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Cover Story January 19, 2006

 

  by lyle e davis

The Blue People of Troublesome Creek


The theatrical ‘blue people’ - part of the Blue Man Group that
frequents Las Vegas Show Lounges.

 

 

by lyle e davis

 

Blame it on a French orphan by the name of Martin Fugate.

 

He claimed a land grant in 1820 and settled on the banks of eastern Kentucky’s Trouble-some Creek.  No mention of his skin color is made in the early histories of the area, but family lore has it that Martin himself was blue. The odds against it were incalculable, but Martin Fugate managed to find and marry a woman who carried the same recessive gene. His red-headed American wife, was the former Elizabeth Smith, whose skin, it is said “was as pale as the mountain laurel that blooms every spring around the creek hollows.”

 

The Fugates had seven children, four of whom were reported to be blue in color. They were away in the remote section of Kentucky and folks just naturally tended to marry folks that lived closest to them.  Never mind the fact that these newlyweds were, more often than not, kin to one another.  Likely first cousins at least.

 

Fugates would marry other Fugates  . . . but sometimes they’d branch out and marry Combses, Smiths, Ritchies, and Stacys. 

 

They all lived in isolation from “the real world,” bunched up in log cabins up and down the hollows so it just seemed natural to marry your neighbor.  As a result, the clan kept multiplying and intermarrying and interbreeding.

 

"When they settled this country back then, there was no roads. It was hard to get out, so they intermarried," says Dennis Stacy, a 51-year-old coal miner and amateur genealogist who has filled a loose-leaf notebook with the laboriously traced blood lines of several local families.

 

Stacy counts Fugate blood in his own veins. "If you'll notice," he observes, tracing lines on his family's chart, which lists his mother's and his father's great grandfather as Henley Fugate, "I'm kin to myself."

 

It had been 162 years since the phenomenon first was noticed to the point where an explanation for the phenomenon developed.  And an apparent cure.

 

Martin and Elizabeth Fugate's blue children had multiplied in this natural isolation tank.

 

Carrie Lee Kilburn, a nurse at the rural medical center called Homeplace Center referred to the daughter of Levy Fugate, Luna, as "the bluest woman I ever saw. Luna was bluish all over. Her lips were as dark as a bruise.."

 

Levy had married a Ritchie girl and bought 200 acres of rolling land along Ball Creek. The couple had 8 children, including Luna. A fellow by the name of John Stacy spotted Luna at Sunday services of the Old Regular Baptist Church before the turn of the century. Stacy courted her, married her, and moved from Troublesome Creek to make a living in timber on her daddy's land. John Stacy still lives on Lick Branch of Ball Creek. Stacy recalls that his father-in-law, Levy Fugate, was "part of the family that showed blue. All them old fellers way back then was blue. One of em - I remember seeing him when I was just a boy - Blue Anze, they called him. Most of them old people went by that name - the blue Fugates. It run in that generation who lived up and down Ball Creek".

 

"They looked like anybody else, ‘cept they had the blue color," Stacy said.

 


The Fugate Blue Family - circa 1900. Taken from Science82: November, 1982

 

Stacy says, sitting in a chair in his plaid flannel shirt and suspenders, next to a cardboard box where a small black piglet, kept as a pet, is squealing for his bottle, "I couldn't tell you what caused it."

 

The only thing Stacy can't or won't remember is that his wife Luna was blue. When asked ahout it, he shakes his head and stares steadfastly ahead. It would be hard to doubt this gracious man except that you can't find another person who knew Luna who doesn't remember her as being blue.

 

Luna Stacy possessed the good health common to the blue people bearing at least 13 children before she died at 84. The clinic rarely saw her and never for anything serious.

 

Benjy Stacy was born in a modern hospital near Hazard, Kentucky, not far from Troublesome Creek. He inherited his father's lankiness and his mother's red hair but what he got from his great, great, great grandfather was dark blue skin! The doctors were astonished, not so the parents, but the boy was rushed off to a medical clinic in Lexington (University of Kentucky Medical School). Two days of tests showed no cause for Benjy's blue skin.

 

Benjy's grandmother Stacy asked the doctor's if they had heard of the blue Fugates of Troublesome Creek. Put on that track, they concluded that Benjy's condition was inherited. Benjy lost his blue tint within a few weeks and now he is about as normal a 7-year old boy as you might imagine. His lips and fingernails still turn a purplish blue when he gets cold or angry and that trait was exploited by the medical students back when Benjy was an infant.  They would gather around his crib and try to get him to cry so they could witness the lips and fingernails turn blue.  Dark blue lips and fingernails are the only traces of Martin Fugate's legacy left in the boy; that, and the recessive gene that has shaded many of the Fugates and their kin blue for the past 162 years.

 

Then, in 1999, after having read a genealogy of the Fugate family, Benjamin Stacy sent the following message:

 

From: Benjamin Arnold Stacy

Subject: Fugate Pedigree

Date: Sun, 14 Mar 1999

 

To Whom It May Concern:

 

My name is Benjamin Arnold Stacy, Ben for short. I am the "Benjy" (misspelled in the original article in Science 82) mentioned in the article. My mother's maiden name is Hilda B. Godsey.

 

I was surprised to see that someone had taken the time to map the genealogy of my family. I would like to thank you for taking that interest, because it was something that I had wanted to do my self.

 

I do not know that much about methemoglobinemia and happened to come across this web site while searching.

 

Just for your information, I am 24 years old now and am in my senior year of college at Eastern Kentucky University. The color of my lips and finger nails usually draws some attention, but mostly out of concern for my health or curiosity. I have had no major health problems related to the disorder and simply try to live an average life in spite of being "blue." Again, thank you for your efforts.

 

Benjamin Arnold Stacy

bstacy@zeus.chapell.com 

stustacb@acs.eku.edu 

 

Madison Cawein began hearing rumors about the blue people when he went to work at the University of Kentucky's Lexington medical clinic in 1960. "I'm a hematologist, so something like that perks up my ears," Cawein says, sipping on whiskey sours and letting his mind slip back to the summer he spent "tromping around the hills looking for blue people."

 

Cawein would drive back and forth between Lexington and Hazard, an eight-hour ordeal before the tollway was built, and scour the hills looking for the blue people he'd heard rumors about. The American Heart Association had a clinic in Hazard, and it was there that Cawein met "a great big nurse" who offered to help. 

 

Her name was Ruth Pendergrass, and she had been trying to stir up medical interest in the blue people ever since a dark blue woman walked into the county health department one bitterly cold afternoon and asked for a blood test.

 

"She had been out in the cold and she was just blue!" recalls Pendergrass, who is now 69 and retired from nursing. "Her face and her fingernails were almost indigo blue. It like to scared me to death! She looked like she was having a heart attack. I just knew that patient was going to die right there in the health department, but she wasn't a'tall alarmed. She told me that her family was the blue Combses who lived up on Ball Creek. She was a sister to one of the Fugate women." About this same time, another of the blue Combses, named Luke, had taken his sick wife up to the clinic at Lexington. One look at Luke was enough to "get those doctors down here in a hurry," says Pendergrass, who joined Cawein to look for more blue people.

 

Trudging up and down the hollows, fending off "the two mean dogs that everyone had in their front yard," the doctor and the nurse would spot someone at the top of a hill who looked blue and take off in wild pursuit. By the time they'd get to the top, the person would be gone. Finally, one day when the frustrated doctor was idling inside the Hazard clinic, Patrick and Rachel Ritchie walked in.

 

"They were bluer'n hell," Cawein says. "Well, as you can imagine, I really examined them. After concluding that there was no evidence of heart disease, I said 'Aha!' I started asking them questions: 'Do you have any relatives who are blue?' then I sat down and we began to chart the family."

 

Cawein remembers the pain that showed on the Ritchie brother's and sister's faces. "They were really embarrassed about being blue," he said. "Patrick was all hunched down in the hall. Rachel was leaning against the wall. They wouldn't come into the waiting room. You could tell how much it bothered them to be blue."

 

After ruling out heart and lung diseases, the doctor suspected methemoglobinemia, a rare hereditary blood disorder that results from excess levels of methemoglobin in the blood. Methemoglobin which is blue, is a nonfunctional form of the red hemoglobin that carries oxygen. It is the color of oxygen-depleted blood seen in the blue veins just below the skin.

 

If the blue people did have methemoglobinemia, the next step was to find out the cause. It can be brought on by several things: abnormal hemoglobin formation, an enzyme deficiency, and taking too much of certain drugs, including vitamin K, which is essential for blood clotting and is abundant in pork liver and vegetable oil.

 

Cawein drew "lots of blood" from the Ritchies and hurried back to his lab. He tested first for abnormal hemoglobin, but the results were negative.

 

Stumped, the doctor turned to the medical literature for a clue. He found references to methemoglobinemia dating to the turn of the century, but it wasn't until he came across E. M. Scott's 1960 report in the Journal of Clinical Investigation (vol. 39, 1960) that the answer began to emerge.

 

Scott was a Public Health Service doctor at the Arctic Health Research Center in Anchorage who had discovered hereditary methemoglobinemia among Alaskan Eskimos and Indians. It was caused, Scott speculated, by an absence of the enzyme diaphorase from their red blood cells. 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 hemoglobin. Scott also concluded that the condition was inherited as a simple recessive trait. In other words, to get the disorder, a person would have to inherit two genes for it, one from each parent. Somebody with only one gene would not have the condition but could pass the gene to a child.

 

Scott's Alaskans seemed to match Cawein's blue people. If the condition were inherited as a recessive trait, it would appear most often in an inbred line.

 

Cawein needed fresh blood to do an enzyme assay. He had to drive eight hours back to Hazard to search out the Ritchies, who lived in a tapped-out mining town called Hardburly. They took the doctor to see their uncle, who was blue, too. While in the hills, Cawein drove over to see Zach (Big Man) Fugate, the 76-year-old patriarch of the clan on Troublesome Creek. Zach took the doctor even farther up Copperhead Hollow to see his Aunt Bessie Fugate, who was blue. Bessie had an iron pot of clothes boiling in her front yard, but she graciously allowed the doctor to draw some of her blood.

 

"So I brought back the new blood and set up my enzyme assay," Cawein continued. "And by God, they didn't have the enzyme diaphorase. I looked at other enzymes and nothing was wrong with them. So I knew we had the defect defined.''

 

Just like the Alaskans, their blood had accumulated so much of the blue molecule that it overwhelmed the red of normal hemoglobin that shows through as pink in the skin of most Caucasians.

 

Once he had the enzyme deficiency isolated, methylene blue sprang to Cawein's mind as the "perfectly obvious" antidote. Some of the blue people thought the doctor was slightly addled for suggesting that a blue dye could turn them pink. But Cawein knew from earlier studies that the body has an alternative method of converting methemoglobin back to normal. Cawein chose methylene blue because it had been used successfully and safely in other cases and because it acts quickly.

 

Cawein packed his black bag and rounded up Nurse Pendergrass for the big event. They went over to Patrick and Rachel Ritchie's house and injected each of them with 100 milligrams of methylene blue.

 

''Within a few minutes. the blue color was gone from their skin," the doctor said. "For the first time in their lives, they were pink. They were delighted."

 

"They changed colors!" remembered Pendergrass. "It was really something exciting to see."

 

The doctor gave each blue family a supply of methylene blue tablets to take as a daily pill. The drug's effects are temporary, as methylene blue is normally excreted in the urine. One day, one of the older mountain men cornered the doctor. "I can see that old blue running out of my skin," he confided.

 

Before Cawein ended his study of the blue people, he returned to the mountains to patch together the long and twisted journey of Martin Fugate's recessive gene. From a history of Perry County and some Fugate family Bibles listing ancestors, Cawein has constructed a fairly complete story.

 

The story demonstrated that as coal mining and the railroads brought progress to Kentucky, the blue Fugates started moving out of their communities and marrying other people. The strain of inherited blue began to disappear as the recessive gene spread to families where it was unlikely to be paired with a similar gene.

 

Cawein and his colleagues published their research on hereditary diaphorase deficiency in the Archives of Internal Medicine in April, 1964. He hasn't studied the condition for years. Even so, Cawein still gets calls for advice.

 

The doctor was later approached by the producers of the television show "That's Incredible." They wanted to parade the blue people across the screen in their weekly display of human oddities. Cawein would have no part of it, and he related with glee the news that a film crew sent to Kentucky from Hollywood fled the "two mean dogs in every front yard" without any film. Cawein cheers their bad luck not out of malice but out of a deep respect for the blue people of Troublesome Creek.

 

"They were poor people," concurs Nurse Pendergrass, "but they were good."

 

The doctor never did reveal the location of the blue people and what with a daily dose of methylene blue, it may be that no one will ever find them again.

 

References:  

 

THE BLUE PEOPLE OF TROUBLESOME CREEK

The story of an Appalachian malady, an inquisitive doctor, and a paradoxical cure.

by Cathy Trost

©Science 82, November, 1982

 

Cawein, Madison, et. al. "Hereditary diaphorase deficiency and methemoglobinemia". Archives of Internal Medicine, April, 1964.

 

Scott, E.M. "The relation of diaphorase of human erythrocytes to inheritance of methemolglobinemia", Journal of Clinical Investigation, 39, 1960.

 

Cawein, Madison and E.J. Lappat, "Hereditary Methemoglobinemia" in Hemoglobin, Its Precursors and Metabolites,” ed. by F. William Sunderman, J.B. Lippincott Co., Philadelphia PA, 1964.

 

 

 

 

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