Intersex - The Third Gender

Boy or girl? It's the first question we ask when a baby is born if it hasn't already been announced in grand fashion by the beaming parents. Just about every birth announcement card carries the essential information on the front cover. "It's a Boy!" or "It's a Girl!" Defining ourselves as one or the other is what gives us status as humans. An it becomes a he or a she. There is no gender-neutral pronoun for humans, at least in the English language. We live in a bi-gendered world and you're either pink or you're blue. There's no room for mauve.

But what do you call a baby with a vagina and a penis? Is that baby a he, a she, or something else? You may consider this to be an academic question, but for over two million people worldwide (one out of every 2000-4000 babies born), the gender line is blurred to the point of non-existence. Most of us have probably had a conversation with a person that is genetically or morphologically neither male nor female � a condition known as intersex. You're not likely to have been aware of it at the time because long before they could speak, most intersex children were assigned to one gender or the other.

The "optimal gender of rearing" protocol was established in the mid-sixties by psychologist John Money, on the understanding that gender identity was to a large extent socially defined. Gender assignment was usually reinforced by surgery and because it's a whole lot easier to construct a vagina than to enlarge a micro-penis, even infants that were predominantly male were usually feminized. Since few things are as unfeminine as a penis (however small), if the baby was sporting one... off it came. The Money protocol also extended to those infants who were clearly boys but whose stretched penis was less than 1 inch. Better to have no dick than a small dick, seemed to be the thinking.

A major problem with this approach, of course, is that the penis (or clitoris) is the source of orgasmic sensation. Even modern-day efforts to retain the glans of the penis and reduce the shaft often result in the loss of orgasmic ability. This may seem like a reasonable tradeoff when weighed against the shame and stigma of going through a life that resembles The Crying Game. But assigning gender is more complicated than pink clothes, Barbies and a little surgical nip and tuck. Gender identity, as it turns out, is not just a social construct. It involves a deeply personal sense of self that has roots far deeper than our ability to manipulate.

While to the outside world they were unquestionably girls, many intersex children felt inside that they were boys. They would spend their childhoods in isolation, rejected by their natural gender and unable to read the social cues that would allow them to bond with their adopted gender. For some, the situation was made infinitely worse as they entered adolescence and their bodies were subject to a surge of masculinizing hormones. Their voices would deepen, hair would grow on their faces and chests and quite suddenly these "girls" revealed themselves to be men. For those that had been spared feminizing surgery, the transition usually involved moving to a different school and changing one's name from Jackie to Jack. For those that had their pee-pee's excised... well, there wasn't much that could be done.

Given the obvious failings of the Money protocol and the dramatic improvements in our understanding of the molecular underpinnings of sexual development disorders, it's surprising how long the protocol has been accepted. Despite intense lobbying by intersex advocacy groups such as the Intersex Society of North America (formed by Cheryl Chase in 1993), it was only this last summer, with the publication of the "Consensus Statement on the Management of Intersex Disorders" in the journal Pediatrics, that the Money protocol was officially abandoned.

The new protocol for dealing with intersex conditions still embraces the idea that every infant should be assigned a gender as soon as possible after birth. The difference now is that no decision is made until the child is subjected to a battery of tests to determine the underlying gender indicators. Prognosis depends on this diagnosis. Sexual ambiguity is not the product a single condition, it arises from any of a dozen different conditions, each with its own developmental characteristics, prognosis and method of treatment (if any).

For those who are genetically male (XY), ambiguity is usually the result of a deficiency of masculinizing hormones at a critical stage in development. For genetic females (XX), it's the converse. The root causes are complex and varied. In XY children the deficiency can arise from diminished testicular development, Leydig cell aplasia (which impairs testosterone production), androgen insensitivity, 5 alpha reductase deficiency or environmental exposure to hormones (as can happen if mom doesn't know she was pregnant and keeps taking birth control pills).

While the sex chromosomes can give some indication as to which gender the child is inclined, they are not the whole story. XY babies with complete androgen insensitivity can safely be raised as girls. For those with 5 alpha reductase deficiency, or whose ambiguity arose as a result of environmental exposure, the picture is murkier. Ditto for XX children, who suffer from a disorder of the adrenal gland known as congenital adrenal hyperplasia (CAS). CAS is the most common condition resulting in genital ambiguity. It has variations that affect both XX and XY, but it manifests itself more commonly in females. CAS children are deficient in an enzyme that converts the steroid hormone pregnenolone into cortisol and aldosterone. The excess hormone is instead converted to testosterone, wreaking havoc on the developing fetus. Depending on the severity of the condition, CAS children can end up anywhere on the gender spectrum. Typically, the vagina is closed over and the clitoris is greatly enlarged (often more closely resembling a penis), yet they often retain a functioning vagina and a uterus and ovaries. Interestingly, CAS girls are typically boyish in their behavior and are sexually attracted to women.

Given the variability and inherent unpredictability of these disorders, it seems logical to leave the child to develop naturally without forcing it into one box or the other. Certainly no surgery should be performed until the prognosis is certain. Unfortunately, when it come to our children's genitals, logic doesn't seem to come into play. While XY infants with diminished genitalia are no longer automatically feminized, CAS girls are still routinely subjected to clitoral reductions. These are never performed for anything other than cosmetic/social considerations and the results can be devastating to the child's future sexual happiness, but many parents find the idea of their baby girl sporting a quasi-penis too upsetting to bear. It's understandable I suppose, given the stigma attached to genital deformity.

But while we may have a natural revulsion to the very idea of ambiguous genitalia, it's also true that we have a natural revulsion towards many things we eventually get used to - Brussels spouts being one example. Why can't there be a neutral sex? I don't foresee a day when parents run into the hospital waiting room proudly shouting; "It's an It!" but I do think it's possible for intersex children and adults to be accepted for exactly who they are. Pink, blue or mauve; it should be up to them to decide what color suits them best.

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