Introductions to Intersex Traits: What is Androgen Insensitivity Syndrome?

This is the second in a series of posts in which I cover the basics of a particular intersex trait. For the purposes of this blog an intersex trait is defined as a bodily characteristic or set of bodily characteristic that have been determined to not be standard for male or female. This determination can be primarily chromosomal, hormonal, gonadal, and/or genital. Some examples include Turner syndrome, Congenital Androgen Hyperplasia, and cliormegaly. In this post I will focus on Androgen Insensitivity Syndrome (AIS).

AIS is an inherited intersex condition (expect for the occasional spontaneous mutation) that occurs in approximately 1 in 20,000 live births and results in partial to complete inability of the body’s cells to respond to androgens, which are the hormones commonly associated with male physiological development. Depending on the extent of the insensitivity, AIS can present in a variety of ways. Complete Androgen Insensitivity Syndrome (CAIS) in most cases results in a 46XY karyotype, undescended or partially descended testes, a short vagina without a cervix and no uterus or fallopian tubes. In some cases infants born with CAIS do not show obvious signs of the condition until puberty and are assigned female at birth without any question. In other cases the signs of the condition are obvious from birth. During puberty those with complete CAIS develop breasts as the body converts some testosterone into estrogen. However, they do not start a menstrual cycle without a uterus. Also, due to their insensitivity to testosterone, they grow little to no armpit and pubic and do not have any acne. Partial Androgen Insensitivity (PAIS) often results in an individual with a 46XY karyotype and ambiguous genitalia that can either be described as a large clitoris or a small penis. Here it is also important to note PAIS may actually be quite common and has been suggested to be the cause of infertility in many men whose genitals are typical in appearance.

Some of the most common medical responses to AIS involve medically unnecessary surgical interventions. These procedures involve removing internal testes and/or modifying genitals to appear more male or female. Along with these surgical interventions, there is also a history of parents and medical professionals refusing to disclose an AIS diagnosis to affected individuals. As well-intentioned as these responses may be they are actually problematic for many reasons, the most obvious being the fact these surgical interventions are mostly performed on infants and children who cannot consent. While it is true removal of internal testes is advisable in some instances due to cancer risks, cases of testicular cancer before puberty are extremely rare and there is no reason why such surgeries cannot be delayed until the individual is an adult with the ability to make an informed decision. As for genital surgeries, being born with genitals deemed to be ambiguous does not pose any physical dangers in and of itself. Unless the genitals in question have formed in such a way that bodily functions like urination are disrupted and the child’s life is threatened there is no medical reason to modify an infant’s genitals. Thus the reasons behind the surgeries performed on infants and children with AIS and other intersex conditions are purely cosmetic and often rooted in cisheteropartriarchy. A good example of this is the practice of lengthening the vaginas of infants and children with CAIS. The most commonly cited reason for vaginoplasty in these cases is to enable the female-assigned infant to one day have cis/heteronormative, penetrative sex as an adult. Under this line of reasoning there is absolutely no consideration of the various ways the individual’s gender and sexuality may develop over time let alone any concern for their future sexual pleasure should the procedure fail. After all she’s going to grow up to be a married woman and it’s all about her future husband’s pleasure, right? Once again such surgeries should not be imposed on anyone. Instead they should be offered later when the person is able to make an informed decision.

Beyond the physical and mental repercussions of nonconsensual surgeries there are the psychological consequences of secrecy regarding a diagnosis of AIS. While nondisclosure is done in the interest of sparing the individual unnecessary upset and confusion the actual lived experiences of those in the AIS community indicate such secrecy is actually harmful. Most people diagnosed with AIS who were not told of their condition have reported feelings of shame and confusion upon discovering they have AIS and advise full and complete disclosure from the beginning. A prominent example of this can be found in the narrative of Georgiann Davis, who tells of her experiences with AIS, nonconsensual childhood surgeries, and nondisclosure in Contesting Intersex: The Dubious Diagnosis as part of her investigation into intersexuality, intersex rights movements, and current diagnostic nomenclature. Along with her own story Davis also includes insights from interviews she conducted with intersex people on their experiences with medical terminology and practices.

Rather than approach AIS with secrecy and nonconsensual, medically unnecessary surgeries medical professionals, families, and individuals with AIS should work together for the well-being of those diagnosed with AIS in ways that respect their bodily autonomy and humanity. This means ending the imposition of medically unnecessary surgeries on infants and children and creating supportive environments free of shame, stigma, and secrecy. Above all individuals with AIS should take the lead in these efforts, challenging abuses of medical authority as necessary and supporting one another.

Access to support groups has also proven to be helpful for many individuals with AIS and their families, especially when first adjusting to a new diagnosis. An excellent example of such a group working today is the DSD-AIS Support Group. In this case DSD stands for Differences in Sex Development as opposed to the highly medicalized Disorders of Sex Development terminology that has been highly debated within the intersex community over the past decade. The group started out as a support group for women with AIS, but recent decades has branched out into a larger support group with a mission of, “Promoting support, education, and outreach to foster healthy outcomes for adults, youth, children, and families affected by Intersex/Differences of Sex Development.” This includes not only intersex women with AIS, but also intersex men, transgender, and nonbinary people with AIS and anyone else with an intersex trait and their families. Such support groups help individuals and families realize they are not alone, find resources, and exchange information. They can also be valuable educational spaces for medical professionals and others who wish to be allies to the intersex community.

Sources and Further Reading 

  1. http://www.isna.org/faq/conditions/ais
  2. http://www.isna.org/faq/conditions/pais
  3. https://www.childrens.com/specialties-services/specialty-centers-and-programs/gynecology/what-we-treat/androgen-insensitivity-syndrome
  4. http://aisdsd.org/
  5. Contesting Intersex: The Dubious Diagnosis Georgiann Davis

 

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Book Review–Contesting Intersex: The Dubious Diagnosis by Georgiann Davis

Georgiann Davis’ Contesting Intersex: The Dubious Diagnosis is a compelling sociological assessment of the intersex rights movement in the United States and is an excellent read for anyone who wants to learn more about the current state of intersex activism. Using insights from the sociology of diagnosis, interviews with various stakeholders in intersex health care, and the work of major intersex organizations Davis effectively argues the medical profession introduced Disorders of Sex Development (DSD) nomenclature in order to reassert their jurisdiction over the intersex body after activists had successfully challenged their authority.

In her examination of the strategies currently employed within major intersex organizations such as Accord Alliance and the AIS-DSD Support Group Davis finds today’s intersex activists tend to take on strategies of contested collaboration with medical professionals rather than collectively confront medical authority like they have in the past. As part of this shift many organizations focused on intersex activism have adopted DSD nomenclature over the last decade in spite of how it inevitably pathologizes intersex bodies. While these less confrontational strategies have been useful for opening up conversations between intersex activists and the medical professionals who wish to care for them as well as for increasing access to biological citizenship for certain intersex people, there is always the danger that working with medical professionals may turn into working for medical professionals. Meanwhile intersex infants and children are still subjected to medically unnecessary surgeries with lifelong consequences despite formal changes on paper strongly advising against such “treatments.” Only now medical professionals can justify these practices in reframing their actions as part of treating abnormalities under the umbrella term Disorders of Sex Development. These findings act as a warning to activists that while collaborative strategies such as the use of DSD nomenclature may get us in the room and help some of us gain access to biological citizenship they can easily be co-opted, used to strengthen medical jurisdiction over intersex bodies , and create divisions within the community. To avoid these pitfalls Davis recommends cautious flexibility when it comes to terminology choices and collaborating with medical professionals and argues for strategic combinations of confrontational and collaborative strategies when seeking to transform intersex medical care.

Along with this analysis Davis also offers her own perspective as an intersex person diagnosed with Androgen Insensitivity Syndrome. Throughout her analysis Davis shares aspects of her own experiences with intersexuality and how her personal experiences with the topic affect her work as a sociologist. This makes Contesting Intersex a very personal work as well as an academic one. Such a personal touch is excellent for readers of similar intersex experiences.

Then there is the analysis of the various views and linguistic strategies of intersex people. Over the course of the research process Davis interviews members of various intersex organizations on their views concerning terminology. These views ranged from those who take on the new DSD nomenclature to those who embrace an intersex identity to those who are flexible in their language choices. Together these interviews yield important insights into the wider debate over terminology within the community at a macro-level as well as how individuals approach language on a micro-level. This includes the benefits and drawbacks of each position for individuals and communities, especially when interacting with families and medical professionals. The one limitation of these interviews is the participants are predominately white, upper to middle class, college educated adults with access to support group memberships, conferences, medical services, and the internet. Hence the issues intersex people who do share in these privileges are left out. However, even with these limitations, this portion of Davis’s investigation does generate questions for further research and offers important insights into intersex issues and community at this point in history.

Beyond the intersex community other stakeholders in intersex health care may find Contesting Intersex valuable for expanding their knowledge of the community and what it means to be intersex. This includes parents and medical professionals brave enough to listen. For this reason I would recommend Contesting Intersex  to those who are intersex  as well as those who love them and wish to care for them.