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Article turned oxygen info w/Nitrates & 4 rx's named

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I can't help it, I've just got to post this article, which for me turned into

oxygen info...What caught my interest in this article was the oxygen info

w/Nitrates, 2 anesthetic (benzocaine and xylocaine) drugs, 2 antibiotics

(dapsone and chloroquine).

Making it personal for me was I've been told has low natural oxygen

carrying abilities w/his blood (possibly genetic, possibly because of all the

toxins his body fights...the good news is that Immusist & oxygenated water like

the Kangen have helped some w/this). The fact that I react so poorly to nitrates

myself....I wonder if some of the " brain fog " (turning to a major stressful

histamine response of hives & w/enough that has included tonge swelling &

breathing problems)....may this just be the additional body stress from the

oxygen inhibitor properties these produce...hummmm. Either way, for my family

anyway, I think it's best I stay away from these items, including the rx's

named.

Statement of oxygen interaction info from the article (about 1/2 way into the

article): " 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. "

Article source (on Yahoo news today):

http://gma.yahoo.com/fugates-kentucky-skin-bluer-lake-louise-200247843--abc-news\

..html

Fugates of Kentucky: Skin Bluer than Lake Louise

By SUSAN DONALDSON JAMES | Good Morning America – 22 hours ago

" Benjy " 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 Appalachia was tinged blue. Their ancestral line began six

generations earlier with a French orphan, 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 the University of 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.

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 than about 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.

Fugate came to Troublesome Creek from France in 1820 and family folklore

says he was blue. He married , 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, , Ritchie and .

Benjy's father, Alva showed Trost his family tree and remarked, " If you'll

notice -- I'm kin to myself, " according to Trost.

One of and 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 E. and they had 13 children.

Benjy descended from the line.

Modern Fugates Still in Kentucky

ABCNews.com was unable to determine if 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 , 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 his Lexington clinic 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 of Hazard, 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 and 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. , who worked in public health

at the Arctic Research Center in Anchorage, 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.

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 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 in Kentucky in 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 family was

upset with insinuations about in-breeding that fed into stereotypes of backwoods

Appalachia.

" 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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