In a remote village in the Dominican Republic, children born female undergo a transformation into boys during puberty due to a rare genetic disorder.
Salinas Village in the Barahona province of southwestern Dominican Republic resembles many other distant lands in the Caribbean. The friendly locals, white sandy beaches, and laughter fill the air at sunset. However, a strange phenomenon is taking place here that has baffled many scientists: the gender transformation of girls at puberty. Girls affected by this syndrome are typically born without testes and possess an organ that resembles a vagina. However, as they reach puberty, elevated levels of testosterone cause male genitalia to develop.
Essentially, Johnny, 24, is entirely a boy in both physical and physiological terms. However, notably, Johnny was born as a girl. This is one of the cases observed in Salinas, an isolated village in the southwestern Dominican Republic, where many children born as girls have transitioned to boys during puberty.
Although Johnny’s story may sound miraculous, for the residents of Salinas Village, such cases have become familiar. This phenomenon occurs at a rate of 1 in 90; for every 90 girls in Salinas, one will undergo transformation by the age of 12. These children are nicknamed “guevedoces”, which means “penis at age 12.”
“I was born at home instead of in a hospital, and my family had no idea about my gender before birth. I remember wearing a dress to school,” Johnny shared. “However, I never wanted to dress like a girl. When my parents bought dolls, I wasn’t interested in playing with them. What I wanted was to play with boys.”
According to scientists, this rare genetic disorder occurs due to the absence of a specific enzyme that hinders the production of a certain form of male hormone, dihydrotestosterone, while in the womb.
Johnny, 24, was born a girl but became a boy at the age of 7.
All babies in the womb, whether male or female, have endocrine glands known as gonads. Around the 8th week of gestation, infants with a Y chromosome begin to produce dihydrotestosterone in large quantities to form a penis.
However, in many cases, male fetuses lack the 5-alpha-reductase enzyme necessary to increase male hormones, resulting in these fetuses being born female due to the absence of a penis and testes. Yet, by puberty, a significant mutation causes testosterone to be produced in large amounts, allowing male reproductive organs to emerge. Consequently, these girls will become boys with complete penises, accompanied by a deepening voice.
In Johnny’s case, this transformation occurred at the age of 7. Johnny expressed that he is truly happy to have fully transitioned into a boy.
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Another case in Salinas is that of a girl named Carla, who is beginning a similar transition at the age of 9. Although born a girl, Carly is gradually adopting a male lifestyle as she grows up.
Many who transition also change their names to better match their new gender, such as Johnny, whose new name replaced the old name Felicia, given by his parents when he was born a girl. However, many still choose to retain their original names given by their parents even after transitioning to boys.
The “guevedoces” syndrome was first identified by Dr. Julianne Imperato, an endocrinologist at Cornell University, in the 1970s when she traveled to the Dominican Republic after hearing rumors about cases of girls turning into boys.
Decoding the “Self-Transition” Phenomenon
From a medical perspective, these cases are referred to as “pseudohermaphroditism” or “intersex”. This term describes atypical developmental states of sex and physiology in the body. These can include unusual characteristics both externally and internally in the reproductive organs, such as ovaries, uterus, testes, sex chromosomes, endocrine glands, or sex hormones. This is a very rare genetic disorder; statistics show that the average occurrence of “intersex” is about 1 in 2,000 births.
However, in Salinas, this rate is 1 in 90. One of the first researchers on this condition was Dr. Julianne Imperato-McGinley from Cornell University Medical College in New York in the 1970s. She ventured into the remote areas of the Dominican Republic after reading reports about the cases of girls transitioning into boys there. Her investigation revealed that most gender transition cases live as fully-fledged men in terms of personality and sexual relationships. Some choose surgery to revert to female, as they were at birth. Other cases have also been recorded in the Sambian village of Papua New Guinea.
Currently, scientists have completely decoded this rare condition. The cause is a form of rare genetic disorder. In reality, during the first few weeks of pregnancy, the genitalia of both boys and girls are indistinguishable. By around the 8th week of pregnancy, male infants begin to produce dihydrotestosterone in large quantities, leading to the formation of genital organs including: the penis, urethra, and prostate.
However, during the formation of external genitalia, some boys lack the 5-α-reductase enzyme, which reduces the levels of the male sex hormone dihydrotestosterone, disrupting the process of penis formation. This results in these boys being born with genitalia that resemble a vagina. Later, at puberty, these “girls” will produce a large amount of the male hormone testosterone, causing their male genitalia to further develop into a penis. Essentially, the development of these children’s reproductive organs should have occurred in the womb but was delayed for several years. Pharmaceutical companies worldwide are still researching to find a cure for this condition.
Despite living like typical men, some small differences persist as they enter adulthood. Most cases have less facial hair and smaller-than-average prostates. Scientists believe that the isolated lifestyle of the villagers is what has allowed this condition to persist across generations. This unique aspect has attracted numerous tourists to Salinas each year.
