Definition of Intersex

"Intersex” is a blanket term used to describe a constellation of congenital conditions that express themselves as physiological divergences from that which is accepted under the standard definition of “male” or “female.” Included are conditions that cause discordance between one’s genetic sex (chromosomes) and phenotypic sex (sex organ appearance), as well as conditions that cause ambiguity in genital or gonadal differentiation. Approximately one in 2,000 babies are born visibly intersexed, although sometimes intersexuality is not discovered until puberty, when sexual development does not follow its expected course. “Disorders of sexual development” is the technical term for intersex.

Biology Behind Intersex Conditions

Early on in sexual development, all embryos contain the precursors for both male and female sex organs. In most cases, during the third month of gestation, one distinct trajectory is followed. At about five to six weeks of gestation, when gonads are first formed, they are undifferentiated, as in, neither male nor female. Chromosomes guide prenatal genital development, by way of predisposing hormonal secretions, resulting in the organization of a fetus’ sex organs and brain as either “male” or “female.”

The precursors to the internal female sex organs, called the “Mullerian system,” do not require the presence of hormones to develop. The ovaries, uterus, fallopian tubes, and external genitalia will develop even in the absence of estrogen and androgens. On the other hand, the precursors to the internal male sex organs, called the “Wolffian system,” require the testes to produce large amounts of testosterone between the sixth and 12th week of gestation. Testosterone stimulates development of the epididymis, vas deferens, seminal vesicles, and external genitalia and initiates the production of Mullerian inhibiting substance, which prevents the female system from developing. Nature’s default is in the direction of female development, as the fetus’ body will be organized as female in the absence hormonal activation.

A few of the more common conditions that cause intersexed appearance are as follows:

Androgen Insensitivity Syndrome (1 in 13,000 births)

Androgen insensitivity syndrome (AIS) occurs in fetuses who are genetically male and whose bodies produce normal amounts of androgens (testosterone), but whose target cells are unresponsive due to lack of an androgen receptor gene. The ducts in the Wolffian system are insensitive to androgens, so the genetic male’s internal sex organs do not develop. However, the ducts of the Mullerian system are successfully inhibited by Mullerian inhibiting substance, as they would be in a typical male fetus, so the child is born lacking any internal reproductive system. Because they are insensitive to testosterone, which is needed for male organization, those with AIS appear to have female genitals externally, including a normally sized clitoris and shallow vagina.

Congenital Adrenal Hyperplasia (1 in 13,000 births)

Congenital Adrenal Hyperplasia (CAH) occurs when the adrenal glands of either a genetically male or genetically female fetus overproduce testosterone. In genetic males, this overproduction can result in premature puberty, as early as age five. In genetic females, the overproduction of testosterone can result in masculinized and ambiguous genitals, including an enlarged clitoris resembling a miniature penis. Additionally, the labia may be fused, resembling a scrotum without testes.

Micropenis

A penis is classified as a “micropenis,” if it measures at or below 2.5 standard deviations from the mean for age and stage of development. In normal penile development, androgens are secreted twice during gestation—the first time to shape the basic structures of the penis and scrotum and the second time to enlarge the penis. A micropenis results from normal androgen secretion in the first stage, but little to no secretion in the second stage. Although small, shape and function is normal.

Traditional Treatment of Intersex Individuals

In the early 1950s, after writing a dissertation on intersex, then called “hermaphroditism,” Dr. John Money was invited to become a psychological researcher at John Hopkins, where he created the Psychohormonal Research Unit and became the preeminent authority on disorders of sexual development. Money and his colleagues created “matched pairs” of intersexed individuals who had been diagnosed with identical genitalia, but who had been assigned to live as opposite sexes. Money claimed there was evidence that children are psychosexually undifferentiated at birth and the primary factor determining their gender identity is not biology, but rearing and socialization. He believed that on the recommendation of surgeons and endocrinologists, parents could choose to steer their children in whichever direction they wished.

His only stipulation was that gender designation and sexual reassignment surgery must be completed during the first two and a half years of life (although corrective surgery would need to be performed thereafter, in many cases), a time period after which a child’s gender orientation would become fixed. Parents and doctors needed to be unwavering as to their decision, lest they introduce ambiguities into their children’s learned conceptions of themselves as either masculine of feminine. Money generalized his theory—widening its application—to include that every child is born gender neutral, regardless of the appearance of his/her genitals, and it is society that shapes his/her gender identity, rather than an innate or instinctual sense. His theory was only affirmed upon exposure to a male-to-female transsexual whose inner sense of self was in direct contradiction to her chromosomal, gonadal, hormonal, and anatomic sex.

The pioneer of intersex research, John Money gained prominence within the scientific community when he was assigned the case of Bruce Reimer, who was born an anatomic male, but who lost his penis to a circumcision accident. This case was unique in two ways: 1) Bruce was born with normal genitalia, rendering irrelevant allegations that intersex children’s brains could be predisposed to be more neutral than those of normal children, and 2) he was also born an identical twin, creating a naturalistic “matched pair.”

Upon receiving Money’s expert advice, Bruce’s desperate parents agreed to rechristen him “Brenda,” raise him as a girl, and subject him to genital normalizing surgery, altering his genitals to the best approximation of normal female genitals given the primitive technology available at the advent of these procedures. Money assumed that Brenda would readily accept her gender assignment—with the cooperation of the eager family—along with role-playing protocol that would serve to enforce her gender role. He forcefully encouraged her to wear feminine clothing, engage in gender-stereotypical activities, and comply with the unorthodoxies of sexual rehearsal play, genital inspection, and exposure to sexually explicit material.

Despite his unconventional and ethically questionable methodologies, Money’s theory of sexual development and his guidelines for treating intersex patients was touted and indisputably accepted in the medical and academic communities alike. Among the members of the women’s movement, who had been arguing against biological sex differences for years, the matched twins case was regarded as proof that the differences between men and women reflected social expectations and prejudices, not biological imperatives. Upon publicly debuting the case in 1973, Money proclaimed that it “provides strong support for a major contention of women’s liberationists: that conventional patterns of masculine and feminine behavior can be altered. It also casts doubt on the theory that major sexual differences, psychological as well as anatomical, are immutably set by the genes at conception.”

Money continued reporting the success of his gender assignment cases, despite the fact that he did not track the progress of most of his subjects longitudinally. Most glaringly, he distorted the facts surrounding his most prominent case even after Brenda reverted back to her birth sex of male, proving that her transition had been a failure. Money was not met without skepticism.

Dr. Michael Diamond became fascinated with the organizing role hormones play in utero, questioning whether the prenatal effects hormones have on anatomy are mirrored in the brain. He, along with mentor and anatomist William C. Young’s staff, created a cohort of intersex guinea pigs by injecting large doses of testosterone into their mother’s wombs. The results were that the female guinea pigs were born with clitorises enlarged to meet the size of normal penises, and their masculinized anatomy corresponded to masculinization of sexual behavior. They assumed male, rather than female sexual posture, despite being chromosomally female. Disregarding this and other breakthrough research on the biological underpinnings of sexual behavior, the view that prenatal factors limit the effects of learning and environment on gender identity never gained ground within the scientific community. This is because John Money explicitly and directly interfered with the process of scientific inquiry, blocking any competing or contradictory theories from being published, denigrating the methodology and purpose of his competitors, and going as far as threatening scientific journals with lawsuits and scientist with fists.

The New Era

Despite resistance from the scientific community, Michael Diamond continued his research on the prenatal organization of the sexual nervous system and his studies affirmed his belief that neither intersex nor normal children are born psychosexually neutral. This led to his questioning the efficacy and ethics of the growing and accepted practice of infant sexual reassignment surgery. In response to the discovered failure of the twins case, previously reported as a success, he commented, “As for the twin, it is scientifically regrettable that so much of a theoretical and philosophical superstructure has been built on the supposed results of a single, uncontrolled and unconfirmed case.”

In the early 1990s, Diamond, along with the twin’s psychiatrist Sigmundson, drafted a new set of guidelines for treating children born with ambiguous genitalia. They recognized that babies must be assigned to one gender or the other, but recommended that doctors defer cosmetic surgery until children are old enough to evidence and articulate what gender they relate to the most closely. Of course, this does not account for genital-normalizing surgery that is necessary for the performance of biological functions, such as, urination. Diamond believed that if doctors continued to perform sexual reassignment surgery on infants, before their gender identity became apparent, gender identity would be incorrectly assigned approximately half of the time. After going through painful, scarring surgery, patients would end up with bodies that were not in accordance with their self-concepts.

Since the 1997 publication of Diamond and Sigmundson’s new guidelines, intersex activist groups have gained widespread recognition. The Intersex Society of North America contends that while provisional gender assignment should be made so that children can fit in in a society where binary gender concept is accepted, genital-normalizing surgery should be deferred, if performed at all, until a child is able to consent. Dr. Howard Devore, a psychologist who studied under John Money and was born with acute hypospadius, believes that the normalizing surgeries he endured throughout childhood were traumatizing, he still does not have a penis that resembles that of a normal male, and the psychological scarring would have been less had he foregone unnecessary cosmetic treatments and instead been raised with counseling to accept his atypical genitals.

Cheryl Chase, the founder of ISNA, born intersex and lied to about her condition, denounces and aims to abolish any “barbaric,” medically unnecessary genital-normalizing surgery on performed on infants, as it has the potential to irreversibly interfere with erotic and reproductive functioning. She expresses regret not only with the removal of her clitoris, leaving her unable to orgasm, but also with the “shame and secrecy” suffered by many intersexuals, partially due to a misguided but well-meaning attempt by their families to encourage them to live normal lives.

Although other intersex members of ISNA complain about physical and emotional scarring, the loss of sexual sensation and reproductive capacities, and the lack of choice in their sexual destinies, Dr. John Gearhart, head of pediatric urology at John’s Hopkins, dismisses the members of ISNA as “zealots” with obsolete concerns. He insists that while sex reassignment surgery is a viable option for boys born with functioning yet small penises or those who have lost their penises due to injury, had the Bruce Reimer case surfaced in the 1990’s, he would have made a different recommendation based on how far penis reconstructive technology has advanced. For similar reasons, he considers ISNA’s complaints to be irrelevant; while the amputations of their genitals was “wrong,” modern reconstructive surgery maintains sensation. Cheryl Chase openly rejects the claim that it is possible to maintain clitoral sensation while reducing the size of the organ.

Specific protocol for intervention in intersexual infants remains controversial, but there is a consensus that doctors and parents be mindful of both the biological and psychological implications of sexual reassignment surgery. Gender identity is not solely determined by the dedicated and well-intentioned socialization of a child as one sex or the other, nor is surgery reversible. A child must be raised as a gendered entity, not an “it,” to live a satisfying life in our society, and it is clear that many who underwent genital normalizing surgery without their consent are bitter.

However, as few intersex individuals have escaped genital-normalizing surgery, the long-term social and psychological effects of avoiding surgery remain unknown and, thus, it is impossible to compare the prognoses of those who avoided and those who underwent reconstructive surgery. It is likely that psychological adjustment is partially dependent on feeling of personal choice and support of family, doctors, and therapists, in addition to actual biological outcome.