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Polygamist community faces rare genetic disorder

By Jason Szep Thu Jun 14, 11:01 AM ET

COLORADO CITY, Arizona (Reuters) - In a dusty neighborhood under sheer

sandstone cliffs studded with juniper on the Arizona-Utah border, a rare

genetic disorder is spreading through polygamous families on a wave of

inbreeding.

The twin border communities of Hildale, Utah, and Colorado City, Arizona, have

the world's highest known prevalence of fumarase deficiency, an enzyme

irregularity that causes severe mental retardation brought on by cousin

marriage, doctors say.

"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," he said.

The community of about 10,000 people, who shun outsiders and are taught to

avoid newspapers, television and the Internet, is home to the Fundamentalist

Church of Jesus Christ of Latter Day Saints (FLDS), a sect that broke from the

mainstream Mormon church 72 years ago over polygamy.

The group, who wear conservative 19th-century clothing, is led by Warren Jeffs,

who was arrested in August and charged as an accomplice to rape for using his

authority to order a 14-year-old girl against her wishes to marry and have sex

with her 19-year-old cousin.

Doctors in the area declined requests for interviews and families refuse to

talk to reporters. But former FLDS members, independent doctors and authorities

say the disorder appears to have struck at least 20 children in the past 15

years.

"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. Doctors worldwide had only studied about 10 cases just

a decade ago.

"Once you get people within in the same community marrying, then the chances

grow of having two people carrying the exact same mutation."

'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."