Why Was He a Girl?

An earlier post ended with pre-med students, enrolled in my Medical Ethics course, wondering how a 14 year-old boy from India turned out to be a girl, even though she had a penis.

Someday, after my students became doctors, they might need to explain the same condition to other parents. What should they say?

Certain known biological events likely occurred in her mother's womb after the girl was conceived. . . .

Women are made to make more women

Women's reproductive systems are programmed to give birth to girls. Their ovaries are charged with XX chromosome eggs that are intended to develop into females.

So when the mother's egg is fertilized by a sperm from the father that carries a matching X chromosome, a female embryo starts to grow, as intended.

Nine months later a girl is born. The infant's sex organs are visibly female. Invisibly, she carries female genes, chromosomes, and hormones. She will grow up to have a woman's body and a woman's brain.

The Y chromosome has a plan of its own

However, there's a 51% chance the mother's egg will be fertilized by a sperm that carries the male Y chromosome. Immediately the initial female development plan gets overwritten.

Shortly after conception the Y chromosome begins to stimulate the production of male hormones called androgens, most notably testosterone. The androgens drive the new plan. They cause the fetus's incipient female sex organs to grow into male sex organs instead.

This process explains the "undescended testicle" for instance. Androgens cause the incipient ovaries to develop into testicles while migrating down into the scrotum. The scrotum is the male version of the organ that, in a female fetus, becomes the vaginal labia.

Sometimes testicles can become stranded higher in the abdomen, perhaps incompletely formed; undescended in other words.

The androgens affect not only the genitals. They also drive the development of male genes, chromosomes, and hormones. A boy is born. He is biologically destined to grow into the form of a man, with the psyche of a man.

Things can go awry

Sometimes the process wanders off track. In a male embryo the development of fully male traits can be retarded by low androgen levels at critical times. Or excessive androgens, perhaps produced by an overactive adrenal gland, may cause a female embryo to develop male traits­—likely what happened to the girl from India.

Congenital sex development can go awry for a number of other reasons as well, leaving a variety of symptoms, some readily visible, some undiagnosed for the person's entire life.

Of every hundred infants born, one is likely to have a body that is different in some way from the familiar male or female.

"Is it a boy or a girl?" The newborn's sex is determined in the delivery room on the basis of a single criteria—the appearance of the genitals.

Occasionally, however, the actual sex of the infant—including genes, chromosomes, hormones, general anatomy, and psyche—cannot be readily detected at birth and may not become apparent for years.

So a child can be a girl, or an adult can be a woman, even though she has a penis, fully formed or vestigial. And a child or adult with a vagina, or a body feature approximating one, can be a male.

The sex organs can also develop so ambiguously sometimes that doctors can't tell, by looking, whether the newborn is a boy or girl.

So my students learned the biological explanation for the case of the "boy" from India whose female hormones triggered breast growth and menstruation when "he" entered puberty. What about medical ethics?

What's a doctor to do?

The parents insisted the doctors in New York "correct" their daughter's female traits with radical surgery, principally removal of her breasts and ovaries. And they should not inform the child.

What, in accordance with medical ethics, should the doctors do?

The March 25 post will answer that question. Three choices will be considered then:

(a) The doctors should respect the cultural values of the foreign parents and perform the surgery. The parents have full authority to make medical decisions for their minor child.

(b) The doctors should propose instead to perform corrective surgery to match the girl's genitals with her actual sex. That would involve removing the penis and replacing it with a surgically constructed vagina.

 (c) The doctors should advise the parents to delay any irreversible action until tests determine the true sex of the child; until the medical options have been determined; and until the child is sufficiently knowledgeable to participate in the decision.

NEXT POST:

The Parents Want the Doctors to

Cut Out Some of Their Daughter's Organs