Saturday 19 December 2009

A Gaza Story



From CNN:
Both Nadir and Ahmed were born with a rare birth defect called male pseudohermaphrodism.

Deficiency of the hormone 17-B-hydroxysteroid dehydrogenase (17-B-HSD) during pregnancy left their male reproductive organs deformed and buried deep within their abdomens.

At birth, doctors identified Nadir and Ahmed as girls, because they appeared to have female genitalia.

As a result, they spent the first 16 years of their lives dressing and acting like girls. It was a role that grew increasingly difficult to play, as they hit puberty and their bodies began generating testosterone, resulting in facial hair and increasingly masculine features.
...
Dr. Jehad Abudaia, a Canadian-Palestinian pediatrician and urologist practicing in Gaza, says he has diagnosed nearly 80 cases like Nadir's and Ahmed's in the last seven years.

"It is astonishing that we have [so] many cases with this defect, which is very rare all over the world," Abudaia says. He attributes the high frequency of this birth defect to "consanguinity," or in-breeding.

"If you want to go to the root of the problem, this problem runs in families in the genes." Abudaia says. "They want to get married to cousins... they don't go to another family. This is a problem."
...
Abudaia's first advice to patients with the disorder is to immediately adopt male clothing and hair cuts, and then to plan for a sex-change operation.
*SIGH* Which means a significant number will become TS as the result...
This unusual ritual has been performed several times in the extended family of Nadir and Ahmed, where sex differentiation is a recurring disorder.

Nadir's 21-year-old brother Midyam and his 32-year-old cousin Ameen Abd Hamed share the same condition of male pseudohermaphrodism. As adolescents, they too underwent the gender identity transformation process the family refers to as "the transfer."

Some back of the envelope calculations:

Gaza Demographics:
Population ~400,000
Birth Rate ~0.04
Infant Mortality rate ~0.02

Expectation of cases of 17BHDD over 7 years
1 in 100,000 x 7 x 400,000 x 0.02 = About 0.5
Actual cases recorded by one doctor : 80

So 17BHDD is 100-200 times more likely than expected. Call it 2 orders of magnitude.

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