The Blue Fugates of Troublesome Creek
The Fugate family in Kentucky are known for sharing an unusual blue hue to their skin, going back for generations. A French orphan named Martin Fugate settled in Troublesome Creek in the hills of eastern Kentucky in 1820, where he married a woman named Elizabeth Smith, who was said to have red hair and very pale skin. Both possessed a rare recessive gene that led to four of their seven children being born with blue skin.
In those days in rural eastern Kentucky, there were no roads, and railroads wouldn’t even reach that part of the state until the early 1900's. As a result, many of the Fugates began to marry and have children within their own bloodline, concentrating the “blue gene”. In the late 1800's, inbreeding wasn't quite the scandal it is today. Zachariah Fugate, Martin and Elizabeth's son and one of the first known Blue Fugates, married his aunt; one of their sons, Levy, married a close cousin. They had eight children together and one of their children married another cousin. Over the next hundred or so years, the Fugates continued to live in relative isolation and were accepted by the people of Troublesome Creek. But that acceptance would not last long.
One of the women in the family was described as: “Luna was bluish all over. Her lips were as dark as a bruise. She was as blue a woman as I ever saw.” However, by the early 1960's, some members of the Fugate clan had begun to resent their cobalt-tinted skin. Not only did their skin mark them as different, but people had already begun to associate their skin color with the family’s history of inbreeding.
Two family members, siblings Patrick and Rachel Ritchie, sought help from a hematologist at the University of Kentucky’s medical clinic in search of a cure. Dr. Martin Cawein was able to conclude from other isolation studies with indigenous people in Alaska that the Fugates carried a rare hereditary blood disorder that causes excessive levels of methemoglobin in their blood. Methemoglobin is a nonfunctional blue version of the healthy red hemoglobin protein that carries oxygen. In most Caucasians, the red hemoglobin of the blood in their bodies shows through their skin giving it a pink tint. For the Fugate family, the excessive amount of blue methemoglobin in their blood turned their skin color blue. Although the disorder could cause developmental delay and seizures, none of the Fugates suffered poor health or lived in pain. The condition had only a cosmetic effect, though the family had come to endure psychological pain of the years from their outsider status. The family had developed a stigma in the community, and were ashamed of their complexion.
The hematologist devised a cure for this disorder: more blue. Counter-intuitively, the best chemical for activating the body’s process of turning methemoglobin to hemoglobin is methylene blue dye. The Fugates he treated ingested this dye and within a few minutes, the blue coloration of their skin disappeared, and their skin turned pink for the first time in their entire lives. As long as they kept ingesting pills of the substance regularly, since the body eliminates the dye on a regular basis, these blue people of Kentucky could live their lives normally.
The last known Blue Fugate, Ben Stacy, was born in 1975. The child looked almost purple at birth, alarming doctors, but his grandmother shared the story of their unusual heritage before the doctors could prepare a blood transfusion. The staff concluded the child had inherited the same rare condition. The blue faded from the child's skin over the next few weeks. Today, Ben appears to have a normal skin tone, though his lips and nails continued to turn purple whenever he got cold or angry.
As the railroads and new highways of the modern era began to connect Troublesome Creek with the rest of the nation, people began leaving the area and the gene is no longer concentrated. Even though most of the Fugate family descendants have lost their blue coloring, for some the tint still comes out in their skin when they are cold or flush with anger.