma. Annual Review of Psychology, 56, 393–421.

    Article  PubMed  Google Scholar 

  22. Masnari, O., Schiestl, C., Rössler, J., Gütlein, S. K., Neuhaus, K., Weibel, L., … Landolt, M. A. (2013). Stigmatization predicts psychological adjustment and quality of life in children and adolescents with a facial difference. Journal of Pediatric Psychology, 38, 162–172. doi:10.1093/jpepsy/jss106.

  23. Meyer-Bahlburg, H. F. L. (2002). Gender assignment and reassignment in intersexuality: Controversies, data, and guidelines for research. Advances in Experimental Medicine and Biology, 511, 199–223.

    Article  PubMed  Google Scholar 

  24. Meyer-Bahlburg, H. F. L. (2014). Psychoendocrinology of congenital adrenal hyperplasia. In M. I. New, O. Lekarev, A. Parsa, T. T. Yuen, B. O’Malley, & G. D. Hammer (Eds.), Genetic steroid disorders (pp. 285–300). San Diego: Academic Press/Elsevier.

    Google Scholar 

  25. Meyer-Bahlburg, H. F. L., Dolezal, C., Baker, S. W., Ehrhardt, A. A., & New, M. I. (2006). Gender development in women with congenital adrenal hyperplasia as a function of disorder severity. Archives of Sexual Behavior, 35, 667–684.

    Article  PubMed  Google Scholar 

  26. Meyer-Bahlburg, H. F. L., Khuri, J., Reyes-Portillo, J., & New, M. I. (2017a). Stigma in medical settings as reported retrospectively by women with congenital adrenal hyperplasia (CAH) for their childhood and adolescence. Journal of Pediatric Psychology, 42, 496–503. doi:10.1093/jpepsy/jsw034.

    PubMed  Google Scholar 

  27. Meyer-Bahlburg, H. F. L., Reyes-Portillo, J., Khuri, J., Ehrhardt, A. A., & New, M. I. (2017b). Syndrome-related stigma in the general social environment as reported by women with classical congenital adrenal hyperplasia. Archives of Sexual Behavior, 46, 341–351. doi:10.1007/s10508-016-0862-8.

    Article  PubMed  Google Scholar 

  28. Money, J. (1991). Biographies of gender and hermaphroditism in paired comparisons. New York, NY: Elsevier Science Publishers B. V. (Biomedical Divisions).

  29. Money, J., Hampson, J. G., & Hampson, J. L. (1955). Hermaphroditism: Recommendations concerning assignment of sex, change of sex, and psychologic management. Bulletin of the Johns Hopkins Hospital, 97, 284–300.

    PubMed  Google Scholar 

  30. Mouriquand, P. D., Gorduza, D. B., Gay, C. L., Meyer-Bahlburg, H. F., Baker, L., Baskin, L. S., … Lee, P. (2016). Surgery in disorders of sex development (DSD) with a gender issue: If (why), when, and how? Journal of Pediatric Urology, 12, 139–149. doi: 10.1016/jpurol.2016.04.001.

  31. New, M. I., Lekarev, O., Mancenido, D., Parsa, A., & Yuen, T. (2014). Congenital adrenal hyperplasia owing to 21-hydroxylase deficiency. In M. I. New, O. Lekarev, A. Parsa, T. T. Yuen, B. O’Malley, & G. D. Hammer (Eds.), Genetic steroid disorders (pp. 29–51). London, Waltham, San Diego: Academic Press/Elsevier.

    Google Scholar 

  32. O’Connor, M. (2016). The treatment of intersex and the problem of delay: The Australian senate inquiry into intersex surgery and conflicting human rights for children. Journal of Law, Medicine & Ethics, 23, 531–543.

    Google Scholar 

  33. Oliveira, Mde S., de Paiva-e-Silva, R. B., Guerra-Junior, G., & Maciel-Guerra, A. T. (2015). Parents’ experiences of having a baby with ambiguous genitalia. Journal of Pediatric Endocrinology and Metabolism, 28, 833–838. doi:10.1515/jpem-2014-0457.

    Article  Google Scholar 

  34. Patton, M. Q. (2014). Qualitative research & evaluation methods: Integrating theory and practice (4th ed.). Thousand Oaks, CA: Sage Publications.

    Google Scholar 

  35. Preves, S. E. (2003). Intersex and identity: The contested self. Piscataway, NJ: Rutgers University Press.

    Google Scholar 

  36. Rolston, A. M., Gardner, M., Vilain, E., & Sandberg, D. E. (2015). Parental reports of stigma associated with child’s disorder of sex development. International Journal of Endocrinology, 980121. doi:10.1155/2015/980121.

  37. Rosenfeld, A., Siegel-Gorelick, B., Haavik, D., Duryea, M., Wenegrat, A., Martin, J., & Bailey, R. (1984). Parental perceptions of children’s modesty: A cross-sectional survey of ages two to ten years. Psychiatry, 47, 351–365.

    Article  PubMed  Google Scholar 

  38. Speiser, P. W., Azziz, R., Baskin, L. S., Ghizzoni, L., Hensle, T. W., Merke, D. P., … White, P. C. (2010). Congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency: An Endocrine Society clinical practice guideline. Journal of Clinical Endocrinology and Metabolism, 95, 4133–4160. doi:10.1210/jc.2009-2631.

  39. Suorsa, K. I., Mullins, A. J., Tackett, A. P., Reyes, K. J., Austin, P., Baskin, L., … Mullins, L. L. (2015). Characterizing early psychosocial functioning of parents of children with moderate to severe genital ambiguity due to disorders of sex development. Journal of Urology, 194, 1737–1742. doi:10.1016/j.juro.2015.06.104.

  40. Wisniewski, A. B. (2017). Psychosocial implications of disorders of sex development treatment for parents. Current Opinion in Urology, 27, 11–13. doi:10.1097/MOU.0000000000000344.

    Article  PubMed  PubMed Central  Google Scholar 

  41. Wisniewski, A. B., & Sandberg, D. E. (2015). Parenting children with disorders of sex development (DSD): A developmental perspective beyond gender. Hormone and Metabolic Research, 47, 375–379. doi:10.1055/s-0034-1398561.

    Article  PubMed  Google Scholar 

Download references

Acknowledgements

The study was supported in part by USPHS Grant HD-38409 and Dr. Khuri by a postdoctoral fellowship T32 MH18264. Susan W. Baker, Ph.D., served as the primary interviewer. We thank all participants for their contribution of effort, time, and data to this study.

Author information

Affiliations

Authors

Corresponding author

Correspondence to Heino F. L. Meyer-Bahlburg.

Rights and permissions

Reprints and Permissions

About this article

Verify currency and authenticity via CrossMark

Cite this article

Meyer-Bahlburg, H.F.L., Khuri, J., Reyes-Portillo, J. et al. Stigma Associated with Classical Congenital Adrenal Hyperplasia in Women’s Sexual Lives. Arch Sex Behav 47, 943–951 (2018). https://doi.org/10.1007/s10508-017-1003-8

Download citation

Keywords

  • Stigma
  • Disorders of sex development
  • Intersexuality
  • Congenital adrenal hyperplasia