The Blue Family of Kentucky
The Fugate Family
In an isolated area of Kentucky’s countryside known as Troublesome Creek, there lived a real family with blue skin. They were known by many names, The Blue Family of Kentucky, The Fugate Family, The Blue Combses, The Huntsville subgroup, and the Blue People of Troublesome Creek.
These Appalachian families have been passing on a hereditary blood disorder that is so very rare that it turns their skin a shocking shade of blue for more than a century.
Because they were ashamed of the bluish tint of their skin, the families isolated themselves even farther from society, which made the condition even worse.
They were cut off from interaction with the larger population, so they married relatives who were already closely linked to them, such as cousins, aunts, and other relatives. This dramatically increased the chances of acquiring the illness.
If you select an individual at random from the population, the likelihood of that individual carrying this gene is somewhere between one in 100,000 and not even that many.
On the other hand, the odds are one in eight if you marry your cousin. The danger goes way up if you’re exchanging blood.
Two Strangers Lose The Genetic Lottery
In the year 1820, Martin Fugate made his way to the uncharted territory on the frontier of Kentucky. He was an orphan in France who had no knowledge of his family history.
According to urban legend, Martin himself may have had a blue tinge to his complexion, although it was not as dark blue as the hue of the Fugates’ skin in later generations.
Martin wed a red-headed American woman named Elizabeth Smith, and the couple established property on the banks of Troublesome Creek close to Hazard County in the Commonwealth of Kentucky.
Elizabeth’s complexion was very pale and appeared practically see-through. What neither Sarah nor Martin could have known is that they both possessed the recessive gene for a rare inherited blood condition known as methemoglobinemia.
Neither of them could have been aware of this fact.
As a result of the fact that rail lines and paved roads did not reach Troublesome Creek for almost a century, the blue recessive gene was passed down through generations of the Fugate family and families living in the surrounding area.
For almost 200 years, the Kentucky Blues have been mostly sealed from the outside world, having passed down their blue skin generation after generation in isolated pockets of the Kentucky countryside.
These individuals became known collectively as the blue people of Kentucky.
What Caused Their Blood to Turn Blue?
Methemoglobinemia is a disorder that affects the blood rather than the skin. It has nothing to do with the amino acid melanin, which is responsible for the darker skin tones that certain people have.
For the Fugates, an excess amount of blue Methemoglobin in their blood turned their skin blue. A recessive genetic condition caused the blue members to lack this enzyme; since their methemoglobin was not convertible, a high volume built up on the blue Fugates, before they were born, giving them an extremely noticeable blue colour on their skin. In this case, the inherited diaphorase deficiency NADH-cytochrome B5 reductase (methemoglobin reductase), diaphorase, caused members of a single family to develop blue skin.
People who have methemoglobinemia have skin that looks blue because the veins just under the surface of their skin are filled with blood that has a dark blue colour.
If anyone paid attention during biology class in high school, one might recall that the reason red blood cells are packed with proteins called haemoglobin is that blood is a red colour due to this.
The iron atom that is found in the molecule known as heme is what gives haemoglobin its characteristic red hue. Because of this iron atom’s ability to form bonds with oxygen, red blood cells are responsible for transporting oxygen throughout the body.
In patients who have methemoglobinemia, the colour of their blood changes from red to blue as a result of oxygen, or more specifically, the absence of oxygen.
Because of a defective gene, their bodies produce an extremely uncommon type of haemoglobin known as methemoglobin, which is incapable of forming a link with oxygen.
If sufficient amounts of this defective kind of haemoglobin are infected in the blood, the colour of the blood will shift from red to an almost purple-ish-dark blue. Different members of the Fugate family displayed variable levels of the gene’s expression.
In the case of the Fugates, different members of the family displayed variable levels of expression of the gene. People whose blood included a larger percentage of methemoglobin appeared vivid blue from head to toe when exposed to cold weather, in contrast to those whose blood contained a lesser concentration of the pigment.
This disorder is still present today, and in fact, a person’s skin with this condition will often look blue-tinged. For over a century, families in Appalachia have passed on a genetic blood condition, an extremely rare one, that causes their skin to become an unnerving shade of blue.
Is Blue Skin Curable?
Methemoglobinemia is a very uncommon hereditary disorder that can be remedied with the use of a single oral medication. The disease did not appear to be associated with any particular health issues.
Although it was obvious that the condition was genetic, reading stories of hereditary methemoglobinemia in isolated Inuit cultures in Alaska, where people married within their own families, provided scientists with a crucial piece of information.
A lack of an enzyme that transformed methemoglobin into haemoglobin was identified as the root cause of the issue that plagued the Inuit population by scientific researchers.
Scientists came to the conclusion after conducting research on the issue that they could convert methemoglobin to haemoglobin in the absence of the enzyme.
All that was required was a chemical that could give a free electron to the methemoglobin, which would then enable the methemoglobin to form a connection with oxygen.
Methylene blue, a popular type of dye, turned out to be the answer, which was really peculiar.
After injecting the subjects with methylene blue, they observed an eased in their symptoms and reduced the blue colouring of their skin.
What Happened to The Blue People of Troublesome Creek?
During the middle of the 20th century, when young people began leaving the farms that were located in the area around Troublesome Creek, they took their recessive blue genes with them.
As coal mining came to Kentucky in 1912, and the Fugates moved out of Troublesome Creek, the blue folks began to fade away. Most descendants of the Fugates have lost the blue colouration, the hue still appearing on their skin when cold or reddened by anger.
The number of kids who were born with blue cheeks decreased with time, and those who were given a tablet containing methylene blue once daily helped restore the pink colour to their faces.
There are, however, additional ways to develop blue skin without inheriting it from their parents. A person may develop methemoglobinemia as a result of an adverse reaction to a topical analgesic medication, such as benzocaine or xylocaine.
And in at least one well-known instance, a man’s skin turned a permanent shade of blue as a result of him consuming an excessive amount of colloidal silver pills and applying colloidal silver cream to his skin the condition is called argyria or silver poisoning.
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