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Archive for February, 2012

Well, I don’t know about all of those folks that ventured to the South to escape cold weather this winter, but you perhaps should have saved your money and stayed home.  Todays temp where I live was 68 degrees and nice.  Of course I am guessing that is warm down there, wherever that might be.  Whatever your spring and summer plans are, I hope you are considering attending the FGHS Alumni reunion and Fort Gay Heritage Day on Labor Day weekend this year.  Planning will begin in April and if you would like to have a part in planning the event, just get in touch with suggestions or plan on attending our first alumni board meeting sometime in April.

I had the good fortune of being in the Fort Gay area a couple of weeks ago doing some  traveling around with Jarrett Peters and visiting several local cemeteries.  There is so much history among all of those old tombstones scattered about in the cemeteries.  Jarrett, Dan Watts and a few others are attempting to create a history of the Fort Gay and Bartram cemeteries and bring about a perpetual care program for the two.  You will probably be hearing more about this from Jarrett or Dan.

Fort those who have struggled in the past to obtain housing for the Labor Day weekend there is GOOD NEWS.  There is a brand new motel now open in Louisa.  It is a Best Western and is located across U.S. Route 23 from the Super 8 motel.  I stopped by and it appears to be an excellent facility with very nice rooms plus a full breakfast is provided with your room.  I am sure it will fill rapidly for that Labor Day weekend so I would advise making a reservation either there or at the Super 8 at your earliest opportunity.  The phone number is 606 638 3420.  I checked the Best Western web site on-line and it indicated that the motel was not available for that weekend but I believe that information is incorrect.  I would suggest you call the motel direct at the above number.

While at Fort Gay, we went by the High School athletic field and site preparation is underway for the new middle school building.  I don’t know what the target date if for completion but I have seen the plans and it is certainly going to be a nice addition to the town of Fort Gay.  It does appear that we will have the old FGHS building available for the reunion banquet this year.  After 2012 I suppose we will just have to wait and see.

 

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Upon moving to Kentucky in the early 1960’s and traveling all of Eastern Kentucky and dealing with the medical community, I heard stories of the “Blue People” of Eastern Kentucky.  I had a county health officer physician friend tell me of this oddity and  invitedd me to travel his circuit through the hollows of his county and he would introduce me to some of them.  That never happened and over time I had forgotten it all untill today.   I read the following narrative of the occurrence and it’s cause.  I found the story rather fascinating and thought perhaps some of you reading this might also find it so.

Many of us who grew up in Appalachia can understand the seclusion that the isolated hollows and communities provided and were able to keep their secrets over the years.  Not secret by intent, but secret because of the geography and isolation that the rural areas provided.  I can remember many terms, words, and practices used by my father that I have not heard in any other area in which I have spent my life.  Search your memories and I am sure you will also remember these things.

Benjamin “Benjy” Stacy so frightened maternity doctors with the color of his skin — “as Blue as Lake Louise” — that he was rushed just hours after his birth in 1975 to University of Kentucky Medical Center.

As a transfusion was being readied, the baby’s grandmother suggested to doctors that he looked like the “blue Fugates of Troublesome Creek.” Relatives described the boy’s great-grandmother Luna Fugate as “blue all over,” and “the bluest woman I ever saw.”

In an unusual story that involves both genetics and geography, an entire family from isolated Appalachiawas tinged blue. Their ancestral line began six generations earlier with a French orphan, Martin Fugate who settled in Eastern Kentucky.

Doctors don’t see much of the rare blood disorder today, because mountain people have dispersed and the family gene pool is much more diverse.

But the Fugates’ story still offers a window into a medical mystery that was solved through modern genetics and the sleuth-like energy of Dr. Madison Cawein III, a hematologist at theUniversityof Kentucky’s Lexington Medical Clinic.

Cawein died in 1985, but his family charts and blood samples led to a sharper understanding of the recessive diseases that only surface if both parents carry a defective gene.

Fugate’s great-great-great-great grandson Benjamin “Benjy” Stacy so frightened maternity doctors when he was born in 1975, that they rushed him to a University of Kentucky medical clinic.

As a transfusion was being readied, Benjy’s grandmother suggested to doctors that the boy looked like the “blue Fugates of Troublesome Creek.” Relatives described the boy’s great-grandmother Luna Fugate as “blue all over” and “the bluest woman I ever saw.”

The most detailed account, “Blue People of Troublesome Creek,” was published in 1982 by the University of Indiana’s Cathy Trost, who described Benjy’s skin as “almost purple.”

The Fugate progeny had a genetic condition called methemoglobinemia, which was passed down through a recessive gene and blossomed through intermarriage.

“It’s a fascinating story,” said Dr. Ayalew Tefferi, a hematologist from Minnesota’s Mayo Clinic. “It also exemplifies the intersection between disease and society, and the danger of misinformation and stigmatization.”

Methemoglobinemia is a blood disorder in which an abnormal amount of methemoglobin — a form of hemoglobin — is produced, according to the National Institutes for Health. Hemoglobin is responsible for distributing oxygen to the body and without oxygen, the heart, brain and muscles can die.

In methemoglobinemia, the hemoglobin is unable to carry oxygen and it also makes it difficult for unaffected hemoglobin to release oxygen effectively to body tissues. Patients’ lips are purple, the skin looks blue and the blood is “chocolate colored” because it is not oxygenated, according to Tefferi.

“You almost never see a patient with it today,” he said. “It’s a disease that one learns about in medical school and it is infrequent enough to be on every exam in hematology.”

The disorder can be inherited, as was the case with the Fugate family, or caused by exposure to certain drugs and chemicals such as anesthetic drugs like benzocaine and xylocaine. The carcinogen benzene and nitrites used as meat additives can also be culprits, as well as certain antibiotics, including dapsone and chloroquine.

The genetic form of methemoglobinemia is caused by one of several genetic defects, according to Tefferi. The Fugates probably had a deficiency in the enzyme called cytochrome-b5 methemoglobin reductase, which is responsible for recessive congenital methemoglobinemia.

Normally, people have less approximately 1 percent of methemoglobin, a type of hemoglobin that is altered by being oxidized so is useless in carrying oxygen in the blood. When those levels rise to greater than 20 percent, heart abnormalities and seizures and even death can occur.

But at levels of between 10 and 20 percent a person can develop blue skin without any other symptoms. Most of blue Fugates never suffered any health effects and lived into their 80s and 90s.

“If you are between 1 percent and 10 percent, no one knows you have an abnormal level and this might be the case in a lot of unsuspecting patients,” he said.

Many other recessive gene diseases, such as sickle-cell anemia, Tay Sachs and cystic fibrosis can be lethal, he said.

“If I carry a bad recessive gene with a rare abnormality and married, the child probably wouldn’t be sick, because it’s very rare to meet another person with the [same] bad gene and the most frequent cause therefore is in-breeding,” Tefferi said.

Such was the case with the Fugates.

Martin Fugate came to Troublesome Creek fromFrancein 1820 and family folklore says he was blue. He married Elizabeth Smith, who also carried the recessive gene. Of their seven children, four were reported to be blue.

There were no railroads and few roads outside the region, so the community remained small and isolated. The Fugates married other Fugate cousins and families who lived nearby, with names like Combs, Smith, Ritchie and Stacy.

Benjy’s father, Alva Stacy showed Trost his family tree and remarked, “If you’ll notice — I’m kin to myself,” according to Trost.

One of Martin and Elizabeth Fugate’s blue boys, Zachariah, married his mother’s sister. One of their sons, Levy, married a Ritchie girl and had eight children, one of them Luna. Luna married John E. Stacy and they had 13 children.

Benjy descended from the Stacy line.

Modern Fugates Still in Kentucky

ABCNews.com was unable to determine if Benjamin Stacy is still alive — he would be 37 today. Trost writes that he eventually lost the blue tint to his skin, but as a child his lips and fingernails still got blue when he was angry or cold.

His mother Hilda Stacy, who is 56, appears to still live in Hazard,Ky., but did not answer calls to her home. Other relatives are scattered throughout Virginia and Arkansas.

Most of what scientists know about the family was discovered by Cawein, the grandson of Kentucky’s poet laureate, who had done pioneering research on L-dopa as a treatment for Parkinson’s disease.

Later in 1965 he was famous for another reason. His wife was murdered by chemical poisoning, but no one was ever indicted.

Cawein heard rumors about the Fugates while working at hisLexingtonclinic and set off “tromping around the hills looking for blue people,” according to Trost’s account.

At an American Heart Association clinic in the town ofHazard, Cawein found a nurse, Ruth Pendergrass, and she was willing to assist. She remembered a dark blue woman who had come to the county health department on a frigid afternoon seeking a blood test.

“Her face and her fingernails were almost indigo blue,” she told Trost. “It like 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.”

More families were found — Luke Combs, and Patrick and Rachel Ritchie, who were “bluer’n hell” and embarrassed by their skin color.

Cawein and Pendergrass began to ask questions — “Do you have any relatives who are blue?” — and mapped a family tree and took blood samples.

The doctor suspected methemoglobinemia and uncovered a 1960 report in the Journal of Clinical Investigation. Dr. E. M. Scott, who worked in public health at theArcticResearchCenterinAnchorage, had seen a recessive genetic trait among Alaskans that turned their skin blue.

That suggested an inbred line that had been passed from generation to generation. To get the disorder, a person would have to inherit two genes — one from each parent. When both parents have the trait, their children have a 25 percent chance of getting the disorder.

Scott speculated these people lacked the enzyme diaphorase in their red blood cells. Normally diaphorase converts methemoglobin back to hemoglobin.

All of the blue Fugates he tested had the enzyme deficiency, just like the Alaskans Scott had observed.

Their blood had accumulated so much of the blue molecule that it over-powered the red hemoglobin that normally turns skin pink in most Caucasians.

The bluest of the bunch was Luna, and she lived a healthy life, bearing 13 children before she died at the age of 84.

As coal mining arrived inKentuckyin 1912 and the Fugates moved outside of Troublesome Creek, the blue people began to disappear.

Doctors say Benjy likely carried only one gene for methemoglobinemia, because he eventually had normal skin tones, and the likelihood of him marrying a woman with the same recessive gene would have been small.

By the time reports appeared in the media on the disorder, the Stacy family was upset with insinuations about in-breeding that fed into stereotypes of backwoodsAppalachia.

“There was a pain not seen in lab tests,” wrote Trost. “That was the pain of being blue in a world that is mostly shades of white to black.”

Benjamin “Benjy” Stacy so frightened maternity doctors with the color of his skin — “as Blue asLake Louise” — that he was rushed just hours after his birth in 1975 to University of Kentucky Medical Center.

As a transfusion was being readied, the baby’s grandmother suggested to doctors that he looked like the “blue Fugates of Troublesome Creek.” Relatives described the boy’s great-grandmother Luna Fugate as “blue all over,” and “the bluest woman I ever saw.”

In an unusual story that involves both genetics and geography, an entire family from isolatedAppalachiawas tinged blue. Their ancestral line began six generations earlier with a French orphan, Martin Fugate who settled inEastern Kentucky.

Doctors don’t see much of the rare blood disorder today, because mountain people have dispersed and the family gene pool is much more diverse.

But the Fugates’ story still offers a window into a medical mystery that was solved through modern genetics and the sleuth-like energy of Dr. Madison Cawein III, a hematologist at theUniversityofKentucky’s Lexington Medical Clinic.

Cawein died in 1985, but his family charts and blood samples led to a sharper understanding of the recessive diseases that only surface if both parents carry a defective gene.

Fugate’s great-great-great-great grandson Benjamin “Benjy” Stacy so frightened maternity doctors when he was born in 1975, that they rushed him to a University of Kentucky medical clinic.

As a transfusion was being readied, Benjy’s grandmother suggested to doctors that the boy looked like the “blue Fugates of Troublesome Creek.” Relatives described the boy’s great-grandmother Luna Fugate as “blue all over” and “the bluest woman I ever saw.”

The most detailed account, “Blue People of Troublesome Creek,” was published in 1982 by theUniversity ofIndiana’s Cathy Trost, who described Benjy’s skin as “almost purple.”

The Fugate progeny had a genetic condition called methemoglobinemia, which was passed down through a recessive gene and blossomed through intermarriage.

“It’s a fascinating story,” said Dr. Ayalew Tefferi, a hematologist fromMinnesota’s Mayo Clinic. “It also exemplifies the intersection between disease and society, and the danger of misinformation and stigmatization.”

Methemoglobinemia is a blood disorder in which an abnormal amount of methemoglobin — a form of hemoglobin — is produced, according to the National Institutes for Health. Hemoglobin is responsible for distributing oxygen to the body and without oxygen, the heart, brain and muscles can die.

In methemoglobinemia, the hemoglobin is unable to carry oxygen and it also makes it difficult for unaffected hemoglobin to release oxygen effectively to body tissues. Patients’ lips are purple, the skin looks blue and the blood is “chocolate colored” because it is not oxygenated, according to Tefferi.

“You almost never see a patient with it today,” he said. “It’s a disease that one learns about in medical school and it is infrequent enough to be on every exam in hematology.”

The disorder can be inherited, as was the case with the Fugate family, or caused by exposure to certain drugs and chemicals such as anesthetic drugs like benzocaine and xylocaine. The carcinogen benzene and nitrites used as meat additives can also be culprits, as well as certain antibiotics, including dapsone and chloroquine.

The genetic form of methemoglobinemia is caused by one of several genetic defects, according to Tefferi. The Fugates probably had a deficiency in the enzyme called cytochrome-b5 methemoglobin reductase, which is responsible for recessive congenital methemoglobinemia.

Normally, people have less approximately 1 percent of methemoglobin, a type of hemoglobin that is altered by being oxidized so is useless in carrying oxygen in the blood. When those levels rise to greater than 20 percent, heart abnormalities and seizures and even death can occur.

But at levels of between 10 and 20 percent a person can develop blue skin without any other symptoms. Most of blue Fugates never suffered any health effects and lived into their 80s and 90s.

“If you are between 1 percent and 10 percent, no one knows you have an abnormal level and this might be the case in a lot of unsuspecting patients,” he said.

Many other recessive gene diseases, such as sickle cell anemia, Tay Sachs and cystic fibrosis can be lethal, he said.

“If I carry a bad recessive gene with a rare abnormality and married, the child probably wouldn’t be sick, because it’s very rare to meet another person with the [same] bad gene and the most frequent cause therefore is in-breeding,” Tefferi said.

Such was the case with the Fugates.

Martin Fugate came to Troublesome Creek fromFrancein 1820 and family folklore says he was blue. He married Elizabeth Smith, who also carried the recessive gene. Of their seven children, four were reported to be blue.

There were no railroads and few roads outside the region, so the community remained small and isolated. The Fugates married other Fugate cousins and families who lived nearby, with names like Combs, Smith, Ritchie and Stacy.

Benjy’s father, Alva Stacy showed Trost his family tree and remarked, “If you’ll notice — I’m kin to myself,” according to Trost.

One of Martin and Elizabeth Fugate’s blue boys, Zachariah, married his mother’s sister. One of their sons, Levy, married a Ritchie girl and had eight children, one of them Luna. Luna married John E. Stacy and they had 13 children.

Benjy descended from the Stacy line.

Modern Fugates Still in Kentucky

ABCNews.com was unable to determine if Benjamin Stacy is still alive — he would be 37 today. Trost writes that he eventually lost the blue tint to his skin, but as a child his lips and fingernails still got blue when he was angry or cold.

His mother Hilda Stacy, who is 56, appears to still live in Hazard,Ky., but did not answer calls to her home. Other relatives are scattered throughoutVirginiaandArkansas.

Most of what scientists know about the family was discovered by Cawein, the grandson of Kentucky’s poet laureate, who had done pioneering research on L-dopa as a treatment for Parkinson’s disease.

Later in 1965 he was famous for another reason. His wife was murdered by chemical poisoning, but no one was ever indicted.

Cawein heard rumors about the Fugates while working at hisLexingtonclinic and set off “tromping around the hills looking for blue people,” according to Trost’s account.

At an American Heart Association clinic in the town ofHazard, Cawein found a nurse, Ruth Pendergrass, and she was willing to assist. She remembered a dark blue woman who had come to the county health department on a frigid afternoon seeking a blood test.

“Her face and her fingernails were almost indigo blue,” she told Trost. “It like 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.”

More families were found — Luke Combs, and Patrick and Rachel Ritchie, who were “bluer’n hell” and embarrassed by their skin color.

Cawein and Pendergrass began to ask questions — “Do you have any relatives who are blue?” — and mapped a family tree and took blood samples.

The doctor suspected methemoglobinemia and uncovered a 1960 report in the Journal of Clinical Investigation. Dr. E. M. Scott, who worked in public health at theArcticResearchCenterinAnchorage, had seen a recessive genetic trait among Alaskans that turned their skin blue.

That suggested an inbred line that had been passed from generation to generation. To get the disorder, a person would have to inherit two genes — one from each parent. When both parents have the trait, their children have a 25 percent chance of getting the disorder.

Scott speculated these people lacked the enzyme diaphorase in their red blood cells. Normally diaphorase converts methemoglobin back to hemoglobin.

All of the blue Fugates he tested had the enzyme deficiency, just like the Alaskans Scott had observed.

Their blood had accumulated so much of the blue molecule that it over-powered the red hemoglobin that normally turns skin pink in most Caucasians.

The bluest of the bunch was Luna, and she lived a healthy life, bearing 13 children before she died at the age of 84.

As coal mining arrived inKentuckyin 1912 and the Fugates moved outside of Troublesome Creek, the blue people began to disappear.

Doctors say Benjy likely carried only one gene for methemoglobinemia, because he eventually had normal skin tones, and the likelihood of him marrying a woman with the same recessive gene would have been small.

By the time reports appeared in the media on the disorder, the Stacy family was upset with insinuations about in-breeding that fed into stereotypes of backwoodsAppalachia.

“There was a pain not seen in lab tests,” wrote Trost. “That was the pain of being blue in a world that is mostly shades of white to black.”

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