by By Will Evans — Sacramento Bee on 28 April 2004
David Cameron feels neither completely male nor female. Born with male genitalia, Cameron began growing breasts during puberty and didn’t sprout chest hair until testosterone treatment kicked in. Instead of the typical male XY chromosomes or the female XX set, Cameron has XXY.
“I feel sort of like a blend,” says Cameron, 56, of San Francisco.
Some researchers say that’s a reasonable conclusion. Humans don’t always clearly divide into male and female categories. Some are born with abnormalities that challenge the very definition of sex. The term for them is intersex. Julia, a schoolteacher from a small town in central California, didn’t want to be identified to protect her daughter. Now 4, the girl has a condition that caused an enlarged clitoris.
Doctors couldn’t tell Julia her baby’s sex until after several days of testing. They first came to her with a box of tissues, announcing, “We have a problem.”
Julia felt hot from head to toe from the shock. She remembers the hospital bracelet that said only “baby” instead of “boy” or “girl.” She cried at the thought of her child’s future challenges. “Oh, what a hard life,” she told her husband.
The concept of intersex that links Cameron and the little girl is too blurry to yield a definition with which everyone agrees. Many people with XXY chromosomes, for example, consider themselves absolutely male and distance themselves from the intersex world.
But prominent academics and activists define intersex as anyone whose sex chromosomes, external genitalia or internal reproductive system is not considered standard for male or female.
Peter Trinkl, a computer specialist in Berkeley, doesn’t really know how he looked at birth. All he has to work with are his genital scars, evidence of surgery. His parents didn’t tell him much. In school, he was beaten up and called an alien.
Trinkl, 51, considers himself a heterosexual male, but dating brings up difficult issues, and he hasn’t tried for 20 years.
“If I’m a man or a woman, I don’t want to get too much into that,” he says.
Only recently did Trinkl summon the courage, he says, to research the intersex community and hunt for his medical records.
Some infants are born with ambiguous genitalia while others clearly look male or female and may not find out they are different until they reach puberty. Still others bear a visible difference in anatomy – an enlarged clitoris or a tiny penis – but otherwise can be determined male or female. And some have the standard chromosomes of one sex and the external appearance of the other.
Intersex activists decry the shame and secrecy caging their condition. They urge doctors to avoid cosmetic genital surgery on intersex infants until the children themselves can decide if they want it. Cameron is helping to organize a public hearing on intersex issues to be held by San Francisco’s Human Rights Commission next month.
Children frequently are born with wide-ranging genetic and physical abnormalities. Intersex conditions just happen to manifest in an area that gets at the very definition of who we are.
What defines a person’s sex – their chromosomes, their appearance or their psyche? What if they don’t match?
How can you assign a sex to a child when you don’t really know? How can you not?
What if you surgically reconstruct a baby to look like one sex and the child grows up to identify as the other? What does gay or straight mean, then?
And when everything from color-coded baby presents on out is sexually segregated, is it possible to grow up as an alternative to male or female?
The mind-boggling, gender-bending conundrum plays out in people’s lives.
Intersex people might make up as much as 2 percent of live births, with between 0.1 percent and 0.2 percent of all infants receiving genital surgery, according to a scientific journal article co-written by Anne Fausto-Sterling, a professor of biology and gender studies at Brown University.
“If you look at this from the bigger philosophical view of, ‘Are there really only two kinds of people in the world – either men or women?’ – then the answer to that clearly is no,” she says.
Human sexuality, instead, can be seen as a spectrum or continuum, she says.
The medical profession has traditionally viewed an intersex birth as a “social emergency,” pushing to assign a child’s sex immediately and perform corrective surgery as soon as possible, says Celia Kaye, a professor of pediatrics at the University of Texas Health Science Center at San Antonio. Doctors want to avoid traumatizing parents and help the child grow up normally, without confusion or ridicule, she says.
Kaye helped create the American Academy of Pediatrics’ policy statement on intersex newborns along these lines in 2000. But the academy might revise its guidelines because of a growing number in the field who question whether surgery and sex assignment should take place so early in life.
A baby with an enlarged clitoris or minuscule penis, depending on one’s perspective, conventionally has been more likely to be determined a female because it’s surgically easier to make that happen, Kaye says. But it’s not clear, she says, whether that child will grow to be a happy, functioning woman. Some activists call it “genital mutilation.”
Sonoma County resident Cheryl Chase, 47, had surgery on her enlarged clitoris, leaving a “big, flat scar.” But she says the biggest harm doctors caused was “the idea that this was shameful,” telling her parents to keep it a secret.
In the early 1990s, Chase, who identifies herself as an intersex lesbian female, confronted doctors, called the press and founded the Intersex Society of North America, creating today’s intersex movement.
Because of pressure from advocates, doctors are now more open with patients and more likely to present parents with options rather than telling them what to do, says Amy Wisniewski, who does intersex research at the Johns Hopkins Children’s Hospital.
Julia, mother of the 4-year-old girl, says one of her daughter’s doctors “bullied” her into making a surgery appointment. Some surgery is necessary when the toddler hits puberty, but decreasing her clitoris is optional and cosmetic.
Because doctors can’t guarantee a post-surgery clitoris will retain the same sexual sensation, Julia worried her consent may deprive her daughter of a vital part of life. Julia cried every day until she finally canceled the surgery.
“We’re going to leave the decision up to her and talk to her and support her when she’s old enough to make that decision,” Julia says over the phone.
How old is that? If you can delay surgery, can you also put off assigning a sex?
The questions build quickly, but most people are stuck at the first one: “What is intersex?” The Lesbian, Gay, Bisexual, Transgender Resource Center at the University of California, Davis, held a talk on exactly that as part of its first Intersex Awareness Week earlier this month.
It’s not clear, Wisniewski says, whether rates of homosexuality are higher among intersex people. But because it shares a battle against the closet, the gay community has embraced the intersex populace, with some organizations adding “I” to the alphabet soup of LGBT.
Still, some with sex chromosome variations keep as far away from both communities as possible.
Those with Klinefelter’s syndrome, or XXY, struggle in a world that glorifies a man’s-man masculinity and sexual prowess, mocking androgynous qualities in men as signs of homosexuality. They’re already marked by that extra “female” chromosome and, for some, breast development and smaller genitalia. The last thing many want is to be aligned with the gay community.
Melissa Aylstock of Loomis is clear: Her XXY son is unambiguously male, and most men with Klinefelter’s syndrome don’t consider themselves intersex. Her son’s doctor, Ronald Swerdloff, chief of endocrinology at Harbor UCLA Medical Center, doesn’t consider Klinefelter’s syndrome intersex, either, because it doesn’t produce ambiguous genitalia.
When her son was diagnosed at age 8, Aylstock was “scared to death.” She wrote to Ann Landers, asking that a post-office box address be published for other parents to get in contact. After the letter ran in 1989, Aylstock received 1,000 letters and hundreds of dollars to start an organization. She founded Klinefelter Syndrome and Associates in Roseville.
Testosterone treatment is the norm for Aylstock’s son, now 23. In the school gym, students asked about his patch. He told them it was for nicotine addiction. “Mind you, we’re Mormon,” says his mother. “That just cracks me up. So he handled it.”
The son declined to talk about his condition in the context of the intersex community.
“So many guys are trying to be just normal,” says Robert Grace of Sonora, who found out at 39 he has XXY chromosomes. When he told people, they thought, “Oh, you’re gay,” he says.
When Grace should have been going through puberty, he watched the other boys whistling at girls and thought, “What jerks.” But he wasn’t gay.
His diagnosis popped up during his premarital physical. “I looked at my (fiancï¿½e) and I said, ‘You don’t have to marry me.’ ”
They did marry and have adopted four children, two of whom also have Klinefelter’s syndrome.
“As a general population, we really would like to be accepted,” says Grace, a “stay-at-home Mr. Mom.” “If I sat next to you, you would have no clue that I was XXY, so why do we need another label?”
Cameron, on the other hand, embraces the other label.
Cameron’s birth certificate and driver’s license declare that “he” is male. With a 6-foot-10 build, a balding head, a deep voice and a beard, Cameron could hardly pass for female yet feels more female than male.
When faced with those annoying little boxes designating “M” or “F” on forms and applications, Cameron might check both or write “intersex.” It somehow seems appropriate that Cameron sometimes goes by the nickname “Iris,” after a favorite flower, the bearded iris.
Cameron got the Klinefelter’s diagnosis at 29 and began testosterone therapy. Where before Cameron had a “really nice smooth body,” now everywhere is hair. “I hate it,” Cameron says. “Quite frankly, I would really like the body I had 27 years ago back.”
Cameron has been with the same male partner for 26 years, though before that Cameron had a girlfriend. Earlier this month, the partner dropped to his knees and presented Cameron a diamond ring.
Cameron wants to wed but first is inquiring with civil rights lawyers because of the radical questions the act could provoke.
After all, would it be a standard marriage, a same-sex marriage or something else entirely?
Misused terms add confusion
The term “intersex,” according to advocates and academics, means anyone with sex chromosomes, external genitalia or an internal reproductive system not considered standard for male or female. Here’s what intersex is not.
Hermaphrodite: The medical definition of a true hermaphrodite is someone with both ovarian and testicular tissue. This is rare and only one of various intersex conditions. Many intersex people consider this term offensive.
Homosexual: Some intersex people are gay, some are not. One doesn’t imply the other.
Transgender: This refers to people who are born one sex but identify as the other. Some choose a sex-change operation.
Eunuch: This refers to a castrated male.
Genetic roots of intersex conditions
Intersex conditions vary in their genetic roots and physical manifestations. Here are details of a few conditions.
Androgen insensitivity syndrome: Patients have male chromosomes (XY) but don’t respond to androgens (male sex hormones, including testosterone). They have undescended testes, normal female external genitalia and breast development. Those with partial androgen insensitivity syndrome have ambiguous genitalia.
Gonadal dysgenesis: Patients have XY chromosomes, but their gonads don’t produce androgens. They have female external genitalia. Those with partial gonadal dysgenesis have ambiguous genitalia.
5-alpha-reductase deficiency: Patients have XY chromosomes but can’t produce the sex hormone dihydrotestosterone. They have testes, a penis resembling a clitoris and a scrotum resembling outer labia. They undergo some masculinizing changes during puberty.
Congenital adrenal hyperplasia: Patients have female chromosomes (XX) but produce excess androgens. They have ovaries, an enlarged clitoris and fused labia resembling a scrotum.
Klinefelter’s syndrome: Patients have the sex chromosome variation XXY and are sterile. They have male genitalia, sometimes with smaller sex organs, and sometimes develop breasts at puberty.
Turner syndrome: Patients have the chromosome variation of only one X. They have normal female external genitalia but can have other physical abnormalities. Because they don’t have functioning ovaries, puberty doesn’t cause breast development or menstruation.
Source: The Johns Hopkins Children’s Center
* Bodies Like Ours support group with online forums: www.bodieslikeours.org, (610) 258-7466.
* Intersex Society of North America: www.isna.org.
* Klinefelter Syndrome and Associates: www.genetic.org, (888) 999-9428.
* The Johns Hopkins Children’s Center guide for patients and parents: www.hopkinsmedicine.org/pediatricendocrinology/intersex.