Everyone Is Born Either Male or Female | adult skills

Everyone Is Born Either Male or Female, intersex. Sometimes it seems like every television show and movie we watch has to have a birth scene in it. Whether it’s an hour-long hospital drama (remember Grey’s Anatomy’s Bailey refusing to push because her husband was having brain surgery down the hall), a network sitcom (poor How I Met Your Mother’s Lily went into labor while her husband was falling down drunk in Atlantic City), a Disney channel half-hour comedy made for tweens (the mom on Good Luck Charlie has now been seen giving birth twice), or the climax of a blockbuster movie (who can forget the famous crotch/crowning shot in Knocked Up), women are constantly giving birth on the big and little screen.

Everyone Is Born Either Male or Female | adult skills
Everyone Is Born Either Male or Female | adult skills

The scene in the hospital room (or the car or the elevator — because fictional babies always seem to come super-fast and at the worst moments) plays out differently depending on whether the goal is to mimic real life drama or go for the cheap laughs, but it always ends the same way with someone happily proclaiming: “It’s a Boy!!!” (Okay, sometimes it ends with “It’s a Girl!” But you get our point).

In Hollywood, this big moment is simple; the doctor peeks between the baby’s legs and makes the all-important proclamation and the parents are always surprised (even though many of today’s parents know the sex months before delivery through prenatal tests) and delighted (that is after the dad who dreamt of a son melts the first time he holds his daughter).

For some parents in real life, however, it not possible for the doctor to proclaim boy or girl just by looking at their infant. Ultimately, our gender is based on biological sex which is determined by our chromosomes, hormones, and internal and external genitalia. As we grow, we learn how males and females are expected to behave (known as gender roles), decide how we want to present our gender to the world (gender expression), and develop our own internal sense of whether we are male or female (gender identity). Though much of society — from the media, to the fashion industry, to every modern toy store — would like us to believe that there are only two choices, many think that gender rests on a continuum with male and female at either end and many (if not most) people residing somewhere in between.

Everyone Is Born Either Male or Female | adult skills

In this entry, we are going to focus on variations of biological sex and what happens when someone’s biological sex is not firmly male or female. Later in the book (Myth #9) we cover gender roles and gender identity in more detail and look at what happens when a person is transgender, which means that their internal sense of gender identity does not match their biological sex or the gender roles society expects of them. Though these two issues are inextricably linked for some people, we believe it is important to look at them separately.

Variations in Biological Sex
As we often do when addressing a long-standing myth in human sexuality, we have to start this entry with a review of basic biology. We apologize if we are repeating things you learned in ninth grade but we are pretty sure that you didn’t hear it all back in high school.

Sperm and ova (eggs) are known as gametes. They each carry 23 individual chromosomes that, when they come together, become the 23 pairs of chromosomes that make up our unique genetic identity. One of those pairs of chromosomes determines our biological sex.

Ova always (or almost always — more on that later) carry one X chromosome while sperm typically carry either an X or a Y chromosome. When they come together and everything goes according to plan, an embryo with XX chromosomes develops into a female while one with XY chromosomes develops into a male.

All fetuses start out with two undifferentiated gonads and certain homologous structures that can become either male or female sex organs. There is a specific section of the Y chromosome (called the TDF or testes-determining factor region) that sends a signal to the gonads telling them to develop into testes.

The testes in turn begin to produce testosterone which sets the rest of the fetal development on a path toward becoming male. This means that homologous structures in the fetus (structures that start out the same) will turn into the internal reproductive system (such as the Cowper’s gland, prostate gland, seminal vesicles) and the external male genitalia (the penis and scrotum).

Everyone Is Born Either Male or Female | adult skills

Without TDF, the gonads become ovaries and produce estrogen instead of testosterone. The fetus then develops along a female path meaning that these homologous structures will become a uterus, fallopian tubes, and a vagina internally, and externally they will develop to form the labia minora, labia majora, clitoris, and the vaginal opening. (Since we are reviewing here, let us remind you that these are not all of the internal and external parts in either male or female reproductive systems.)

Though the majority of babies born will have followed one of these two paths, there are many points along the way where things can diverge from these paths.

Chromosomal Anomalies
Like life itself, these anomalies can start with the egg or the sperm. Though each gamete is supposed to have only one sex chromosome, things can happen during meiosis (the process of cell separation) that cause them to have more than one or none. This means that an embryo can begin to develop with too many or too few sex chromosomes. These are some of the ore common conditions that can result.

Turner syndrome, XO, develops when either the egg cell or the sperm cell does not have a sex chromosome at all. Individuals who are born with Turner syndrome have external female genitalia and internal female reproductive organs (ovaries, uterus, and fallopian tubes) but the ovaries are not functional and do not produce estrogen during development. Females with Turner syndrome are usually short, have a broad chest with the nipples way out to the sides, and have low ears. They may have congenital heart defects as well.

They will not develop secondary sex characteristics on their own. They are often given human growth hormone during childhood to help them grow taller and estrogen therapy after puberty to increase their development of secondary sex characteristics (Mader, 1992).

Everyone Is Born Either Male or Female | adult skills

Klinefelter syndrome, XXY, happens when an egg with two X chromosomes is fertilized by a sperm with a Y chromosome or an egg with an X chromosome is fertilized by a sperm with both an X and a Y. These individuals will appear male when born but some abnormalities including small testes, a female pattern of pubic hair, poor muscle development, and a lack of facial hair may begin to be noticed as the individual reaches puberty (Mader). Males with Klinefelter syndrome tend to have long limbs, broad hips, and some learning disabilities (especially in verbal skills).

These men are also infertile. In fact, many men with the condition find out they have it as adults because they are having trouble conceiving. Those who find out at birth or during puberty can be treated with testosterone to help bring on secondary sex characteristics (Kelly, 2010).

Supernumerary Y syndrome, XYY, happens when an egg with one X chromosome is fertilized by a sperm with two Y chromosomes. These males are usually taller than average but have no other outward symptoms. Most males with XYY syndrome have normal sexual development and are fertile.

However, males with XYY may be at higher risk for learning disabilities, including delayed development of speech and language skills. They may also face delayed development of motor skills, weak muscle tones, hand tremors, and other motor tics. The syndrome has also been associated with behavioral and emotional difficulties. It was once suggested that these males had higher aggression and were more likely to be in prison than other males but that theory has since been disproven.

Triple X syndrome, also known as meta-females, XXX, develops when an egg cell that has two X chromosomes is fertilized by a sperm cell with an X chromosome of its own. Individuals with three X chromosomes may never know it as they most often have no physical abnormalities. However, they might have menstrual irregularities, suffer from fertility issues, and go through menopause at an early age (Mader).

There are other variations as well. A fetus could have XO/XY which would mean it will develop either male or female genitals or some combination but likely have no other issues. The fetus could also have both XX/XY which would mean that it could develop some combination of ovaries and testes and would likely have a uterus. The external genitals could be male or female or some combination. At puberty, individuals with XX/XY usually grow breasts and begin to menstruate (Kelly).

It is important to note that there is no syndrome from embryos with YO sex chromosomes because at least one X is required for survival. Any embryo that has just a Y chromosome will result in a spontaneous abortion or miscarriage.

Hormonal Anomalies
While chromosomes set a fetus on the path toward its biological sex, how the fetus develops is also very much controlled by the hormones it is exposed to while in utero (and after birth) and how the body processes these hormones. Again, there are many things that can go differently during development but these are some of the most common.

Androgen insensitivity syndrome (AIS) occurs when the body cannot process male sex hormones (known as androgens). When this happens in someone who is genetically male (XY), the process of developing a male reproductive system and male genitalia is interrupted. The TDF region will tell the undifferentiated gonads to become testes and the testes will produce testosterone but, because the body cannot process this hormone, the fetus will develop along a female path and will appear female when born with their external genitalia appearing as labia. The internal reproductive organs, however, will not have developed completely. The baby will be born with a shortened vagina and will not have a uterus (Blonna and Levitan, 2005).

Everyone Is Born Either Male or Female | adult skills

The baby will also have testes which will most often be located somewhere in the abdomen. After puberty, individuals with AIS are often given estrogen replacement therapy to help develop female secondary sex characteristics. They may also have to have their testes removed as these can become cancerous (Kelly). Though people with AIS are genetically male, most identify as female.

Congenital adrenal hyperplasia (CAH) is a disorder of the adrenal glands (there is one adrenal gland located on each of our kidneys) which causes a buildup of androgens in a fetus and infant. If this happens in someone who is genetically male it can cause sex characteristics to appear too early. Males with CAH may have a deepening voice, an enlarged penis (though normal testes), pubic hair, armpit hair, and well-developed muscles before puberty.

Genetic females (XX) with some forms of CAH will usually have normal internal reproductive systems (ovaries, uterus, and fallopian tubes) but may have an enlarged clitoris at birth. In some instances the clitoris may be so large that it is mistaken for a penis. They may also develop pubic hair and armpit hair before puberty and may develop facial hair as well. There is also evidence that females with CAH tend to develop a male gender identity

DHT deficiency is a problem that affects genetic males (XY). In utero these males do not produce enough of a hormone called dihydrotestoster one (DHT) which has a critical role in male sexual development, including the development of the external genitalia. Many people with this deficiency are born with external genitalia that look female, others are born with external genitalia that appear male but are unusually small (sometimes called a micropenis).

Still others will be born with what is called ambiguous genitalia where it is hard to tell whether they are male or female just by looking. During puberty, people with this condition often develop male secondary sex characteristics such as increased muscles, deeper voices, and pubic hair. Their penis and scrotum often grows larger as well. Despite the fact that many have been raised as females, they do not develop breasts or other secondary sex characteristics (Kelly).

Everyone Is Born Either Male or Female | adult skills
Everyone Is Born Either Male or Female | adult skills
Everyone Is Born Either Male or Female | adult skills

Evolving Language
In the old days, anyone born with ambiguous genitals of any kind would have been called a hermaphrodite. The medical definition of a true hermaphrodite, however, means someone who has both ovaries and testes. Most people with the conditions mentioned above do not fit into that category. Some medical professionals make the distinction of pseudohermaphrodites noting that someone with testes (but no ovaries) and some female genitalia would be a male pseudohermaphrodite and someone with ovaries (but no testes) and some male genitalia would be a female pseudohermaphrodite.

Everyone Is Born Either Male or Female | adult skills

In 1993, Anne Fausto-Sterling, a biologist who frequently writes about gender, wrote an article for The Sciences magazine in which she suggested we should recognize five biological sexes. In addition to males and females, she suggested adding “herms” (named after true hermaphrodites), “merms” (named after male pseudohermaphrodites), and “ferms” (named after female pseudohermaphrodites).

In a follow-up essay, she admits that her “tongue was planted firmly in cheek” when she suggested these names but that her point was clear; the two-sex system that is so deeply embedded in our culture is not sufficient to “encompass the full spectrum of human sexuality” (Fausto-Sterling, 2000).

Though the concept of five sexes might seem crazy to some of us, other cultures have been more open to recognizing those who do not fit into the male and female categories. There is a village in the Dominican Republic, for example, where a genetic mutation has meant that many babies are born with DHT deficiency. These babies have genitals that look female during childhood but once exposed to testosterone during puberty they develop male secondary sex characteristics. Villagers have given these men the name guevedoche, which means “balls at twelve.” They are also sometimes referred to as machi-embra (male-female) (Herdt, 1990). The important part is that over the generations they have been accepted at least to some degree as a third sex.

The Sambia tribe of Papua New Guinea also has a similar genetic mutation and has seen many children born with genitals that appear to be female at birth but eventually develop male secondary sex characteristics. These individuals are assigned the name kwolu-aatnwik and are not expected to take on either male or female gender roles in the society. They are allowed to become spirit doctors or shamans (Kelly).

In the Western world there is no real agreement even now as to what we should call an individual whose biological sex is not easily categorized. The same year that Fausto-Sterling suggested five sexes, activists founded the Intersex Society of North America (ISNA).

According to its website: “The Intersex Society of North America (ISNA) is devoted to systemic change to end shame, secrecy, and unwanted genital surgeries for people born with an anatomy that someone decided is not standard for male or female.” As its name suggested, the ISNA proposed using the term intersex as “general term used for a variety of conditions in which a person is born with a reproductive or sexual anatomy that doesn’t seem to fit the typical definitions of female or male.” While this terminology became popular for a number of years some still found it to be “pejorative to patients” and “confusing to practitioners and parents alike” (Lee et al., 2006).

Everyone Is Born Either Male or Female | adult skills

New guidelines put out by experts in the field and supported by the ISNA (which has now closed its doors) propose using the term “disorders of sex development” (DSD) and defining this as “congenital conditions in which the development of chromosomal, gonadal, or anatomic sex is atypical”.

Not everyone is happy with this language either, primarily because it begins with the term disorders. Some activists and people living with DSD believe that this further marginalizes their identities.

Evolving Treatment
If we don’t have the language to even talk about those born with disorders of sex development, imagine how hard it must be for parents of these infants to figure out what to do. In the United States there has historically been a rush to fix that which is not “normal.” In many cases this has meant that parents are told that their infant needs to have surgery to make their genitals appear more like an average clitoris or penis. In truth though, it has always been much easier to create labia and a vagina than it has been to create a penis (especially one that functions).

Most of the time, therefore, doctors would recommend that parents surgically create female genitalia and begin to raise the infant as a girl regardless of the chromosomal sex or what hormones the infant was exposed to in utero. This is what happened to Cheryl Chase, the founder of ISNA. She was born in 1956 with ambiguous genitalia — she had what could have been an enlarged clitoris or a micro penis and something that appeared to be a vaginal opening.

At first doctors recommended that she be brought up as a boy so she went home from the hospital with the name Charlie. But her parents were concerned about the appearance of her genitals and consulted another team of experts when she was 18 months old. Based on the fact that she had a fairly normal vagina, these experts recommended surgery to make her external genitals look more female.

She underwent a clitoridectomy and was sent home as Cheryl. Her parents never told her what had happened and she remembers many unexplained surgeries and genital exams during her childhood. She also remembers not fitting in with the other girls: “I was more interested in guns and radios and if I tried to socialize with any kids, it was generally boys, and I would try to best my brother” (Colapinto, 1997).

Everyone Is Born Either Male or Female | adult skills

Fausto-Sterling notes the case of Max Beck who was born with disorders of sex development and was surgically assigned female. Though comfortable in that identity through her teens, Max first came out as butch lesbian in her twenties and then in his mid-thirties became a man. He is now married to a female partner and they have had children (through reproductive technologies).

Interestingly, the most famous test case of gender reassignment in children did not involve someone who had a disorder of sex development at all. Instead, the case involved identical twin boys who were born in 1965 with identifiably male sex organs. At 8 months old they underwent circumcision because they were suffering phismosis (a condition in which the foreskin will not pull back).

There was a serious accident during the procedure and one twin essentially lost his penis. His parents had few choices and little hope as the doctors they saw all said they could not rebuild a penis that would either look normal or function properly.

The parents then found out about a doctor at Baltimore’s Johns Hopkins Hospital who was researching and performing sex reassignment surgeries. Dr. John Money was a pioneer in the field of gender and had a theory that gender was a purely cultural concept that came from how kids were raised especially early in their lives. He believed that infants are born as blank slates and it is not until their parents and society imprint them with gender that they begin to see themselves as either male or female.

Money met with the parents of the infant (then named David) and assured them that if they allowed surgeons to construct external female genitalia and then raised the child as a girl, “she” would be capable of growing into a well-adjusted young women. Money was particularly interested in this case because as an identical twin David came with a control group. If “she” could be successfully raised as a female while her brother (who had her exact genetic make-up) was successfully raised as a male, it would go a long way toward proving Money’s blank slate theory.

The parents took Money’s advice, the surgery was carried out, and they proceeded to raise David as Brenda. Money followed the case, which he always referred to as “John/Joan,” and repeatedly published articles about its success in which he described “Joan” as a happy, well-adjusted girl who had easily adopted more female roles and characteristics.

In 1973, Time magazine pointed to Joan saying, “This dramatic case provides strong evidence for a major contention of women’s liberationists: that conventional patterns of masculine and feminine behavior can be altered. It casts doubt on the theory that major sexual difference, psychological as well as anatomical, are immutably set by genes at conception” (Colapinto, 1997).

Everyone Is Born Either Male or Female | adult skills

It is unclear if Money failed to notice the truth or deliberately ignored it but Brenda’s family tells a very different story. Her brother tells it this way: “I recognized Brenda as my sister but she never, ever acted the part. She’d get a skipping rope for a gift, and the only thing we used it for was to tie people up, whip people with it.” He went on to say,

“When I say there was nothing feminine about Brenda, I mean there was nothing feminine. She walked like a guy. She talked about guy things, didn’t give a crap about cleaning house, getting married, wearing makeup” (Colapinto, 1997). Brenda’s mother claims that the very first time she put a dress on her, the little girl tried desperately to pull it off.

Everyone describes a miserable childhood of feeling different and not fitting in anywhere. Brenda realized early on that she was not a girl in many ways starting perhaps with her preference for peeing standing up. When Brenda imagined her future, she pictured herself living as a man married to a woman. Though she reluctantly began taking hormones as a young teenager and as a result grew breasts, Brenda refused to see Dr. Money any more and began to be treated by doctors and psychologists closer to home.

One day when she was 14, she met with a doctor who was trying to convince her to have the surgery that would be needed to finish creating a vagina but Brenda kept refusing. The doctor asked in exasperation “Do you want to be a girl or not?” Brenda answered, loudly, “No.” The doctor then told her parents that he thought it was time to tell Brenda the truth.

David remembers hearing the story from his father that afternoon and feeling a number of conflicting emotions, the overwhelming one, however, was relief. He instantly changed his name back to David and began the process of going back to living as a boy. He had his breasts surgically removed and then had a rudimentary penis constructed right before he turned 16.

Another operation in his twenties created a better looking and more functional penis. When the real result of John/Joan’s case became public in the mid-1990s, David was 31, married to a woman, and raising the children she had from previous relationships as his own. He said he was happy living as a man but acknowledged that getting there was not easy and that he had even contemplated suicide a number of times. Unfortunately, the happiness did not last; in 2004 David Reimer took his own life (Colapinto, 2004).

Today’s Thinking
Based on cases like Cheryl Chase and John/Joan, the prevailing wisdom today suggests that rushing into surgery — which has permanent repercussions — for purely cosmetic reasons is a bad idea.

Some conditions do require early surgery for functional reason (such as separating the vagina from the urethra) or safety (such as removing testes located within the abdomen as they can become cancerous. ISNA helped to convene an International Consensus Conference on Intersex. One of the major outcomes of the conference was a consensus document written by the Lawson Wilkins Pediatric Endocrine Society in the United States and the European Society for Pediatric Endocrinology.

The overall advice is to take it slow and not treat the situation as a medical emergency that requires immediate intervention. The consensus statement suggests that health care providers should avoid making any gender assignment before an expert evaluation is conducted and that such an evaluation should be made at a center with an experienced multidisciplinary team (ideally that team would include pediatric endocrinologists, surgeons, urologists, psychologists, gynecologists, geneticists, and neonatologists who have experience with DSD).

Everyone Is Born Either Male or Female | adult skills

The team will make a diagnosis based on anatomy, genetics, imaging (such as ultrasound or MRIs), and measurement of various hormone levels. In a culture that instantly categorizes everyone based solely on appearance and doesn’t even have the proper pronouns to talk about someone without referring to gender, the task of raising a child without an identified gender is practically impossible and unadvisable as it can be troubling to the family and the child. Instead, the guidelines suggest that every infant be assigned a gender. This assignment should be made based on the diagnosis, genital appearance, surgical options, need for lifelong replacement therapy (such as estrogen shots), potential for fertility, and the views of the family and culture.

However, the idea is that this gender assignment can change if it turns out to be inconsistent with how the baby feels as he or she grows up. The consensus statement explains that atypical gender behavior is common in those born with DSD and should not be seen as an indicator that the gender was wrongly assigned or should be changed.

That said, if affected children or adolescents report “significant gender dysphoria” (a sense that their gender identity does not match the gender they’ve been assigned), they should be given a comprehensive psychological evaluation and an opportunity to explore gender issues with a qualified professional over time. “If the desire to change genders persists, the patient’s wish should be supported and may require the input of a specialist skilled in the management of gender change” (Lee).

Everyone Is Born Either Male or Female | adult skills
Everyone Is Born Either Male or Female | adult skills
Everyone Is Born Either Male or Female | adult skills

These new guidelines recognize that the process of developing a gender identity (even for people born with a clear biological sex) is complicated and ever evolving. The approach that they are recommending therefore is one of caution which leaves all irrevocable decisions to be made by the individual when he/she has developed an internal sense of who they are.

These guidelines represent a major step forward in how we think about biologic sex and how we handle differences. Nonetheless, we are still a society that tries to categorize people as male or female and masculine or feminine.

While we certainly tolerate more variations in gender roles and expressions than we once did, we still hold onto many stereotypes about how an individual should look and behave. As we see later in the book when we explore gender roles, gender identity, and transgender issues more closely, we still have a lot of work to do in dispelling myths about what makes someone a man or a woman. This much is clear — it is more than just what is between someone’s legs.

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