Polygamy and genetics: Short Creek, Utah’s inbreeding mutation epidemic

[clueliss]

https://geneticliteracyproject.org/2017/10/18/polygamy-genetics-short-creek-utahs-inbreeding-mutation-epidemic/

This article popped up on my Facebook Newsfeed yesterday.  They're seeing a cluster of a rare childhood genetic disorder in Short Creek.  

Spoiler

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Polygamy and genetics: Short Creek, Utah’s inbreeding mutation epidemic

Zaria Gorvett | October 18, 2017 | BBC

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[In 1990], 10-year-old boy was presented to Theodore Tarby, a doctor specialising in rare childhood diseases.

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oon Tarby had diagnosed a total of eight new cases, in children ranging from 20 months to 12 years old.

In every case, the child had the same distinctive facial features, the same delayed development – most couldn’t sit up, let alone walk – and, crucially, they were from the same region on the Arizona-Utah border, known as Short Creek.

Even more intriguingly, this region is polygynous. In this small, isolated community of Fundamentalist Church of Jesus Christ of Latter-Day Saints (FLDS) – a group that split from the LDS in the early 20th Century – the likelihood of being born with fumarase deficiency is over a million times above the global average.

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“With polygyny you’re decreasing the overall genetic diversity because a few men are having a disproportionate impact on the next generation,” says Mark Stoneking, a geneticist at the Max Planck Institute for Evolutionary Anthropology, Germany. “Random genetic mutations become more important.”

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Since inbreeding tends to uncover “recessive” mutations that would normally remain in hiding, studying these communities has helped scientists to identify many disease-causing genes.

The GLP aggregated and excerpted this blog/article to reflect the diversity of news, opinion, and analysis. Read full, original post: The polygamous town facing genetic disaster

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[Carm_88]

That's just heartbreaking. Those poor kids.  

[Buzzard]

Thats pretty interesting, but I'm sure they wont identify the carriers and use any medical means to ensure the health of their children.

[Tatar-tot]

There was issue concerning with an Amish community in Ohio.  It is just horrible.    

[Meowkitties]

Interesting to see a detrimental physical health effect of this religious practice. Those poor kiddos, and imagine the stress on the women in these relationships and carrying then caring for these children  

[JermajestyDuggar]

They also have a high number of still births and infant mortality. It could be genetically related if it could be a lack of prenatal care. Or a mix.

[OyToTheVey]

It's the same with the Ultra Orthodox Jews. Here in Brooklyn, you can see the actual genetic disorders. It goes from something small like every kid in the family has thick glasses on to multiple kids in wheelchairs. They don't do genetic testing and prefer to marry within the community. Other Jews, before marriage, take the Tay-Sachs test. It's a requirement. I don't know a single Jewish couple that hasn't gotten that test. It's a horrible disease. Tay-Sachs kids suffer so much.

[Nikedagain?]

I live in NC and worked at a school that served kids with special needs years ago. We had one family (first cousins married) and they had 10 kids. 4 have a severe form of something like fragile X (but it's not, the tests were negative, it's an unknown...) 2 died ( one was in an accident, one as a newborn) and the rest have varying degrees of developmental delay. Very sad. The dad (Ironically named SQUIRREL) fell out of a tree stand and died. The mom, who was just very sweet and simple, absolutely fell apart after that and DSS stepped in. There are those families you remember forever, this is one of mine. 

 

[apandaaries]

@Nikedagain? wow.  There is so much wrong with all that. But I just Googled and discovered that first (but not double) cousins CAN marry in NC (also, that query will help me garner the weirdest ads, I just know).  TEN kids? The dad was named Squirrel? And he fell out of a tree stand?  You know how sometimes in life things are just too weird and you think, no one would ever believe that if it happened in a book or a movie? THAT is how that read to me.

But that series of tragedies  That's awful.  It's just wrapped up in so much WTF, though.

[Nikedagain?]

1 hour ago, amandaaries said:

@Nikedagain? wow.  There is so much wrong with all that. But I just Googled and discovered that first (but not double) cousins CAN marry in NC (also, that query will help me garner the weirdest ads, I just know).  TEN kids? The dad was named Squirrel? And he fell out of a tree stand?  You know how sometimes in life things are just too weird and you think, no one would ever believe that if it happened in a book or a movie? THAT is how that read to me.

But that series of tragedies  That's awful.  It's just wrapped up in so much WTF, though.

It was CRAZY TOWN I tell ya. They just kept having baby after baby. The mother would say "I JUST LOVE BABIES". She is very sweet, very child like. They both were.

The oldest daughter was probably the least affected and she is around 22 or 23. I remember that she had severe learning disabilities and speech issues. She moved out with some guy after her dad died and has kids (she friended me on fb) and one of her daughters has a very serious, "possibly genetic" syndrome. I am not sure what it is, but the little girl is in a wheelchair and on a vent 24/7. I think they are still trying to figure her out. I think that it will be likely just end up another  case of "(*their last name*) Syndrome".

So sad. 

[Rosalie]

Quote

On November 1st, 1700, an entire dynasty of kings came to a crashing end with the death of Charles II of Spain. Charles had neither a pleasant life nor a successful reign. He was physically disabled, mentally retarded and disfigured. A large tongue made his speech difficult to understand, he was bald by the age of 35, and he died senile and wracked by epileptic seizures. He had two wives but being impotent, he had no children and thus, no heirs. Which is what happens after 16 generations of inbreeding.

How inbreeding killed off a line of kings

On 20.10.2017 at 4:30 PM, clueliss said:

https://geneticliteracyproject.org/2017/10/18/polygamy-genetics-short-creek-utahs-inbreeding-mutation-epidemic/

This article popped up on my Facebook Newsfeed yesterday.  They're seeing a cluster of a rare childhood genetic disorder in Short Creek.  

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Longer article reg. the illness caused by inbreed in Short Creek:  http://www.bbc.com/future/story/20170726-the-polygamous-town-facing-genetic-disaster?ocid=fbert

[keen23]

My husband's first cousins had kids together ("we don't even need to get married, we already have the same last name!" for reals). The first one is seemingly ok. The second one suffers from some type of auto immune issue where his body attacks his own immune system and his blood doesn't clot correctly. But it's not HIV and it's not hemophilia, it's something all together different, but it acts like it sometimes. He's in and out of the hospital all the time. Respiratory issues galore. Kid is 8, looks like a much younger child, because he's physically and mentally behind his peers.