Saturday, September 09, 2006

Inbreeding and polygamy

Inbreeding amongst polygamists near the Utah-Arizona border has lead to a massive increase in cases of children suffering from the extremely rare genetic disease fumarase deficiency.

The Phoenix New Times reports that reseachers discovered in the late 1990s that fumarase deficiency occurred in the greatest concentration in the world among the Fundamentalist Mormon polygamists of northern Arizona and southern Utah, and that because of inbreeding, the recessive gene responsible was rapidly spreading to the entire community.

About half of the secretive polygamist community which settled in the desert plateau near the Vermillion Cliffs in the 1930s are blood relatives of two of the founding families. With a population of about 8,000 people, there are thousands at risk of passing on the genetic disease to their offspring.

The Phoenix New Times says:

Religious leaders control all marriages in the community, and many of these relatives have married or likely will marry in the future. Some of these marriages will include parents who both are carriers of the fumarase deficiency gene, making it certain that more children will be afflicted with the disease.

"We have and will have a continual output of children with this condition," [Dr. Theodore] Tarby says.

In this isolated religious society north of the Grand Canyon, few secrets have been more closely guarded than the presence of fumarase deficiency. Fundamentalist Church of Jesus Christ of Latter-day Saints elders, who control the community, have labored to keep the public from finding out why the disorder is manifesting. Many members of the fundamentalist community don't even know it's occurring.

The state of Arizona is contributing to the secrecy. The state Department of Health Services and the Department of Economic Security have been quietly providing services to assist the children and families of fumarase victims for more than 15 years. Both DHS and DES officials refused repeated requests from New Times to document the type and cost of services the state is providing to treat fumarase deficiency. The agencies claim that federal health laws prohibit them from releasing records or allowing their authorities to comment on the situation.

The effects of the disease are terrible. There is no cure, and no way to treat the disease, merely to try to fix some of the symptoms. Victims suffer from a range of ailments, including physical deformities, failure to grow normally, lack of muscle control and inability to walk or even to sit upright, epileptic fits, severe speech impediments and brain damage. Victims essentially require full-time attention just to survive.

The leaders must also understand the ethical considerations of continuing behavior, [Dr. Kirk A. Aleck] says, that is bringing children into the world who suffer tragic deformities.

"They have the authoritarian structure necessary to keep this from happening, but I don't think they have the advanced thinking," Aleck says.

"Advanced thinking"? It is hardly rocket science to realise that if you love your children and want what is best for them, you will do whatever you can to stop them from being born with a horrible genetic disease that condemns them to a short, painful life. Perhaps what Dr. Aleck was thinking was, these Fundamentalists have the authoritarian structure necessary to keep it from happening, but not the common human decency.

Which, of course, is far from common.

Not surprisingly, where you have suffering, you have people using "divine revelation" to decide matters:

The ultimate decision on marriages rests with FLDS Prophet Warren Jeffs. And Jeffs so far has shown no indication that he is concerned about the increasing prevalence of fumarase deficiency children in the community, former FLDS member Isaac Wyler says.

Even if a genetic screening test were available, Wyler says, Jeffs would have to be cautious about how he allowed it to be implemented. If the FLDS faithful believed that Jeffs was relying on science to determine marriages rather than divine revelation from God, he could lose control of the church.

"Warren has to be really careful that he doesn't lose his position as a god to these people," Wyler says.


Warren Jeffs, like Joseph Smith before him, has emphasized the importance of obedience among members of the church. Jeffs is following a long-established practice -- started by Smith 170 years ago -- of excommunicating those who do not strictly adhere to church leaders' commands.

"The 'gene' that Warren is really selecting for," Wyler says, "is the 'obedience gene.'

"Joseph Smith was also selecting for the 'obedience gene.' He was kicking people out, too, who weren't obedient.

"I hate to talk like this about my own genealogy," Wyler says, "but, literally, they are keeping all the breeding stock -- the women, the [strictly faithful] men -- and weeding out the disobedient men."

The ultimate goal of the breeding program, Wyler says, is to create the perfect race.

This is what happens when you allow folk-stories of "blood" to determine your actions. We don't see farmers breeding sicker and sicker cattle or sheep, convinced that if only they keep "purifying the bloodline" they will magically become perfect. No, for that sort of stupidity, you need folklore, religion and superstition.

(Note: Warren Jeffs, one of the FBI's ten most wanted, was recently arrested after a routine traffic stop.)

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