The Gender Blur

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We don't do these experiments in humans—for obvious reasons—but there are naturally occurring instances that prove the same point. For instance: In the fetal testes are a group of cells, called Leydig cells, that make testosterone. In rare cases, the fetus doesn't make enough of these cells (a defect known as Leydig cell hypoplasia). In this circumstance we see the limited power of the XY chromosome. These boys have the right chromosomes and the right genes to be boys; they just don't grow a penis. Obstetricians and parents often think they see a baby girl, and these children are routinely raised as daughters. Usually, the "mistake" is caught about the time of puberty, when menstruation doesn't start. A doctor's examination shows the child to be internally male; there are usually small testes, often tucked within the abdomen. As the researchers put it, if the condition had been known from the beginning, "the sisters would have been born as brothers.

Just to emphasize how tricky all this body-building can get, there's a peculiar genetic defect that seems to be clustered by heredity in a small group of villages in the Dominican Republic. The result of the defect is a failure to produce an enzyme that concentrates testosterone, specifically for building the genitals. One obscure little enzyme only, but here's what happens without it: You get a boy with undescended testes and a penis so short and stubby that it resembles an oversized clitoris.

In the mountain villages of this Caribbean nation, people are used to it. The children are usually raised as "conditional" girls. At puberty, the secondary tide of androgens rises and is apparently enough to finish the construction project. The scrotum suddenly descends, the phallus grows, and the child develops a distinctly male body--narrow hips, muscular build, and even slight beard growth. At that point, the family shifts the child over from daughter to son. The dresses are thrown out. He begins to wear male clothes and starts dating girls. People in the Dominican Republic are so familiar with this condition that there's a colloquial name for it: guevedoces, meaning "eggs (or testes) at 12."

It's the comfort level with this slip-slide of sexual identity that's so remarkable and, I imagine, so comforting to the children involved. I'm positive that the sexual transition of these children is less traumatic than the abrupt awareness of the "sisters who would have been brothers." There's a message of tolerance there, well worth repeating, and there are some other key lessons too.

These defects are rare and don't alter the basic male-female division of our species. They do emphasize how fragile those divisions can be. There is no supermale security in possessing a Y chromosome, no femininity guarantee with the double X. Biology allows flexibility, room to change, to vary and grow. With that comes room for error as well. That it's possible to live with these genetic defects, that they don't merely kill us off, is a reminder that we, male and female alike, exist on a continuum of biological possibilities that can overlap and sustain either sex.

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