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Birth defect is plaguing children in FLDS towns

Fumarase Deficiency afflicts 20, is linked to marriages of close kin

Published: Thursday, Feb. 9 2006 12:00 a.m. MST

It's one of the darkest secrets of the Warren Jeffs polygamist community.

An especially severe form of birth defect is on the rise and may mushroom in coming generations.

"I don't want to describe it in too much detail," said Isaac Wyler, who was related by marriage to some of the victims. "It's not a real pretty sight."

According to experts and former Jeffs followers, the cause of the birth defect is clear: 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.

The children live in the polygamist community once known as Short Creek that is now incorporated as the twin towns of Hildale, Utah, and Colorado City, Ariz. Tarby believes the recessive gene for Fumarase Deficiency was introduced to the community by one of its early polygamist founders.

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.

For many years, Bistline was a member of the Fundamentalist Church of Jesus Christ of Latter-Day Saints (FLDS), which today reveres fugitive polygamist Warren Jeffs as a prophet.

"They claim to be the chosen people, the chosen few," Bistline said. "And their claim is they marry closely to preserve the royal bloodline, so to speak."

Wyler, who says Jeffs kicked him out of the FLDS group two years ago, has observed some of the "Fumarase children" in their home environment.

"I've seen some children that can talk and communicate a little," Wyler said. "And I've seen others that are totally laid out. They have no movement. They can't do anything by themselves. Literally, if they're 8 years old, it's like taking care of a baby."

Tarby saw the first "Fumarase child" in the community 15 years ago. He said the oldest victim is now about 20 years old. In March 2000, Tarby co-authored an article in the medical journal "Annals of Neurology" describing eight new cases of Fumarase Deficiency in the Southwest. It has now grown to 20 known cases in the polygamist community on the Utah-Arizona border.

Tarby said children suffering from Fumarase Deficiency have unusual facial features and frequent "grand mal" epileptic seizures. The children require constant care from parents and close relatives. "In some ways, they are really kind of remarkable people," Tarby said. "They do treat these kids pretty well."

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