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Polygamist Community Faces Rare Genetic Disorder

By Carolyn E. Price     Jun 14, 2007 in Health
The twin communities bordering Utah and Arizona, Hildale in Utah and Colorado City in Arizona, have the most cases in the world of fumarase deficiency, an enzyme irregularity that causes severe mental retardation. Why? Simply put, cousin marriage.
This community is home to the Fundamentalist Church of Jesus Christ of Latter Day Saints (FLDS) who are led by Warren Jeffs. The FLDS broke away from the regular Mormon Church more than seventy years ago over the very issue of polygamy.
Mr. Jeffs was on the FBI's Most Wanted List and was apprehended and arrested last August. He was charged as being an accomplice to rape because he used his position to force a 14-year-old girl to marry (and consequently have sex with) her 19-year-old cousin.
There are about 10,000 people who live in this "closed" community. They shun outsiders, avoid newspapers, television and the internet and wear conservative, 19th-century clothing.
Doctors say that a rare genetic disorder is spreading through polygamous families on a wave of inbreeding. Fumarase deficiency is an enzyme irregularity that causes severe mental retardation and doctors believe it is brought on in this community because of "cousin marriage".
"Arizona has about half the world's population of known fumarase deficiency patients," said Dr. Theodore Tarby, a pediatric neurologist who has treated many of the children at Arizona clinics under contracts with the state.
"It exists in a certain percentage of the broader population but once you get a tendency to inbreed you're inbreeding people who have the gene there, so you markedly increase the risk of developing the condition."
As recently as ten years ago, doctors had only been able to study about 10 case in the whole world. Former FLDS members, independent doctors and authorities are saying that there are at least 20 children in this community who have the disease.
"The disease itself is very rare in the rest of the world," said Dr. Vinodh Narayanan of Arizona's St. Joseph's Hospital & Medical Center and Barrow Neurological Institute. "Once you get people within in the same community marrying, then the chances grow of having two people carrying the exact same mutation."
Approximately 75 to 80 percent of people in this community are related by blood to two men -- John Y. Barlow and Joseph Smith Jessop. These two originally founded the sect in the early 1930's on this remote desert plateau.
"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. Tarby first came across a child with the disease when a FLDS couple came to his clinic in Phoenix with their 10-year-old son. He had been suffering from some sort of degenerative disease that noone could put a finger on. Dr. Tarby sent the boy's urine out to a lab in Colorado for an in-depth analysis and was stunned when he received the diagnosis back.
People who have fumarase deficiency are missing an enzyme in their system that is needed to generate energy from food. Consequently, brain cells fail to receive enough fuel to grow, multiply and function properly. This causes severe mental retardation and muscle control problems.
The disease also causes unusual facial features, epileptic seizures, comas and early death.
Dr. Tarby, who is retired, met with members of the FLDS community last November and tried to explain to them that the disease in cause by cousins marrying each other. There was a rumor going around the community that the disease was caused by drinking tainted water.
"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."
More about Polygamist, Community, Genetic disorder