The Blue Skin People of Kentucky

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

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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.

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

The Fender Benders

http://darkrose_42345.tripod.com

Sunsets and Fairydusts
(not a music site, my home page
http://darkrose_42345.tripod.com/ter...istsongwriter/

ORIGINAL MESSAGE:

--- In Shakti_Sadhana (AT) yahoogroups (DOT) com, "Eric Otto"
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.

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!!!




--- In Shakti_Sadhana (AT) yahoogroups (DOT) com, "Teresa"
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
>
> The Fender Benders
>
> http://darkrose_42345.tripod.com
>
> Sunsets and Fairydusts
> (not a music site, my home page
> http://darkrose_42345.tripod.com/ter...istsongwriter/