Wednesday, July 30, 2014

Founder Effect among the Amish

"The Amish make up only about 10 percent of the population in Geagua County in Ohio, but they're half of the special needs cases. Three of the five Miller children, for example, have a mysterious crippling disease that has no name and no known cure.

...The three Byler sisters were all born with a condition that has no cure and mysteriously leads to severe mental retardation and a host of physical problems. Last year, doctors figured out the girls have the gene for something called Cohen Syndrome; there are only 100 known cases worldwide. 

..."Nobody knew it was around here and we found, what, 20 to 30 cases in this area now that they didn't realize. Nobody knew about it," says Erwin Kuhns.

...The genetic problems come down to something called the "founder effect" because the nearly 150,000 Amish in America can trace their roots back to a few hundred German-Swiss settlers who brought the Amish and Mennonite faiths to the United States in the 18th century. Over generations of intermarriage, rare genetic flaws have shown up, flaws which most of us carry within our genetic makeup but which don't show up unless we marry someone else with the same rare genetic markers.

...While 60 Minutes Wednesday was in Ohio, Dr. Wang [a pediatrician hired by the community] made a house call to check on the Miller children. Bobby Junior, the sickest, can't tell Wang what's bothering him because he can't even talk.

And the doctor was treating these challenging cases under the most rudimentary conditions since Amish custom prohibits electricity. Still, he doesn't complain. In fact, he calls the heritage beautiful and says, "We are not come here to change them."

..."I knew as soon as I had the third one, I knew," [the mother of the Byler sisters] says. "They kept telling me, 'No, she's OK.' No, she wasn't. I could hear by her cry that she was gonna be like the others. Their cry is different. You can tell. After you've lived with it that long, you know."

Now, when she needs to go to the doctor, she wheels the girls into her van. She's left buggy rides, and the whole Amish lifestyle, behind. But the price was being shunned forever by the community, as well as her ex-husband and her two healthy adult children.

Irma's now tuned in to the 20th century, and Iva's plugged into the 21st. Using a genealogy Web site, she's figured out she and her ex-husband were distantly related, something that appears to be common among the Amish.

"I don't think the Amish really understand that it's a genetic disorder that causes the handicapping condition," Byler says.

The Amish think it is God's will; "Gottes Wille" is how they describe it."

-CBS News

25 comments:

YoungHegelian said...

It's impossible for the average American, as ethnically mixed as we are, to understand that for many of the world's communities, marrying in the community may not be out & out incest, but genetically, it's pretty damn close. For example, everyone in Corsica are cousins of some distance or another.

I've thought for a long time that someone should write a history of the American nation from a socio-biological viewpoint, and investigate if much of the 19th & 20th century strength of character came from genetic mongrelization masking over a lot of bad recessive characteristics.

Synova said...

Understanding that it's a genetic condition is not incompatible with accepting it as God's will.

Michael Haz said...

I hope Fr. Martin weighs in regarding the concept of Gid's will.

I just don't see God willing that some people are diseased, or poor, or successful, or attractive, or whatever. I don't think He chooses winners and losers like that.

I can understand acceptance of a condition or circumstance by calling it God's will. There's som peace and relief in doing that. But is it accurate, theology wise?

Beloved Commenter AReasonableMan said...

Michael Haz said...
I hope Fr. Martin weighs in regarding the concept of Gid's will.


From Protestant stock I married two Catholics (sequentially). My kids are fine. Intermarriage is a good thing - hybrid vigor.

deborah said...

That sounds reasonable, YH. And ARM took the words right out of my mouth, hybrid vigor.

I think war and conquest serves the same purpose. The conquerors eventually become absorbed into their acquisitions.

Along the same lines, the military, even in peace time, and kids intermixing at college serve the same purpose.

Synova said...

I know we've got actual theologians around...

My feeling about "God's Will" is that it's His will that the world contain evil, sickness and death, because the alternative is a world without freedom or choice. Could we even have life if we didn't have death?

But picking and choosing individuals? No.

Why would a just God allow a little child to be born with a painful disease? Because all of it is important to being human, even the bad parts.

XRay said...

Somehow I got here months ago:

hbdchick.wordpress.com/

Human Biodiversity.

It appears to be a highly controversial area of discussion.

A huge premise based on cousin marriage, or lack and movement therefrom. Intensely interesting.

XRay said...

As for the religious angle, well, would it be wrong to say that God creates, and then simply leaves his creations to themselves. Rise or fall on your own. Freewill?

Titus said...

I did an x-amish once who was all about experiencing the world. He was boring and his dick was so white and no shaving. The pubes were overwhelming the hog and it was like hard to find through the brush. I was like no.

tits.

Bleach Drinkers Curing Coronavirus Together said...

Have claims of this effect in Muslim communities marrying their cousins at such a high rate also been corroborated?

Synova said...

XRay... except that "leaves them to themselves" isn't right either, because God gives us strength and help through adversity and hope in Jesus Christ.

deborah said...

Excellent link, Xray. I'd heard of parallel vs. cross cousin marriages before, the latter {a male marrying his mother's brother's daughter) being the lesser of two evils because his patriarchal lineage isn't reinforced, but what I do not get is how all this close breeding doesn't lead to genetic problems...or does it? Perhaps endemic paranoia and hostility. Physically, they seem a healthy lot.



deborah said...

YH, more along the lines you were saying.

rcommal said...

Man, oh day, what part of God decides do you not get? What we see or do not see has naught to do with it. What part of *that* do you also not get?

Just askin'.

rcommal said...

Holy shit.

Can it actually be true that people, nowadays, only use religion to bolster their own beliefs as to how their lives ought to go, politically- and administratively- and [above all] rhetorically-speaking?

This is a good question that ought be asked by people of themselves before it gets asked by people of others.

rcommal said...

Does God decide, or does God not.

Are God's faithful accountable or are they not.

Is there a way to to be adamantly saved on account of a path.

How can it be that God-in-a-box is not an indictment of believers.

etc.

etc.

etc.

rcommal said...

If God is all-powerful, why couldn't God pick and choose individuals? Of course, God could--and not only that, God could also choose not to. God's choice, either way.

If one believes that God is all-powerful, then I can't see how (much less why) one can manage to attenuate that--

--other than to betray the very principle.

rcommal said...

Also, it's clear that Biblical literalism does not reign here, in this discussion (if that weren't so, how could a discussion of Adam and Eve, not to mention the re-population of the earth post-Noah, not take place, in context).

I find that invigorating, and I am grateful for it.

Eric the Fruit Bat said...

"Why, then, ’tis none to you, for there is nothing either good or bad, but thinking makes it so. To me it is a prison."

-- Hamlet, Act II, Scene II

Mitch H. said...

The numbers in that article are wildly inaccurate. There are closer to 250k Amish in North America, not under 150k, and there were two Amish immigration waves, one of about 500 and the other in the Nineteenth Century of about 3000. Since the Puritan and Quaker immigration waves were only on the order of 40k or so, we're not talking about unusually small founding populations.

The Amish demographic problem has more to do with their shunning practices, which tends over time to fragment their communities into mutually unsociable shards. For instance, Kishacoquillas Valley over in Mifflin County has a dozen mutually noncommunicative sects in a fairly small valley bottom (~30 miles long by 4 miles wide).

Michael Haz said...

There have been several articles published describing the high level of birth defects among Muslims because of their intermarriage with relatives. Some of the defects are physical in nature, while others include mental illness and reduced IQ levels.

The high level of birth defects was observed by physicians in UK hospitals. It occurs most in the Afghani population due to a cultural bias toward marrying cousins, or even closer relatives in that culture.

The birth observed defects include psychoses, something hard to ignore when the behavior of jihadis is observed.

deborah said...

Fascinating, Mitch, thanks. None of them seem concerned about the sin of pride.

Haz, I will def look into that.

Sydney said...

There's a pediatrician in Pennsylvania who has done amazing work with genetic disorders among the Amish. He even has his own gene sequencer, bought and paid for with support from the Amish community he serves. See more here.

Beloved Commenter AReasonableMan said...

The Amish demographic problem has more to do with their shunning practices, which tends over time to fragment their communities into mutually unsociable shards. For instance, Kishacoquillas Valley over in Mifflin County has a dozen mutually noncommunicative sects in a fairly small valley bottom (~30 miles long by 4 miles wide).

This is both sad and hilarious, a bit like New Guinea highlands tribesmen.

deborah said...

Thanks, sydney :)