||January 19, 2006
by lyle e davis
The Blue People of
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
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
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,"
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
From: Benjamin Arnold
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
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
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
"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
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.
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
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
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!"
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.
THE BLUE PEOPLE OF TROUBLESOME
The story of an
Appalachian malady, an inquisitive doctor, and a paradoxical cure.
by Cathy Trost
©Science 82, November,
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.