Although Ray was born in Mexico City, his father was a prominent medical doctor in the southern U.S., and Ray lived with his family through high school in the Carolinas. He stayed in the South to get his undergraduate degree in biology from the University of Kentucky.
As a small town southern boy, one of the local traits that Ray picked up that continues to define him as an adult is storytelling, at which traditional Southerners in the U.S. often excel.
Today Ray told me about the blue people of Kentucky.
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I mentioned that one of my closest lifelong friends still teaches high school in rural Kentucky and has incredible stories to tell about moonshiners and pot farmers, Kentucky being one of the pot growing Capitols of our fair nation east of the Mississippi. Ray said my friend wouldn't be a real Kentuckian unless he knew about the blue people.
I thought he was joking, but it turns out there really are blue people in remote eastern Kentucky, in an appropriately named area called Troublesome Creek. There are also blue people in Alaska!
In Kentucky, the Fugate and Combes families from the hills and hollows around Troublesome and Ball Creeks trace their blue ancestry back at least 162 years. Among long time residents of the area, they are simply known as the "blue people," and it is common knowledge that they have traditionally preferred to live where they do because it is so isolated, home to the only blue folks in a world full of white, black and brown people.
Their skin coloration has been described as the "...color of a bruised plum," and a local nurse named Ruth Pendergrass and others who worked with them have described some people as so dark blue they were "...almost purple," with blue lips and fingernails too.
Their isolation was preserved for years because there were few roads into the Cumberland Plateau, and the railroad didn't come into eastern Kentucky until coal mining started on a large scale in about 1912, although even then, it took a few decades for roads to be built along local creek beds.
Local stories about blue people are legion and are filled with names and tales as colorful as their skin; stories about Aunt Bessie Fugate up Copperhead Hollow, the Zacariah and Levy Fugate clans, blue farmer John Stacy's black piglets kept as pets up on the Lick Branch of Ball Creek, blue patients showing up for treatment at Homeplace Clinic and Hazard Clinic and lists of blue ancestors written in old family Bibles.
 Map courtesy of www.nationsonline.org |
A fascinating article in the November, 1982 issue of Science magazine (link below) recounts the history of the blue people quite nicely while also explaining the hematological abnormality that causes their unique color. A young hematologist named Madison Cawein at the University of Kentucky's Lexington medical clinic spent an entire summer in the 1960's tramping around the hill country of eastern Kentucky in search of blue people. He found so many that he eventually had to map out detailed family histories and lineage charts.
Cawein, who helped develop an antidote for cholera, had specialized in hematology because "Blood cells always looked so beautiful to me." Just the kind of guy you would want on this case. After ruling out heart and lung disease, he began to suspect methemoglobinemia, a rare hereditary blood disorder caused by excess levels of methemoglobin in the blood, which is caused in turn by abnormal hemoglobin.
Methemoglobin is blue! It carries blood that has been depleted of oxygen, which is what we see in the blue veins just under the surface of our skin. If the blue people had abnormal hemoglobin, Cawein thought he might be able to prove methemoglobinemia as the cause of their unique coloration.
Cawein drew blood from dozens of blue people far into the hill country around Troublesome and Ball Creeks, but tests for abnormal hemoglobin were negative. Stumped, he began researching exhaustively and discovered a 1960 report about blue Alaskan Eskimos in the Journal of Clinical Investigation, written by Dr. E.M. Scott of the Arctic Health Research Center in Anchorage. The Eskimos were blue and they had methemoglobinemia, but it didn't show up in regular hemoglobin tests because it was a recessive hereditary trait that caused the enzyme diaphorase to be missing from their red blood cells. Diaphorase converts blue methoglobin back to hemoglobin. Without sufficient diaphorase in your blood, you develop methemoglobinemia.
An absence of diaphorase turned out to be the cause among the blue people of Kentucky as well. It is much more likely to occur in isolated and often inbred groups. Because the recessive trait found in the blue people requires that both parents have the gene in order for the third, the child, to be born blue, this is a rare kind of methemoglobinemia. Interestingly, the blue people of Kentucky were in generally robust health, often living well into their 80's with the women bearing large numbers of children.
Although there are still blue people in America, their numbers are dwindling now that their isolation has lessened dramatically and their inter-breeding with non-blue people has accelerated. Cawein and his team published their findings in the Archives of Internal Medicine in April, 1964.
One of the interesting aspects of this story is that the blue people of Kentucky were reported to feel enormous embarrassment about their abnormality, which further increased their isolation. They thought there was something wrong with them as people, not just medically. They also lived in poverty, although it is doubtful they thought of it in that way.
To his enormous credit, Cawein turned down an offer from the producers of a TV show called "That's Incredible." They wanted to make a spectacle out of these beautiful blue human beings, and he would have none of it.
What is America? It is beautiful blue people in Alaska and Kentucky, an omnivorous media obsessed with spectacle and a principled doctor with an abiding respect for the dignity of his patients.
Thank you Ray! We will follow up on that story about Kentucky's midget village another day.
Notes
A reprint of the 1982 Science article can be found on the Roots Web site. It has full attribution for the articles noted above.
http://www.rootsweb.ancestry.com/~kyperry3/Blue_Fugates_Troublesome_Creek.html
