COLORADO CITY, Arizona - 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.
A sign marks the town of Hildale, Utah, home to the nation's
largest polygamist community - the Fundamentalist Church of Jesus Christ
of Latter Day Saints (FLDS), a polygamist sect that broke from the
mainstream Mormon church 72 years ago, in this photo taken May 31, 2007.
"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
"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
"Once you get people within in the same community marrying, then the chances
grow of having two people carrying the exact same mutation."