Monday, February 13, 2006

Birth Defects Up in Polygamous Area (Utah/Arizona Border)

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Birth Defects Up in Polygamous Area
ASSOCIATED PRESS
SALT LAKE CITY (AP)
- A rare, severe birth defect is on the rise in an inbred polygamous community on the Utah-Arizona border, according to a doctor who has treated many of the children.
Intermarriage among close relatives is producing children who have two copies of a recessive gene for a debilitating condition called fumarase deficiency. The enzyme irregularity causes severe mental retardation, epileptic seizures and other effects that often leaves children 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, the neurologist told Salt Lake City television station KSL-TV.
The children live in the twin polygamist communities of Hildale, Utah, and Colorado City, Ariz.
Tarby believes the recessive gene was introduced by one of the community's polygamist founders.
Community historian Ben Bistline said 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.
"Ninety percent of the community is related to one side or the other," said Bistline, a former member of the sect, the Fundamentalist Church of Jesus Christ of Latter-Day Saints.
"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."
Isaac Wyler, also a former follower of the church led by fugitive Warren Jeffs, told KSL that he was related by marriage to some of the victims.
"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 child with the deficiency in the community 15 years ago. He said the oldest victim is now about 20.
In March 2000, Tarby co-authored an article in the medical journal "Annals of Neurology" describing eight cases of fumarase deficiency. It has now grown to 20 known cases in the community.
Tarby said the victims require and receive constant care from parents and close relatives.
"They would just assume they've been given a test and they need to pass this test," Wyler said. "And it's their lot in life to take care of a child like this. And they'll give it everything they've got. And they'll do a good job."
In the FLDS community, marriages with cousins and other relatives are common, Bistline said.
It's believed that more than half the residents carry the recessive gene. That means the number of cases likely will grow. Tarby said there could be hundreds of victims in coming generations.
Tarby said he urges residents to undergo genetic screening before marriage, but is largely ignored.
He said medical care for the children is frequently borne by taxpayers

What is Fumarase Deficiency? Fumarase deficiency is a very rare metabolic disorder causing severe neurological disease and often death in early childhood. Massachusetts General Hospital

Fumaric aciduria is transmitted as an autosomal recessive trait and is due to fumarase deficiency. Fumarase catalyses the transformation of fumarate into malate in the Krebs cycle. Clinical signs have an early onset but are non-specific: hypotonia, psychomotor retardation, convulsions, respiratory distress, frequent cerebral malformations and polyhydramnios. Chromatography of organic acids evidences the excreted fumaric acid, often associated with succinic acid and alphacetoglutaric acid. Hyperlactacidemia and moderate hyperammonemia are other common findings. Diagnosis can be confirmed by measuring fumarase in leukocytes or cultured fibroblasts. There is no efficient treatment, but prenatal diagnosis is available. * Author: Pr. J-M. Saudubray (March 2004) *

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