Polygamist community faces rare genetic disorder


sanctus
#1




By Jason Szep
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."
Reuters 2006.
 
karrie
#2
Those poor people. A jaded little part of me has a comment on the tip of its tongue about the efficiency of natural selection at weeding out the crazies, but, more rationally I know all of these people were born into and raised with the influences of this sect, making them think it's okay. Poor poor people.
 
Johnnny
#3
most not all of the worlds genetic dsorders are from inbreeding, for example the azshkanti jews in europe inbred with each other back in the day to keep there wealth jewish and as a result had a higher rate of cystic fibrosis and tay sachs.
 
Johnnny
#4
This is an example of the FOunder effect and another reason to support immigration cause we need those newer genes
 
Pangloss
#5
I don't feel the least bit sorry for these self-selecting fools. They have chosen a way of life that will indeed weed themselves out of the gene pool - once they're extinct, the issue is gone. Natural selection wins over empty-headed, man-made spiritualism again.

I do honestly believe it is a form of child abuse to indoctrinate children with religious fantasies (Really - eternal torture after death? How truly horrific!), but once those kids grow to reproduction age, they are responsible for their actions and their beliefs. Each generation bears its own responsibility.

Another example of religion being the cause of stupid and hurtful behaviour.

Pangloss
 
Johnnny
#6
the local menanites from esponala will pay you cash to knock up there wives, they said my genes would make a perfect addition to there community, wtf!!?? lol
 
Dreadful Nonsense
#7
Quote: Originally Posted by PanglossView Post

I don't feel the least bit sorry for these self-selecting fools. They have chosen a way of life that will indeed weed themselves out of the gene pool - once they're extinct, the issue is gone. Natural selection wins over empty-headed, man-made spiritualism again.

I do honestly believe it is a form of child abuse to indoctrinate children with religious fantasies (Really - eternal torture after death? How truly horrific!), but once those kids grow to reproduction age, they are responsible for their actions and their beliefs. Each generation bears its own responsibility.

Another example of religion being the cause of stupid and hurtful behaviour.

Pangloss

As Karrie pointed out they were born into it, hindered from outside influence and basically brainwashed ....so self selecting fools is a lil harsh considering the facts.

Again once they reach the reproductive stage they are still brainwashed....It's not like the indoctrination and mind abuse suddenlt wears off with onset of puberty......
 
Dreadful Nonsense
#8
Quote: Originally Posted by JohnnnyView Post

the local menanites from esponala will pay you cash to knock up there wives, they said my genes would make a perfect addition to there community, wtf!!?? lol

what do the broads look like?
 
Zzarchov
#9
Actually, if you wanna get technical....


If they keep this up sooner or later the diseased branches of the family tree will whither and fall off, and leave a group who are actually healthier than the populace at large in most areas.

This is how animal husbandry works.

That would require them to be exceedingly cruel and callous to their "faulty" offspring, but that is not out of the question given their history of how they deal with "excess sons" in those families.
 
karrie
#10
Quote: Originally Posted by PanglossView Post

I don't feel the least bit sorry for these self-selecting fools. They have chosen a way of life that will indeed weed themselves out of the gene pool - once they're extinct, the issue is gone.

You don't know much about cultural imprinting if that's what you truly think pangloss. These 'fools' haven't self selected anything. They were born into it, and have had it normalized around them ALL THEIR LIVES. Add to that the likely sheltering from outside media sources and education, and just HOW do you think they should know better?
 
karrie
#11
Quote: Originally Posted by JohnnnyView Post

the local menanites from esponala will pay you cash to knock up there wives, they said my genes would make a perfect addition to there community, wtf!!?? lol



I think you're mixing up menonite and hutterite. Menonites are a widespread religion, and are generally not subject to the small breeding pool that hutterite colonies are. Your story is one that almost EVERY young man tells about hutterite colonies, because their buddies before them told it, and they don't want to be the only ones who weren't appropriate genetic material.
 
Twila
#12
Quote:

Originally Posted by Pangloss
I don't feel the least bit sorry for these self-selecting fools. They have chosen a way of life that will indeed weed themselves out of the gene pool - once they're extinct, the issue is gone.


Quote:

You don't know much about cultural imprinting if that's what you truly think pangloss. These 'fools' haven't self selected anything. They were born into it, and have had it normalized around them ALL THEIR LIVES. Add to that the likely sheltering from outside media sources and education, and just HOW do you think they should know better?

So where does the responsbility lie? Because it has to lie with someone. It's not like it was 50 years go with access to knowledge being the hinderance.
 
Pangloss
#13
Quote: Originally Posted by karrieView Post

You don't know much about cultural imprinting if that's what you truly think pangloss. These 'fools' haven't self selected anything. They were born into it, and have had it normalized around them ALL THEIR LIVES. Add to that the likely sheltering from outside media sources and education, and just HOW do you think they should know better?

So one's history is one's destiny? If I am beaten as a child I am not responsible for the children I beat when I become an adult?

Sure, cultural imprinting is a powerful force - so is introspection, reflection, curiosity and free will.

Saying the adults bear little or no responsibility for their present circumstances infantalizes them. I respect the strength and independance of the human mind too much to do that to anyone.

Of course there is the case of the person who gives up their free will and hands their autonomy over to a group.

In that case, if you're gonna be that stupid and lazy, you deserve to get what's coming to you.

Pangloss
 
karrie
#14
Quote: Originally Posted by TwilaView Post

[/i]


So where does the responsbility lie? Because it has to lie with someone. It's not like it was 50 years go with access to knowledge being the hinderance.

There's a reason that there are actual foundations started to try to get these people out, and that's because it IS like 50 years ago. They DON'T have access to the knowledge you and I have. The average person takes access to things like TV, newspapers, public libraries, internet, and education for granted. If you were raised thinking these things are normal, to not strive for mental stimulation, to not seek information about the outside world, then the chances of you doing so would be minimal. That's what's happened to a LOT of these people.

You have to consider also that by the time they are old enough to learn these things on their own, it's too late sometimes. They've already fallen in love and married, or already had kids. Simply 'snapping out of it' isn't an option when it's what your whole life has been. What everything you've got is built on.

The 'responsibility' lies with the founders. The misfortune lies with their progeny. The chance for change lies with them as well thankfully, and with foundations like the one this article discussed, devoted to drawing them out.

But, condemning the brainwashed for having been brainwashed, well, you certainly wouldn't catch me doing it.
 
triedit
#15
Quote:

Arizona has about half the world's population of known fumarase deficiency patients

Finally something to come back with when someone tells a West Virginian joke.
 
eh1eh
#16
OK. This just has to be said.


Ba Da ding ding ding ding ding ding ding ding, Ba ding ding ding dong dong dong dong dong.
 
Pangloss
#17
What kind of brainwashing? Fundamentalist religion? What about materialism? What about the shallow narcissism of our times? When are we supposed to wake up?

Ok, I'll grant you that some cultures are more coercive than others. That still doesn't excuse handing over the autonomy of one's own mind.

Excusing weakness is encouraging weakness. That's immoral. Our entire culture needs to re-embrace personal responsibility and the autonomy of the individual - no more excuses that "my mom made me this way" or "my parents failed me so that's why I'm like this" at 55 years old. We are becoming ever more narcissistic, materialistic and childlike all at the same time, all because we are obsessed with our rights and freedoms and not our responsibilities and obligations.

Not the least of which is to be a grown up and take responsibility for our lives.

Ok, I know that was a way over the top rant, but I do mean what I write - even if it was too much for the point I am trying to make.

Pangloss
 
Dreadful Nonsense
#18
I agree if you were abused as a child it's no exuse to abuse kids as an adult. Get help.If you don't it still is no real exuse to do what was done to you...You know it's wrong you did not like it happening and you know if you do the same what it entails for that person.

This doesn't really seem the case here. We are talking about an entire group of people being brainwashed generation after generation with only a few escaping....It's very different than one on one for people are reinforcing this lyfestyle as normal...

what irked me in the posts was that for some reason these people are expected to just know whats right and wrong in their community as they come of age and grow up.....

A child raped knows as it grows up in society that other kids are not ahving this done to them.they know there is a difference.

These people are isolated and insulated from the real world.

It's not unlike certain cultures living in modern cities, going to school , working and all of a sudden wham!!! yer marrying Zia the guy you have been calling uncle since a kid.....and you do it. the girl is just doing what is expected of her. Brow beaten into an arranged marriage, accepting it...

I used Zia for i know a case and his name is Zia...
 
Summ3rDaze
#19
I love how the Darwin effect will get you every time.
 
Twila
#20
Another question: Do these people not have doctors?
 
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