'Closed door'

Local historian Benjamin Bistline said 75 to 80 percent of people in the area are blood relatives of two men - John Y. Barlow and Joseph Smith Jessop - who founded the sect on the remote desert plateau in the early 1930s.

"There aren't any new people coming in. It's a closed door and that gene just keeps getting passed around," said Bruce Wisan, a court-appointed accountant overseeing a trust of the sect's assets.

Dr. Leslie Biesecker, chief of the Genetic Disease Research Branch at the National Institutes of Health, said the bad gene could have been introduced after the original founding families settled there. "Any person who joined that community could have brought that mutation with them," he said.

Tarby, who has recently retired, said he first observed the problem when an FLDS couple came to a Phoenix clinic about 15 years ago with a 10-year-old boy suffering from a degenerative condition. He sent a urine sample to a lab in Colorado for analysis and was stunned by the diagnosis.

Since then, increasing numbers of children in the community have been stricken with the disease, which causes unusual facial features, frequent epileptic seizures, episodes of coma and possibly early death.

In the disorder, brain cells fail to receive enough fuel to grow, multiply and function properly because of a missing enzyme needed to generate energy from food, causing severe mental retardation and muscle control problems.

Tarby met with about 150 FLDS members in November, explaining that the disorder was not caused by tainted drinking water as rumored but by cousin marriage.

But even with that knowledge, it is still hard for people to leave the sect, said Brenda Jensen, 55, who fled the FLDS several years ago and now works for the Utah-based HOPE Organization, which helps women leave.

"If they are willing to marry their cousin, or unwilling but do it anyway, or even in a relationship that is closer than that, it can be very hard for them," Jensen said.

And local habits, are deeply ingrained, authorities say.

"They will tell you if that's what God wants for you than that's what you will get," said Gary Engels, an investigator assigned to Colorado City by the Mohave County attorney's office. "They don't think too much about marrying cousins and things like that."

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