Genetic disease is ravaging Lancaster County's Amish, and helping to change medicine for all of us

[TexasTowelie]

Read more: http://democratsforever.freeforums.net/thread/3664/genetic-disease-ravaging-lancaster-countys

[madokie]

1. Here's a direct link to the article

http://www.pennlive.com/news/2016/02/genetic_disease_is_ravaging_la.html

The condition, an inherited disorder of the immune system known colloquially as "the bubble boy disease," is present in roughly one in every 40,000 to 70,000 live births in the U.S., according to the National Institutes of Health.

Currently, more than 50,000 Lancaster County Amish can trace their lineage to just 80 ancestors, less than a tenth of what that number would be outside of the religious community in the 250 years, or 10 generations, since its founding.

Fewer ancestors mean more sharing of genetic material and any genetic defects — the same linked to potentially fatal hereditary diseases like SCID — contained therein.

This is the founder effect, and evidence of it has been found in Amish and Mennonite populations from Pennsylvania and Ohio to Ontario, Canada.

We have a large Amish and Mennonite presence here in northeast Oklahoma also. I suspect to combat this the Amish here send their girls back east to marry and the Amish back east send many of their men here to live and marry. Not sure how many exchanges are made but I'm aware that they do this.

[madokie]

3. Here is some more from the article

Morton determined all but one shared GA1 or Glutaric Aciduria, Type 1, an inherited metabolic disorder that leads to a build up of potentially harmful acids in the blood. Unchecked, it can prompt a metabolic stroke that leaves Amish children paralyzed or "locked-in" — able to think and feel, but not to speak or move.

The condition is present in one in 400 Amish births in Lancaster County, and almost non-existent outside of it.

Through the clinic's development of new diagnostic tools and treatments, as well as its groundbreaking use of genetic mapping, Morton was able to zero-out what was once a 93 percent injury rate.

He did the same with Maple Syrup Urine Disease, a recessive and potentially fatal metabolic disorder named for the sweetish odor it lends to bodily fluids like urine and earwax.

[malaise]

5. Wow

Inbreeding is always bad in the long term

[mrs_p]

2. The full article is interesting

http://www.pennlive.com/news/2016/02/genetic_disease_is_ravaging_la.html

STRASBURG — Soon after birth, Daniel and Rebecca Stoltzfus's first child fell ill.

Fearing pneumonia, her parents rushed her to a hospital where she later died.

Testing revealed Severe Combined Immunodeficiency (SCID), a rare genetic disease, as the cause of death.

The condition, an inherited disorder of the immune system known colloquially as "the bubble boy disease," is present in roughly one in every 40,000 to 70,000 live births in the U.S., according to the National Institutes of Health.

[pansypoo53219]

4. i was at a northern WI reservation. the natives knew this. no marrying in your clan. they explained

that is why no deer clan.

[Amishman]

6. ravaging is too strong of a word

it is happening, but overall I think the amish communities are far healthier than the general population. Cancer rates in particular are much lower.

[LisaL]

7. Not true for genetic disorders.

"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."


http://www.cbsnews.com/news/genetic-disorders-hit-amish-hard/

[ChairmanAgnostic]

9. Yeah, those jerks also don't pay state liquor or cig taxes, either.

Cuz they don't drink or smoke.
That would have no impact, right?

[Mendocino]

8. Certain Mormon communities

have a problem caused by inbreeding. Before 1990 there was only 13 known cases of Fumarase Deficiency, a condition that causes severe mental problems and seizures. Some victims have measured IQs as low as 25. Since that time 20 more cases have been diagnosed in the polygamous mormon communities of Colorado City AZ and Hilldale UT, which border each other. Nearly everyone's lineage in these towns trace back to just two ancestors.