Hello, Lovelies! Welcome to Tiffany’s episode featuring Guevesdoces! Which, as mentioned during the episode, roughly translates to “penis at twleve”. To the left we have the Intersex Pride Flag in honor of this episode and below you’ll find information on the show featured during this week’s drink break and Tiffany’s thorough episode notes.

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Show Notes

Let’s take a trip back in time, ladies.  Close your eyes and think back to what you were like around the age of 12.  Puberty is hard. You have all these thoughts and emotions swirling around in your head.  No one understands what you’re going through. Your parents are super embarrassing and hyper critical.  A small glance from that cute stranger is enough to make every fiber of your being cry out with love and ecstasy.  You’re on a rollercoaster of pure chaos and terror and there is no end in sight! 

And then you grow a penis…

We are going to be learning about the Guevedoces (roughly translates to “penis at 12”).  I want to start by saying that gender is a spectrum. For this topic, I am going to use male/female or boy/girl in relation to anatomical sex (for the most part).

Let’s switch gears and take a geographic trip to Las Salinas, a remote village lying in the SW of the Dominican Republic.  Children in this region have a decent chance of being born with this condition (roughly 1% which sounds small but not when you think about it as 1 out of every 100 children… We would have had at least 20 in my graduating class alone).

Guevedoces, officially known as 5-alpha reductase deficiency, is a condition that affects male sexual development before birth and during puberty.  As the child develops in the womb, a genetic mutation prevents their bodies from converting the male hormone testosterone into a hormone called dihydrotestosterone, or DHT. Because DHT is responsible for the development of male sex organs, the lack of DHT means the male organs don’t develop properly.  

Imagine a pool floaty that is slightly deflated and folded in half.  Now poke a pool noodle through the top until just a little is poking out… This is what we all have before our sex is assigned.  If you get XX chromosomes, the pool noodle becomes your clitoris and the pool floaty becomes your labia. No huge change takes place.  If you get XY chromosomes, the pool noodle grows into a penis and the pool floaty reshapes to form testicles. DHT needs to work its magic for this to happen.

When children with this condition are born, their external genitals look female, or in some cases, ambiguous.  They are raised believing they are female and everyone else believes this too. Around the time that they start to go through puberty, an increase in the levels of male sex hormones leads to the development of some secondary sex characteristics, such as increased muscle mass, deepening of the voice, development of pubic hair, and a growth spurt. The penis and scrotum (the sac of skin that holds the testes) grow larger. Unlike many men, people with 5-alpha reductase deficiency do not develop much facial or body hair. 

Although this is common in this village, it’s not always an easy transition when Felicita comes back to school as Johnny.  “They used to say I was a devil, nasty things, bad words and I had no choice but to fight them because they were crossing the line.”  You can hear Johnny’s story, along with Carla who, at the age of seven, is on the brink of changing into Carlos, in the BBC Documentary Countdown to Life: The Extraordinary Making of You (2015). (Anything by the BBC must be 100% legit, right?)

Since this condition is inherited in an autosomal recessive pattern, meaning both parents must carry the gene (don’t mean the dad had this condition himself), it’s not uncommon for a Guevedoces to have family members with this.  Carla/Carlos cousin, Catherine, is a Guevedoces. He chose not to change his name but assumes a male role in his village.

This condition is not to be confused with Androgen Insensitivity Syndrome.  This is basically where everything from the outside looks female but internally, everything is male, testicles and all.  Breast enlargement occurs during puberty, but menstruation does not. In fact, a physical examination may reveal a shortened and blindly ending vagina.

Hermaphrodite is not the appropriate term for either.  In fact, it is considered by most to be a derogatory word.  Intersex people are individuals born with any of several variations in sex characteristics including chromosomes, gonads, sex hormones, or genitals that “do not fit the typical definitions for male or female bodies”.  Roughly 1 out of every 2,000 people are intersex. For perspective, intersex is more common than cystic fibrosis, a condition most people have heard of.  (The disease occurs in 1 in 2,500 to 3,500 white newborns. Cystic fibrosis is less common in other ethnic groups, affecting about 1 in 17,000 African Americans and 1 in 31,000 Asian Americans.)

People with 5-alpha reductase deficiency who were raised female but identify as male are not transgender, because in this case, they identify with their biological sex. 

There is no need to label them one way or the other.  We are all humans.

…drink break…

So why is it so prevalent here and not elsewhere?  Well it is found elsewhere. There are reported cases in Papua New Guinea, Turkey, and Egypt.  But the reason for its prevalence is thought to be because of “founder effect” or “bottleneck effect”, with the villagers being interrelated because they are geographically isolated and descending from an individual who carried the genetic mutation.

5-alpha reductase deficiency was discovered/first diagnosed by Dr. Julianne Imperato-McGinley, an endocrinologist from Cornell University, who went there to investigate the case during the 70’s.  (Endocrinologist is a medical practitioner qualified to diagnose and treat disorders of the endocrine glands and hormones. Endocrine glands are glands of the endocrine system that secrete their products, hormones, directly into the blood rather than through a duct.) She was the person who discovered that all of this stemmed from this enzyme deficiency.  She surmised that 12 families out of 13 have at least one or more male who carries the mutation. However, being carriers doesn’t necessarily mean all of them are affected. On an average, 1 out of 90 males is an affected carrier, and the rest are either not carriers or not affected.

Dr. Imperato-McGinley also noted that Guevedoces have smaller prostates than non-affected males. This observation, made in 1974, was picked up by Roy Vagelos, head of research at a multinational pharmaceutical company. He thought this was extremely interesting and set in progress research which led to the development of what has become a best-selling drug, finasteride, which blocks the action of 5-alpha-reductase, mimicking the lack of dihydro-testosterone seen in the Guevedoces. It is an effective way to treat benign enlargement of the prostate, a real curse for many men as they get older. Finasteride is also used to treat male pattern baldness.