The blue people of troublesome creek. Troublesome Creek 2023-01-06
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The Blue People of Troublesome Creek is a rare genetic condition that results in the affected individuals having blue-tinted skin. The condition is caused by a deficiency in the enzyme responsible for breaking down methemoglobin, a form of hemoglobin that carries oxygen in the blood. When the body is unable to break down methemoglobin, it accumulates in the blood and gives the skin a bluish tint.
The Blue People of Troublesome Creek were a family living in the Appalachian Mountains of Kentucky in the early 20th century. The family's blue skin was passed down through the maternal line, and several generations of the family were affected by the condition. The family lived in isolation in the mountains and were not discovered by the outside world until the 1930s.
The condition that caused the blue skin of the Blue People of Troublesome Creek is now known as methemoglobinemia, and it is a rare genetic disorder that affects only a small number of people worldwide. It is caused by a deficiency in the enzyme that breaks down methemoglobin, which results in the accumulation of methemoglobin in the blood. The condition is usually inherited and is caused by mutations in the gene that codes for the enzyme.
Symptoms of methemoglobinemia include a blue or grey tint to the skin, shortness of breath, fatigue, and a rapid heart rate. The condition can be treated with medications that help the body break down methemoglobin and increase the oxygen levels in the blood. However, if left untreated, methemoglobinemia can lead to serious health problems and even death.
The Blue People of Troublesome Creek were a fascinating and mysterious group of people who lived in isolation in the Appalachian Mountains. Their rare genetic condition gave them a unique appearance that set them apart from the rest of the world. Today, methemoglobinemia is a rare but treatable genetic disorder that affects only a small number of people. Despite the advances in medicine that have allowed for the treatment of methemoglobinemia, the Blue People of Troublesome Creek will always be remembered as a reminder of the mysteries of genetics and the human body.
The Science Behind The Mysterious Blue People Of Kentucky
If the condition were inherited as a recessive trait, it would appear most often in an inbred line. The family were embarrassed and discriminated against by their local community because of their skin color. The first thing I asked myself after I confirmed that it was real was how the heck have I not heard of this before? Luna has been dead nearly 20 years now, but her widower survives. And in fiction writing particularly, copyright infringement is hard to prove. Eventually, the recessive gene began to disappear once coal mining and the railroad opened the community to outsiders. The blood disorder is inherited as a simple recessive trait - meaning that to get the disorder, a person would have to inherit two genes, one from each parent.
Methemoglobin which is blue, is a nonfunctional form of the red hemoglobin that carries oxygen. Such people tend to be very blue only at birth, probably because newborns normally have smaller amounts of diaphorase. As the story goes, Martin Fugate, a French orphan, settled on the banks of eastern Kentucky's Troublesome Creek around 1820 to claim a land grant. It was a remarkable coincidence with a bizarre result: four of the seven Fugate children were born with bright blue skin that lasted their entire lives. There was just a big fear. Book is out of print, borrowed from Special Collections at the St. Scott also concluded that the condition was inherited as a simple recessive trait.
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. Such people tend to be very blue only at birth, probably because newborns normally have smaller amounts of diaphorase. The gene would most likely appear in an inbred line. Note: In this instance the reason for cyanosis was not methemoglobinemia but Rh incompatibility. Oh, yeah, and four of their kids were blue. This information supplied by John Graves whose uncle was the father of the child.
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They went over to Patrick and Rachel Ritchie's house and injected each of them with 100 milligrams of methylene 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. 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. Cawein decided to investigate the Blue people of Troublesome Creek. 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. Yet, to the surprise of his parents and the hospital's staff, Stacy inherits the family's distinctive blue coloring. There was a Zachariah Fugate who was married to a Mary Smith; both carried the gene which resulted in blue children.
Hemoglobin can be converted into a slightly different molecule called methemoglobin. Normally diaphorase converts methemoglobin back to hemoglobin. Of their seven children, four were reported to be blue. It was caused, Scott speculated, by an absence of the enzyme diaphorase from their red blood cells. This baby is just the devil to settle right now and I was wondering if they had anything would help? The year is 1820 and a French orphan called Martin Fugate has just arrived in Troublesome Creek, a remote and sleepy settlement in eastern Kentucky. Blue People Genealogy Not myth or legend; The Blue People of Kentucky were an isolated enclave of Appalachian people who lived with an embarrassing skin discoloration until a young hematologist took notice and found a solution. 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.
Stacy counts Fugate blood in his own veins. Days of testing provided no answers; but then Benjy's grandmother told the doctor a story about the "blue" Fugates. He was a French orphan who knew nothing of his lineage. The Blue People of Troublesome Creek The Blue People of Troublesome Creek seem to be normal, but there are many things that are hidden behind the blue skin. It is documented that Fugates were born in the colonies in the mid 1600's but the original progenitor has not been confirmed.
Luna is buried at the top of the hollow. Methemoglobinemia can also be caused by. Even so, Cawein still gets calls for advice. Cawein advised giving the child methylene blue and not worrying about it. Martin and Elizabeth had seven children, four being born blue; Zachariah, who was blue, married his mother's sister.
She told me that her family was the blue Combses who lived up on Ball Creek. Elizabeth Smith, apparently, was as pale-skinned as the mountain laurel that blooms every spring around the creek hollows. Some of the blue people thought the doctor was slightly addled for suggesting that a blue dye could turn them pink. All he needed was a substance that could "donate" a free electron to the methemoglobin, allowing it to bond with oxygen. There were several suspects, including abnormal hemoglobin formation and excessive vitamin K consumption, but blood tests eventually revealed the true culprit: The blue Fugates lacked the enzymediaphorase. This caused them to seek greater social isolation, which somewhat ironically, exacerbated the problem. It was real bad in her," Alva Stacy, the boy's father, explained.
