Intersex is an umbrella term that typically is used to refer to a wide variety of conditions in which a person (or another animal) is born with something other than the anatomy considered species-typical for males or females. A person born with genitals that are between the typical male and typical female types usually is considered intersex, as is a person born with the external anatomy of one sex but most of the internal anatomy of the other sex. Many people also consider intersex any person with atypical sex chromosomes, for example, a person with mosaic sex chromosomes in whom some cells contain XX (female-typical) chromosomes and the rest contain XY (male-typical) chromosomes or a person with more or fewer than two sex chromosomes in each cell. (Sex chromosomes is a misleading term because genes on other chromosomes also make critical contributions to sex development and sex physiology and because some genes on the sex chromosomes code for traits that are not related to sex development or sex physiology.) The term intersexuality generally is disfavored because it suggests that the issue is eroticism or sexual orientation (sexuality), when in fact intersex is primarily an issue of biology (sex).
DEFINITION AND INCIDENCE
In practice, intersex activists and advocates have used the definition provided in the first sentence of this entry but have recognized that the category remains fuzzy because of the imprecision of the concept of "species typical" and the challenge of deciding what counts as critical sex anatomy so that variation from it can constitute intersex. Many intersex activists and advocates have attempted to distinguish intersex from transgender, with the latter term typically referring to people for whom the gender category assigned to them at birth does not reflect their sense of self. Most intersex activists and advocates argue that in the absence of evidence that a person's congenital anatomy differed at birth from that of the standard male or standard female, that person should not be counted as intersex.
Approximately one in two thousand children are born with obvious genital ambiguity. Significantly more are born with intersex conditions that are less obvious and may not be detected until well into adulthood, if ever. Some but not all intersex conditions are associated with metabolic problems such as critical hormone imbalances and pathological development of the urinary system. For this reason, when intersex is suspected, expert physicians should be consulted. This is especially true for newborns because congenital genital ambiguity may signal a case of congenital adrenal hyperplasia (CAH), a condition that can be fatal if it is not treated.
Exactly which conditions count as intersex remains a subject of debate in medicine in large part because of disagreement about what constitutes a sufficiently significant deviation from the standard male or standard female. Some physicians claim that a stretched-penis length less than 2.5 centimeters at birth constitutes "micropenis" and a stretched-clitoris length greater than 1 centimeter at birth constitutes "clitoromegaly," and the patients of those physicians have been treated as intersex and subjected to "normalizing" genital surgeries. Other physicians consider small penises and large clitorises benign variations of masculinity and femininity. Similarly, some physicians consider as intersex only people whose brains were exposed prenatally to the hormone mix typical of the development of the "opposite" chromosomal sex, but many physicians find that definition inadequate because it limits intersex (and thus sex) to the prenatal history of the brain. Some physicians consider variations from the typical sex chromosome complements to be intersex—thus, they count people with XXY (Klinefelter syndrome) and X(O) (Turner syndrome) as intersex—but others disagree.
In the past the term hermaphrodite was used to refer both in medicine and in the general culture to many people with intersex. The popular imagination presumes that hermaphroditism endows a person with all the parts and functions of both males and females. However, this is a physical impossibility because one body cannot create and maintain both a complete complement of male sex anatomy and physiology and a complete complement of female sex anatomy and physiology.
In the late nineteenth century there was a medical consensus that people with sexual ambiguity who had testes would be called male pseudohermaphrodites, people with sexual ambiguity who had ovaries would be called female pseudohermaphrodites, and people with both ovarian and testicular tissue would be called true hermaphrodites. That terminology held sway for generations and was employed in a well-known and politically important 1993 article, "The Five Sexes," published in The Sciences by the biologist and feminist critic Anne Fausto-Sterling. However, that terminology with its tripartite division has largely fallen out of favor because it lacks scientific rigor (it ignores many important aspects of sex other than gonadal structure), is clinically confusing (some people who are obviously girls and women may be labeled male pseudohermaphrodites, and people who are girls and boys may be labeled true hermaphrodites), and is unnecessarily stigmatizing.
Since the early 1990s the use of the alternative umbrella term intersex has enabled effective political organizing of people with diverse biological and medical histories. However, there has been the start of a movement in some sections of the medical and intersex activist/advocate communities to replace that umbrella term with disorders of sex development (DSDs). The reasons for this include the fact that (1) some people with DSDs and some parents of children with DSDs find that the term intersex stigmatizes them and implies gender confusion and (2) physicians cannot agree on what conditions they are talking about when they talk about intersex. Detractors object that the term DSDs implies that a person with intersex has a disorder.
TREATMENT OF INTERSEX IN THE PAST AND INTERNATIONALLY
The treatment of people with intersex has varied from culture to culture. In many times and places people with intersex apparently blended into the general population. For example, the American Billy Tipton (1914–1989) worked as a minor jazz artist without many contemporaries knowing that he was intersex. The small segment of the modern medical literature that has traced the lives of people who have grown up with "ambiguous" genitalia and other forms of intersex suggests that in general people with intersex have led largely unremarkable lives.
The Western cultural approach to intersex has been motivated largely by the desire to maintain heterosexual norms. Lorraine Daston and Katherine Park have found that in Renaissance France people labeled as hermaphrodites were required to adhere to one gender and partner only with someone of the other gender to avoid the appearance of homosexuality. Alice Dreger (1998, 1999), Christine Matta, and Elizabeth Reis have shown that a similar system was employed in Europe and the United States throughout the nineteenth century.
In other times and places "third sex" or "third gender" categories were available to (or possibly thrust upon) people with intersex and sometimes on nonintersex people who had same-sex attractions or in some other way did not fit the more common sex/gender categories. In India the caste of hijras includes some people born intersex as well as biological males who in the West would be called transgendered. Hijras typically live in groups and undergo ritual castration. They perform certain religious ceremonies for the non-Hijra population and have a relatively low social status. Before colonialism certain Native American cultures included people called "two-spirit" (labeled by anthropologists as berdache). Two-spirit individuals appear to have been intersex in some cases but often to have been people born non-intersex; the basic idea was that those individuals had both male and female spirits. They seem to have been considered to have had special strengths and powers, often performing particular spiritual ceremonies.
5-alpha-reductase deficiency is an enzyme deficiency that causes male fetuses to develop externally to look female. Thus, at birth they look mostly like girls. At puberty these children masculinize: Their clitorises grow to look more like penises, their testes descend into their labia, and their bodily conformation in terms of muscular, fat, and hair development looks classically masculine. Because of intermarriage the genetic frequency of 5-alpha-reductase deficiency is elevated in at least two cultures, and there categories have been created for affected individuals. Herdt traced what he said was called the guevedoche ("balls at twelve") system in the Dominican Republic, and Imperato-McGinley's work focused on the "turnim-man" of Papua New Guinea.
WELL-KNOWN CASES OF INTERSEX
Cal (also known as Caliope), the protagonist in Jeffrey Eugenides's Pulitzer Prize-winning novel Middlesex, has 5-alpha-reductase deficiency, and millions of people have come to know about intersex through Cal's story. However, since the advent of the modern gay and lesbian rights movement and then the intersex rights movement (in 1993) many people also have come to know the stories of real people with intersex. Michel Foucault published the memoirs of the French Herculine/Abel Barbin with an extensive commentary in 1980. Barbin was born in 1838 and died of suicide in 1868. Raised a girl, she ended up undergoing an essentially masculine puberty and falling in love with a woman. Exposed to the religious, medical, and legal authorities, she had her sex legally changed to male and became ensconced in scandal.
Probably the most famous case associated with intersex did not involve a person born intersex. David Reimer was born a nonintersex identical twin boy in 1965. When he was age eight months, a doctor botched a circumcision on Reimer and essentially burned off his penis. Reimer's parents were advised by John Money, a well-known psychologist at Johns Hopkins University, to sex reassign David surgically and raise him as a girl. Money saw the case as a perfect experiment to prove that gender is the result of nurture; after all, David was born nonintersex and had an identical male twin. Money reported again and again that the case—known as "John/Joan"—was a success and that his theory of gender identity development was therefore well supported.
Many years later the sex researcher Milton Diamond followed up that case and discovered that David had never settled into the female gender. Money had lied; his own records of the case show that David's behaviors, interests, and life differed dramatically from Money's public reports. When David had become suicidal as a teenager, his parents decided to tell him the truth about his medical history, and David immediately resumed life as a boy. He later married a woman and adopted her children. His story was documented in As Nature Made Him by John Colapinto. Reimer killed himself in 2004.
Cheryl Chase founded the intersex rights movement in 1993. Chase was born in 1956 with ambiguous genitalia, and her parents were advised to raise her as a boy. When she was eighteen months old, a different team of doctors decided she would be better off as a girl, and they removed her clitoris and explained to her parents that they had discovered she was "really" a girl. Even when she was an adult, Chase repeatedly was denied her medical history and records. As a lesbian woman Chase became politically conscious of queer rights as well as the women's health movement, and in her mid-thirties she began seeking out other people with intersex.
ADVOCACY AND ACTIVIST GROUPS
In 1993 Chase founded the Intersex Society of North America (ISNA), the first support and advocacy group for intersex people. The first national announcement of ISNA came in Chase's letter to The Sciences responding to Fausto-Sterling's "Five Sexes" article. The mission of the ISNA of "building a world free of shame, secrecy, and unwanted genital surgeries for people born with atypical sex anatomies" was mirrored by other groups that formed later.
Chase remains an internationally recognized scholar and advocate. Several other intersex scholar-activists have risen through the ranks of the movement, including Emi Koyama and Iain Morland. A number of nonintersex academic advocates have aided the intersex rights movement, including Alice Dreger, Anne Fausto-Sterling, Suzanne Kessler, and Sharon Preves. There are many diagnosis-specific support groups, including the CARES Foundation (for congenital adrenal hyperplasia) and the Androgen Insensitivity Syndrome Support Group (AISSG). Nationally based intersex advocacy groups have been founded around the world, including groups in Japan, Poland, New Zealand, South Africa, and Germany.
The intersex rights movement began largely as a response to the medical management of intersex in the United States. The approach used by John Money in the case of David Reimer was developed in the 1950s as a way to manage intersex children. Money, John Hampson, Joan Hampson, and other colleagues at Johns Hopkins University formulated what came to be known as the optimum gender of rearing model. That model held that gender is primarily a product of nurture and that as long as a child's social gender identity (including genital appearance) was stabilized as male or female before about age eighteen months and the child was raised unambiguously, the child would grow up to be a typical man or woman. Consistent with the long-running heterosexist approach to intersex in the West, at Hopkins the idealized outcome generally was understood as a straight and straight-acting man or woman.
Money and his colleagues believed that sex (i.e., physical) ambiguity necessarily would lead to gender (i.e., psychosocial) ambiguity, and so they recommended that children with intersex be subject early to "normalizing" genital surgeries designed to make their genitals appear typically male or typically female. Because surgeons found it easier to make a believable-looking girl than a believable-looking boy, most children with ambiguous genitalia were surgically constructed to appear female. (Cheryl Chase was treated according to this model.) Children who were subjected to removal of their gonads also were given hormone supplements at the age of puberty and beyond to further their sex-appropriate development.
Like Money, many medical professionals who treated children with intersex misled or misinformed their patients even when the patients reached the age of maturity. This appears to have been done out of the belief that knowledge of their intersex history would cause patients confusion or distress, but judging from the recollections of intersex people and their parents as well as older medical professionals, it probably also was done because medical professionals found intersex a shameful sexual matter.
Intersex activists and advocates raised numerous objections to the optimum gender of rearing model. Chief among their criticisms was that this system generally resulted in a shroud of shame and secrecy and, more specifically, that childhood genital surgeries interfered with the health and well-being (both physical and mental) of many patients. Critics also pointed out that the "standard of care" subjected children to irreversible surgeries and hormone treatments because of specious social norms regarding sex, gender, and sexuality; did not address parental distress and anxiety directly; treated children labeled boys and those labeled girls according to asymmetrical values (sexual potency was valued for boys, and reproductive potential and sexual receptiveness for girls); was not based on meaningful evidence that it was necessary, safe, or effective; and condoned and sometimes even seemed to require deception of patients.
For some time the medical establishment largely ignored the criticisms of the intersex rights movement or misunderstood them, believing that activists were upset because they felt they had been assigned the wrong gender. In fact, relatively few people with intersex feel that the gender assignment given to them at birth was the wrong one, and the critiques of the optimum gender of rearing model went well beyond a criticism of Money's nurture-heavy theory of gender.
Within a few years of the founding of ISNA and the intersex rights movement, advocates managed to cultivate a few allies in the medical profession. The most focused and influential of them have been the American pediatric urologist and surgeon Justine Schober, the British gynecologist Sarah Creighton, and the American pediatric urologist turned pediatric psychiatrist William Reiner. More recently, in the United States the French-born geneticist and pediatric urologist Eric Vilain has been influential in arguing for improved treatment of intersex children, including an understanding of the prenatal development of brains in terms of gender identity.
In the early twenty-first century the medical establishment recognizes some of the core problems with the optimum gender of rearing model, including the role biology must play in gender identity development, the fact that lying to patients or withholding critical medical history is wrong, and the fact that there is an ethical duty to find out about the long-term effects of this practice. Controversy remains in regard to how children with intersex and their parents should be treated. Many surgeons refuse to believe that the genitoplasties done for appearance-normalizing reasons will result in the dysfunction and sense of shame many intersex adults report. Meanwhile, many psychosocial specialists have grown impatient with the institutional failure to provide professional mental health and social work services for these families from the start.
THE PATIENT-CENTERED MODEL OF CARE
In 2006 a consortium consisting mainly of three stakeholder groups (adults with DSDs, parents of children born with DSDs, and clinicians who treat people with DSDs) issued clinical guidelines for a "patient-centered model of care" meant to replace the optimum gender of rearing model. This was based on the patient-centered model of care of the ISNA, but the independent Consortium on the Management of Disorders of Sex Development (DSD Consortium) included the founders and leaders of most of the major support and advocacy groups as well as clinicians from all the major disciplines involved in DSD care. The tenets of patient-centered care are as follows:
- Providing medical and surgical care when dealing with a complication that represents a real and present threat to the patient's physical well-being.
- Recognizing that what is normal for one individual may not be what is normal for others; care providers should not seek to force the patient into a social norm (e.g., for phallic size or gender-typical behaviors) that may harm the patient.
- Minimizing the potential for the patient and family to feel ashamed, stigmatized, or overly obsessed with genital appearance; avoiding the use of stigmatizing terminology (such as pseudo-hermaphroditism) and medical photography; promoting openness (the opposite of shame) and a positive connection with others; and avoiding a "parade of white coats" and repetitive genital examinations, especially those involving measurements of genitalia.
- Delaying elective surgical and hormone treatments until the patient can participate actively in decision making about how his or her own body will look, feel, and function; when surgery and hormone treatments are considered, health-care professionals must ask themselves whether they are truly needed for the benefit of the child or are being offered to allay parental distress; mental health professionals can help assess this.
- Respecting parents by addressing their concerns and distress empathetically, honestly, and directly; if parents need peer support or professional mental health care, helping them obtain it.
- Directly addressing the child's psychosocial distress (if any) with the efforts of psychosocial professionals and peer support.
- Always telling the truth to the family and the child and answering questions promptly and honestly, which includes being open about the patient's medical history and about clinical uncertainty where it exists.
The DSD Consortium also recommends that the parents and physicians in consultation assign a gender (as boy or girl) to newborns on the basis of the likely ultimate gender identity of the newborn, recognizing that no baby's gender identity can be predicted with certainty. Reception among medical professionals of the DSD Consortium's guidelines has been extremely positive; this suggests that a consensus is building around this approach. A consensus also is forming around using a dedicated multidisciplinary team that provides long-term integrated care.
As can be seen in the work of the DSD Consortium, the goal of the intersex rights movement has been to try to demedicalize intersex while engaging in medical reform. Some intersex advocates have been working specifically to make intersex understood primarily as an identity rather than a medical issue. Indeed, some have agitated to add an "I" to the GLBT/Q (gay, lesbian, bi, transgender, queer) acronym. This approach has met considerable resistance in the medical profession, which tends to see intersex as a medical problem and to maintain a substantial level of homophobia, particularly in male-dominated fields such as pediatric urology. It also has met resistance among some people with intersex who do not think of themselves as personally or politically queer and among people with intersex who feel that intersex is not a big enough part of their personal or political history to justify the status of an identity.
Nevertheless, intersex advocates are likely to continue their relationship with advocates for GLBT/Q rights because of the shared sense of what it means to be oppressed for being a sexual minority. The idea that intersex rights are a matter of civil rights was made manifest in the 2005 decision by the San Francisco Human Rights Commission (SFHRC) to declare the treatment of people with intersex a human rights issue (Arana 2005). The investigation conducted by the SFHRC was initiated through the work of intersex activists David Iris Cameron and Thea Hillman and coordinated by Marcus de María Arana of the SFHRC. Partly in response, Sally Gross, the founder of the Intersex Society of South Africa, has been working with the South African Human Rights Commission on producing a similar declaration.
Arana, Marcus de María. 2005. A Human Rights Investigation into the Medical "Normalization" of Intersex People. A Report of a Public Hearing by the Human Rights Commission, City & County of San Francisco. Available from http://www.sfgov.org/humanrights.
Balen, Adam H.; Sarah M. Creighton; Melanie C. Davis; et al. 2004. Paediatric and Adolescent Gynaecology: A Multidisciplinary Approach. Cambridge, UK: Cambridge University Press.
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Chase, Cheryl. 1998. "Hermaphrodites with Attitude: Mapping the Emergence of Intersex Political Activism." GLQ: A Journal of Gay and Lesbian Studies 4(2): 189-211.
Colapinto, John. 2000. As Nature Made Him: The Boy Who Was Raised as a Girl. New York: Harper Collins.
Consortium on the Management of Disorders of Sex Development. 2006. Clinical Guidelines for the Management of Disorders of Sex Development in Childhood. Available from http://www.dsdguidelines.org.
Daston, Lorraine, and Katherine Park. 1995. "The Hermaphrodite and the Orders of Nature: Sexual Ambiguity in Early Modern France." GLQ: A Journal of Gay and Lesbian Studies 1: 419-38.
Dreger, Alice D. 1998. "'Ambiguous Sex'—or Ambivalent Medicine? Ethical Problems in the Treatment of Intersexuality." Hastings Center Report 28(3): 24-35.
Dreger, Alice D., ed. 1999. Intersex in the Age of Ethics. Hagerstown, MD: University Publishing Group.
Fausto-Sterling, Anne. 1993. "The Five Sexes: Why Male and Female are Not Enough." The Sciences 33 (2):20-25.
Foucault, Michel. 1980. Herculine Barbin: Being the Recently Discovered Memoirs of a Nineteenth-Century Hermaphrodite, trans. R. McDougall. New York: Colophon.
Herdt, Gilbert, ed. 1994. Third Sex, Third Gender: Beyond Sexual Dimorphism in Culture and History. New York: Zone Books.
Imperato-McGinley, Julianne, et al. 1986. "The Prevalence of 5-Alpha Reductase Deficiency in Children with Ambiguous Genitalia in the Dominican Republic." The Journal of Urology 136 (4): 867-73.
Kessler, Suzanne. 1998. Lessons from the Intersexed. New Brunswick, NJ: Rutgers University Press.
Matta, Christine. 2005. "Ambiguous Bodies and Deviant Sexualities: Hermaphrodites, Homosexuality, and Surgery in the United States, 1850–1904. Perspectives in Biology and Medicine 48 (4): 74-83.
Reis, Elizabeth. 2005. "Impossible Hermaphrodites: Intersex in America, 1620–1960." Journal of American History 92 (2): 411-41.
Alice D. Dreger