An Intersex Primer

 

Hermaphroditus–that mythological creature both male and female–the double-sexed child of the Olympian gods Hermes and Aphrodite–does it survive today?

Human sexual development is a complex process involving differentiation into male and female, from fetus to adult. The process can be misdirected genetically, or break down along the way, resulting in a reproductive system that remains unfinished. Intersex is a blanket term for the various conditions resulting in these differences.

Actually, DSD–Disorder of Sexual Development–is the term some prefer, intersex/intersexed being somewhat politically incorrect, and hermaphrodite so often misunderstood.

Although the markers of sex–genetics, gonads, genitals, etc.–aren’t all on the male side or the female side, in intersex the choice of gender isn’t an issue as often as one might think.

Complete Androgen Insensitivity Syndrome (CAIS) leaves the body unable to process male hormones. An XY conception becomes a female child with testes in her abdomen and no ovaries or uterus. The testes don’t produce sperm, but some of the testosterone is converted into estrogen, which gives the child a feminine puberty.

Swyer Syndrome or Pure Gonadal Dysgenesis is a condition in which the testes don’t form; the result is an XY female with a uterus but no ovaries.

Turner Syndrome results from the loss of the second sex chromosome. Turner babies are female, but their ovaries are only streaks of tissue. Because the sex chromosomes help determine height, Turner Syndrome women are short-statured, averaging something like four-foot-eight as adults.

Klinefelter’s(XXY) and Kallman’s are two intersex conditions affecting men. They don’t ordinarily result in genital ambiguity. Because of the extra sex chromosome, the average adult height in Klinefelter’s is greater than in XY.

None of these intersex conditions result in the mythological hermaphrodite, at least not in the sense of having both sex organs. When intersex affects the genitals, they turn out somewhere between male and female. The Quigley Scale describes the range of intersex genital shapes.

Partial Androgen Insensitivity Syndrome(PAIS) results in ambiguous genitals, and breast development at puberty. The severity varies depending on which genetic mutation is responsible.

Congenital Adrenal Hyperplasia can result in masculine genital development in an XX baby, sometimes enough so that the child is raised male. Although CAH can be fatal if not treated, most CAH girls can bear children. One possible complication in CAH children being raised as boys is a feminizing puberty.

A 5-Alpha Reductase deficiency in an XY baby results in feminine genitals, but masculine development at puberty. 5-Alpha children, although sometimes castrated by their doctors and raised as girls, can be fertile males.

Mixed Gonadal Dysgenesis is a partial form of Turner Syndrome. Some cells have a Y chromosome; some don’t. The distribution of the cells during fetal development determines how the gonads develop and how tall the child will grow.

The medical definition of hermaphroditism is having both testicular and ovarian tissue. In Mixed Gonadal Dysgenesis, the child may be born with one testis and one streak ovary. Or they may have two ovatestes. Streak ovaries are non-functional and mixed gonadal tissue often results in cancer.

When a child is born with ambiguous genitals, parents and physicians must decide whether the child should be raised as a girl or a boy. Most experts recommend that a gender be chosen for the baby, but cosmetic surgeries be put off until the child can participate in treatment decisions. Until then, it’s important for parents to preserve their child’s options.

With or without surgical intervention, some children reject the gender assigned them. Determining when a gender change is appropriate may be difficult. Children aren’t always willing to verbalize their feelings and may not be aware of their options.

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