Rare genetic disorder plagues polygamous towns

Colorado City, AZ – The streets of Colorado City, Arizona, are lined with especially large two- and three-story houses. But even the giant homes are dwarfed by the snow speckled cliffs of Zion National Park.

WYLER: That's the ugly side of Zions and that's pretty (laughter). So it's a beautiful place around here.

Isaac Wyler is accustomed to the view. He has lived here all of his life. Sitting in a park in the middle of town Wyler says the children with the disorder are virtually helpless. (Park amby under next graf)

WYLER: They can wiggle around a bit but they're like a large baby A child gets to be 8 years old and can't even sit up. It takes a couple people to get them in a bathtub and get em back out and clean em up. It's bigger than a one person job.

Wyler is related by marriage to some of the victims of the birth defect. Wyler and several others were recently kicked out of the community by leader Warren Jeffs. Jeffs is the head of the fundamentalist Mormon church, which dominates Colorado City and Hildale, Utah. He is a federal fugitive accused of facilitating child sexual abuse. Wyler and other former members say the fundamentalist Mormon group has forced girls as young as 13 to marry men decades older. Most of the community believes Jeffs to be a prophet.

WYLER: Obviously I don't believe he is or I wouldn't be talking to ya. Because if I was talking to you and I believed in him I would get cut off for this.

The group believes Jeffs to be in direct communication with God. And God tells them who should get married -- even if they're first cousins.

Child neurologist Theodore Tarby discovered the first case of the disorder when a Colorado City couple brought their son to him for seizures about ten years ago.

TARBY: I asked if there were any other children who were afflicted by this condition. The parents said no, they had a girl who has cerebral palsy.

As it turns out the daughter also had the condition along with several other children in the community. It's called fumarase deficiency and it's extremely rare. There are ONLY 40 cases in the world and half of those are children living here in Colorado City and Hildale.

TARBY: It's certainly the largest cluster in the world... and they're still making more of them The church teaches them their job is to produce children and they know from demonstrated fact, if nothing else, if they continue to procreate with their kin they will have children with this condition.

But Isaac Wyler says people believe the rare birth defect is God's doing.

WYLER: It would be what you call a test. Around here when people have adverse situations like that and they know God put em together because they believe that a prophet received a revelation to marry them then anything like that that would crop up would be considered a test to overcome and you just deal with it.

Former member and local historian Ben Bistline agrees with Wyler. Bistline lives on the outskirts of Colorado City in a worn down mobile home. He breathes with the help of an oxygen tank. (bring in oxygen sfx and room amby) His two nieces also have fumarase deficiency. But Bistline says people here aren't going to stop having children no matter what genetic disability they might have.

BISTLINE: In Mormon philosophy there's different degrees of glory in heaven and the top kingdom is the celestial kingdom in order to reach the highest degree of that celestial kingdom a man has to be sealed to at least three women three wives. From then on I guess they marry as many as they want because they're also commanded to multiply and have as many children as possible to increase the numbers in the society.

Bistline himself has sixteen children but just one wife. He withdrew himself from the society in the mid 80s. Bistline says the community started out with just four or five families and has multiplied in a short amount of time.

BISTLINE: I would say 3 years ago there was probably 10,000 people living in Colorado City Hilldale. That of course includes the children. The median age is 9 years old, you know. Then Warren started moving people out. I don't think there's 8,000 today so many moved out to different compounds that he has around.

Salt Lake City attorney Scott Berry has worked for the community for almost twenty years, and although he is not a member, he has gotten to know the group very well and supports their lifestyle. Berry disagrees with Dr. Tarby's findings saying it's just one man's opinion.

BERRY: They have this divine revelation view of marriage. I could see how there's a potential problem but I believe these people are sensible generally logical people and if that information is true and commonly known that somehow that information would be reflected in these decisions.

But Doctor Tarby has tried to educate the community, and he says their decisions haven't changed. He recently answered questions about the disorder at a town hall meeting.

TARBY: I have told them there are ways known to prevent the birth of children with the condition. But I also knew the techniques that would have to be used would be things they wouldn't do.

things such as genetic testing, prenatal testing and abortion. The group isn't accustomed to trusting outsiders. Berry says the group has talked to outsiders in the past but they have been burned too many times by the media.

BERRY: They've never believed they've gotten a fair break in any sort of press coverage so they ve just decided to avoid it.

While the community has avoided most outsiders, Tarby says they haven't shied away from outside government assistance. He says much of the fumarase deficiency costs are paid for by state or federal health care programs.

TARBY: The community has a policy an acknowledged policy, which is known as bleed the beast'. They use all efforts that they can to get services from any state, federal, or municipal community to pay for their expenses.

That means tax dollars are paying for many public services there. And Tarby expects to see more and more people from Colorado City and Hildale using taxpayers' money to get care for their children with fumarase deficiency.

For Arizona Public Radio I'm Laurel Druley.