When a mixed-sex child is born, somebody (sometimes the surgeon, sometimes a pediatric endocrinologist, more rarely a trained sex education counselor) explains the situation to the parents.11 A ‘‘normal’’ boy, they say, may be born with a penis (defined as a phallus that has a urethral tube [through which urine flows] running lengthwise through its center and opening at the tip). This boy also has one X and one Y chromosome (XY), two testes
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figure з. 2 : A: Female reproductive anatomy. B: Male reproductive anatomy.
(Source: Alyce Santoro, for the author) descended into scrotal sacs, and a variety of tubing, which in the sexually mature male transports sperm and other components of the seminal fluid to the outside world (figure 3.2 B).
Just as often, the child has a clitoris (a phallus that does not have a urethra) which, like a penis, contains ample supplies of blood and nerves. Physical stimulation can cause both to become erect and to undergo a series of contractions that we call orgasm.12 In a “normal’’ girl the urethra opens near the vagina, a large canal surrounded at its opening by two sets of fleshy lips. The canal walls connect on the inside to the cervix, which in turn opens up into the uterus. Attached to the uterus are oviducts, which, after puberty, transport egg cells from the nearby pair of ovaries toward the uterus and beyond (figure 3.2A). If this child also has two X chromosomes (XX), we say she is female.
The doctors will also explain to the parents that male and female embryos develop by progressive divergence from a common starting point (figure 3.3). The embryonic gonad makes a choice early in development to follow a male or female pathway, and later in development the phallus ends up as either a clitoris or a penis. Similarly, the embryonic urogenital swellings either remain open to become vaginal labia or fuse to become a scrotum. Finally, all embryos contain structures destined to become the uterus and fallopian tubes and ones with the potential to become the epididymis and vas deferens (both are tubular structures involved with transporting sperm from the testes to the body’s
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FIGURE 3.3 : The development of external genitalia from the embryonic period through birth. (Source: Redrawn by Alyce Santoro from Moore 1977, p. 241, with permission from W. B. Saunders) |
exterior). When the sex is chosen, the appropriate structures develop and the rest degenerate.
So far, so good. The doctors have simply recounted some basics of embryology. Now comes the tricky part: what to tell the parents of a child whose development has not proceeded along the classic path. Generally doctors inform parents that the infant has a ‘‘birth defect of unfinished genitalia,’’ and that it may take a little time before they’ll know whether the child is a boy or a girl.13 The doctors can and will, they assure the parents, identify the ‘‘true’’ sex that lies underneath the surface confusion. Once they do, their hormonal and surgical treatments can complete nature’s intention.14
Modern medical practitioners still use the nineteenth-century categories of ‘‘true’’ and ‘‘male pseudo’’ or ‘‘female pseudo’’ hermaphrodites.15 Since most intersexuals fall into the pseudo category, doctors believe that an intersexual child is ‘‘really’’ a boy or a girl. Money, and others trained in his approach, specifically ban the word hermaphrodite from use in conversation with the parents. Instead, doctors use more specific medical terminology—such as ‘‘sex chromosome anomalies,’’ ‘‘gonadal anomalies,’’ and ‘‘external organ anomalies’’16 —that indicate that intersex children are just unusual in some aspect of their physiology, not that they constitute a category other than male or female.
The most common types of intersexuality are congenital adrenal hyperplasia (CAH), androgen insensitivity syndrome (AIS), gonadal dysgenesis, hypospadias, and unusual chromosome compositions such as XXY (Klinefelter Syndrome) or XO (Turner Syndrome) (see table 3.1). So-called true hermaphrodites have a combination of ovaries and testes. Sometimes an individual has a male side and a female side. In other cases the ovary and testis grow together in the same organ, forming what biologists call an ovo-testis.17 Not infrequently, at least one of the gonads functions quite well (the ovary more often than the testis),18 producing either sperm or eggs and functional levels of the so-called sex hormones—androgens or estrogens. In theory, it might be possible for a hermaphrodite to give birth to h/her own child, but there is no recorded case of that occurring. In practice, the external genitalia and accompanying genital ducts are so mixed that only after exploratory surgery is it possible to know what parts are present and what is attached to what.19
Parents of intersexuals often ask how frequently children like theirs are born and whether there are any parents of similar children with whom they might confer. Doctors, because they generally view intersex births as urgent cases, are unaware of available resources themselves, and because the medical research is scanty, often simply tell parents that the condition is extremely rare and therefore there is nobody in similar circumstances with whom they can consult. Both answers are far from the truth. I will return to the question of support groups for intersexuals and their parents in the next chapter. Here I address the question of frequency.
How often are intersex babies born? Together with a group of Brown University undergraduates, I scoured the medical literature for frequency estimates of various categories of intersexuality.20 For some categories, usually the rarest, we found only anecdotal evidence. But for most, numbers exist. The figure we ended up with—1.7 percent of all births (see table 3.2) —
should be taken as an order-of-magnitude estimate rather than a precise
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count.
Even if we’ve overestimated by a factor of two, that still means a lot of intersexual children are born each year. At the rate of 1.7 percent, for example, a city of 300,000 would have 3,100 people with varying degrees of intersexual development. Compare this with albinism, another relatively uncommon human trait but one that most readers can probably recall having seen.
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TABLE 3.1 Some Common Types of Intersexuality
a. Aaronson etal. 1997. b. The story is, of course, more complicated. For some recent studies, see Jacobs, Dalton, et al. i997;Bomanet al. 1998. c. There are a great many chromosomal variations classified as Klinefelter (Conte and Grumbach i989). |
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TABLE 3.2 Frequencies of Various Causes ofNondimorphic Sexual Development
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Albino births occur much less frequently than intersexual births—in only about і in 20,000 babies.22
The figure of 1.7 percent is an average from a wide variety of populations; the number is not uniform throughout the world. Many forms of intersexuality result from an altered genetic state, and in some populations, the genes involved with intersexuality are very frequent. Consider, for example, the gene for congenital adrenal hyperplasia (CAH). When present in two doses (that is, when an individual is homozygous for the gene), it causes XX females to be born with masculinized external genitalia (although their internal reproductive organs are those of a potentially fertile woman) (see table 3.1). The frequency of the gene for CAH varies widely around the world. One study found that 3.3 per thousand Yupik Eskimos born had a double dose of the CAH gene. In contrast, only 0.003/1,000 New Zealanders express the trait. The frequency of a related genetic change that leaves the genitalia unaffected but can cause premature pubic hair growth in children and symptoms such as unusual hair growth and male pattern baldness in young women, also varies widely around the world. These altered genes result in symptoms in 3/1,000 Italians. Among Ashkenazic Jews, the number rises to 37/1 ,ooo.23
Furthermore, the incidence of intersexuality may be on the rise. There has already been one medical report of the birth of a child with both an ovary and testes to a mother who conceived via in vitro fertilization. It seems that two embryos, one XX and one XY, fused after three were implanted into her uterus. Save for the ovary, the resulting fetus was a normal, healthy boy, formed from the fusion of an XX and an XY embryo!24 There is also concern that the presence of environmental pollutants that mimic estrogen have begun to cause widespread increases in the incidence of intersex forms such as hypospadias.25
But if our technology has contributed to shifts in our sexual makeup, it nevertheless also provides the tools to negate those changes. Until very recently, the specter of inter sexuality has spurred us to police bodies of indeterminate sex. Rather than force us to admit the social nature of our ideas about sexual difference, our ever more sophisticated medical technology has allowed us, by its attempts to render such bodies male or female, to insist that people are either naturally male or female. Such insistence occurs even though intersexual births occur with remarkably high frequency and may be on the increase. The paradoxes inherent in such reasoning, however, continue to haunt mainstream medicine, surfacing over and over in both scholarly debates and grassroots activism around sexual identities.
"Fixing” Intersexuals