The Biology of Gender, and When (if Ever) Children Should Be Given Sex Reassignment Surgery

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Analysis Human Rights

The Biology of Gender, and When (if Ever) Children Should Be Given Sex Reassignment Surgery

Martha Kempner

The first words uttered after a child is born are often “It's a boy!” or “It’s a girl!” But sometimes doctors don’t know exactly what to say. How does this happen, and what should parents and doctors do?

Last week, parents in South Carolina filed a lawsuit against the state on behalf of their 8-year-old adoptive son, known as M.C., who was born with both male and female genitalia and was given sex reassignment surgery as a toddler when he was a ward of the state.

In a recent Rewire article, Jessica Mason Pieklo explored the legal issues in the case. Here, I want to look at the myriad other gender-related issues a case like this raises. There are biological issues (How do some babies come to be born with male and female genitalia?), sociological issues (Is our gender born or made, and is it possible to raise a child, even a very young one, in a gender-neutral way?), and psychological issues (What’s in the best interest of a child in this situation?).

Biological Sex

We don’t know the precise medical condition M.C. has, but there are a number of ways he could have been born with genitalia and reproductive organs that at least appear to be both male and female. At one point, M.C. would have been called a hermaphrodite, though that would likely have been inaccurate as the term technically refers to someone who has both ovaries and testes. Within the past few decades the word intersex was introduced as a way to describe individuals with these conditions. More recently, the term “disorders of sexual development” has been used, but some advocates feel this language only further marginalizes the identities of these individuals.

Whatever language we land on, it all starts with sperm and ova (eggs), which 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.

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All fetuses start out with two undifferentiated gonads and certain homologous structures (structures that start out the same) that can become either male or female sex organs. It is up to the chromosomes to start the path toward male or female. Do nothing and the fetus will develop ovaries that begin to make estrogen. The homologous structures will then become a uterus, fallopian tubes, and a vagina internally. Externally they will develop to form the labia minora, labia majora, clitoris, and the vaginal opening.

Add something called TDF (testes determining factor), which is found on the Y chromosome, and the gonads will become testes and start making testosterone. The homologous structures in the fetus will then turn into the internal reproductive system (such as the Cowper’s gland, prostate gland, and seminal vesicles) and the external male genitalia (the penis and scrotum).

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.

Chromosomal Anomalies

Though each gamete is supposed to have only one sex chromosome, problems can occur 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. For example, 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. Klinefelter’s 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. Other combinations, like XYY or XXX, can cause issues after puberty such as infertility and irregular periods but exhibit no outward symptoms in childhood.

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. When the hormones are not produced or the body can’t process them, the fetus develops along a different path.

For example, 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 testes will develop and 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 external genitalia that appear to be 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. The baby will also have undescended testes, which will most often be located somewhere in the abdomen.

Another hormonal issue is called Congenital Adrenal Hyperplasia (CAH). It 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 (XY), it can cause sex characteristics to appear too early. 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.

DHT deficiency is another common anomaly. Genetic males (XY) with DHT deficiency do not produce enough of a hormone called dihydrotestosterone (DHT) while in utero. Some babies with this deficiency are born with external genitalia that look female, while others are born with external genitalia that appear male but are unusually small (sometime 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.

A Rush to Judgement

The first words uttered after delivery are often “It’s a boy!” or “It’s a girl!” But what happens when these conditions make it so doctors don’t know exactly what to say? Such a diagnosis can be difficult for parents, and the path forward unclear.

As with many things in our society, there has historically been a rush to fix that which is not “normal.” In many cases this has meant that parents are told their infant needs to have surgery immediately 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 appears to be what happened to M.C. It is also what happened to Cheryl Chase, who later became an advocate for putting off genital surgery until a child is old enough to make their own decisions. Cheryl was born in 1956 with ambiguous genitalia—she had what could have been an enlarged clitoris or a micropenis and something that appeared to be a vaginal opening. At first doctors recommended that she be brought up as 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, though 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.”

Interestingly, the most famous test case of gender reassignment in children did not involve someone with a disorder of sexual development. Instead, the case involved identical twin boys who were born in 1965 with identifiably male sex organs. At eight months old they underwent circumcision because they were suffering from phismosis (a condition in which the foreskin will not pull back). There was a serious accident and one twin essentially lost his penis. At the time, doctors said they were unable to surgically give the child anything that looked or functioned like a real penis.

The parents turned to Dr. John Money, a pioneer in the field of gender and sex reassignment surgery. Money had a theory that gender is purely a cultural concept that comes from how kids are 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 woman. 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 the exact same 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, did the surgery, and proceeded to raise David as Brenda. Neither she nor her brother was told the truth about the situation.

For years, Money published papers about how well his gender experiment was going, and his research on the “John/Joan case” became the basis for his book, Man & Woman, Boy & Girl. It also made national news, including a full-page story in Time magazine. But the family tells a different story. The twins’ mother claims that the first time she put a dress on Brenda, the child tried desperately to pull it off. Brenda’s brother tells it this way, as quoted in an article for Healthy Place: “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.” And as a teen she refused to go ahead with the surgery that would have created a full vagina.

As soon as Brenda learned the truth, at the urging of a psychologist who saw her as near suicidal, she began once again to live as David. When the real result of the “John/Joan” case became public in the mid-1990s, David was a 31-year-old man and married to a woman. At the time, he said he was happy living as a man but acknowledged that getting there was not easy and that he had contemplated suicide a number of times. Sadly, this happiness did not remain; in 2004 David Reimer took his own life.

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. It is important to note that some conditions do require early surgery for functional reasons, such as separating the vagina from the urethra, or safety reasons, such as removing testes located within the abdomen as they can become cancerous. But in most cases the surgeries that these children undergo—like the one that M.C. had—are about appearance.

A recent consensus document written by the Lawson Wilkins Pediatric Endocrine Society in the United States and the European Society for Pediatric Endocrinology advises parents and physicians to take it slow and not treat the birth as a medical emergency that requires immediate intervention.

Instead, the guidelines suggest that every infant be assigned a gender shortly after birth based on the diagnosis, genital appearance, surgical options, needed for lifelong replacement therapy (such as estrogen shots), potential for fertility, and the views of the family and culture. In our pink and blue society, it is not possible, nor is it advisable, to attempt to raise a child without gender. The idea, however, is that this without surgery this gender assignment can change if it turns out to be inconsistent with how the child feels as he or she grows up. This allows the child (most likely as a teen or an adult) to make the ultimate decisions about gender and genital appearance.

While this approach clearly makes the most sense, the waiting game must be very difficult for parents who will likely have many concerns about whether their child will accept him/herself and how he/she will be treated by others if they look different (think potty training in preschool or high school locker rooms). In M.C.’s case, the guardians may also have worried that the child would not be adoptable without a clear gender and “normal” genitalia.

M.C. is very lucky that he found parents who are strong advocates for his right to be who he wants to be. Hopefully their efforts can help young people and parents who are facing these issues make these difficult decisions as painlessly as possible.