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Within the family her rare condition turned BLUE and blood brown after generations of incest

A FAMILY with a bizarre, rare genetic condition that turned their skin blue passed them down through incest for centuries.

The Fugate family of Troublesome Creek, Kentucky lived in an isolated rural community, resulting in regular crossbreeding between relatives.

Members of the Fugate family of Kentucky have had the disease for centuries

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Members of the Fugate family of Kentucky have had the disease for centuries
The recessive gene for blue skin has been passed down through generations through inbreeding

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The recessive gene for blue skin has been passed down through generations through inbreedingPhoto credit: The Lost Creek Medicine Show
Methemoglobinemia is a rare condition that turns the skin blue

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Methemoglobinemia is a rare condition that turns the skin blue

In a stomach-turning case, one of the Fugate men married his own aunt.

Their story begins in 1820 when Martin Fugate and his wife, Elizabeth Smart, first settled in the remote area of ​​Appalachia that is now Perry County, Kentucky.

Martin was the first known family member with an incredibly unusual genetic defect that led to a condition called methemoglobinemia.

This extremely rare condition, which affects just 0.035 percent of the world’s population, is caused by the blood not carrying as much oxygen around the body as it normally would.

The blood turns brown due to the lack of oxygen-carrying red blood cells.

This, in turn, causes the skin of white patients to turn blue, while the lips take on a purple hue.

Both Martin and Elizabeth carried the recessive gene that causes methemoglobinemia, and four of their seven children were born with blue skin, including their son Zacharia.

Since the gene was recessive, this would not have affected future generations unless they intermarried within their own family.

But because they lived in such an isolated community, the Fugates had limited options and mostly married their cousins, crossing paths with nearby families like the Combs, the Richies, the Smiths and the Stacys.

Zacharia married his own aunt, and one of their sons married a close cousin.

In return, one of their children would marry another cousin.

As a family member Alva Stacy later told a researcher studying the family, “I’m related to myself.”

Of all the Fugates, Luna Fugate was known as the bluest of the family.

She married John Stacy in the late 19th century and the couple had 13 children.

Contemporary accounts describe her as having “lips as dark as a bruise”.

Although methemoglobinemia can cause developmental disabilities and seizures, none of the Fugate children appeared to be affected.

Luna herself lived to the ripe old age of 84 while all her children and John’s were physically healthy despite their unusual appearance.

FINDING THE FUGATA

By the mid-20th century, modernity was beginning to take hold even in rural Kentucky, and the Fugate’s history spread beyond their isolated community.

One person intrigued by the family was hematologist Dr. Madison Cawein of the University of Kentucky.

He heard about the Fugates in the early 1960s and made his way to Perry County in the southeastern state to try to track them down.

On his journey he met a nurse, Ruth Pendergrass. She told him she once worked at the County Health Department when a blue woman walked in asking for a blood test.

Ruth believed the woman was having a heart attack and was scared at first.

But the patient reassured her and said she was from the blue Combs family who lived up on Ball Creek.

She was also the sister of one of the Fugate women.

I am related to myself

Alva Stacy“Blue People of Kentucky”

Finally, Dr. Cawein and Ruth locate some of the surviving Fugate.

The doctor determined that the family’s blood was missing a key enzyme, and to cure them, he injected them with a blue dye, methylene.

Amazingly, it worked and the delighted family members’ skin lost its blue color within a few days.

The effect was only temporary, however, and Dr. Cawein provided the Fugates with methylene tablets daily to keep it going.

They first settled in the rural community in the 1820s

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They first settled in the rural community in the 1820s
dr Madison Cawein studied the fugates in the 1960s

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dr Madison Cawein studied the fugates in the 1960s
The skin appears blue because the blood does not carry enough oxygen

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The skin appears blue because the blood does not carry enough oxygen
dr Cawein treated the fugates with methylene blue dye

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dr Cawein treated the fugates with methylene blue dye
Troublesome Creek in Kentucky was incredibly isolated until recently

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Troublesome Creek in Kentucky was incredibly isolated until recentlyPhoto credit: Kentucky Digital Library

However, life wasn’t always easy for the Fugates. Her blue skin became more of a stigma as it was associated with generations of incest.

They withdrew even more from their community, not even coming into a doctor’s waiting room.

In the last half century the family has started having children outside the family and the blue gene has all but disappeared.

The last known blue-skinned Fugate was born in 1975.

Benjy Stacy was nearly purple when he was born, and panicked nurses rushed him to the hospital for an emergency blood transfusion.

However, his grandmother then explained what had happened.

The blue would eventually fade from Benjy’s skin, but his lips and nails would still turn purple when he got cold or angry.

At least one descendant of the Fugate is said to still live in the Appalachia area, but their life of isolation that created such a bizarre state of affairs has now come to an end.

https://www.the-sun.com/news/5539580/family-skin-turns-blue-incest-fugate-kentucky/ Within the family her rare condition turned BLUE and blood brown after generations of incest

DevanCole

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