Between XX and XY | Notes

Gerald N. Callahan, Ph.D. Between XX and XY: Intersexuality and the Myth of Two Sexes. Chicago Review Press, 2009. (195 pages)



Human sex is not something that switches irreversibly between two poles–male and female–like an on/off switch on a radio. Rather it is like the bass and treble knobs on that radio. Pure bass or pure treble are impossible to achieve, but in between those two exists an infinite number of possible mixtures. Inside that infinity of possibilities each one of us is nestled in the vastness between pure male and pure female. (xii)

| My purpose is not to convince you that we need to imagine more sexes, because the concept of five sexes would be no closer to solving the problem than the idea of two sexes is. Instead, I wish to offer other ways of thinking about sex–ways that aren’t so constraining or exclusive, ways that might even change how we think about ourselves. (xii)

1. The Puzzle of Intersex: The Story of Lenore

Curiously, no one seems to know just how many babies of indeterminate se 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. (7)

| 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. (7)

As a result, for years hardly anyone outside the medical community had heard of the thousands upon thousands of children like Lenore. (8)

| 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. (8)

2. A Brief History of Sex

…according to Aristotle, a castrated male became essentially a female. (10)

The Greeks’ ideas about sex may seem strange or silly to us today, but the accuracy of their beliefs is not the point. It is how the ways we have thought about sex have changed over time that is important. Absurd or not, the Greeks’ views remained dominant among physicians, scientists, and laypeople alike until around the time of the Enlightenment in the eighteenth century. (12)

Renaissance Sex: Leonardo da Vinci (1452-1519) and Andreas Vesalius (1514-1564)

I expose to men the origin of their first, and perhaps second, reason for existing. – Leonardo

…it is interesting that, at the time, Catholic Church doctrine fit a lot better with Leonardo’s image of two tubes through the penis, which would keep pure sperm “unpolluted” by urine. In addition, over a thousand years before Leonardo, Hippocrates had proposed that the brain produced sperm and delivered it to the penis, even though Hippocrates never saw such a duct either. The Church also liked that idea, because it separated the holy act of reproduction from the base function of elimination. Old ideas die hard, and political ideas backed by the strong arm of governing religious institutions fitted with he talons of the Spanish Inquisition also acquire a certain attractiveness they might otherwise lack. (15)

From Vesalius and his disciples this vision spread. Nearly every drawing of women’s genitalia for the next three hundred years would look like those first created by Vesalius and Valverde de Amusco. For most of the first two millennia of recorded history, men and women appeared more alike than they ever have since. (16)

Believing Is Seeing

Looking at some of the drawings of Leonardo, Vesalius, and Valverde de Amusco, my first temptation is to believe that the anatomists who created them simply lied about what they saw–just deleted, reshaped, and created things to suit their expectations and popular beliefs. But these were some of the best-trained and most critical observers alive. They sought the truth,… I have to believe that these drawings reflect what these men truly saw–men and women as very nearly alike. (17)

Similarly, as long as people believed and expected that men and women were more alike than different, as long as it seemed sperm should come from the brain, then that was what people uncovered when they poked around inside the dead. The ideas were the important thing, the corpses and dissections were needed only to confirm those ideas. From Hippocrates to Vesalius, for over two thousand years, that’s how things stood with human sex, until the Western world found itself not he verge of the Enlightenment. (18)

Columbus Discovers the Clitoris: The New World of Sex


It is ‘preeminently the seat of a woman’s delight.’ Like a penis, ‘if you touch it you will find it rendered a little harder and oblong to such a degree that it shows itself as s sort of male member…Since no one has discerned these projections’ workings, if it is permissible to give names to things discovered by me, it should be called the love or sweetness of Venus. – Matteo Renaldus Columbus (or Realdo Colombo)… De Re Anatomica

First, and most important, it seemed to contradict the one-sex hypothesis then popular. Now, according to Columbus, a woman had an exterior counterpart to a man’s penis. How could a woman have “two penises” and still be the perfect homologue of and basically the same as a man? That rattled the foundations of then-current thought. But once everyone took a careful look, they had to agree–the clitoris was in fact there, and it did seem a lot like a little penis. That began to work at people’s minds. Where certainty had ruled for nearly two thousand years, a seed of doubt began to sprout. (19)

Gabriel Fallopius, Observationes Anatomicae, 1561.; G. B. Carcano; Kaspar Bartholin

Eventually, by the beginning of the eighteenth century, the one-sex theory finally began to crumble. Along with that change came changes in our language. Before the eighteenth century, men and women were thought to be so much alike physically that no one thought we needed different words for our similar parts. Now words were invented for the vagina and the uterus, previously unnamed because of their obvious homology to the male penis and the scrotum. The same was true for the ovaries, known until then as female testes. (21)

It is impossible to name with certainty all of the forces behind the shift from seeing ourselves as one to seeing ourselves as two. Some people have argued that the roots are political, others that it was a misguided effort to somehow link physiological differences to social differences–to tie sex to gender. But what we can know is that once human beings thought of themselves very differently than we do now, and that once we were a little more taken with our similarities than with our differences. (21)

[via: אדם vs. איש ואישה]

Sex and Sexual Intercourse: A History of Obsession

R. L. Dickinson.

Interestingly, in none of these studies did the authors think to question whether their findings and conclusions would be meaningful for more than the one man and one woman who had participated in the particular study. No one ever asked whether the obvious differences in genitalia among men and among women might have some relevance, or considered that the anatomy of intercourse might be as varied as the men and women engaging in it. (23)

You’d think that after all these years, after all the drawings and pictures, the ultrasounds and MRIs, we would have it right by now. Yet the sexual anatomy of human beings remains an evolving and sometimes contentious concept. (23)

Changing Times, Changing Sexes: Science as a Moveable Feast

…nested just above human and most other mammals’ hearts is an organ called the thymus. In immunology there is no organ as singularly important as the thymus; within its membranous walls, our immune systems learn the mystery of self/non-self discrimination. Without thymuses, humans and other animals disintegrate under the onslaught of infection. If you transplant skin from, say, a normal chicken onto a normal mouse, within just a few days the (23) mouse will mount a violent immunological attack not he chicken skin and reject it. If you perform the same experiment using normal chicken skin and a mouse without a thymus, the mouse will grow feathers. Mice without thymuses have lost the ability to distinguish themselves from chickens. That is a very serious sort of identity crisis. (24)

Has-Reimer Rotewald.

…less than five years ago, any knowledgeable neuroscientist would have told you unequivocally that brains don’t make new neurons. You were born with all you will ever have, and that’s it. This is absolutely wrong. And until just a few months ago, we were told that most mammals began life with all of the eggs they would ever have, and for at least some mammals that is equally wrong. (24)

I no longer assume that our perception of the sexual anatomies of males and females is static and beyond revision. If such important things as an extra thymus or thymuses or new brain neurons could be ignored into the twenty-first century, what else have we overlooked? What more will we still find among the human rubble we have been staring at so persistently for so long? (25)

Another Side of Sex: A Brief History of Intersex

cf. Brisson, L., Sexual Ambivalence: Androgyny and Hermaphroditism in Graeco-Roman Antiquity.

Sex Under the Microscope

As powerful as the sciences of anatomy and histology were, the false twins of sex disposed of them as quickly and as easily as it had blinded other human eyes. (28)

Sex in the Genes

Karyotyping is an assessment of all of a person’s chromosomes, a measure of each of the wormy curls that wrap themselves inside the nucleus of every one of our cells. (29)

At first karyotyping seemed to have finally laid all ambiguity to rest. But pretty quickly we discovered that whole classes of people have more or fewer than forty-six chromosomes, often including unusual numbers of sex chromosomes. (29)

1945… These researchers concluded that “the hermaphrodite assumes a heterosexual libido and sex role that accords primarily with his or her masculine or physical upbringing,” not his or her chromosomes. (29)

1955… …”the gender assignment in infancy will be the one the patient stays with into adulthood, regardless of the standard biological indicators of sex.” This meant that, to these researchers, it appeared that people tended to end up being what other people told them to be, not what their chromosomes predicted they should be. That fractured a lot of paradigm about childhood development. (30)

| The group at Johns Hopkins, as well as many others dropped the “true sex” policy (such as XX or XY) and adopted the “optimal gender” policy for assigning sex to sexually ambiguous children. According to this policy, proposed by Dr. John Money and his colleagues at Johns Hopkins, “the assignment or reassignment of gender should be based on the expected optimal outcome in terms of psychosexual, reproductive, and overall psychologic/psychiatric functioning.” Translated, that meant that it was up to the pediatricians, endocrinologists, plastic surgeons, and parents–not chromosomes, gonads, or the vagaries of biology–to determine the sex of the child. Furthermore, this protocol suggested surgery as early as possible to quickly unify physical appearance and the gender expectations of all involved. Though many of these ideas have since been discredited, they took hold like grappling hooks in the medical community of the time. (30)

Near the end of the [1950s], a group of scientists studying guinea pigs discovered that early exposure to sex hormones had a major effect on sexual orientation of the brain. (31)

1971… An Enzyme called 17-beta-hydroxysteroid dehydrogenase (17β-HSD) normally helps to convert androstenedione to testosterone in the developing fetus. Several different mutations can cause deficiencies in 17β-HSD. When that happens, there is less testosterone in the developing fetus. Because of that, most of these children–though 46,XY–are born with female genitalia and identified and raised as baby girls. But at puberty, many of these children begin to produce nearly male amounts of testosterone, develop phalluses much like penises, grow beards, acquire large muscles, and speak with the voices of men–they undergo a nearly complete reversal of sex. (31)

| Then in 194 another study appeared that described a second syndrome called 5-alpha reductase deficiency, or 5α-RD. This syndrome results from mutations in a gene encoding 5-alpha reductase (5αR). This enzyme is responsible for the production of another hormone essential for making baby boys. First, like boys with deficiencies in 17β-HSD, (31) genetic males with 5α-RD are born with female external genitalia, and are consistently identified and raised as baby girls. And with 5α-RD, at puberty, these girls again become boys, or nearly so. (32)

Somewhere within that brew of hormones and genes and dolls and toy soldiers there may be some deep secret about how we all come to be who we are. Or maybe not. Regardless, two things are clear. First, no one has yet figured out the list of ingredients needed to make a boy or a girl–neither chromosomes, nor hormones, nor genes, nor family or society or chance, alone or in combination, seems sufficient to explain how one’s sex comes to be. And second, there is no hard reason why we’ve come to believe that people even need to be a boy or a girl, no hard reason whatsoever. (32)

3. Sex Versus Reproduction: Why Are We So Married to the Idea of Two Sexes?

The more of you there are, the more successful you are, biologically. It’s all about domination, and nothing dominates like sheered numbers. Reproduction provides the means; lust provides the motivation. (34)

In reality, if you look closely, you’ll find that in many species, including our own, reproduction is not the only aim of sexual interaction. And in some species, including our own, reproduction does not even appear to be the primary function of sexual interaction. (34)

Our phenomenal population growth rate is more a testament to our fondness for intercourse than it is to fecundity. (35)

Between ages twenty-five and sixty, the percent of people having sex “at least a few times a month” only drops from 80 percent to 65 percent. But during that same period of our lives, essentially all women and many men go from fertile to sterile. It seems that the likelihood of reproduction has little to do with the magnetism of sexual intercourse. (35)

Sexless Reproduction

Bacteria outweigh all other living things combined. (35)

Somewhere around 1952, some folks in Beltsville, Maryland, noticed that about 16 percent of their turkey eggs developed, without fertilization, into brand-new hens. From those hens, the turkey ranchers developed a strain of turkeys whose eggs developed without fertilization about 40 percent of the time. No one expected that animals as complex and modern as turkeys could so easily be pushed into a single-sex way of life. By the criteria of sheer mass and numbers, most of these animals are much more successful than we are. So why do so many animals and so many plants devote so much time and energy to sex? Put most simply, we don’t know. No one has ever unequivocally established why we have sex instead of just splitting in two every once in a while like bacteria. (36)

The Selfish Gene and the Red Queen

The Red Queen hypothesis proposes that the only thing that saves us from this horrible fate is sex. (39)

| Bacteria divide once every twenty minutes or so, and at every division there is the opportunity for genetic change. In humans, it takes roughly twenty years. Clearly, bacteria evolve much faster than humans do. So our immune systems are always shooting at moving targets. Because of that, we must, as Alice and the Red Queen did, run as fast as we can just to stay in place. We must evolve as quickly as possible just to stay alive. If we didn’t do that–if we didn’t continuously reshuffle our genetic decks–human diversity would stagnate. (39)

The Red Queen theory says that our salvation is reproduction–the mixing of the genes that occurs every times perm and ovum come together to start a new life. The more often we shuffle our genetic deck and eat ourselves new hands, the less likely it is that any microbial body will guess just which hand it must play to beat us. Without sex, our similarities would kill us. (40)

| The Red Queen theory seems sensible, but two rather large problems still remain. First, if sex is so critical to survival, why do we still have sponges and starfish and worms and insects and turkeys? It’s not like these animals are struggling to make it; they outnumber us dramatically (except for turkeys). Then there are also all of those asexually reproducing plants that are doing just fine out there, thank you. Second, as discussed earlier, human sex seems in no particular way to focus on the act of reproduction. People have sex all of the time. What is that about/ (40)

Bonobos at Play

cf. Frans deWaal, Bonobo: The Forgotten Ape

Sex Before Surrender

No one fully understands why bonobos exhibit this sexual frenzy, but experts like de Waal have suggested some possibilities. He suggests that, in this instance, sex eases social tension and is a (44) surrogate for confrontation and fighting, which, as he points out, is not unheard of in other species, including humans. (45)

Survival to adulthood depends very much on the nuclear family–mothers and fathers nurse, care for, and protect the babies. The permanence of that family bond is essential to our survival. Perhaps bonobos’ and humans’ interest in sex also serves the purpose of gluing us to one another, of stabilizing the nuclear unit for the well-being of our babies. (45)

The Truth About Human Sex?

Perhaps sex once served the sole purpose of reproduction, but over millions of years has evolved–just as eyes and ears and muscles and fingers evolved–to serve a multitude of more complex functions, only one of which still (45) focuses on reproduction. (46)

Any species seeking to sate its desires, rework its social structure, and avoid confrontation would set itself on a path to oblivion if the sole purpose of sex were reproduction. The fact that we and the bonobos are still here is testament to the fact that as we evolved we co-opted the sexual urge for a multitude of other purposes–purposes essential to our past, our present, and our future. These purposes are essential to the growth and development of our children–purposes of peace, cooperation, entrepreneurship, pleasure, and promise. If that isn’t sufficient reason for all of us to reconsider how we think about ourselves and our sexes, then at the very least it should be cause for reflection on the value and judgments we place on people whose genitalia don’t obviously lend themselves to reproduction. (46)

4. Where Our Sexes Come From: The Abridged Version

The Story of Life and Sex

Female, in other words, is development’s default mode; without orders to the contrary, women’s bodies make more women. Perhaps that isn’t as surprising as it might seem, given that sponges, starfish, whiptail lizards, and others do the same thing–all without males. Since we evolved from one of those all-female species, maybe it isn’t startling that reproduction’s first choice is female. Only the gradual evolution of the Y chromosome (from an X chromosome) changed that. (52)

| Compared to making girls, making boys is harder work. And though many things can go wrong on the way to making girl babies, there re even more potential detours, pitfalls, and wrong turns on the road to making baby boys. (52)

| The human X chromosome is long, wasp-waisted, and full of information. The Y chromosome, on the other hand, is an odd-looking little blob of DNA. But there are genes on the Y chromosome that can make all the difference when it comes to determining sex. One part in particular of the Y chromosome plays a critical role. We call it the sex-determining region of the Y chromosome, or SRY, and it is essential for making baby boys. (52)

| Genes in this part of the Y chromosome operate like switches to turn on other genes that are critical to male development. Without SRY, the primitive gonads do not develop into testes, and sometimes don’t develop at all. With SRY, the gonads, until now amorphous little tissues, transform into testes and by about four months begin to do some of the things that testes do. (52)

…gonado-tropin-releasing hormone (GNRH). (53)

5-alpha dihydrotestosterone,… 5-alpha reductase types 1 and 2. (53)

So every one of those message-carrying molecules has to be in place, on time, and ready to go or else everything produced by the testes or the developing adrenal glands will have no effect on anything. (53)

| That’s a lot–a lot of molecules, a lot of genes, a lot of coordination–that has to occur properly. And all of it happens during the first ten weeks of pregnancy. (53)

In spite of the label the doctor slaps on us at birth like a bar code, this whole sex process isn’t over, not nearly. (54)

Sexual development is a lifelong journey for us all. And though there are some common signposts along the way, such as puberty and menopause, we shall see how the journey is shrouded in mist, and the path is as varied as human beings themselves. (57)

5. Where Our Sexes Come From: The Rest of the Story

The Truth about X and Y

Every step on the way from zygote to adult is directed by one or more proteins. And each protein has its own set of problems and its own peculiar way of dealing with the world. (61)

| That’s a little like having a set of blueprints that change in critical ways every time someone opens them up for guidance. And it’s a lot like having carpenters, electricians, plumbers, and contractors who all speak slightly different languages. So, even if the blueprints were perfect to begin with (which they never are) the project’s possible outcomes are as variable as the people who use those blueprints, as convoluted as spoken languages, as uncertain as a lottery. (61)

| It’s the same with sex, but even more so. (61)

Sex Chromosomes Lost and Found

…some people–genuine human being people–don’t have forty-six chromosomes. (61)

| People with Down syndrome, for example, have forty-seven chromosomes, including an extra copy of chromosome twenty-one. (61)

…nondisjunction–chromosomes that fail to disjoin as the mother cell divides. And we call these people abnormal, because each of their cells holds one extra strand of DNA, because they have forty-seven instead of forty-six chromosomes. (62)

| Nondisjunction can happen with any chromosome, including the sex chromosomes X and Y. (62)

If that alone isn’t enough to derail the simple XX/XY, female/male idea, a mystery known as anaphase lag can also cause developing sperm or ova to lose an X or Y chromosome along the way. And even after fertilization, sex chromosomes can be lost or gained. And even among men with the normal 46,XY karyotype, the size of the Y chromosome can vary. (62)

The end product is a panoply of possible sexes by any definition, an array of human beings as grand and as varietal as the fragrances of flowers: 45,X; 47,XXX; 48,XXXX; 49,XXXXX; 47,XYY; 47,XXY; 48,XXXY; 49,XXXXY; and  49,XXXYY. (62)

People as Syndromes: Dr. Klinefelter’s Discovery

Harry Fitch Klinefelter, 1937…

It turned out that each of these men also had forty-seven chromosomes. Each was 47,XXY. This syndrome became known as Klinefelter syndrome and grew to include all karyotypes with more than one X chromosome plus one or two Y chromosomes. Because of the presence of a Y chromosome (containing SRY), and all of these people have penises. Because of that we call them males, but they all have more than their fair share of X chromosomes. (63)

| Only about 40 percent of fetuses with extra X chromosomes survive to term. Nevertheless, somewhere between 1 in 500 and 1 in 1,000 boys born in any given day have one or more extra X chromosomes. That makes Klinefelter syndrome the most numerous among those syndromes with an unusual number of chromosomes. (63)

The least common form of Klinefelter syndrome is the 48,XXYY karyotype. These people very closely resemble those with the 47,XXY karyotype except 48,XXYY men are taller, nearly five inches taller, on average, than their fathers. Apparently, one of the things Y chromosomes do is provide the necessary genetic material for the tallness of men in comparison to women. And in a double dose, it seems to push people a little higher. (63)

Dr. Turner’s Findings

The second largest group with unusual numbers of chromosomes includes those with the 45,X ckarytopye, comomonly called XO or Turner syndrome. (63)

| When a sperm containing an X chromosome fertilize an ovum with no X chromosome, the zygote that forms has only forty-five chromosomes. Or if a chromosome gets lost because of anaphase lag or some (63) other reason, the zygote that develops will have only forty-five chromosomes, 45,X. (64)

The 45,X karytope appears in about 0.8 percent of zygotes. That makes 45,X the most common human chromosomal anomaly. But only about 3 percent of these fetuses survive to term. 45,X is also the most common chromosomal anomaly found in spontaneously aborted human fetuses–nearly 18 percent. In the end, about one in 2,700 live newborns has the 45,X karyotype. That is about fifteen hundred newborns per year in the United States and perhaps as many as thirty thousand worldwide. (64)

Mosaics: Putting the Pieces Together

Through curious acts of living cells, some people have different number of chromosomes in different cells inside their bodies, like different pieces gathered from different stones. When those cells with differing numbers of chromosomes come from one zygote (one fertilized egg), this is called “mosaicism,” and the affected people are living mosaics in a very real biological sense. When the mosaicism involves the sex chromosomes, we call these people sex-chromosome mosaics. (65)

Like light through a prism, as the X chromosome splinters, so do our old ideas about what makes a man or woman. (66)

Chimeras: When There Are Two of Me

Mosaics develop from a single zygote. Chimeras, on the other hand, arise from two different zygotes. (67)

For example, some eggs with two nuclei survive through to ovulation, and it is sometimes possible for two different sperm to fertilize these binucleate eggs. Occasionally, instead of splitting into two separate individuals, this sort of zygote develops into one person with two different types of cells–46,XX and 46,XY, for example–male and female all at once. On other occasions two separate zygotes may fuse and develop into a single fetus, again with two completely different types of cells–one from zygote A and one from zygote B, each with its own karyotype. (67)

Just how many of us are chimeric? No one knows. Often, sex chromosome chimeras show no symptoms at all. Everything depends on whether the chimerism extends to the gonads. (67)

There is something about sex and sex difference in children that lights a candle in a very dark room inside of many of us, a room we prefer to keep darkened. So when that flame flares, the easiest thing to do for some parents is to simply shut the door and pretend they never saw the things that glistened in that candlelight. (70)

Bits of Y Chromosomes: Lost and Found

Like with X chromosomes, Y chromosomes may also lose parts of themselves, duplicate parts of themselves, and pick up stray pieces of other chromosomes that they find lying about in the nucleus. (750

Occasionally, during development of sperm, a Y chromosome will drop a piece of itself on the cutting-room floor. If that piece happens to include SRY, then this particular Y chromosome has lost its knack for making baby boys. When that sperm hooks up with a normal ovum, the resulting zygote, embryo, fetus, and child will have the 46,XY karyotype, but, in every other way that matters, it will be a girl, or almost. This change comes in two forms called complete and partial gonadal dysgenesis (dysgenesis means abnormal development). (75)

True Hermaphrodites

By definition, a true human hermaph-(76)rodite must have both ovarian and testicular tissues either in the same or different gonads. (77)

Pseudohermaphrodites: Beyond X and Y

Female pseudohermaphrodite have a 46,XX karyotype and have ovaries, but their external genitalia include fully or partly formed penises and scrotums. Male pseudohermaphrodite are (87) 46,XY, but their external genitalia are partly or fully female. (88)

Normal Chromosomes Wearing Abnormal Genes

Like a typewriter with a bad key, sometimes a chromosome taps out a story peppered with mistakes. Every time the writer reaches for an “o,” the typewriter slaps down an “e.” “Looking forward” becomes “leaking forward.” The sense of it blurs and the story takes an abrupt swing. (98)

| When a chromosome does that, people change in unpredictable ways. (98)

Congenital Adrenal Hyperplasia

Within the urogenital ridge–what will become the external and internal genitalia of the fetus–the gonads and related sexual tissues develop close to the kidneys and the adrenal glands. The adrenal glands, like the gonads, produce hormones critical to determining the sex of the developing child. These hormones include cortisol and the adrenal androgens, especially androstenedione, a precursor to testosterone. When the adrenal glands overdevelop–a condition called congenital adrenal hyperplasia–they can produce enormous quantities of cortisol and adrenal androgens. This can cause otherwise normal 46,XX fetuses to develop clitorises that to varying degrees more closely resemble penises and scrotums–female pseudohermaphrodite. (99)

Intersex as a Way of Life Among Other Animals

In effect, half of all spotted hyenas are intersex–40,XX females (100) (forty being the normal number of chromosomes among hyenas) whose genitals look like those of males. (101)

Androgen Insensitivity Syndrome

Children Who Change Their Sexes: 5-Alpha Reductase Deficiencies

Normally, the final strokes in the creation of a baby boy require two hormones, testosterone and a derivative of testosterone called dihydrotestosterone. The developing testes produce testosterone, but an enzyme derived from another source is necessary for the conversion of testosterone to dihydrotestosterone. That enzyme is 5-alpha reductase type 2, normally produced from the SRD5A2 gene at relatively high levels during the sexual development of boys. (109)

| But nearly thirty known mutations in the SRD5A2 gene result in inadequate production of functional 5-alpha reductase type 2. When that happens, 46,XY fetuses develop normal testes, but the testes don’t descend, and their genitals look like those of baby girls. As a result, at birth they are almost always identified as baby girls. And they nearly are, except they have testes, and what seems to be a vagina is only a small closed pouch that leads nowhere. (109)

Fishy Sex: Changing Times

…some wrasse (Labroides dimidiatus) are sequential hermaphrodites–at times female, at other times male. … From veiled damsel to bearded sheik, from a maker of eggs and a receptive mate to a sprayer of sperm and the master of all in a day or two–this is sex as a choice, sex as a consequence, sex as social order. (112)

And then there are species like the saddleback wrasse, midshipman fish, and some species of salmon that have two very different types of males. (113)

No matter how hard we may try to squeeze these fish tales into our human stories, sex remains–as pioneering geneticist J. B. S. Haldance said of all the universe–“not only queerer than we suppose, but queerer than we can suppose.” (114)

6. What We Do About the Ambiguous Child

When it comes to human intersex, many people find themselves in a curious and frightening void. The only way out of that void is through the child. (115)

Limiting the Ambiguity: Assigning a Gender

cf. 2007 “Consensus Statement on Management of Intersex Disorders.” (cf. 2018, “Impact of the consensus statement and the new DSD classification system.”)

First, this group recommended that we change the words we use to describe intersex people. “Terminology such as ‘pseudohermaphroditism’ is controversial, potentially pejorative to patients, and inherently confusing.” They proposed in its place the term “disorders of sex development” or DSDs ” to indicate congenital conditions with atypical development of chromosomal, gonadal, or anatomic sex.” That seems a reasonable conclusion. After all, very few of us, even without years of medical training, feel that the term hermaphrodite adds to our social currency. And this conclusion acknowledges, up front, the crucial role played by language in this whole area. The language we use to describe ourselves and the words that others use to name us change the way we see everything. Words are not innocent bystanders here. (116)

7. Outcomes: Sex, Surgery, and Satisfaction

But does it work? Do these surgeries make for happier people? As previously mentioned, it depends upon whom you ask. Doctors, parents, and patients are all likely to provide different answers to those questions. The aftermath of surgery is often complex, and long-term studies of patient satisfaction are few and frequently contradictory. (127)

When Surgery Is Essential, Does Sex Matter?

Cloacal exstrophy is not truly an intersex condition, but it is one in which sex reassignment through surgery is typically required, not optional. Cloacal exstrophy is the term physicians use to describe a rare type of abnormal fetal development in which the whole bottom portion of the abdominal wall fails to form properly. In these people, where their legs join, there is a hole. Through that hole, portions of the intestines and bladder often protrude outside of the body, and the genitalia, and sometimes the anus as well, do not form at all. (131)

When Surgery Is Optional, Does Sex Matter?

…complete androgen insensitivity syndrome and the women it creates prove one thing–there is more to sex determination than chromosomes. (136)

Sensation after Surgery: The Downside of Clitoral Modification

Concern About Clitoroplasty

Concern About Studies of Sex Satisfaction

In nearly every one of the published studies, the investigators concluded that, because the patients had no significant complaints about their surgeries and where functioning as contributing adults, the physicians’ treatment and management decisions had been the right ones. But Kuhnle and Krahl object to this assumption: “We now believe that this is an unwarranted conclusion, and that the only inference to be drawn from these studies is that the majority of these patients are well adjusted and can somehow live with a handicap. These studies do not answer the question of whether there are other and/or better options for a patients’ life.” (142)

| In other words, perhaps these people are more or less content with their lot in life because they, like many other human beings, are remarkably resilient and have learned to live well with a major problem. Nothing about the way in which any of these studies have been conducted even begins to approach the question of whether these people would be happier if their physicians and patents had chosen differently, or done nothing at all. (142)

8. Alternatives: Other Cultures, Other Sexes

Do people raised with different worldviews see the sexes differently? The answer is an emphatic yes. (144)

The Hijras of India

Men Not-Men Among the Natives of North America

Thinking Inside the Box

What About Their Parents?

No matter who we become, much of what set us upon our paths was out of our hands. Long before we even realized that choices had to be made, our parents were making those choices for us. (150)

How Our Past Becomes Our Future

Only a very few cultures are built on mythologies wise enough and strong enough to comfortably accept intersex children. most of us living in the Americas and Europe have no such traditions. Or maybe I should say that most of us have only a single tradition, a myth handed down to us from Realdo Columbus, who imagined he had discovered the clitoris, and from the other great minds of the Enlightenment. That myth tells us that among humans there are two–and only two–polar opposite sexes. Faced with a child who lives completely beyond what most of us ever imagined possible, that simple myth is a poor guide. (154)

9. Choices: Exploring the Options

In light of the poverty of our single myth, the nature of our world, the skills of our surgeons, and the needs of our children, what can we do? (155)

The thing that people with intersex suffer from most is shame, it’s not surgery. …what people who are anatomically different need the most is the message that they’re human and acceptable and lovable. – Dr. Alice Dreger

By age three, most children relate strongly with one sex and have a finite sense of being a boy or a girl. Interestingly, the genitalia of children and a adults don’t figure preeminently into a child’s decisions about someone’s sex. (157)

And it is at about age five that children come to believe that sex is unchanging with time. (158)

Are Our Attitudes and Our Medicine Changing?

Yes and no. (159)

Epilogue: Untying the Knot

When we divide the world into two groups, male and female, we tend to see all males as beings similar and all females as being similar, and the two categories of “male” and “female” as being very different from each other. In real live, the characteristics of women and men tend to overlap. Unfortunately, however, gender polarization often creates an artificial gap between women and men. – from a publication off the National Honor Society in Psychology

In truth, all of us fall somewhere in between our masculine and feminine ideals. Graphically the human race might look something like this:

Woman ● ● ● ● ● ● ● ● ● ● ● ● ● ● ● ● ● ● ● ● ● ● Man

For some reason, we choose to call only the people who fall near the dead center of this chart intersex. But the center is just as essential as any other part of the continuum–without the middle, neither end is possible. And the middle really has no obvious boundaries. (162)

| In truth, we are all intersex, living somewhere in the infinite, but punctuated, stretch between MAN and WOMAN. (162)

Intersex people are not a few freakish, unfortunate outliers. They are instead the most complete demonstration of our humanity. (163)

What intersex people have shown us is the truth about all of us. There are infinite chemical and cellular pathways to becoming human. Because of that, no two of us are now, or ever were, identical. (163)

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  1. Pingback: Living In A Gray World | Reflections, and Notes & Critique | vialogue

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