This commentary is by Paul Manganiello, M.D., emeritus professor of obstetrics and gynecology at Geisel School of Medicine at Dartmouth, co-medical director of the Good Neighbor Health Clinic in White River Junction, and president of the GunSenseVT Education Fund. He lives in Norwich.
When Judge Ketanji Jackson-Brown was recently going through the Supreme Court nomination process, she was asked this question. “Judge, how do you define a woman?”
Pretty straightforward question, right? Yes, if you are capable of having only binary thought processes, and not having the capacity to have a reality-based, nuanced worldview. Not much in life is black or white; there is a lot of gray in between.
Some would say we shouldn’t complicate these issues, but that is exactly why people need to educate themselves on issues that affect so many of us on a daily basis. We all need to reject simple black or white responses. Fortunately, the judge didn’t take the bait; she was too wise for that.
It doesn’t take much to realize that sex is really, really complicated. Sexuality is a spectrum whether you are talking about “phenotypic “ sex, the observable, outward physical appearance of an individual; chromosomal sex, whether we are talking about the individual’s overall chromosomal makeup, i.e., 46 XX, 46 XY, 47XXY, etc.; or the numerous possible genetic mutations that can affect the genes on the individual’s chromosomes and thus ultimately affect the outward physical appearance of that individual.
To complicate the situation, we need to recognize that sexual orientation is also on a “spectrum” from being exclusively heterosexual, homosexual, or bisexual. Oh, don’t forget to throw in gender identity. Despite what the individual’s outward appearance is, how do they view themselves?
There are literally thousands of genes that can cause differences in sexual development. Many of these cause abnormalities in the production of sex hormones (estrogens, progestogens and androgens), which ultimately affects sexual and physical development. Differences in sexual development, Disorders of Sexual Development (DSD), are not rare, and probably affect more than 1 out of 1,000 individuals.
Let me give you only two examples of intersex conditions that make up DSD.
- An individual with the most common form of congenital adrenal hyperplasia, of which there are several, with a 46XX chromosomal karyotype has an enzymatic defect, causing that individual to be unable to make adequate quantities of adrenal hormones. In order to compensate for this inadequate production, the adrenal gland inadvertently is stimulated to produce excess amounts of other adrenal hormones, sex (estrogens, androgens and progestogens) hormones.
During the affected individual’s intrauterine development, the excess androgens will masculinize/androgenize the development of their sexual organs even though they are chromosomally female.
The extreme form of this condition will result in the enlargement of the clitoris, to the point of it resembling a penis. The lips of the vagina may also be fused, resembling a scrotum. When these individuals approach puberty, because of the body’s exposure to excess sex hormones, they may be shorter than expected, due to the premature closure of the epiphyses of their long bones. They will also experience other manifestations of masculinization, excess unwanted facial and overall body hair.
- Likewise, there are numerous examples of 46XY DSD. An individual with a condition known as the Androgen Insensitivity Syndrome will have a 46XY chromosomal karyotype. They will have testes and have male levels of circulating androgens, but their androgen receptive organs will be unresponsive. This is due to the fact that the cells lack an enzyme, which allows the cells to respond to these circulating androgens. The testes are usually inside the abdomen. The penis and scrotum are undeveloped. The breasts are able to react to circulating estrogens so they develop along feminine lines.
Since Mullerian/Uterine Inhibitory Factor is also secreted from the testis, along with the sex hormones, uterine development is inhibited and doesn’t occur. When the individual enters puberty and becomes sexually active, a vagina can be formed as a result of attempts at penetration from the partner’s penis/or a dilator or through a surgical intervention. Their sexual identity or sexual orientation may or may not correlate with their physical phenotype.
Let’s be clear about our lesbian, gay, bisexual, trans, queer (LGBTQ) neighbors: When it comes to gender identity and sexual orientation, it isn’t a choice. In the beginning of one’s sexual development, those attitudes may be somewhat fluid, but at some point in time, they become “hardwired.”
Personally, at this point in my life, I could not choose to be a homosexual or bisexual. In a similar vein, individuals who present with DSD certainly don’t choose to have their physical disorders. They suffer emotionally from the intolerant and unkind opinions they may encounter on a daily basis. They certainly don’t need government officials dictating how they should be treated.
What they need, however, is to be treated respectfully, both from those of us who interact with them in society, or in a medical setting. We need to be more empathetic and supportive. We need to be able to put ourselves “in their shoes” and help them as they navigate their difficult personal life’s journey.
