Johnny was a grown-up girl. There was no reason not to. He had what looked like a vagina. So he went to school dressed in small red clothes, and used to talk beautiful. But he was never happy being a girl.
“I didn’t like to dress up as a girl and when they bought me toys for girls I never cared to play with them – when I saw a group of boys I would stop playing ball with them, “Johnny tells the bbc,
Once Johnny hit puberty, a penis began to mysteriously appear, and his testicles descended. Apparently, Johnny was always a boy, but a genetic mutation prevented his genitals from developing until puberty.
Catherine and her cousin Carla, Guedoes, in the Dominican Republic.
Johnny, formerly called Felicita, isn’t the only anomaly in Salinas, a small village in the Dominican Republic. The BBC’s filmmakers also talked to Carla, a seven-year-old “girl” who was on the verge of turning into Carlos. Even before she reached puberty, her mother was seeing the changes to come.
“When she turned five I noticed that whenever she saw a guy friend she wanted to fight with him. Her muscles and chest started to grow. You could see she was going to have a boy. I love her I do, however he is. Girl or son, doesn’t matter.”
Johnny and Carlos belong to a small group of individuals who suffer from a rare genetic disorder that inhibits the production of an enzyme called 5α-reductase, which is responsible for the metabolism of testosterone to dihydrotestosterone, or DHT. Since DHT aids in the development of male sex organs, deficiency of this enzyme leads to underdevelopment of male sex organs. Babies born with the condition have female genitals, and are thus raised. At puberty, these boys, like all normal men, get a surge of testosterone. This time the body reacts, and the penis begins to grow and the testes descend into the scrotum. Secondary sexual characteristics, such as increased muscle mass, beard, and deep voice may also appear.
Officially this condition is called 5-alpha reductase deficiency. In the Dominican Republic these men are known as guedoses which means “penis on twelve”. They are also called “machihembras”, which means “first a woman, then a man.” In Papua New Guinea this phenomenon is called Kwalu-Atomwol by the Sambian people. In Salinas where the condition is most prevalent, approximately one in 90 children is affected by the mutation.
Guavedos’ unusual condition has led to the development of a new drug called finasteride that is used to shrink an enlarged prostate, and it works by reducing the amount of the DHT hormone.
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