Kids with Intersex Conditions in the Age of Gender Identity, Part I

Making room for gender *and* sex diversity

Creative Commons/Wikipedia

For the first 24 years of her life, Jo didn’t understand why she was different from the other girls and women. She’d been having occasional surgeries since the day she was born, first to fix hernias and later, during puberty, to remove pre-cancerous ovaries—or so she was told by her parents and doctors. They explained to her that her womb hadn’t developed properly. Even though she’d developed breasts normally, she’d never get her period. She also had no body hair or odor. No acne. What had made her so different?

Jo had been born with hernias, and when doctors operated to repair them, they found they’d been caused by internal testes that had never descended. Jo has CAIS, or complex androgen insensitivity syndrome, a disorder or difference of sex development (DSD), sometimes known as an intersex condition, in which a male XY body can’t react to testosterone and converts it into estrogens. A baby with CAIS may appear female externally but will have typically male chromosomes; this is one of the rare medical conditions in which terminology like “sex assigned at birth” actually makes sense, when one’s phenotype doesn’t match one’s genotype.

Doctors removed Jo’s internal testes because they were thought, at the time, to be a significant cancer risk (these days doctors are likely to keep them in and monitor). Her body no longer produced necessary natural hormones after that, and she would have to take hormone replacement therapy for the rest of her life.

But Jo never knew what had happened, or why. The doctors instructed her parents not to share this information with Jo because there was no room in Western society for such uncommon bodies, no acceptance of intersex variations. Just as there is little tolerance for and understanding of gender nonconformity in our society, there is little tolerance for and understanding of natural sex nonconformity. “When I was born, [my parents] had not heard of [CAIS] or anything like it and knew nobody else dealing with it; it was so completely isolating,” Jo told me. There was nothing but shame and secrecy.

She finally learned her medical history as she was finishing university. The truth of her condition made sense to her. But the fact that everyone had conspired to keep that truth from her didn’t. Jo went on to become a child and adolescent psychiatrist, working with children with DSD and their families, and is also a trustee for charity that supports children with DSD and their families. And although she recognizes that there are some overlapping concerns between transgender people and those with DSD, she has found, as the clinics fill with gender dysphoric children, that including I—for intersex—in the LBGTQIA++ alphabet both co-opts the language of intersex rights and detracts from the needs of kids with intersex conditions.

“For children with DSDs it was very much, You need to fix the body, rather than being truthful, offering psychological support or family support.”

You may have heard that as much as 1.7% of the population has an intersex condition, but that’s because some scholars and activists have widened the category and conception to include conditions like Turner syndrome, when one X chromosome is missing or partially missing. In reality, closer to .02% of people have actual DSD.

In comparison, congenital adrenal hyperplasia—a potentially dangerous condition in which a female fetus is exposed to high levels of testosterone and other androgens—can present at birth with atypical genitalia, for instance lengthening the clitoris into what looks like small penis. (Few such girls or their parents identify as intersex. Like trans, intersex is a term that some people want to expand the boundaries of, while others resist.) Penoscrotal hypospadias, when the opening is at the base, not the tip of the penis, means boys can’t pee standing up. In both cases, the genitalia may be functional but unusual looking, and the latter was what mattered in the past. “For children with DSDs it was very much, You need to fix the body, rather than being truthful, offering psychological support or family support,” Jo said.

But those cosmetic surgeries often left scar tissue that affected sexual function, and rendered these children lifelong medical patients, which is why adult people with DSD and intersex conditions began lobbying to stop medically unnecessary surgeries performed to make a body conform.

At the same time that cultural consciousness about the needs of kids with intersex conditions has been raised, the number of children with gender dysphoria has exploded. “Throughout child and adolescent mental health, we have seen a big increase of children presenting with gender issues,” Jo said.

As the intersex rights movement has pushed us away from performing dangerous or medically unnecessary surgeries on children, the trans rights movement has pushed children back toward them, arguing that we shouldn’t perform these surgeries on infants not because of the medical issues but because these children should be allowed to claim their gender identity later, and change their bodies accordingly then. “There is almost a similar narrative to it, that the body is wrong and needs to be fixed because of psychological issues,” Jo said. “It just really reminded me of how DSD were approached in the previous generation. It’s all about you have an innate gender identity and you need to fix the body.”

When people campaign for no surgical interventions for children with DSD but for younger ages of intervention for gender dysphoria, she says, it doesn’t add up in terms of balancing risks and benefits.

“Most of the issues for surgery are not because you’ve had a wrong gender identity. It’s because they cause long-term problems because it’s an incredibly complex and sensitive area with huge amounts of nerve cells,” she said. “If you have genitals that are functional and work exactly as expected from the perspective of someone with a DSD, it’s great thing not to mess around with.” The importance of fertility and sexual function, she said, can’t be fully understood by most adolescents, and surgery often interferes with them, whether it’s done in infancy or the teen years.

“One of the problems of the I in the big LGBTQ-plus umbrella is that the actual needs of the most vulnerable and the most in need of support or psychological interventions and family support, they kind of get lost,” she said. The inclusion translates the struggles of kids’ with intersex conditions into a facet of queerness and identity, when they are a facet of biology.

For instance, a teenager who has just found out she’s XY may told by our culture that she’s queer or a third sex, which can make a difficult situation worse. “It’s really tough when you’re 14 years old and find you don’t have a uterus. Your vagina is short. If you want to have sex with your teenage boyfriend, then you may need to use dilators,” Jo said. And it’s not something that can just be kind of waved away with a few flags.” We’re told that misgendering is profoundly offensive, she added. But telling a child that they’re not male or female, “is really disrespectful and quite dehumanizing.” Jo is fine with adults identifying however they want, “But we need to ensure that children do not have adult identities forced on them.”

Some adult trans people, meanwhile, arrive at the clinic asserting that they’re intersex. “It’s almost a universal thing, that they kind of use intersex to explain gender dysphoria,” she said. “Androgen insensitivity syndrome was being used as a way to say sex isn’t real.” And intersex has become an identity, instead of a way of characterizing a series of medical conditions. “A lot of people use intersex as an identity term and often without having a DSD,” Jo said.

Just as there is little tolerance for and understanding of gender nonconformity in our society, there is little tolerance for and understanding of sex nonconformity.

These arguments are unscientific and miss the most important point. We shouldn’t operate on children to make them cosmetically conform because it’s physically bad for them. What we need to do is accept these atypical bodies and teach children to accept themselves, too. Focusing on identity obscures the real medical and psychological needs of children with DSD and their families, and doesn’t safeguard children against unnecessary surgery.

Meanwhile, the vast majority of kids with DSD don’t have gender issues, Jo said. “The rates of people [with DSD] wanting to actually change their sex is fairly low.” The issue with the way Jo was raised wasn’t that she was forcibly assigned a gender, rather than able to pick her own. It was the secrecy and shame.

Jo is not against medical interventions per se. She has referred children on to the gender identity clinic when she thought it was appropriate. But she’s against interventions for younger and younger kids, without considering the complications. “You can cause a lot of harm if you [perform medical interventions because of] ideological beliefs.”

She thinks the same cautious approach we now take to children with DSDs should be applied to children with gender dysphoria. “If you are going to use medical interventions, it should be really cautiously done because I think the most fundamental thing in pediatrics is usually to take a watch-and-wait approach and to intervene as little as possible—unless there is really clear evidence of best interests.”

“The rates of people [with DSD] wanting to actually change their sex is fairly low.”

Jo believes that sometimes interventions can be beneficial, and doesn’t support banning medical approaches to treating gender dysphoria, even for young people. “But I do prioritize an evidence-based, cautious approach with young people,” she said. And that’s especially because of the kinds of young patients with gender issues she sees today.

“We are increasingly seeing very complex young people with gender dysphoria and autism.  These young people often have co-morbid eating disorders and significant self-harm, including swallowing magnets, tying ligatures and frequent cutting– and now also presenting with diverse gender identities,” she said.

In the UK, Jo said, clinicians are still fairly cautious, but the U.S. seems to be pushing for ever earlier medical and surgical interventions. “These are frequently very vulnerable young people and any interventions need to be evidence-based and following careful assessment, or there is a risk that interventions offered may not be in their long-term best interests,” she said, even if it is conceived of as an ameliorative intervention. 

For some young people, could a desire for medical interventions be trying to find a simple solution to really complex problems—or even another form of self-harm?

“To make any real change, it needs to be both a medical model alongside more of a psychological, holistic model,” Jo said. Children with DSD need the same thing gender nonconforming children need: acceptance. And they may need what gender dysphoric children need, too: psychological support. 

Accepting a body with a DSD can take time, Jo said, and may need psychological support. “But it is possible, and being born with a DSD should be no barrier to a great life.”