Image: via Appalachian History
Lorenzo and Eleanor Fugate. Lorenzo was also known as ‘Blue Anze’ and was mentioned in Trost’s The Blue People of Troublesome Creek.
Researcher Cathy Trost, who compiled the most comprehensive history of the Fugates to date, says:
“The clan kept multiplying. Fugates married other Fugates. Sometimes they married first cousins. And they married the people who lived closest to them, the Combses, Smiths, Ritchies, and Stacys. All lived in isolation from the world, bunched in log cabins up and down the hollows, and so it was only natural that a boy married the girl next door, even if she had the same last name.”
And so, after ten generations, from Martin Fugates father, ‘blue’ people roamed the hills of Kentucky.
The pattern of inheritance for congenital methemoglobinemia is autosomal recessive, as shown here.
It was only when researchers investigating Benjy Stacy’s case discovered a report in the Journal of Clinical Investigation by EM Scott in 1960 that a cure appeared likely.
The article pointed to an absence of an enzyme from the red blood cells called diaphorase, which Scott found was lacking in some indigenous Alaskans he had studied previously. Trost explains: “In normal people hemoglobin is converted to methemoglobin at a very slow rate. If this conversion continued, all the body’s hemoglobin would eventually be rendered useless. Normally diaphorase converts methemoglobin back to haemoglobin.”
The descendants of the Fugates were then tested, and they too lacked this enzyme. Springing into action, doctors studying the Appalachian clans considered Scott’s findings and found their own methemoglobin converter – a dark blue dye called methylene blue.
Image: Rachel D
Smurfs? These guys clearly aren’t suffering, but methemobglobinemia turns the skin color of those affected quite blue.
Trying to convince members of the blue clan to have blue dye injected into them so they would revert to a natural skin tone must have been harder than trying to find the cure, but one couple conceded. Minutes after the methylene blue was administered the blue tinge to the skin was gone.
Since then, it’s thought that all the Fugates and their relations have been treated – records claim that by 1982 only two of three family members had methemoglobin. We’re guessing they’ve been sorted by now.
So, the next time you’re feeling slightly persecuted, spare a thought for the Blue Fugates, a small populace in America who had skin as blue as blueberries and no doubt endured taunts that only Willy Wonka’s Violet Beauregarde could understand.