Between XX and XY

Intersexuality and the Myth of Two Sexes

Chicago Review Press Incorporated

Contents

The Puzzle of Intersex: The Story of Lenore

It might have been one of those Los Angeles days when the soup rolled in off the sea and sopped up the sky, one of those days when people were left with nothing more than sputtering electric fans and limp palm trees curdling in the oily light. It might have been, but the report doesn't mention any of that. So it might have been otherwise.

For certain, it was the summer of 1952. Harry Truman was still president, the Dodgers were in the process of losing more road games than they've lost since, and the Ford Motor Company was preparing for its fiftieth anniversary. That summer, in the city of angels, a baby was born to two very proud parents. I will call them Frank and Laura. The baby — let's call her Lenore — was the second child in what would grow to be a family of twelve, and Frank and Laura were first cousins. Maybe that's important; maybe their genetics had something to do with the way Lenore turned out. Maybe not.

At birth, Lenore was everything everyone had hoped: all her digits present in the proper places and numbers, beautiful eyes and hair, pink gums and stubby toes. Everything about her looked perfect, with one tiny exception. Well, actually, not so tiny. Lenore's clitoris was a little too big for a baby girl. "Hypertrophied" was what the doctor called it. But after some further probing the doctor found what seemed to be a vagina, so he announced, with a big smile, "It's a girl."

As the doctor's words split the air that day, one door opened and another one closed. No one noticed.

Wrapped in pink, Lenore went home, and for the next several years things seemed just fine. Lenore did all the things a baby girl should do. Then, when she was six years old, Lenore — like many kids her age — got the measles. Her mother took her to see the doctor. Once again, nothing about her physical exam seemed out of order, except for that clitoris thing. It still seemed a little big, but not too big, at least not so big that anyone felt compelled to do something about it.

By age thirteen, Lenore had begun to develop pubic and underarm hair, just as any girl her age should. Curiously, though, unlike her friends, Lenore's chest remained as flat as a boy's, and she had not begun to menstruate.

That same year, Lenore developed tonsillitis and went to the hospital to have her tonsils removed. During Lenore's intake exam, the doctor who examined her — a particularly thorough sort, apparently — again noticed the hypertrophy of Lenore's clitoris. However, unlike the others who had noted the same excess, this doctor felt the need to do something more. When he looked at her more closely, he found that Lenore's vagina was only rudimentary — too short and too small to be functional.

This, combined with Lenore's flat chest and lack of menstruation, puzzled the doctor. So he referred Lenore to the Adolescent Children's Unit of Childrens Hospital of Los Angeles for further tests. A pediatrician there performed a complete physical evaluation. In the fall of 1966, Dr. Betty Suits Tibbs wrote up the course of that evaluation, and the story of Lenore's short life to that point, in the clinical journal Pediatrics. Dr. Tibbs's tale is the foundation for this story. Since Dr. Tibbs's report doesn't name Lenore's physician at Childrens Hospital of Los Angeles, I'll call him Dr. Brown. Here is his description of Lenore:

Pertinent physical findings revealed moderate hirsutism of the face [facial hair] with slight acne. The thyroid gland was not palpable. Examination of the chest revealed a normal contour but no breast development. There were no cardiac murmurs and the heart was not enlarged. Axillary hair was ample and the pubic hair formed a female escutcheon. The clitoris was hypertrophied, appearing as a small penis with a urethral opening at its base. There was a small vaginal opening posterior to the urethral opening.

According to Dr. Brown, when Lenore appeared before him, her outward physical appearance and mannerisms seemed more boyish than girlish. In addition, Lenore's vagina was only about 2.5 centimeters deep — about an inch — and, palpate as he would, Dr. Brown could not find a uterus in Lenore's abdomen. All of this led him to suspect that in spite of her history, Lenore was in fact a boy. That seemed worth following up on, so he scraped some cells out of Lenore's mouth and sent them off for analysis.

A few days later the results of Lenore's tests came back. The genetic expectation for girls is that each cell will have two X chromosomes. Normally, early in the embryonic development of girls, in every cell of the embryo one of those two X chromosomes is inactivated and condenses into a small inert lump called a Barr body, or sex chromatin. The presence of sex chromatin usually identifies a cell as female. Dr. Brown could find no evidence of any sex chromatin in any of Lenore's cells. So, at her next visit, Dr. Brown took some blood from Lenore and sent the white cells off for analysis. The results of those tests showed that most of Lenore's cells had the right number of total chromosomes, forty- six, but the tests also showed that buried inside of every one of Lenore's cells there was an X and a Y chromosome. Lenore's cells were genetically male. Analysis of Lenore's hormones revealed a shortage of female hormones. It all fit, except for the fact that Lenore still thought of herself as a girl.

Because of his findings, Dr. Brown referred Lenore for psychiatric evaluation. Apparently, he wanted to find out how well Lenore's mind had adapted to life as a girl in spite of her Y chromosomes.

Dr. Tibbs reported the results of those tests:

The results of the psychiatric evaluation revealed a low average intelligence. ... The patient never actually expressed confusion on direct query as to whether she was male or female, but did demonstrate some masculine aggressive behavior patterns and fantasies. Dreams and projective tests interpreted by both the psychologist and psychiatrist also revealed some ambivalence in her gender identity. On the conscious level, the identity patterns conformed to the assigned sex.

In other words, on a conscious level Lenore acted like a girl. But subconsciously she behaved more like a boy, which fit with the way she looked, not to mention her mysteriously silent Y chromosomes.

After that analysis, it appears that Dr. Brown suggested to Lenore's parents that their daughter might be different from other children. But the parents' discomfort with the subject outweighed their curiosity. Frank and Laura seemed to have little interest in exploring the possibility of Lenore's apparent intersex condition. According to the report, Frank and Laura were "in denial." Dr. Brown's findings did, however, make Lenore's parents uneasy enough to ask if Lenore was "all boy or all girl." Dr. Brown and the doctors he worked with decided not to push the matter. They assured the parents that the physicians were just following up on the diagnosis of amenorrhea and hirsutism and that there were many possible causes of these symptoms, "rather than dwelling on the possibilities of intersex and their ramifications." Dr. Brown and his colleagues never again raised the specter of intersex with Lenore's parents.

Lenore was not the first person Dr. Brown had seen or read about with a condition like this. By the 1960s, intersex was certainly a known human syndrome, and the medical literature, albeit a patchwork of fact and opinion, contained many descriptions of intersex children. It was clear that children didn't always come in tidy packages. What was far less clear was how best to treat such babies and children. At the time, some physicians thought they could do just about anything with a child's sex. Give any baby the right clothes, the right toys, the right parents, and the right instructions, and that baby would become a boy or girl. Other physicians who studied intersex weren't so certain, especially with children as old as Lenore.

Lenore's age complicated matters. Still, Dr. Brown felt he had to act. Almost everyone accepted that something had to be done. The child had to be either a "she" or a "he." But before he made his final decision, Dr. Brown wanted to investigate further. He told Frank and Laura that the doctors needed to do a laparotomy on Lenore so they could determine why she wasn't menstruating. A laparotomy is an invasive procedure requiring a sizable incision through the abdominal muscles and some degree of poking around inside the patient's abdominal cavity. Many important things reside inside that cavity, such as the liver, pancreas, and gall bladder, so there is significant risk associated with a laparotomy. Regardless, at their doctor's suggestion, Frank and Laura agreed to the surgery. So did Lenore. They all wanted to know why she wasn't menstruating. In fact, Lenore, now fourteen, went so far as to thank the doctors for "making themselves available to her at this time."

During the exploratory surgery, the doctors discovered that Lenore had two undescended testes. They also found a uterus of sorts and rudimentary Fallopian tubes. That confirmed everything Dr. Brown and the others suspected — that Lenore, who had been raised for fourteen years as a girl and whose genitalia were mostly those of a girl's, in fact had not only the chromosomes but also the gonads of a boy.

Some minds were probably made up even before the surgery. But now the doctors agreed on the spot: Lenore's life might just as well continue as a girl's life. So during the laparotomy, the doctors took it upon themselves to remove Lenore's testes. They later told Lenore and her parents that her gonads were abnormal, so the doctors removed them. It isn't clear whether the doctors ever told the parents or anyone else that those "abnormal" gonads were, in fact, testes and not ovaries. They did say that it was because of this abnormality that Lenore did not menstruate. Once again the doctors assured the parents that there was no doubt about Lenore's sex. The orchidectomy, or removal of the testicles, was the first step in shoring up that reassurance. Further procedures would set it in concrete.

Three months after the laparotomy, the doctors took Lenore back into surgery (the report is a little vague about the justification for this surgery) and performed a clitorectomy; that is, they cut off Lenore's too-small penis or too-large clitoris (whichever it was) to make her look more feminine. Then Dr. Brown administered a full course of estrogen therapy.

To the doctors, it seemed that the only remaining problem was Lenore's lack of ovaries. Simply by providing her with the hormones that nature had denied her, the doctors could give Lenore that final push down the road to womanhood. In part, at least, the doctors were right. Injections would provide Lenore with the estrogen she lacked and stimulate secondary sexual development, especially breast formation. But beyond that no one knew for sure what all those hormones might do.

It appears that each of the physicians involved felt certain that Lenore's best future was as an assigned and surgically simplified, chemically enhanced female, regardless of her Y chromosomes and her testes. We can't know all of the factors involved in their decision, but it's probable that Lenore's fourteen years spent as a girl played a part. It's also likely that the difficulty of reconstructive surgery to make genitalia appear more masculine influenced them as well. And perhaps there were other factors. One thing is clear in Dr. Tibbs's report: no one considered the option of leaving Lenore as they found her. Lenore, as she came to them, didn't fit into either of the two spaces human minds reserve for human beings — she wasn't clearly a boy or a girl. Empathy, morality, and kindness surely played a role in the doctors' decisions, but so too did a certain deep predisposition.

Even though Lenore had been swimming in a pond of male hormones for over a dozen years, the doctors scalpeled and hormoned Lenore — a 46,XY person — into a girl. With the hormones given her, Lenore's facial hair cleared up and her breasts began to grow, gracing her chest with one of the most noticeable badges of womanhood.

But of course none of this really solved the problem, if in fact there ever was a problem that needed solving. In the end, to some Lenore was clearly a girl. At the same time, to others Lenore remained a boy. But Lenore was neither.

What, then, should we call Lenore?

Dr. Betty Suits Tibbs's report ends with Lenore in the tenth grade — maybe sixteen years old. Dr. Tibbs observed, "The patient is in tenth grade at present and has made a very good adjustment. It is felt that with her drive and capacities, the prognosis for her identity as a woman is quite good." The rest of Lenore's story we can only imagine.

Every year in the United States, approximately one thousand babies are born with cystic fibrosis and about four hundred are born with hemophilia. Few of us have to ask what hemophilia or cystic fibrosis are. We might not fully understand what causes these disorders, but we know that either one can make a person's life very difficult. Curiously, no one seems to know just how many babies of indeterminate sex like Lenore are born in the United States every year. Estimates range from one thousand to fifteen thousand. It seems probable that the correct number is nearer to the lower estimate than the higher one. Regardless, it is a substantial number of people. We now refer to these people collectively as intersex, or people with disorders of sex development (DSDs). The birth of an intersex child is a difficult event for family and physicians. They must select, from very few options, the least-bad alternative with the hope that, even in their ignorance, even with the paucity of language available to speak about these children, even under the weight of history and fear, they may create a better future for their new child.

Surprisingly, until very recently, standard practice usually excluded the child and the parents from the decision-making process. The physicians made the choice of boy or girl and did what they could do to ensure that the child would walk that path for the rest of his or her life. Physicians believed they knew best and that the input of others was unnecessary. They believed that the fewer who knew about what had happened at birth, the less likely it was that someone might raise the veil so carefully woven and placed by their hands.

As a result, for years hardly anyone outside the medical community had heard of the thousands upon thousands of children like Lenore.

And even today, most of us hear little about these people who, like Lenore, fall through the cracks in our language and raise serious questions about our cast-iron ideas about two opposite sexes. Maybe we don't hear much about these people simply because we don't want to hear about them. They make us even more uneasy about things we are already sufficiently uneasy about — things like human sex. But regardless of our discomfort, these are people, and their stories are important, because wrapped up inside of them is a secret that all of us should know, a secret about what it truly means to be human.

A Brief History of Sex

In many ways Lenore's future was laid out years before she was born. As Dr. Brown and his colleagues pondered Lenore's situation, millennia of human thought about sex and intersex molded their ideas. As long as human beings have walked upright, we've been thinking about how we acquire sex, how we have sex, and why we need sex. And though most of us may believe that humans have always thought in more or less the same ways about the sexual character of human beings, history doesn't support that assumption. In fact, we have not even always believed that humans come in two, and only two, opposite sexes.

Ancient Greek Sex (c. 450 B.C.–A.D. 200): The Power of One

Our access to musings about the biology of human sex begins about twenty-four hundred years ago with people like Hippocrates (c. 460–c. 377 B.C.), Aristotle (384–322 B.C.), and Galen (A.D. 129–c. 199). Hippocrates, the father of medicine, forced the first separation of religion and science. He argued for the natural origins of disease and death and shunned the gods. He was a logical man and a careful observer of humankind. Nothing he proposed originated from fancy or spur-of-the-moment decisions. He was thorough and methodical.

Hippocrates proposed that menstrual blood and sperm were in essence the same substance. Women shed menstrual blood, he said, when an excess of nutrients accumulated in the blood. Men, instead, refined blood foam into sperm and passed it along to the brain. The sperm then made its way through the spinal marrow, into the kidneys, to the testicles, and finally into the penis itself. Hippocrates clearly saw differences between men and women, but for him the differences were of process — like subject and exposition, parts of a single fugue — not basic differences in nature, and certainly not opposite sexes. He even proposed that both men and women produced sperm, or seed, and that only the strength of their seed differed. Even then, he said, sometimes men produced strong sperm and at other times weak sperm, and the same was true for women. Men, of course, because they were stronger, came from the strong sperm. Nevertheless, to Hippocrates, men and women came from a single mold.