Image: via NClark
Illustration of Martin Fugate and his family. Some reports say Martin was not blue but was a carrier of the methemoglobinemia gene.
If a good friend of usually normal intelligence suddenly insisted they saw little blue men, you’d be inclined to think they had lost all their faculties and would carefully hide all alcohol from sight, that or they’ve been watching too many reruns of the Smurfs. But until only recently the chance of seeing not only blue men but blue women was a very high likelihood, especially if you frequented the Appalachian Hills of Kentucky. In fact, you’d see whole families of blue people.
Image: via Wunderkabinett
Ingesting elemental silver can also turn the skin blue. This man, Paul Karason, rubbed colloidal silver on his face and skin many years ago to treat a skin condition, which made him slowly turn a bluish/grey color.
Stemming from one French immigrant, Martin Fugate, who moved to Kentucky in 1820, the blue families were to become legends in their own rights, all because of the color of their skin.
Their story came to the attention of medical researchers in the early 1980s when 9th generation Fugates (now with the surname Stacy, through marriage) gave birth to a child named Benjy. He was a dark blue color immediately after birth, which caused panic among the medical team, and after carrying out a hoard of tests, mainly for heart and lung problems, the doctors found nothing of note. The only clues to the child’s diagnosis was when the grandmother piped up, “Have you ever heard of the blue Fugates of Troublesome Creek? My grandmother Luna on my dad’s side was a blue Fugate. It was real bad in her.”
“Blue Man” Paul Karason during an interview on September 10, 2009.
Born with the condition methemoglobinemia, the Fugates and their affected descendents suffered from a rare hereditary blood disorder where there is excessive methemoglobin in the blood. The American Heritage® Medical Dictionary states methemoglobin is “a brownish-red crystalline organic compound formed in the blood when hemoglobin is oxidated either by decomposition of the blood or by the action of various oxidizing drugs or toxic agents. It contains iron in the ferric state and cannot function as an oxygen carrier.” Because the circulating blood is less oxygenated those with the disorder appear various shades of blue, depending on how seriously they are affected.
Methemoglobinemia is a recessive gene, which means it can only be passed on if both parents carry the gene. So the chances of Martin Fugate meeting and marrying someone who carried the ‘blue’ gene were pretty slim, but that’s what happened. Martin Fugate and Elizabeth Smith went on to have seven children, four of them reported to be blue.
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.