Androgen Insensitivity Syndrome Or AIS, Have You Ever Hear Of It?

Chances are you have not, it is one of the secret little medical conditions that no one likes to talk about. However, this morning on ABC’s “Good Morning America” they had a segment about it and this evening they are having a long segment on "Medical Mysteries," 10 p.m. ET.

Women With Male DNA All Female
Women with Rare Syndrome Learn to Live with Male Chromosomes
By MARY HANAN
Aug. 11, 2008

For musician Eden Atwood, there have been few signs of the dark secret concealed within her body. A secret so seemingly monstrous, Eden wasn't supposed to know.
Androgen Insensitivity Syndrome is a rare genetic condition.

"If my mother didn't think I should know, and my father didn't think I should know, and the doctors didn't think I should know," Atwood said, "I shouldn't let anybody else know."

What could possibly be so wrong? Throughout her life, Atwood appeared to be the picture of health. She blossomed from an adorable young girl into a striking beauty. She even became a model, an actress and an accomplished jazz singer.
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True Diagnosis

Atwood is not a freak -- nor is she half-man, half-woman. But her DNA says she's a man. That's because she has male chromosomes, an X and a Y, instead of two Xs, like most females. It's a disorder of sexual development in the womb called Androgen Insensitivity Syndrome, or AIS. It can be passed down through the mother or occur as a spontaneous mutation.

"There are probably about seven-and-a-half thousand people, women, in the U.S. with the condition," said Dr. Charmian Quigley, a pediatric endocrinologist.

Despite the male chromosomes, Quigley said, women with AIS are just that -- women.
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Androgen Insensitivity Syndrome

All of us, men and women, have a mix of male and female hormones running through our systems. And as you might expect, the testes of women with AIS produce huge amounts of the typically male hormone testosterone. But here's the hitch: their bodies can't process any of it. And amazingly, they turn it into the typically female hormone estrogen, giving them much more estrogen than the average woman.

These women don't get acne, and have no body odor and minimal sweating. In essence, they are the furthest thing from a male that there could be.

So, why keep it a secret from them? Quigley explained that there was a concept that "if you told them that they had a Y chromosome, or a testicle inside them, but they were externally female, they would completely meltdown."

She even showed ABC News a 1970s medical textbook that says, "It is of no benefit to disclose that the gonads were testes instead of ovaries."

It's a lie doctors have been telling since about 1953, when the syndrome was formally identified. For Atwood, it was the discovery of that lie that shattered her self-image and drove her to sleep with many men in an effort to prove her femininity.

And as for the act of sex, it's pretty much the same. Women with AIS can have orgasms just like the rest of us. But they say the lies about their conditions can interfere with intimacy and become far more toxic than the actual diagnosis.
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Finally Healing

It's taken more than 20 years, but Atwood is finally more comfortable talking about her long-kept secret. She shared it with Bruce Anderson, who eventually married her. But Atwood says the lies told to her about her AIS gave her intimacy issues; she and Bruce eventually divorced.

They have, however, remained close. In fact, Anderson is still often found in the kitchen, making meals with Ben, the 4-year-old son they adopted as an infant.

Thanks to hormone therapy, this woman -- with male chromosomes and no womb -- was actually able to breast-feed. Atwood said it was an incredible act that "sewed up some of the Swiss cheese of [her] soul.

"I check the box every time it comes up: Male or Female? Female, " she said confidently. "But only because the box is there."

This story is about Complete Androgen Insensitivity Syndrome (CAIS) but there is also Partial or Incomplete Androgen Insensitivity Syndrome where the effects are not a pronounced as CAIS, where some of the over four hundred androgen receptors in body are insensitive to androgens. When this occurs in the BSTc region of the brain it is thought to cause transsexualism (for a technical discussion read “Male-to-Female Transsexuals Have Female Neuron Numbers in a Limbic Nucleus”), this is now being called Harry Benjamin Syndrome in Europe and is considered a medical condition and a form of Intersex.

Nature is not just black and white, but all the shades of grey. Gender does not just come in male and female, but a whole multitude of blends male and female.