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Author Topic: Amish People Inbred With Genetic Ailments
Morrigan
Happy Holly Days


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This is from the book "Weird Pennsylvania on page 65.

"....Over hundreds of years, this inbreeding leads to all kinds of genetic ailments, some of which cripple or kill them in slow and painful ways. One tragic condition that occurs is commonly called maple syrup disease because of the sweet smell of the urine and earwax of its suffers. A child with this condition reacts very powerfully to normal childhood illnesses, like cols and ear infections. Temperatures spike very high, which causes seizures and brain damage. Almost invariably, a series of minor chidhood ailments will lead to severe brain damage, spasticity, and ultimately death.

Another ailment that's rife is a liver defect that causes a chronic jaundice that usually kills sufferers before they reach their mid-teens. It's called Crigler-Nagar syndrome, and it can only be held at bay by ultraviolet light treatments for up to 14 hours per day. Many women suffer from a deformed chromosome that leads to a condition called chicken breast disease. The deformed gene hardens and expands the muscle fibers in the chest, making the sufferers barrel-chested."

Any ideas?

Morrigan

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Rhiandmoi
Ding Dong! Merrily on High Definition TV


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Article on Amish and Crigler Najjir syndrome

Chicken Breast Syndrome = Amish Nemaline Myopathy
http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=605355

Maple Syrup Disease= Branched-Chain Ketoacid Dehydrogenase
http://rarediseases.about.com/od/rarediseases1/a/062004.htm


http://www.clinicforspecialchildren.org/labmolgen.html

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Izzy Quigley
Jingle Bell Hock


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When I took Biology 101, the textbook mentioned the Amish of Pennsylvania as an example of a genetic bottleneck (I think). It said that this Amish group has a higher than usual rate of dwarfism and polydactylism as a result of inbreeding. There was nothing about the disorders mentioned in this thread, though.

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Auntie Witch
It Came Upon a Midnight Clearance


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I don't know about that, but the community I live near has some pretty high stats. I don't remember exactly what they are, but my Director of SPED said once that their rate of Down's alone was three to four times higher than the rest of the community. There were several other disorders that by law, we had to offer SPED services for, and they declined them. All the numbers she cited me were shockingly high for such a small community.

[hijack] One Amish woman that my grandma befriended had a sister move to Pennsylvania to marry a man out there, because there weren't any her age left here that she wasn't directly related to.

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happyholidaysfrog
Jingle Bell Hock


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I'm pretty lucky as I am inbred myself, also due to a small religious community, but not the Amish but rather Old German Baptist. My parents are fourth cousins, but they didn't know until after they were married. Both sets of my grandparents were raised OGB, and my mothers and fathers famlies knew each other before they married, my father at four years old saw my mother when she was a newborn.

There were three marriages between the two sides of my family, I've always thought that was odd, My fathers sister married my mothers brother and had three sons (my "quasi-brothers") and my mothers uncle married my fathers sister.

My family tree doesn't branch so much as it wreaths.

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Magdalene
Happy Holly Days


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quote:
Originally posted by happyholidaysfrog:
There were three marriages between the two sides of my family, I've always thought that was odd, My fathers sister married my mothers brother and had three sons (my "quasi-brothers")

Double first-cousins, actually. *grin*

Magdalene

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Lainie
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quote:
Originally posted by happyholidaysfrog:
There were three marriages between the two sides of my family, I've always thought that was odd, My fathers sister married my mothers brother and had three sons (my "quasi-brothers")

One of the Anne of Green Gables books refers to such a relationship as "double cousins." The fact that there was a term for it makes me think it may not have been that uncommon.

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Spamamander in a pear tree
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It would seem unusual for Down syndrome to have a higher prevalence in a community unless there was a large number of women over the age of 40 giving birth, and even then it increasing exponentially like that seems very odd. The only way I could see it happening is if there were a large number of persons with the mosaic form of trisomy 21 who were having children. The mosaic type is unusual in that the extra chromosome only appears in some of the cells, so it may be completely unknown that they are a carrier. Most of the men with it would likely be infertile (as pretty much all males with Down syndrome are) but nature played an interesting little prank in making no adverse effects on most women with Down syndrome's fertility.

I'm rambling and I'm certainly not a scientist or geneticist, I would just be really fascinated if a study was made in that community regarding karyotypes to see how that might occur.

I can definitely see how a closed community like the Amish might well develop a series of recurring genetic anomalies because there would be closer blood relationships in most families and very little new genetic input. The royal families of Europe had a few traits that popped regularly because of the close intermarrying.

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Auntie Witch
It Came Upon a Midnight Clearance


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quote:
Originally posted by Spamamander in a pear tree:
It would seem unusual for Down syndrome to have a higher prevalence in a community unless there was a large number of women over the age of 40 giving birth, and even then it increasing exponentially like that seems very odd. The only way I could see it happening is if there were a large number of persons with the mosaic form of trisomy 21 who were having children. The mosaic type is unusual in that the extra chromosome only appears in some of the cells, so it may be completely unknown that they are a carrier. Most of the men with it would likely be infertile (as pretty much all males with Down syndrome are) but nature played an interesting little prank in making no adverse effects on most women with Down syndrome's fertility.

I'd say it's a combination of inbreeding and the fact that the Amish women tend to have babies as often and as long as they can.

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Magdalene
Happy Holly Days


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quote:
Originally posted by Lainie:
quote:
Originally posted by happyholidaysfrog:
There were three marriages between the two sides of my family, I've always thought that was odd, My fathers sister married my mothers brother and had three sons (my "quasi-brothers")

One of the Anne of Green Gables books refers to such a relationship as "double cousins." The fact that there was a term for it makes me think it may not have been that uncommon.
If you were a "Little House On The Prairie" freak at any point....then here's trivia for you. Laura Ingalls Wilder had *two* sets of double-first cousins: One of Charles Ingalls brothers married one of Caroline Ingalls sisters, and one of Caroline Ingalls brothers married one of Charles Ingalls sisters.

The books always spoke of Charles' 'wanderlust' and wanting to be 'away from people'. After finding out that, I couldn't help but wonder if he really wanted to get his daughters a little further away from their male cousins-probably-gentically-closer-to-being-brothers. [Big Grin] [Big Grin] [Big Grin]

ETA a question: In many states, it is legal to marry your first cousin....but what in the event of double-first cousins? Do the states look at things like that before issuing out marriage licenses, or not?

Magdalene

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Setzer
I'll Be Home for After Christmas Sales


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quote:
Originally posted by happyholidaysfrog:
I'm pretty lucky as I am inbred myself, also due to a small religious community, but not the Amish but rather Old German Baptist. My parents are fourth cousins...

Someone correct me if I'm wrong, but doesn't legal relations only extend to 3rd cousin? While being related in any sense would discourage most (Supression of memories nonwithstanding), this would be legal, and you not "inbred".

P.S. What states permit 1st cousins? I know of old laws where exocutioners were allowed to marry that closely (European law from hundreds of years ago), but nothing that close for "regular" families, much less modern ones.

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Esprise Me
We Wish You a Merry Giftmas


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As of April, 2002:
quote:
Twenty-four states prohibit marriages between first cousins, and another seven permit them only under special circumstances. Utah, for example, permits first cousins to marry only provided both spouses are over age 65, or at least 55 with evidence of sterility. North Carolina permits first cousins to marry unless they are "double first cousins" (cousins through more than one line). Maine permits first cousins to marry only upon presentation of a certificate of genetic counseling. The remaining nineteen states and the District of Columbia permit first-cousin marriages without restriction.
Source

Cecil's take on cousin marriage (with some legal info)

It's legal where I live.

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Pondicherry Pi
Deck the Malls


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quote:
Originally posted by Izzy Quigley:
When I took Biology 101, the textbook mentioned the Amish of Pennsylvania as an example of a genetic bottleneck (I think). It said that this Amish group has a higher than usual rate of dwarfism and polydactylism as a result of inbreeding. There was nothing about the disorders mentioned in this thread, though.

I do believe we might have had the same biology textbook. [Wink]

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Izzy Quigley
Jingle Bell Hock


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Maybe so. Was yours light blue with a bald eagle on the front?

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Buzzkiller
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How were laws about marrying blood relatives enforced back when less documentation was required to obtain a marriage license?

My husband and I recently found out that the parents of his 80-year-old father were first cousins. His dad mentioned it in such an off-handed way, we almost missed it. He failed to see why we found this information so...interesting. His family is all from Missouri, so I am assuming they married in Missouri in the 1920s or perhaps the 1910s. I'm wondering what sort of documentation was required for a marriage license that long ago. When my parents married in Missouri in the 1950s, I'm pretty sure they didn't have to show birth certificates. The reason I can say this with relative certainty is that I remember when my mother saw her birth certificate for the first time, and it was in the late 1980s. (She had to obtain an official copy--for insurance purposes, I think--and it was a big deal because she found out that her legal name wasn't what she thought it was.)

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Elwood
Little Sales Drummer Boy


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My brother is married to Mooommmeee's sister, so we refer to our kids as double-cousins of there, as opposed to simply cousins of my sister's kids. I'm told the double-cousins are extremely close genetically, more so than siblings, which is fascinating if true.

Its the inter-generational relationships that always confuse me. What is my relationship to my father's cousin? And his sons? We call the father's cousin my second cousin, but what does that make the son?

Once marriage is involved, I lose track pretty fast. We have a genealogist in the family that prints out the entire family tree on my paternal Grandmother's side literally from the Mayflower to the present every year. It is as complete as could reasonably be expected and displayed every year at the family reunion. Now THAT is a massive document. Printer with maybe 8-point font on a large-format plotter, it still goes on for around 75 feet. Since even the reunion is working from my grandmother's parents on down, there are predictably a lot of people at the reunion that don't know each other, perhaps even of each other--not that we'd be able to recognize impostors gathered for a free meal! [Wink]

Edit: Likewise, there has got to be some minor imbreeding in there somewhere in pockets where certain portions of the family wound up in very small, isolated towns for a while. Every family probably has some of that, it's just that with the Amish, they've been isolated for generations upon generations rather than just a couple of decades.

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Lainie
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quote:
Originally posted by Elwood:
Its the inter-generational relationships that always confuse me. What is my relationship to my father's cousin? And his sons? We call the father's cousin my second cousin, but what does that make the son?

Your second cousin once removed, IIRC.

Buzzkiller, it's possible that being first cousins wasn't even an issue. According to EspriseMe's source, it's illegal for first cousins to marry in 24 states, which means it's legal in 26 states.

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Buzzkiller
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quote:
quote:
Its the inter-generational relationships that always confuse me. What is my relationship to my father's cousin? And his sons? We call the father's cousin my second cousin, but what does that make the son?
Your second cousin once removed, IIRC.
Okay, I learned this stuff in a cultural anthropology class back when I was in college. First cousin, second cousin, and so on denotes "parallel" cousins who are the same number of generations removed from the original, common relative. E.g. the child of my parent's sibling is my first cousin; my child and my first cousin's child are second cousins.

Once removed, twice removed, and so on refers to situations where two relatives are not parallel in the family tree and indicates the number of generations' difference between the two. For instance, my first cousin's child is my first cousin, once removed; my grandchild and my first cousin are first cousins, twice removed; my dchild and my cousin's grandchild are second cousins, once removed.

Confused yet?

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Morgaine La Raq Star
The "Was on Sale" Song


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I have some experience with Mennonite communities & they are making an effort to expand their gene pool by finding other communities & 'swapping'. A group of men will go to an area (I know that a group of men from Oklahoma went somewhere in Canada) to find wives. I don't know if some men from Canada did the same thing but it wouldn't surprise me.
I personally see a benefit to this in addition to new genes as it might be easier to leave your family if several women from your community are going with you.

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Clarity
Toys to the World


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You've got it right, Buzzkiller. Your cousin's child is your first cousin once removed, and your father's cousin is also your first cousin once removed. But your father's cousin's child is your second cousin. Fun stuff.
I've been seeing the word "cousin" so much on here that it is starting to look unfamiliar and I'm questioning my spelling. That ever happen to you?

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Bramble Silvertree
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I also have a double cousin. Our moms are sisters and our dads are brothers. Meaning my mom married her inlaw, no shared genes, so no problems. Since my cousin and I share the same grandparents, we are as close to siblings as you can get without sharing parents, but we aren't closer related or inbred or anything else like that. We just happen to look somewhat alike, but no more so than most siblings. If anyone's a fan of teh show Roseanne, same thing happened towards the end of the series. Darlene and David had a kid, and Becky and Mark had one on the way.
(I'm very ashamed that I know that [Embarrassed] )

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Pogue Ma-humbug
Happy Christmas (Malls are Open)


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quote:
Originally posted by Esprise Me:
It's legal where I live.

It ain't in Kentucky.

Pogue

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Buzzkiller
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Totally off topic, but I know a married couple who are stepbrother and stepsister...which would be realllllly creepy were it not that his mother and her father got together after their children's wedding. What's weird, though, is that the younger couple's children have but one set of grandparents--twice over.
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