This is the tale of two children, born with the outward appearance of girls, but lacking the inner passageways of either sex: no cervix, uterus or fallopian tubes, and no seminal vesicles, vasa deferentia or prostate either. They did have gonads, though: one had a boy's testes, the other had a girl's ovaries.
Both the children were reared the way their appearance demanded as girls. Both were traumatized by their physical abnormality and by society's response to it. But one of them found comfort in her role as a girl, and she eventually grew into a woman whom everyone now recognizes and treats as such. The other was a tomboy who rebelled against everything girlish; eventually she demanded sex reassignment and is a man today. So which was which?
That's obvious, you'll say: the child with the ovaries became a woman, and the child with the testes became a man. Hormones, after all, are the ultimate arbiters of our sexual fate, so how could it be otherwise?
Well, it was otherwise. The child with the testes became a woman, and the child with the ovaries became a man. This is the story of how that happened. And, more generally, it's about the biological complexity, the emotional turmoil and the medical politics of intersexes people who can't be shoehorned into the comfortable categories of male or female.
Sherri Groveman, a forty-one-year-old tax lawyer, lives alone in a ranch-style home in a sun-baked suburb of San Diego. She's a pleasant, forthright, freckle-faced, womanly woman. So why, within a few minutes of the start of our interview, did I innocently ask: "And did you enjoy playing with other boys girls, I mean?"
I blushed and apologized, but Sherri wouldn't let me get away with it. It was a Freudian slip par excellence evidence that a part of me considered her a male, regardless of all the evidence to the contrary. And the reason? Because I knew that she had been born with testicles, and that every cell in her body contained an X and a Y chromosome the genetic signature of a male.
Sherri has a medical condition known as androgen insensitivity syndrome, or AIS. Androgens are the "male-making" hormones, of which testosterone is the leading member. Normally, androgens do their business by attaching to "receptor" molecules in target cells throughout the body and brain, in the same way as a key fits in a lock. In genetic males with AIS, a mutation changes the shape of those receptors, so that androgens no longer "fit" them. An affected fetus may be awash in testosterone, secreted by the developing testes, but the hormone has no effect, so the fetus develops with the outer appearance of a female. In addition, the male reproductive tract (prostate, seminal vesicles and vasa deferentia) fails to develop.
If Sherri's entire body had been female, all might have been well. But there's another hormone that is normally secreted by the developing testis, whose role is to switch off the development of the female reproductive tract: the upper part of the vagina, the cervix, uterus and fallopian tubes. This hormone does function normally in fetuses with AIS, so Sherri ended up lacking the reproductive tract of either sex.
Sherri's condition was recognized a few weeks after birth, because her testes, in the attempt to migrate down into her non-existent scrotum, became lodged in her groin, where they could be felt as lumps. They were removed surgically, out of concern that they might become cancerous. (This is a legitimate concern in all cases of undescended testicles, but such cancers rarely occur during childhood, so the operation could have been put off for many years.) Sherri was brought up thinking that she had had hernia surgery, but an occasional remark from her mother, to the effect that "not all girls could become mothers," hinted that more might be amiss.
Because Sherri now lacked gonads of any kind, she would need hormone replacement to bring about the bodily changes associated with female puberty. When she was eleven, her mother prepared her for this by telling her something slightly closer to the truth: that she had been born with "twisted ovaries" that had been removed to prevent cancer. Sherri learned that she would not menstruate and could never bear children, but she did not learn the real cause, nor was she told of possible difficulties in her sex life. "The twisted-ovaries story is a lie heard round the world," she says. "The medical literature recommends that doctors and parents should lie about it the thought being that we would kill ourselves if we were told the truth." After this ten-minute conversation, she did not discuss the matter with either of her parents for the next twenty-eight years.
With puberty, her private shame threatened to become a public one. She developed most of the outer features of a woman, but she did not grow pubic hair, which depends on androgens in both men and women. So she had to conceal her lower body in school showers and locker rooms, and from future boyfriends. Worse, she found that her vagina was too narrow and short. "Everyone was using tampons," she says, "so I bought some and tried putting one in, but it hurt like hell, because of the narrow entry-way." Her vagina was less than two inches long too short for intercourse.
Convinced that she had a dirty secret for which she was herself to blame, Sherri became a "gregarious loner" outwardly sociable, but shunning intimacy with anyone. The only time her condition was discussed was at her periodic endocrinological check-ups. The discussions were not with Sherri, though, but among the doctors and medical students who gathered around her crotch. "They were talking amongst themselves about my genitals," she says, "but I had no idea what they were saying." She was told nothing about her condition, and she was asked nothing about her psychological health.
At age twenty-one, Sherri figured out the cause of her condition on her own by rummaging through textbooks in a medical library. She was devastated not so much at discovering her intersexed status but at discovering she had been lied to for two decades by parents and doctors. This led to her complete estrangement from the medical profession: she stopped taking estrogens and didn't see a doctor for another fourteen years.
"Finding out about myself in the way I did really damaged me," she says. "I thought, 'How can I embark on a relationship and not tell my partner? I'd be participating in that same lie that was told to me.' And yet I thought I would have to lie. After all, no one told me the information, so I thought it must be so horrible, such a hideous and freakish thing, that if I told anyone else they would bolt."
At the age of thirty-five, however, she did go back to a doctor; she was in a tentative relationship with a man and wanted to know if anything could be done to help her have vaginal intercourse. The doctor confirmed that Sherri had AIS, and put her back on estrogens to correct the severe osteoporosis that had been brought on by the lack of hormones. She also recommended that Sherri use vaginal dilators plastic test-tubes that she was to sit on for fifteen minutes twice a day. Although these dilators do help some women, Sherri did not like them, and she eventually broke off the relationship with her boyfriend.
All those years, Sherri had never met anyone resembling herself, and this, she says, was the deepest cause of her loneliness and shame. A few months after the break-up with her boyfriend, however, she came in contact with an AIS support group that was forming in England, and she flew over to attend the inaugural meeting. "There was nothing I would not have given to participate," says Sherri. "I would have cut off my arm I was that desperate to look into someone else's eyes and have them know what I was experiencing." She has been back for ten subsequent meetings, and she has herself founded a U.S. support group that works closely with its U.K. counterpart.
Although Sherri identifies as a woman, her identity as an intersex is now just as important to her. Indeed, she is waging a small campaign to have the intersex status more widely recognized: in the "M/F" box on the census form she wrote, "I (intersex)", and she is trying (unsuccessfully so far) to have the same designation included on her driver's license. "I'm not asking for unusual accommodations," she says. "I just want people to acknowledge the reality of intersexes."
James Benson is a forty-five-year-old neuroscientist at a prestigious university. With ample beard, muscular build and male dress and mannerisms, Benson is unquestionably a man, but he was born a girl. Jennifer, as she was named, seemed to be a physically normal girl throughout her childhood, but she was anything but a typical girl in her behavior. She wanted to play with boys and do all the hyperactive, mucky things that boys do. She did not want to associate with girls or do anything remotely feminine. As her childhood progressed, she became more and more of a social pariah, ostracized by her peers of either sex.
At puberty, Jennifer developed breasts, but failed to menstruate. When she was sixteen, her mother took her to a gynecologist, who prescribed estrogen shots to "get her going," but these didn't work. Jennifer was not especially keen to menstruate anyway, so she stopped the treatment after about a year. In high school her social life was essentially non-existent. Instead she "over-achieved" academically, and entered MIT a male-dominated school a year early.
In college, she fell in love with a man, and they attempted intercourse, but without success: Jennifer, it turned out, had no vagina. This fact had not only escaped her own attention for twenty years, it had also been missed by the doctor who had examined her four years before ("A typical bozo gynecologist," Benson reminisces).
Jennifer went to see her college gynecologist, who arranged for exploratory surgery. It was found that Jennifer had ovaries but no reproductive tract. It emerged that Jennifer's mother had taken progestin hormones during pregnancy, but a connection between that and Jennifer's condition is uncertain. (Jennifer has a twin sister, who was exposed to the same drug in utero but who did not develop Jennifer's condition.) The surgeon recommended that Jennifer have a vagina constructed by plastic surgery, telling her that this operation would make her "normal."
"I remember thinking, 'I'm not going to be normal,'" says Benson, "but back then I was very intimidated by doctors and I was confused by what was going on. I didn't spend time arguing with them like I would now." A tunnel was opened up between her rectum and her bladder, and it was lined with skin taken from her buttocks. It did in fact work as a vagina, after a fashion, but Jennifer's problem was much deeper than that: she did not feel psychologically comfortable having sex as a woman. Eventually she ended the relationship with her boyfriend and invested all her time and energy in her career. Luckily, it was a career in which dress codes and gender expectations were pretty much non-existent.
At about the age of forty, Jennifer was diagnosed with breast cancer, which runs in her family. The surgeon recommended that she have the affected breast removed, but Jennifer persuaded him to remove both breasts, in part for prophylactic reasons but also because she had never liked them. "When my mother had to have her mastectomy, it was this incredibly devastating experience, which is typical for most women," says Benson. "But I was delighted when my breasts were taken off. I never wore any prostheses. This got me to realizing that there was something unusual about my gender identity."
Thus, Jennifer had the first stage of sex-reassignment surgery almost by subterfuge. The rest ovariectomy, testosterone treatment and change of name and legal sex followed a few years later. James did not have a phalloplasty the construction of a penis. "I do wish I had one," he says, "but 100,000 dollars for something that can't get an erection and has little feeling and can lead to all kinds of complications? No, thank you." Since James is still sexually attracted to men, he is now a gay man and is tentatively exploring that identity.
At a recent sexology conference in Spain, I had the opportunity to discuss the surgical issues with physician Jos Megens, who runs the celebrated sex-reassignment clinic at the Free University of Amsterdam. Megens conceded that the construction of normal male genitalia in an intersexed or female patient leaves a lot to be desired. For that reason, he said, his clinic has recently begun to offer a surgical procedure which creates a "micropenis" out of the patient's own clitoris. In this operation, the clitoris is lengthened by exteriorizing the portion of the clitoral shaft that is usually hidden within the body, and a newly-fashioned urethra is embedded within it. The resulting organ is no more than four to six centimeters long and is not adequate for vaginal penetration, but it is erectile and it usually has good erotic sensitivity with the capacity for orgasm. Even this small penis can have problems, however: in about half the patients some kind of repair surgery is eventually necessary.
Luckily, surgical perfection is not the key factor. Even without a penis of any kind, James Benson feels like a new man. "The thing that's so hard to describe is the relief," he says. "I feel it every day, every minute. There's no longer this conflict between my inner self and that outer woman. Years ago, I read about people who changed sex, and I thought they were some kind of perverts I'm embarrassed to say that now. But changing sex isn't about sexuality, it's about who you feel you are."
At some levels, Sherri Groveman and James Benson are very different. Certainly they now look very different one an unmistakable woman, the other an unmistakable man. Also, Sherri has a clear-cut syndrome, AIS, whereas the underlying cause of James' condition the unusual combination of absent reproductive tract and cross-gender identity remains something of a mystery.
At a deeper level, though, Sherri and James share a sense that they have been wounded by more than the hand nature dealt them. They feel that they were violated by doctors who saw them purely as medical problems to be solved as people to be made normal, or as close to normal as was possible, by surgery or hormones. Little or no attention was paid to the people behind the genitalia.
More positively, however, both Sherri and James share a sense of triumph: a sense that, against all the odds, they have found their way to who they really are. In the process, they have overcome life-destroying shame and loneliness. Both want to communicate their stories out of a desire to ease the lives of others like them.
Intersexes are just beginning to build a community and to become socially and politically engaged. The Intersex Society of North America (ISNA) seeks to provide not only a support group for intersexes but also education and advocacy that will help break down the shame and silence that surrounds them. ISNA's founder, Cheryl (née Charlie) Chase, was born with gonads containing both ovarian and testicular tissue making her what used to be called a "true hermaphrodite." She also had a small penis, but doctors later reassigned her to be female, whereupon the penis became an excessively large clitoris, and it was cut off. In consequence, Cheryl grew up without the capacity to experience orgasm.
To Chase, the overriding priority is to overcome the sense of shame associated with intersexuality. This shame is not just psychologically damaging; it is also the main motivation behind the "corrective" surgery done on intersexed children. Most of this surgery is unnecessary, or could be left until the child is old enough to make decisions for him- or herself, says Chase, but doctors and parents want above all to get rid of anatomical deviations; they want to "normalize" the child. Yet this cannot be with any confidence until the child is able to communicate what is "normal" for him or her.
Unlike Sherri Groveman, with her one-person war against the "M/F" dichotomy, Chase acknowledges the biological and cultural primacy of the two sexes. "I'm not a radical postmodern theorist who wants to deconstruct sexuality," she says. "I don't think it's realistic or beneficial to encourage parents to bring up children with an intersex identity." What she does want is for doctors to communicate with parents, parents with children and adult intersexes with the public. She herself is a tireless communicator, churning out articles, books and sound-bites, and speaking at meetings with everyone from pediatricians to church folk. Although she faces considerable resistance, there is a gradual movement among pediatricians to rethink the traditional notion of early surgical intervention in the treatment of intersexed kids.
To their credit, none of the intersexes I've met attribute their problems simply to victimization by society. "It's going to be totally shitty no matter what, there's no way around it," says Sherri. "But I was never sorry that I was born intersexed. I wouldn't trade. This is who I was meant to be in this world."
DEFINING SOME TERMS
Gonadal intersex (or "true hermaphrodite"): Rare condition in which the child has both ovarian and testicular tissue, either in separate gonads or as hybrid "ovotestes." Reproductive tracts, genital anatomy and gender identity are variable.
Congenital adrenal hyperplasia (or adrenogenital syndrome): Genetic error in adrenal glands causes excessive secretion of androgens during fetal life. Genitals of female children are masculinized to a variable degree. Psychological effects include an increased likelihood of same-sex attraction in adulthood.
Androgen insensitivity syndrome (AIS, or testicular feminization): An XY (chromosomally male) fetus lacks sensitivity to testosterone, so develops external anatomy and gender identity of female, but lacks internal reproductive tract of either sex.
Partial AIS: Like AIS, but with reduced rather than absent sensitivity to androgens. Child may partially virilize at puberty if testes are not removed.
Hypospadias: Condition in male children in which the urethral meatus (opening) is located on the underside or at the base of the penis. Represents an incomplete male differentiation of the genitalia, but it is not necessarily considered an intersexed condition, especially in its milder forms.
Mayer-Rokitansky-Kustur-Hauser syndrome: Failure of development of vagina, cervix, uterus and fallopian tubes in genetic females. Cause not known. James Benson may have had this syndrome, although his male gender identity is atypical.
Vaginal agenesis: Failure of formation of vagina, for a variety of reasons, including MRKH syndrome.
Cloacal exstrophy: Severe congenital malformation of pelvis, including (in males) lack of a penis. These boys usually develop a male identity even if castrated and surgically reconstructed as females.
Klinefelter's syndrome: Genetic males with one or more extra X chromosomes (XXY, XXXY). Anatomically male but infertile.
Turner's syndrome: Affected individuals have one X and no Y chromosome. Ovaries degenerate during fetal life, in other respects anatomically female. Short stature and lack of secondary sexual characteristics without treatment.
Hermaphrodite: Old medical term for an intersexed person, given new life by some intersex activists.
Intersex Society of North America
AIS Support Group
Mayer-Rokitansky-Kustur-Hauser Syndrome Support Group
Simon LeVay and Nerve.com