Posted on 06/11/2005 1:55:31 PM PDT by NYer
It doesn’t get much more peaceful than the simple life among the Amish in rural Ohio. They have no cars, no electricity, no televisions.
But their children have medical conditions so rare, doctors don’t have names for them yet, reports correspondent Vicki Mabrey.
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.
Their father, Bob Miller, says he realizes there is a crisis in the community, which is why he and two other fathers, Irwin Kuhns and Robert Hershberger, have agreed to break a strict Amish rule that forbids them to appear on camera. The three sat for an informal interview.
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.
Since then, more than a dozen other cases of Cohen’s have been discovered in Ohio Amish country.
“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 Irwin Kuhn.
But for so many years, the Amish have had no names for these disorders. It was simply a mystery why half the headstones in Amish cemeteries were headstones of children.
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.
Kuhn and Miller admit these conditions have gotten more widespread in recent years. So much so that concerned families pulled together, held an auction and raised enough to build a clinic within buggy range of all the Amish. They also hired a pediatrician and researcher named Dr. Heng Wang to start caring for their children.
Kuhn’s daughter isn’t doing well at the moment, but now he can take her to the clinic every day, if needed, and the doctor has even made house calls at his home.
While 60 Minutes Wednesday was in Ohio, Dr. Wang 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.”
Certain homes, like the Miller’s, have taken small steps toward change. Some with lifesaving medical equipment have asked for special dispensation from the Amish bishop to install solar panels to run the machines.
Iva Byler, mother of the three girls with Cohen Syndrome, made an even more drastic change eight years ago, after her third child in a row showed signs of this crippling disorder.
The eldest, Betty Ann, is 24 and functions at a 9-month level. Irma is 21 and functions as a 5-year-old; Linda, at age 18, can’t even sit up.
“I knew as soon as I had the third one, I knew,” she 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.
Dr. Harold Cross, who’s from an Amish background himself, has heard that for more than 40 years, since he first discovered the high incidence of genetic problems in the Amish in the 1960s.
“Although we used state-of-the-art medical technology and genetics technology at the time,” he says, “we didn't know about the human genome. We weren't able to--to drill down and get to the specific molecular defects. So I always felt like we hadn't finished the job that we had started doing.”
He’s finishing the job now, learning by examining some of the children in Geauga County, Ohio, and teaming up with researchers in a London lab to find the actual genes that are causing the Amish disorders.
“What we're really trying to do eventually by pinning down the mutation is to find some kind of treatment,” he says. “If we can find out what went wrong, we might be able to correct it.”
They’ve already identified genes to several rare conditions, including this debilitating seizure disorder found in only 12 people worldwide, all Amish children.
There are no cures in sight yet, but these doctors are able to offer the next best thing: pre-marital testing, to help future parents avoid these tragedies.
It’s a powerful new tool for the Amish, if they choose to use it.
Despite the illnesses in his family, Miller would not use such tests. “That's our-- our lifestyle is that way. We-- we trust God to take care of that, you know? We just, just the way we—we - live.”
Joyce Brubacker, who comes from the slightly less-orthodox Mennonite faith, says at minimum the Amish and Mennonites should be testing their children as soon as they’re born. That’s what saved her daughter Shayla’s life. After her first child, Monte, died of an unusual-sounding genetic condition called Maple Syrup Urine Disease, Joyce had Shayla tested, and she was positive.
With Maple Syrup Urine Disease, the body turns protein into poison, causing brain damage. Shayla was immediately put on a strict low-protein diet. Now, she’s 20 years old and healthy.
If she had not been tested, Shayla says, “I probably would have been in a coma or died at that point, or had brain swelling which would have took me very dramatic. I mean it would just-- boom, boom, boom and I woulda been dead.”
Right now, the best prevention for many of these mutations is to prevent intermarriage, which is hard to do.
Marrying outside the faith could create a healthier gene pool, but it would also ultimately destroy the very essence of what it means to be Amish.
“I have a son that married a girl, they share the same great-great grandfather,” says Iva Byler. “And when he called me to tell me that he was gonna get married, I said, ‘Do you realize that you already stand a big chance to have a handicapped child since you have three siblings.’ And he says, ‘Yes, I know.’ He got married anyway.”
One of our catholic freepers attended an Indult Tridentine Mass in her community last year. She witnessed exactly the same thing. The freeper brought her children there to 'experience' the pre-Vatican II mass. They gasped when they witnessed this woman slap her infant across the face simply because he was crying. Amazingly, no one else even batted an eyelash. There are extremes in all religions.
Don't know if this is the story you're thinking of. Don't know that this one is traced to Portugal.
He is quite brilliant but quirky. He took up with a group whose faith is based on some odd notion. Such a waste of talent! He has always impressed me as lost and confused.
ping
You'll find this interesting.
Interesting quoate form the article, "She was as blue a woman as I ever saw."
'quoate'?
There are places in the US where the people have intermarried for more than 350 years. The same 30 families or so. Talk about needing "special" schools.
The Amish and Mennonites aren't the only communities with these problems. I can name at least two that are distinctly Catholic. And they aren't necessarily ultra-trads, either.
I was unaware the Dunkards still existed. The only reference I knew about them was their church along Antietam Creek in Maryland, which was a landmark during the Battle of Sharpsburg in 1862.
Thanks for the indirect update! :-)
Tay-Sachs is prevalent among certain communities that are descended from Eastern European (Ashkenazim) Jews.
Though the Orthodox are complex in certain ways.
The Satmar a very insular, but the Lubavitcher are very progressive in some ways.
I suppose both have problems with intermarriage, though to a much lesser degree.
After all, I don't think that you can convert into being a Mennonite or Amish, while Reformed and Conservative Jews can-theoretically-convert to Orthodoxy if they so desire.
Re: education: a very good point. It is true that they do not go to school past 8th grade. In PA where we live, however, you must have a minimum of a high school diploma to homeschool, and some Amish do homeschool, though most go to some kind of parochial school. This would be true of the "plainer" Mennonites as well. "Plainer" group such as the Eastern Mennonites and Horning Mennonites are very much against homeschooling but the more "liberal" ones do homeschool (mind you, these are still VERY conservative people! Except they do not by and large vote, though this is changing. Many Amish voted in the last election and voted for Bush.)
Many Amish use alternative medicinal treatments and seem to want to know about health issues so it surprises me they aren't more keen on this issue of inbreeding. But the point comes to this: what are you going to do when your daughter Anna Stolzfus meets her 3rd cousin Amos Stolzfus at a Sunday meeting and they fall in love? It has been done before, what is the big deal?
And actually, in most of people with defects that I personally know, the relationship between the married couples was not very close (not any cousins that they were aware of). My friends who are the 3rd/4th cousins have 6 healthy children although she did just have a miscarriage. (That can happen to anyone).
I just wonder if the genes of the original 1500 Anabaptist people (from whom I am also descended) just keep popping up genetically from time to time across the board with these people.
One of my friends has 10 children but she is VERY aware of the symptoms of MSUD (Maple Syrup Urine Disease) and has looked for signs and symptoms with each child. If you catch it within the first 24 hours, the child will not end up a brain-damaged vegetable. My one friend has 3 cousins in one family with MSUD. Imagine having three vegetable children to raise. THREE.
The real problme is that thes "quaint" people are in violation of the commandments of God by turning their backs on society and creating a world unto themselves, not bringing in new blood, literally and spiritually. They reap the consequences in their bodies.
Any isolated genetic group (for whatever reason) is going to have these problems cropping up.
In the old days (like the "Blue People") it was physical isolation. These days, it's most likely a cultish sort of isolation.
It may wind up being a Delayed Shaker Effect. The effect was pretty immediate with the Shakers, since they didn't believe in marriage or intercourse and practiced complete separation of the sexes. Since none of these children with the serious genetic problems are going to reproduce, it will take a little longer but the effect will be the same. Eventually the offspring will be unable to replace themselves. I think there's ONE Shaker left at Sabbathday Lake, Maine (a late convert).
This is true. When the Amish youth go out and party, they REALLY go out and party. It is called "wilding" (sound familiar?).
You are fine as long as (and until) you actually "join" the church. They assume you don't know better until then. After you join, they literally have your soul.
AND the problem lies in this: whom will you marry?Most Amish are not going to attract normal gals. They are usually nice but ignorant, smelly and weird socially. They will usually join the church so that they can get a spouse through the Amish community (albeit a cousin).
Their doctors and midwives need to educate them more on cause and effect.
Betcha their dynamite on the banjo!
It's that last phrase that's the kicker. No cousins they were aware of. If you go back into the family tree, you'll probably find multiple cousin marriages further up the line, say 6-7 generations back. Most are unknown to the descendants. My great aunt married her fourth cousin, and they did not know this until after she started researching D.A.R. stuff. They are both descended from the same Revolutionary soldier. They never had children. Probably no connection, because the rest of the family tree is generously out-crossed.
Problem is, if you have multiple cousin marriages even if very far back, the effect is exponential, not just simply cumulative. What you have is not just "double cousins" (where a couple are cousins through two different branches of the family tree) but triple and quadruple cousins. If there are only 1500 ancestors (not all of whom successfully reproduced) back in the 1600s, by now you have people who are, say, not just sixth or seventh cousins but five or six times over. In the genealogy trade, this is known as "pedigree collapse". Or, in more humble jargon, "the family tree don't fork."
We've seen this in cats and in dogs, when a popular bloodline starts cropping up all across the pedigree eight or ten generations later. Even if you don't have any in- or linebreeding in the five-generation pedigree, if there's multiple use of a single bloodline the defects will start to crop up.
they can sure cook, though...
You are absolutely right. It seems that the pedigree is collapsing pretty hard in recent generations because it seems I hear of health problems if one sort or another in almost every Amish or Mennonite family (if not THAT family, a cousin's family).
What I always tell my kids is, say hi to to the Amish because they are your cousins. Now, my it was my great-great grandmother who was the last Amish, but there have been Mennonites and Dunkard Brethren going back to the 18th century and I have traced our family tree back to the 1500's in Germany through one line. I tell my kids that we are cousins because if we go back that many generations we ARE related, we all have some common ancestor who came to Pennsylvania from Germany and Switzerland and intermarried for many generations. My mother's family eventually settled in Goshen, Indiana, another large Amish enclave but they all come from the original stock that settled in Lancaster and Berks counties in the 18th century.
Genetics is indeed a fascinating subject...
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