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[escepticos] incesto: gente azul


Como ejemplo de problema genético asociado al incesto, he visto
citado con frecuencia el caso de la "gente azul de Kentucky". 
No es una historia especialmente trágica, pero sí muy... eh...

El llamativo color que denunciaba consanguinidad era causado por 
la metemoglobinemia, una enfermedad genética recesiva relacionada 
con la hemoglobina de la sangre que le da a la piel un color azul.

Una búsqueda en google ("Fugate Kentucky blue") proporciona una 
multitud de enlaces. Ninguno de los que he mirado yo duda de la 
autenticidad del caso.

Un buen sitio parece ser 


It all started over 6 generations ago after a French orphan named 
Martin Fugate claimed a land grant in 1820 and settled on the banks of 
eastern Kentucky's Troublesome Creek, with his red-headed American 
bride, the former Elizabeth Smith, whose skin was as pale as the 
mountain laurel that blooms every spring around the creek hollows. The 
Fugates had seven children, four were reported to be blue. The clan 
kept multiplying. Fugates married other Fugates. Sometimes they 
married first cousins. And they married the people who lived closest 
to them, the Combses, Smiths, Ritchies, and Stacys. All lived in 
isolation from the world, bunched in log cabins up and down the 
hollows, and so it was only natural that a boy married the girl next 
door, even if she had the same last name.
"When they settled this country back then, there was no roads. It was 
hard to get out, so they intermarried," says Dennis Stacy who counts 
Fugate blood in his own veins. 

Martin and Elizabeth Fugate's blue children multiplied in this natural 
isolation tank. The marriage of one of their blue boys, Zachariah, to 
his mother's sister triggered the line of succession that would result 
in the birth, more than 100 years later of Benjy Stacy. When Benjy was 
born with purple skin, his relatives told the perplexed doctors about 
his great grandmother Luna Fugate. One relative described her as "blue 
all over" and another calls Luna "the bluest woman I ever saw". Luna's 
father, Levy Fugate, was one of Zachariah Fugate's sons. Levy married 
a Ritchie girl and bought 200 acres of rolling land along Ball Creek. 
The couple had 8 children, including Luna. A fellow by the name of 
John Stacy spotted Luna at Sunday services of the Old Regular Baptist 
Church before the turn of the century. Stacy courted her, married her, 
and moved from Troublesome Creek to make a living in timber on her 
daddy's land. John Stacy still lives on Lick Branch of Ball Creek. 
Stacy recalls that his father-in-law, Levy Fugate, was "part of the 
family that showed blue. All them old fellers way back then was blue. 
One of em - I remember seeing him when I was just a boy - Blue Anze, 
they called him. Most of them old people we by that name - the blue 
Fugates. It run in that generation who lived up and down Ball Creek".

"They looked like anybody else, cept they had the blue color," Stacy 

"The bluest Fugates I ever saw was Luna and her kin," said Carrie Lee 
Kilburn, a nurse at the rural medical center called Homeplace 
Center. "Luna was bluish all over. Her lips were as dark as a bruise. 
She was as blue a woman as I ever saw."

Luna Stacy possessed the good health common to the blue people bearing 
at least 13 children before she died at 84. The clinic rarely saw her 
and never for anything serious.

Benjy Stacy was born in a modern hospital near Hazard, Kentucky, not 
far from Troublesome Creek. He inherited his father's lankiness and 
his mother's red hair but what he got from his great, great, great 
grandfather was dark blue skin! The doctors were astonished, not so 
the parents, but the boy was rushed off to a medical clinic in 
Lexington (University of Kentucky Medical School). Two days of tests 
showed no cause for Benjy's blue skin.

Benjy's grandmother Stacy asked the doctor's if they had heard of the 
blue Fugates of Troublesome Creek. Put on that track, they concluded 
that Benjy's condition was inherited. Benjy lost his blue tint within 
a few weeks and now he is about as normal a 7-year old boy as you 
might imagine. His lips and fingernails still turn a purplish blue 
when he gets cold or angry and that trait was exploited by the medical 
students back when Benjy was an infant.