This Amish girl suffers from a genetic disorder.
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.”
Don't the Muslims also inter marry? Definitely the straight path to obsolesence.
That's what I was thinking, but I didn't want to say it.
I see this, not to such an extent, in the Orthodox Jewish Community too.
Since Muslims cross ethnic backgrounds, I don't see it would be an issue. You are more likely to see problems with small and/or remote villages that "stick to their own kind" where you may see more distant cousin/distant relative marriage.
Some small-island Greeks have issues like this likely also caused by small gene pool.
I recall reading, many years ago, about a group of Americans who also suffered from some genetic disorder. It was traced to one village in Portugal.
I have Amish ancestry on my mother's side and live among the Amish,as well as Mennonites and Dunkard Brethren today in central Pennsylvania.
I have so much to say on this topic, I hope I can keep my head on straight. I intend to write a book about this someday: "Amish, Mennonites and a Dried Cultic Root" or something like that.
First of all, almost all the Mennonites we know out here are related to each other. Some are 2nd and 3rd cousins. This is pretty close considering the rest of the world's gene pool. A very close couple to us are 3rd cousins on one side and 4th cousins on the other side. Her maiden name was Martin and she married a Martin. She is now XXXX Martin Martin. Her mother was also a Martin who married a Martin.
I have also seen this with the Amish where every family in our area either has the surname of Fisher or Byler. Every single one of those families are related and all their children are close cousins.
In our children's school (another anabaptist school) the majority of familes are all cousins. So far I have not heard of any intermarriage between the cousins but nothing would surprise me.
God never intended a Christian community to be a closed community. The heavy-handed tactics of the Amish and the more legalistic Mennonite leadership encourage people to remain in their communities, and marry within them, lest they literally "lose their salvation." Indeed, salvation is dependent upon exceedingly strict adherence to various codes of behavior and dress (I think dress is the most important, since Amish children drink and party, and often get pregnant. But they better LOOK right!)
Because of these tactics (which are very cultic if you look at what constitutes cultic actions of leadership), the Amish continue to remain Amish. As a result, few if any every join their groups from outside the original gene pool of a couple hundred years ago when they emigrated here. They are not just closely related; these people are more like siblings to each other. There is no strength in the gene pool and they are LOADED with deformities.
I see the deformed Amish and Mennos all the time. One poor Amish soul I have seen for years is highly cripped but manages to slide himself around; one poor Mennonite teenager had a cleft lip that his parents never sewed shut; he used to work at our local store. Another Mennonite boy has several fingers missing. A Horning Mennonite family we are close to just had a boy born with his feet on backwards. Another Mennonite family has a baby girl who was born blind and retarded. I am friends with a former Beachy Amish woman whose cousins are Maple Syrup Urine diseased. It runs in her family. And on and on. As diseased physically as they are spiritually; it is the mirror of their condition.
This is the result or turning away from the call of God to reach the world with the gospel. The Amish and Mennonites claim to be followers of Jesus Christ, yet the majority are insular, non-evangelistic, and incestuous. This is NOT the way it was meant to be.
If it were not for their incestuous marriages and cultic leadership, they would have died out along ago, assimilated into the rest of Christianity by bringing lost souls from all nations into their fold.
Moreover, to strain out a gnat and swallow a camel is a way of life. The Amish will pay to drive in people's cars or vans but they cannot own their own. Yet, some groups will allow tractors, as long as the rubber is off the tires. Horning Mennos who must drive black cars ONLY (to be "plain") drive around in shiny, beautiful, late-model Ford Mustangs, Acuras, Saturns. I always remark to my dear Mennonite brethren when I see their cars like this: "Oh, was a beautiful FANCY car you have!" It about stops them dead.
I could go on but then it will be my book. Someday.....
I read an article in National Geographic a few years ago about Iraq. It said that the amount of inbreeding is pretty high throughout the Muslim world. Between substandard medine and a general lack of cultural empathy, one can only imagine the sad lives of those with congenital problems.
***I recall reading, many years ago, about a group of Americans who also suffered from some genetic disorder. It was traced to one village in Portugal.***
I remember that. I think 60 Minutes did a show on it.
Thanks for encouragement. I would need to find the right publisher for this kind of book.
And most cases of Huntingdon's Chorea can be traced to a single individual from Venezuela.
I think a lot of people view isolated inbred populations as "quaint", and don't really consider all the consequences.
It's not so much the 3rd or 4th cousin marriages, as that in a population this small and isolated you have multiple layers of cousin marriages. The potential effect multiplies with every layer.
I used to breed and show Siamese cats, and I can tell you I was VERY careful with ANY linebreeding, and I NEVER inbred. The chances of something horrible happening are just too great.
Thanks. It might be an interesting summer project. It has been on my mind for years. I can speak from personal experience. My natural mother did not raise me but when I was in my 20's I met her and found out about my Amish/Mennonite heritage. Ironically I joined a very strict Mennonite community some years later and experienced first-hand the cultic nature of these groups, what happend when you first join, if you disagree, and when you leave. And moreover the abuse of children and women that I saw in the name of "submission." Tiny babies being spanked for crying. Babies left to wail because they had been fed "only a little while ago." Women told what to wear, who to be friends with, children harshy disciplined, sexual abuse covered over.
Want more?
Do you think some of the unwillingness to deal with the root problem (inbreeding) is due to a low level of education? I read that many Amish only attend school to the 8th grade, and don't consider education worth pursuing. Maybe if they understood more about DNA and genetics it would make a difference?
Any church that exercises principles of what used to be called "strict watch care" is going to be antithetical to modern American notions of freedom.
You would probably be horrified at the lot of women in a small rural Alabama Baptist congregation circa 1840 . . . my gg grandfather was a deacon in one. I've read all the minutes.
Welcome to the nineteenth century.
Intermarriage is common throughout Africa, the Middle East and Southeast Asia, although the rate of marriages between first cousins, second cousins and other relatives in the Persian Gulf region, estimated at more than 55 percent in Saudi Arabia, is considered high by world standards.
Pinar Ozand, a Turkish doctor who has led research into genetic errors at the King Faisal Specialist Hospital and Research Centre here, said problems that occur once in every thousand births in a robustly mixed gene pool are being found as frequently as once in every 50 births in Saudi Arabia.
What went wrong: inbreeding.
How to correct it: marry outside the family community.
I was reading about how the Amish from one area will trade young men, say a couple of dozen, with an Amish group from another area just to help solve this problem. Does that really happen?
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