ALTERNATE ORIGINS

If the blue people did have methemoglobinemia, the next step was to find out the cause. It can be brought on by several Martin and Elizabeth set up housekeeping on the banks of Troublesome and began a family. Of their seven children, four things: abnormal hemoglobin formation, an enzyme deficiency, and taking too much of certain drugs, including vitamin K, which were reported to be blue.

is essential for blood clotting and is abundant in pork liver and vegetable oil.

The clan kept multiplying. Fugates married other Fugates. Sometimes they married first cousins. And they married the people Cawein drew “lots of blood” from the Ritchies and hurried back to his lab. He tested first for abnormal hemoglobin, but the who lived closest to them, the Combses, Smiths, Ritchies, and Stacys. All lived in isolation from the world, bunched in log cabins up results were negative.

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.

Stumped, the doctor turned to the medical literature for a clue. He found references to methemoglobinemia dating to the turn

“When they settled this country back then, there was no roads. It was hard to get out, so they intermarried,” says Dennis 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) Stacy, a 51-year-old coal miner and amateur genealogist who has filled a loose-leaf notebook with the laboriously traced blood that the answer began to emerge.

lines of several local families.

Scott was a Public Health Service doctor at the Arctic Health Research Center in Anchorage who had discovered hereditary Stacy counts Fugate blood in his own veins. “If you’ll notice,” he observes, tracing lines on his family’s chart, which lists his methemoglobinemia among Alaskan Eskimos and Indians. It was caused, Scott speculated, by an absence of the enzyme diapho-mother’s and his father’s great grandfather as Henley Fugate, “I’m kin to myself.”

rase from their red blood cells. In normal people hemoglobin is converted to methemoglobin at a very slow rate. If this conversion The railroad didn’t come through eastern Kentucky until the coal mines were developed around 1912, and it took another 30

continued, all the body’s hemoglobin would eventually be rendered useless. Normally diaphorase converts methemoglobin back or 40 years to lay down roads along the local creeks.

to hemoglobin. Scott also concluded that the condition was inherited as a simple recessive trait. In other words, to get the disorder, Martin and Elizabeth Fugate’s blue children multiplied in this natural isolation tank. The marriage of one of their blue boys, a person would have to inherit two genes for it, one from each parent. Somebody with only one gene would not have the condition Zachariah, to his mother’s sister triggered the line of succession that would result in the birth, more than 100 years later, of but could pass the gene to a child.

Benjamin Stacy.

Scott’s Alaskans seemed to match Cawein’s blue people. If the condition were inherited as a recessive trait, it would appear When Benjamin was born with purple skin, his relatives told the perplexed doctors about his great grandmother Luna Fugate.

most often in an inbred line.

One relative describes her as “blue all over,” and another calls Luna “the bluest woman I ever saw.”

Cawein needed fresh blood to do an enzyme assay. He had to drive eight hours back to Hazard to search out the Ritchies, who Luna’s father, Levy Fugate, was one of Zachariah Fugate’s sons. Levy married a Ritchie girl and bought 200 acres of rolling 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, land along Ball Creek. The couple had eight children, including Luna.

Cawein drove over to see Zach (Big Man) Fugate, the 76-year-old patriarch of the clan on Troublesome Creek. His car gave out A fellow by the name of John E. Stacy spotted Luna at Sunday services of the Old Regular Baptist Church back before

on the dirt road to Zach’s house, and the doctor had to borrow a Jeep from a filling station.

the century turned. Stacy courted her, married her, and moved over from Troublesome Creek to make a living in timber on her Zach took the doctor even farther up Copperhead Hollow to see his Aunt Bessie Fugate, who was blue. Bessie had an iron daddy’s land.

pot of clothes boiling in her front yard, but she graciously allowed the doctor to draw some of her blood.

Luna has been dead nearly 20 years now, but her widower survives. John Stacy still lives on Lick Branch of Ball Creek.

“So I brought back the new blood and set up my enzyme assay,” Cawein continued. “And by God, they didn’t have the

His two room log cabin sits in the middle of Laurel Fork Hollow. Luna is buried at the top of the hollow. Stacy’s son has built a enzyme diaphorase. I looked at other enzymes and nothing was wrong with them. So I knew we had the defect defined.’’

modern house next door, but the old logger won’t hear of leaving the cabin he built with timber he personally cut and hewed for Just like the Alaskans, their blood had accumulated so much of the blue molecule that it overwhelmed the red of normal Luna and their 13 children.

hcmoglobin that shows through as pink in the skin of most Caucasians.

Stacy recalls that his father-inlaw, Levy Fugate, was “part of the family that showed blue. All them old fellers way back then Once he had the enzyme deficiency isolated, methyleneblue sprang to Cawein’s mind as the “perfectly obvious” antidote.

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 Some of the blue people thought the doctor was slightly addled for suggesting that a blue dye could turn them pink. But Cawein by that name the blue Fugates. It run in that generation who lived up and down Ball [Creek].”

knew from earlier studies that the body has an alternative method of converting methemoglobin back to normal. Activating it

“They looked like anybody else, ‘cept they had the blue color,” Stacy says, sitting in a chair in his plaid flannel shirt and requires adding to the blood a substance that acts as an “electron donor.” Many substances do this, but Cawein chose methylene 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 blue because it had been used successfully and safely in other cases and because it acts quickly.

caused it.”

Cawein packed his black bag and rounded up Nurse Pendergrass for the big event. They went over to Patrick and Rachel

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 Ritchie’s house and injected each of them with 100 milligrams of methylene blue.

stares steadfastly ahead. It would be hard to doubt this gracious man except that you can’t find another person who knew Luna

‘’Within a few minutes. the blue color was gone from their skin,” the doctor said. “For the first time in their lives, they were who doesn’t remember her as being blue.

pink. They were delighted.”

“The bluest Fugates I ever saw was Luna and her kin,” says Carrie Lee Kilburn, a nurse who works at the rural medical center

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

called Homeplace Clinic. “Luna was bluish all over. Her lips were as dark as a bruise. She was as blue a woman as I ever saw.”

The doctor gave each blue family a supply of methylene blue tablets to take as a daily pill. The drug’s effects are temporary, Luna Stacy possessed the good health common to the blue people, bearing at least 13 children before she died at 84. The as methylene blue is normally excreted in the urine. One day, one of the older mountain men cornered the doctor. “I can see that clinic doctors only saw her a few times in her life and never for anything serious.

old blue running out of my skin,” he confided.

As coal mining and the railroads brought progress to Kentucky, the blue Fugates started moving out of their communities Before Cawein ended his study of the blue people, he returned to the mountains to patch together the long and twisted journey and marrying other people. The strain of inherited blue began to disappear as the recessive gene spread to families where it was of Martin Fugate’s recessive gene. From a history of Perry County and some Fugate family Bibles listing ancestors, Cawein has unlikely to be paired with a similar gene.

constructed a fairly complete story.

Benjamin Stacy is one of the last of the blue Fugates. With Fugate blood on both his mother’s and his father’s side, the boy Martin Fugate was a French orphan who emigrated to Kentucky in 1820 to claim a land grant on the wilderness banks of

could have received genes for the enzyme deficiency from either direction. Because the boy was intensely blue at birth but then Troublesome Creek. No mention of his skin color is made in the early histories of the area, but family lore has it that Martin recovered his normal skin tones, Benjamin is assumed to have inherlted only one gene for the condition. Such people tend to be himself was blue.

very blue only at birth, probably because newborns normally have smaller amounts of diaphorase. The enzyme eventually builds The odds against it were incalculable, but Martin Fugate managed to find and marry a woman who carried the same recessive to normal levels in most children and to almost normal levels in those like Benjamin, who carry one gene.

gene. Elizabeth Smith, apparently, was as pale-skinned as the mountain laurel that blooms every spring around the creek hollows.

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