Mystery deaths among Amish people caused by genetic mutation

It's easy to imagine the Amish leading a harshly charmed existence. Different sects have their own sets of restrictions, per Penn State News, but the version that emerges in pop culture leads an unflinchingly Luddite reality devoid of vehicles, electricity, and even fashionability, and lives in tight-knit isolation from the rest of the world. Their faith demands that they stick together and look after each other, so much so that many sects are exempt from paying Social Security taxes (with some important exceptions) because they sued for the right to provide their own forms of health insurance for their members.

While it's important not to paint the Amish with too broad a brush (some use public electricity and drive cars, for instance), there are certain circumstances in which the social isolation associated with the Amish lifestyle creates far more harm than charm. One of the more horrifying examples was highlighted by a 2020 study that investigated a spate of largely unexplained deaths that plagued two Amish families.

The heart of a heart problem

The deaths struck unexpectedly. As Gizmodo details, in a four-month span, two young Amish siblings died suddenly of cardiac arrest while exercising. Eight years later, two children from the exact same family died almost the exact same way: either playing or exercising. This grief-afflicted family wasn't alone. A second Amish family also struggled with abrupt deaths of young relatives. Between the two families, a total of 18 people died, and none were younger than 12.

According to a report in the American Medical Association's publication, JAMA Cardiology, between 2004 and 2019, researchers collected genetic samples from the two families and identified the culprit as a genetic mutation that causes the cardiac muscle the have too few calcium release channels (CRCs). As a result, the heart can't respond properly to stress. Hence, the cardiac arrest.This CRC deficiency was present in 23 people in the two Amish families, including the 18 who died. Sadly, the lack of genetic diversity in often isolated Amish communities heightened the likelihood that these families would suffer from CRC deficiency.

Sadly, this isn't an isolated case of genetic difficulties for the Amish. In 2016, reported on an Amish family in Lancaster County, Pennsylvania, that was grappling with Severe Combined Immunodeficiency (SCID). A rare condition that affects only one in every 40,000 to 70,000 U.S. births in general, it manifested in three out of the 12 children born to Daniel and Rebecca Stoltzfus. One of those three tragically died. That stunningly high occurrence could be explained by the fact that 50,000 Amish families in Lancaster County "can trace their lineage to just 80 ancestors."