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The Blue Skin People of Kentucky

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Thought you might find this interesting. I grew up in Kentucky and

remember hearing stories about this when I was a child from my father

who was a pharmacist. He may have known the Dr. Madison Cawein

mentioned in the article. When I look at the pictures of Shakti, the

story of the blue skin people comes to mind.

 

EO

 

--------------------

 

 

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

 

Six generations after a French orphan named Martin Fugate settled on

the banks of eastern Kentucky's Troublesome Creek with his redheaded

American bride, his great-great-great great grandson was born in a

modern hospital not far from where the creek still runs.

 

The boy inherited his father's lankiness and his mother's slightly

nasal way of speaking.

 

What he got from Martin Fugate was dark blue skin. "It was almost

purple," his father recalls.

 

Doctors were so astonished by the color of Benjy Stacy's skin that

they raced him by ambulance from the maternity ward in the hospital

near Hazard to a medical clinic in Lexington. Two days of tests

produced no explanation for skin the color of a bruised plum.

 

A transfusion was being prepared when Benjy's grandmother spoke

up. "Have you ever heard of the blue Fugates of Troublesome Creek?"

she asked the doctors.

 

"My grandmother Luna on my dad's side was a blue Fugate. It was real

bad in her," Alva Stacy, the boy's father, explained. "The doctors

finally came to the conclusion that Benjy's color was due to blood

inherited from generations back."

 

Benjy lost his blue tint within a few weeks, and now he is about as

normal looking a seven-year-old boy as you could hope to find. His

lips and fingernails still turn a shade of purple-blue when he gets

cold or angry a quirk that so intrigued medical students after

Benjy's birth that they would crowd around the baby and try to make

him cry. "Benjy was a pretty big item in the hospital," his mother

says with a grin.

 

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.

 

They're known simply as the "blue people" in the hills and hollows

around Troublesome and Ball Creeks. Most lived to their 80s and 90s

without serious illness associated with the skin discoloration. For

some, though, there was a pain not seen in lab tests. That was the

pain of being blue in a world that is mostly shades of white to

black.

 

There was always speculation in the hollows about what made the blue

people blue: heart disease, a lung disorder, the possibility proposed

by one old-timer that "their blood is just a little closer to their

skin." But no one knew for sure, and doctors rarely paid visits to

the remote creekside settlements where most of the "blue Fugates"

lived until well into the 1950s. By the time a young hematologist

from the University of Kentucky came down to Troublesome Creek in the

1960s to cure the blue people, Martin Fugate's descendants had

multiplied their recessive genes all over the Cumberland Plateau.

 

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 is no stranger to eccentricities of the body. He helped

isolate an antidote for cholera, and he did some of the early work on

L-dopa, the drug for Parkinson's disease. But his first love, which

he developed as an Army medical technician in World War II, was

hematology. "Blood cells always looked so beautiful to me," he says.

 

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. His car gave out on the dirt road to Zach's

house, and the doctor had to borrow a Jeep from a filling station.

 

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 over- whelmed the red of normal hcmoglobin 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. Activating it requires adding to the blood a

substance that acts as an "electron donor." Many substances do this,

but 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.

 

Martin Fugate was a French orphan who emigrated to Kentucky in 1820

to claim a land grant on the wilderness banks of Troublesome 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. Elizabeth

Smith, apparently, was as pale-skinned as the mountain laurel that

blooms every spring around the creek hollows.

 

Martin and Elizabeth set up housekeeping on the banks of Troublesome

and began a family. Of their seven children, four were reported to be

blue.

 

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.

 

"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."

 

The railroad didn't come through eastern Kentucky until the coal

mines were developed around 1912, and it took another 30 or 40 years

to lay down roads along the local creeks.

 

Martin and Elizabeth Fugate's blue children multiplied in this

natural isolation tank. The marriage of one of their blue boys,

Zachariah, to his mother's sister triggered the line of succession

that would result in the birth, more than 100 years later, of Benjy

Stacy.

 

When Benjy was born with purple skin, his relatives told the

perplexed doctors about his great grandmother Luna Fugate. One

relative describes her as "blue all over," and another calls

Luna "the bluest woman I ever saw."

 

Luna's father, Levy Fugate, was one of Zachariah Fugate's sons. Levy

married a Ritchie girl and bought 200 acres of rolling land along

Ball Creek. The couple had eight children, including Luna.

 

A fellow by the name of John E. Stacy spotted Luna at Sunday services

of the Old Regular Baptist Church back before the century turned.

Stacy courted her, married her, and moved over from Troublesome Creek

to make a living in timber on her daddy's land.

 

Luna has been dead nearly 20 years now, but her widower survives.

John Stacy still lives on Lick Branch of Ball Creek. 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 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 Luna and their 13 children.

 

Stacy recalls that his father-inlaw, 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

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.

 

"The bluest Fugates I ever saw was Luna and her kin," says Carrie Lee

Kilburn, a nurse who works at the rural medical center 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."

 

Luna Stacy possessed the good health common to the blue people,

bearing at least 13 children before she died at 84. The clinic

doctors only saw her a few times in her life and never for anything

serious.

 

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.

 

Benjy 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 could have

received genes for the enzyme deficiency from either direction.

Because the boy was intensely blue at birth but then recovered his

normal skin tones, Benjy is assumed to have inherlted only one gene

for the condition. Such people tend to be very blue only at birth,

probably because newborns normally have smaller amounts of

diaphorase. The enzyme eventually builds to normal levels in most

children and to almost normal levels in those like Benjy, who carry

one gene.

 

Hilda Stacy (nee Godsey) is fiercely protective of her son. She gets

upset at all the talk of inbreeding among the Fugates. One of the

supermarket tabloids once sent a reporter to find out about the blue

people, and she was distressed with his preoccupation with

intermarriages.

 

She and her husband Alva have a strong sense of family. They sing in

the Stacy Family Gospel Band and have provided their children with a

beautiful home and a menagerie of pets, including horses.

 

"Everyone around here knows about the blue Fugates," says Hilda Stacy

who, at 26, looks more like a sister than a mother to her

children. "It's common. It's nothing.''

 

Cawein and his colleagues published their research on hereditary

diaphorase deficiency in the Archives of Internal Medicine (April,

1964) in 1964. He hasn't studied the condition for years. Even so,

Cawein still gets calls for advice. One came from a blue Flugate

who'd joined the Army and been sent to Panama, where his son was born

bright blue. Cawein advised giving the child methylene blue and not

worrying about it. Note: In this instance the reason for cyanosis was

not methemoglobinemia but Rh incompatibility. This information

supplied by John Graves whose uncle was the father of the child.

 

The doctor was recently 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."

 

References

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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I live in Kentucky now. I am 38, and from Muhlenberg County. My

great-grandmother was a blue lady. I always thought she was sick and

old. But when I read the explanation, it all makes so much sense. Now

I know why my fingersnails, fingers and toes turn blue when I get

cold. lol

My great-grandparents were raised in a coal mining hollar called

Skebo. The family all married first cousin's and the like.

I am so glad my grand parents moved to another state to bring in

some fresh blood.lol

I have uncles and aunts, that are first cousins now.

 

It's the land that time forgot, or at least left behind 25 years.

 

Your Kentucky Friend,

Teresa

 

<B>The Fender Benders<B>

 

http://darkrose_42345.tripod.com

 

<B>Sunsets and Fairydusts<B>

(not a music site, my home page

http://darkrose_42345.tripod.com/teresafordvocalistsongwriter/

 

<B>ORIGINAL MESSAGE:<B>

 

, "Eric Otto" <mkultra@f...>

wrote:

> Thought you might find this interesting. I grew up in Kentucky and

> remember hearing stories about this when I was a child from my

father

> who was a pharmacist. He may have known the Dr. Madison Cawein

> mentioned in the article. When I look at the pictures of Shakti,

the

> story of the blue skin people comes to mind.

>

> EO

>

> --------------------

>

>

>, 1964.

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Guest guest

Hi Teresa -

 

Gee, you know that having that problem and not knowing it could be a

real problem. My fingersnails went blue when I was in the hospital

some time ago and it's automatic for them to put you on oxygen. If

people don't know it, that could make for an interesting diagnosis if

one is cyanotic naturally.

 

My dad wondered if the aristocrats and royals when they were

called "blue bloods" if the condition might have been the reason

why. It is such an odd expression. With them it is mostly cousins

too to "keep up the blood lines" like happy thoroughbred horses.

Which always gets me wondering about if the aristocrats describe

themselves as horses and cattle, what do they think about the rest of

us? LOL Is the idea of the blue bloods something deep in our

unconscious that kind of shows up in the language?

 

Reading on Buddhist tantra, the author was talked about the yogini's

putting ashes over themselves and I think she thought that was the

origin of the blue skin idea. Being Americans we love scientific

explanations for everything. They're gods afterall and perhaps their

skin is blue.

 

Yes, I grew up in northern Kentucky near Cincinnati. I live in

Cincinnati now. Even in Campbell, Boone and Kenton counties there

were families that advised their children to find husbands and wives

in the next county to avoid marrying cousins. Other states make fun

of it but it can be a real problem in some of the "down counties" of

the state.

 

Your Ohio (kind of**) Friend,

 

Eric

 

PS Cyanosis is from the Latin from the Greek KyanOsis. The Osis

looks a lot like Isis doesn't it?

 

 

** I'll always be a Kentuckian!!!

 

 

 

 

, "Teresa" <teresarford>

wrote:

> I live in Kentucky now. I am 38, and from Muhlenberg County. My

> great-grandmother was a blue lady. I always thought she was sick

and

> old. But when I read the explanation, it all makes so much sense.

Now

> I know why my fingersnails, fingers and toes turn blue when I get

> cold. lol

> My great-grandparents were raised in a coal mining hollar called

> Skebo. The family all married first cousin's and the like.

> I am so glad my grand parents moved to another state to bring in

> some fresh blood.lol

> I have uncles and aunts, that are first cousins now.

>

> It's the land that time forgot, or at least left behind 25 years.

>

> Your Kentucky Friend,

> Teresa

>

> <B>The Fender Benders<B>

>

> http://darkrose_42345.tripod.com

>

> <B>Sunsets and Fairydusts<B>

> (not a music site, my home page

> http://darkrose_42345.tripod.com/teresafordvocalistsongwriter/

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