Tuesday, February 14, 2006

Problems in the Colorado Strip

When I was doing genealogical research on my family, I discovered Judge Drake, who, in his capacity as the first federal judge for Utah, was a royal pain in Brigham Young's backside. I wonder what he would make of this?:
...Intermarriage among close relatives is producing children who have two copies of a recessive gene for a debilitating condition called Fumarase Deficiency.

They predict the scale of the problem will increase dramatically in the future. Wyler, who has lived in the polygamist community most of his life, said he expects residents to continue marrying close relatives.

"Around here," Wyler said, "you're pretty much related to everybody."

Fumarase Deficiency is an enzyme irregularity that causes severe mental retardation, epileptic seizures and other cruel effects that leave children nearly helpless and unable to take care of themselves.

Dr. Theodore Tarby has treated many of the children at clinics in Arizona under contracts with the state. All are retarded. "In the severe category of mental retardation," the neurologist said, "which means an IQ down there around 25 or so."

Until a few years ago, scientists knew of only 13 cases of Fumarase Deficiency in the entire world. Tarby said he's now aware of 20 more victims, all within a few blocks of each other on the Utah-Arizona border.

...According to community historian Ben Bistline, most of the community's 8,000 residents are in two major families descended from a handful of founders who settled there in the 1930s to live a polygamist lifestyle.

"Ninety percent of the community is related to one side or the other," Bistline said.

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