Inside family so inbred their rare condition turned skin BLUE & blood brown after generations of incest | The SunJune 11, 2022
A FAMILY with a bizarre rare genetic condition that turned their skin blue passed it down for centuries due to generations of incest.
The Fugate family of Troublesome Creek, Kentucky, lived in an isolated rural community, which led to regular interbreeding between relatives.
In one stomach-churning case, one of the Fugate men married his own aunt.
Their story begins back in 1820 when Martin Fugate and his wife Elizabeth Smart first settled in the remote area of Appalachia, which today is in Perry County, Kentucky.
Martin was the first known family member to have an incredibly unusual genetic defect resulting in a condition called methemoglobinemia.
This extremely rare disorder, which only affects 0.035 per cent of the population globally, is a result of the blood not carrying as much oxygen around the body as it normally does.
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The blood turns brown due to the lack of oxygen-carrying red blood cells.
This in turn leads to the skin of white patients turning blue, while the lips take on a purple hue.
Both Martin and Elizabeth carried the recessive gene which cause methemoglobinemia, and four of their seven children were born with blue skin, including their son Zacharia.
Because the gene was recessive, this wouldn't have affected future generations if they hadn't married within their own family.
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But because they lived in such an isolated community, the Fugates had limited options and mostly married their cousins, interbreeding with nearby families including the Combs, the Richies, the Smiths, and the Stacys.
Zacharia married his own aunt, and one of their sons married a close cousin.
In turn, one of their children would marry another cousin.
As one family member Alva Stacy would later tell a researcher studying the family: "I'm kin to myself."
Of all the Fugates, Luna Fugate was known as the bluest family member.
She married John Stacy in the late 19th century and the pair had 13 children.
Contemporary reports describe her as having "lips as dark as a bruise".
However, although methemoglobinemia can cause developmental disorders and seizures, this didn't seem to affect any of the Fugate children.
Luna herself lived until the ripe old age of 84, while all of her and John's children were said to be physically healthy despite their unusual appearance.
FINDING THE FUGATES
By the middle of the 20th century, modern times had started to come even to rural Kentucky, and the story of the Fugates spread beyond their isolated community.
One person who was fascinated by the family was haematologist Dr Madison Cawein at the University of Kentucky.
He heard about the Fugates in the early 1960s and set off to Perry County in the southeast of the state to try and track them down.
On his journey, he met a nurse Ruth Pendergrass. She told him that she had once been working at the County Health Department when a blue woman came in asking for a blood test.
Believing the woman to be having a heart attack, Ruth was initially terrified.
But the patient reassured her and said she was part of the blue Combs family who lived up on Ball Creek.
She was also a sister to one of the Fugate women.
I'm kin to myself
Eventually, Dr Cawein and Ruth were able to track down some of the surviving Fugates.
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 thrilled family members' skin lost its blue colour in just a matter of days.
The effects were only temporary, however, and Dr Cawein supplied the Fugates with daily methylene tablets to keep up the effects.
However, life wasn't always easy for the Fugates. Their blue skin became more of a stigma as it came to be associated with generations of incest.
They withdrew even more from their community and wouldn't even come to a doctor's waiting room.
Over the past half-century, the family have started to have children outside the family, and the blue gene has all but disappeared.
The last-known Fugate with blue skin was born in 1975.
Benjy Stacy looked almost purple at birth, and panicked nurses rushed him to hospital for an emergency blood transfusion.
However, his grandmother then explained what had happened.
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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 descendent of the Fugates is said to still live in the Appalachia area, however, their life of isolation which created such a bizarre condition has now come to an end.
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