"U.S. polygamist community faces rare genetic disorder"

Headlines tend to change on Yahoo news, but that's the actual headline of this moment, 9:29 AM, Thursday, on this story about fumarase deficiency striking children in Arizona and Utah.

Fumarase deficiency causes severe mental retardation.

But the first question I had was not about the disorder.  My first question was, "The US has a polygamist community?"

Of course I knew that all the polygamists in the country aren't on Big Love.  But there's a whole community?

Yep.  It comprises the twin border towns of Hildale, Utah and Colorado City, Arizona:

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.

Medical researchers say the high incidence of fumarese deficiency is due to inbreeding.

"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."

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.

The condition is degenerative and it's awful:

"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...

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.

And of course, once again, religion is getting in the way of tackling the problem:

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."

I really have nothing much to say about this, except that the next time some opponent of gay marriage tries to make the case that if we allow gays to marry then we can't stop polygamy and incest either I'm going to point out that the arguments against polygamy and incest aren't arbitrary moral arguments and then I'm going to bring up fumarase deficiency.

Comments

[joanr16]

[T]he arguments against polygamy and incest aren't arbitrary moral arguments....

There is also a legal argument, Lance, but it's a bit complicated for most homophobes to follow.

While churches may define marriage as a sacrament, the law can only view it as a contract. Certain types of contracts can have exceptional definitions, and still be valid and legal. For example, contracts involving the sale of real estate must be in writing. Marriage contracts already have a lower age requirement than any other type of contract; the contract could be further defined as between two persons only, and persons who don't fall within certain degrees of consanguinity.

Your point about fumarese deficiency is one of many social arguments that could be used to exclude polygamous and incestuous unions from the legal definition of the marriage contract. Restricting the marriage contract purely on the basis of gender, however, probably violates already-existing civil rights law.

[SDoyle]

Make sure you pronounce it right when you bring it up. Except for the quotes it's spelled wrong every time.

"fumarase", like many enzymes it ends in -ase

[Lance]

SDoyle,

Thanks for the edit. I fixed it.

[mac macgillicuddy]

I'm probably going to get flamed here as usual, but I'm also a glutton for punishment, so here goes:

I read this story too, and religion has nothing to do with it. An egomanical leader of a backwoods cult is standing in the way of addressing the issue. I'm not an expert in the Church of Jesus Christ of the Latter Day Saints, and I don't even know exactly what the faithful's belief in polygamy is, but I do know a few members of the church and none would condone, or even relate to (pardon the unintentional pun), the polygamous practice described here. And they certainly wouldn't defend the horrific outcome as "God's will."

[joanr16]

mac, perhaps you missed this?

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 official LDS Church, which banned polygamy in the late 19th Century, isn't mentioned or implied anywhere in Lance's post, although I can't speak for the original article as a whole.

And yes, it is a religious practice among the FLDS to take multiple wives, marry very young girls, and see that those girls have as many babies as possible. I recommend Jon Krakauer's book Under the Banner of Heaven, for proper context and scope.

[Linkmeister]

There was a solidly political reason for banning polygamy in the late 19th century, too; Utah was not going to be admitted to the Union unless its inhabitants renounced the practice.

Which may explain part of this sect's holdout: they're the same sort of people that refuses to recognize US or even State laws as ones which must be obeyed.

[mac macgillicuddy]

Joan,

I didn't miss it. That was my point: that this is a cult, started by two egomaniacs, and still led by an egomaniac more interested in a bizarre notion of personal power and control than faith--as cult leaders typically are; and it is NOT the Church of Jesus Christ of the Latter Day Saints. But thanks for making it more clear.

[J]

Of course I knew that all the polygamists in the country aren't on Big Love.

My understanding is that Juniper Creek is based on Colorado City.

Beyond that, I don't see why we should forbid people from marrying on the basis of eugenics and potential birth defects potential children might have. That seems fairly outrageous to me. If a brother and sister want to get married, and it's genuinely consensual, I think that's totally gross, but I don't see what business the state has getting involved. Certainly I don't see how the state's involvement ought to be premised on some supposed state interest in preventing inbred children.

Polygamy is different, I think, in that it is essentially an entirely different sort of social system, and I don't see why the state should have to endorse that, especially since it's a social system that tends pretty strongly towards the "horribly exploitative."

[ajay]

That was my point: that this is a cult, started by two egomaniacs, and still led by an egomaniac more interested in a bizarre notion of personal power and control than faith--as cult leaders typically are; and it is NOT the Church of Jesus Christ of the Latter Day Saints.

No, it's not the LDS. Lance never said it was. It's not the Baptists or the Shia Muslims or the Swedish Lutherans either. It's the FLDS. But it's still a religious issue, because the FLDS is a church, and it's their religious beliefs that are causing this problem.