Amy Bloom - Normal

Or let’s say that what the parents see is a baby girl with a larger than standard clitoris (more than one centimeter in length). You might not think that this is a problem of “doubtful sex” or confusing genitals, but in infants the gap between clitoris and penis is only about half a centimeter, so the large clitoris that doctors fear will worry her parents every time they change the diaper, and will alarm or even dissuade her future husband, also requires the surgical solution, as early as possible. The surgeries include “clitoral reduction,” and if necessary, some enlargement of the vaginal cavity by metal dilators inserted by the parents daily for six months, beginning two weeks postoperatively. Monthly dilation of the seven- or eight-year-old continues into adolescence to prevent narrowing or closure of the vaginal cavity. (The standard for a “good” vagina is one that can be penetrated adequately.) And then, perhaps, following the early vaginoplasty, further molding of delicate and cosmetically pleasing labia may be required.

“Ambiguous genitals,” “doubtful sex,” “intersexed babies,” “male and female pseudohermaphroditism,” “true hermaphroditism”—these phrases sometimes describe the same conditions, sometimes very different conditions. Some conditions require hormonal treatment or surgery or both; some require no treatment at all except counseling and time. Symptoms range from the physical anomaly — an unusual-looking set of genitals — to symptoms that will not become apparent until adolescence, to symptoms that will never be apparent from the outside. Some anomalies are defects in the plumbing; others are simply unusual fixtures.

There is a range of medical conditions that fall under the umbrella term “congenital anomalies of the reproductive and sexual system.” Boys may suffer from hypospadias, meaning in mild cases that the urethral opening (the “pee hole”), which is supposed to be at the tip of the penis, is perhaps in the glans, on the underside of the penis, or in more severe cases is open from mid-shaft out to the glans, or is even entirely absent, with urine exiting the bladder from behind the penis. Hypospadias sometimes results in ambiguity as to sexual organs, as does Klinefelter’s syndrome, which is quite common, occurring in one in five hundred to one in a thousand male births. Most men inherit a single X chromosome from their mother and a single Y chromosome from their father. Men with Klinefelter’s inherit an extra X chromosome from either father or mother, and their testes often produce smaller than average quantities of testosterone, so that they don’t virilize (develop facial and body hair, muscles, deep voice, larger penis and testes) as strongly as other boys at puberty. (Many also develop small breasts, one of Nature’s variations that is often found in those with no intersex conditions at all.) Despite an absence of sperm in their generally small, firm testes, many men with Klinefelter’s are never diagnosed because their genitals are typical in appearance.

In androgen insensitivity syndrome (AIS), the body of an XY individual lacks a receptor that enables it to decode messages from androgens (virilizing hormones). AIS results in people with male chromosomes and obviously female bodies; although they produce male hormones, their cells are not sensitive to those hormones, and their bodies never masculinize. There is also partial androgen insensitivity syndrome (PAIS), which typically results in “ambiguous genitalia.” The clitoris is large or, alternatively, the penis is small and hypospadic (two different ways of labeling the same anatomical structure). PAIS seems to be quite common, and has been suggested as the cause of infertility in many men whose genitals are typically male.

Among the most prevalent causes of intersexuality among XX (usually female) people is congenital adrenal hyperplasia (CAH), in which the adrenal gland produces an excess of androgens but feminizing occurs at puberty because the ovaries function normally. When excess androgens are produced in utero (sometimes not because of CAH but because an unborn XX baby’s metabolism converts hormonal drugs such as progestin, which was frequently administered to prevent miscarriage in the 1950s and 1960s, into an androgen), the female baby may be born with an enlarged clitoris and fused labia that look very much like a scrotum. Sometimes the genitals look typically female, with barely perceptible variations. Sometimes the babies appear to be healthy boys without testes, and it may be that no one in the delivery room thinks anything is amiss. And less often, the babies’ genitals are not just misleading but the hallmark of what has historically been called hermaphroditism: truly ambiguous genitals, both male and female, although not a complete set of either.

Monsters, freaks, prophets, border-crossers, portents of disaster — hermaphrodites have been disturbing people for a long time. Ovid wrote of handsome Hermaphroditos, son of Hermes and Aphrodite, whose beauty so dazzled the nymph Salmacis that she longed — as lovers do — to be joined with him. The gods granted her wish, in their quirky fashion, and two turned into one — one Hermaphrodite, now both man and woman. In another version of the myth, Hermes and Aphrodite’s child is so completely a mix of both parents, both male and female, that they cannot agree on its sex, and name it Hermaphroditos. In the first version, love flowers so fully that one body can contain two sexes and two souls — a happy ending. In the second, the gods are so baffled that they can do no more than name their child and move on; we hear no more of poor Hermaphroditos, not of love, nor power, nor family life.

The Hippocratics suggested a continuum of gender, much as many contemporary gender theorists do: masculine male at one end, feminine female at the other, masuline women and feminine men along the road, and hermaphrodites smack dab in the middle. Aristotle held that the hermaphrodite was a kind of incomplete twin in whom the ambiguous genitals signaled that almost enough body had been created for two babies, but not quite. The excess body parts, like a sixth toe or a third nipple, were odd and undesirable but irrelevant to the person’s “true” sex, which was determined, Aristotle believed, not by genitalia but by the “heat” of the heart.

The search for the “true” sex of the individual seems to me the model that most contemporary physicians have been following. This search, like the search for “true” love, seems composed of equal parts convention, social man-date, human need, and commitment to a dream, and despite all of our trouble and technology, we not only get it wrong more often than not, we cannot even acknowledge that Nature’s answers are much more sophisticated than our questions. As Anne Fausto-Sterling writes so neatly in Sexing the Body , “Different countries and different legal and religious systems [in seventeenth- and eighteenth-century Europe] viewed intersexuality in different ways. The Italians seemed relatively nonplussed by the blurring of gender borders, the French rigidly regulated it, while the English, although finding it distasteful, worried more about class transgressions.”

By the late nineteenth century, physicians were dividing hermaphrodites into three categories based on the identification of gonadal tissue. Individuals having testicular tissue were defined as “male pseudohermaphrodites,” and individuals with ovarian tissue were “female pseudohermaphrodites,” regardless of the form of the genitals. Only individuals having both ovarian and testicular tissue were “true hermaphrodites.” The result of this typology, which relied on “scientific” tissue analysis, was that fewer “true hermaphrodites” appeared: “A body with two ovaries, no matter how many masculine features it might have, was female. No matter if a pair of testes were nonfunctional and the person possessing them had a vagina and breasts, testes made a body male,” Fausto-Sterling says. “Additionally,” Dreger writes, “given that biopsies of gonads were not done until the 1910s and that Victorian medical men insisted upon histological proof of ovarian and testicular tissue for claims of ‘true hermaphroditism,’ the only ‘true hermaphrodites’ tended to be dead and autopsied hermaphrodites.” Whatever the intent of the doctors and scientists, in the 1870s people of truly mixed sex, who had been sufficiently common to merit discussion in every country’s medical texts, and in the Talmud and Tosefta as well (couldn’t shave, like men; couldn’t inherit, like women), began to disappear from the records, and took with them their troubling tendency to blur the social issues of the day.