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The story of the 5-alpha-reductase deficiency children.

(names have been changed, but all information is factual)

Dominican Republic

Little Josefina witnessed the birth of her six younger sisters and younger brother before she turned twelve. She grew up with dolls and dresses like any other girl in her town. At that point, her body began to undergo some changes. First, she began to grow hair under her arms and on her pubic region. Then her voice deepened, and her legs and chest grew some hair as well. Her shoulders broadened, and her testicles began to descend. Within a few months, little Josefina had a penis, forcing her parents to rename her Jose.

Two years later, towards the end of her younger sister’s eleventh year, Jose had a new playmate. Her new 11 year old brother.

Only one of Jose’s sisters remained female.

Being male is valued very highly in the Dominican Republic. So much that people are ready to accept the fact that these little girls are becoming men. The children simply take on a new identity right away. And society fully supports this. “We are so proud to have five healthy men in our family. Now we have someone to take care of us,” said Jose’s parents.

Jose and most of his siblings suffered from 5-alpha-reductase deficiency, which was thought to have come from a common ancestor, Altagracia.

In 5-alpha reductase deficiency the body does not convert testosterone into the necessary androgen dihydrotestosterone (DHT), the chemical that, at conception, masculinizes the baby. The result is feminine looking organs at birth (although the children are technically male).

At around the age of 12, there is a surge of DHT in the body, and the 5-alpha-reductase deficient children become normal, perfectly healthy males.

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