Klinefelter syndrome (XXY) is a Medical and Genetic Condition

Stop Exploiting Us

Last spring we had a big wake up call with my youngest child, and we realized just how vulnerable she was to the influence of gender ideology, even in elementary school. I discovered PITT while trying to navigate through these experiences, and have spent the last year trying to understand how my kids' imaginations have been captivated to such a great extent. 

But today I’m writing about something different within my family that makes our challenges in gender all the more difficult: My son has Klinefelter syndrome—his genetic karyotype is 47XXY. Most males are 46XY and most females are 46XX. Most people have never heard of Klinefelter syndrome, and this unfamiliarity has created a vacuum where others have seized the opportunity to capitalize on assumptions about what it means to be XXY.

For the past few years as the mom of a boy with XXY, I've been "reading the room" and I've witnessed how others cite my son's genetic condition as "proof" that gender exists on a continuum. It is actually quite offensive how these outsiders have leveraged his genetic condition to further their social cause. I've seen how they could care less about his developmental challenges and health concerns. I wish people would approach his genetic condition with actual curiosity not foregone conclusions about something they know nothing about.

So, here’s my essay on this issue—I hope it adds to the overall conversation.

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A while back, National Public Radio ran a story about the skeletal remains of a medieval warrior who, through genetic testing, was discovered to have Klinefelter syndrome, also known as XXY.

My teenage son is XXY, and we are connected with a whole network of people who are quite familiar with the ups and downs of Klinefelter syndrome. A common theme emerged as we discussed our reactions to the NPR story: we felt the story did not accurately represent the realities of life with XXY. Multiple people affected by XXY contacted NPR to request that the story be retracted because of its poor research, misrepresentations, and the story’s speculation that everyone with XXY is “non-binary”. They informed NPR that the story harmed men and boys with XXY through its inaccuracies, and did a great disservice to the XXY community. NPR ignored these requests. 

An estimated 1 in every 650 males is born with an extra X chromosome.

The XXY community finds itself in the middle of the cultural fray, with others constantly pushing an agenda at their expense…an agenda that seeks to prove that people are gender fluid and that more than two sexes exist. 

The reality is a far cry from this magical thinking, that those with chromosomal differences are evidence of a third sex. Those looking for my son (and others like him) to be their poster boys for gender and sex fluidity, or on a spectrum are bound for disappointment, and XXY people have more pressing issues to deal with than gender expression.

What does life look like for those with this condition?

First, XXY people often have developmental issues. When my son was a small child (and before he was diagnosed), he struggled with so many things. He had trouble latching on at the breast, low muscle tone, apraxia of speech, poor memory, inattentive ADD, minor autistic like traits, and tooth decay. We used to wonder why it was so hard for him to formulate the words that were swirling around in his head and to kick a ball smoothly “across the midline” like the other kids. He has suffered from low energy and fatigue his whole life. His hands and feet often shake involuntarily, known as essential tremors. As he grows older he will be more susceptible to diabetes, certain cancers, autoimmune and heart diseases, and osteoporosis.

Around puberty the hallmark symptom of Klinefelter syndrome sets in: hypogonadism, also known as testicular failure. This does not mean my son is not male. It means that, for reasons that researchers still cannot explain, the testicles don’t get the message from the brain to make enough Testosterone. The male body needs testosterone for dozens of proper metabolic, executive, and bodily functions. To treat this deficiency, my son needs to give himself a painful Testosterone injection once a week for his short and long term health.

Despite these medical challenges, my son understands that his condition is specific to males, and that his extra sex chromosome and testicular failure do not make him some sort of hybrid male/female person, nor do they make him neither male nor female—non-binary in the common non-scientific parlance. He is not confused about his sex/gender. Society’s ignorance and mischaracterization of this condition, as promulgated by NPR and activists is inexcusable and we should not be allowing this sort of disinformation. It is directly harmful to my son and others.

The XXY community has its work cut out for itself in terms of raising awareness, raising funds for research, undoing outdated stereotypes, and sharing best practices with one another. But we don't make progress when we are constantly reacting to labels that we didn’t choose. Many XXY guys still hide their condition because outsiders have swept them up in the culture wars, speculating about them and their condition as if they are some sort of curiosity in a carnival act instead of individual human beings with their own concerns. 

People are naturally curious about the “novelty” that is XXY. That’s okay. But using them to prove gender theory is not okay. It's not right to use other people’s health conditions to promote what you wish were true, and the continuous positioning of XXY as an identity issue causes harm to those who are actually living with this medical condition. We need to be focusing instead on the issues that are most central to boys and men born with XXY—namely good medical and dental care, speech and occupational therapies, hormonal support, ongoing research, fertility treatments, and educational and workplace accommodations.

I used to think that we could protect our son by being discreet about his condition. I now think that the best thing we can do for his future is to get out in front of it, to dispel myths, and to actively shape this conversation.