The True Story of Kentucky’s Blue People: The Avatars of Real Life

By | May 18, 2023

Embarrassed by the shade of blue, families further withdrew from society, exacerbating the problem. Cut off from contact with the general population, they married cousins, aunts and other close relatives, greatly increasing their chances of inheriting the condition.

As scientists discovered in the 1960s, the mutation that makes skin look like Smurfs has a recessive gene, and it takes two people with the same gene to create one blue boy.

“If you take any random person in the population, maybe one in 100,000 would carry that gene, if that’s that many,” says Ricky Lewis, a science writer and author of the textbook Human Genetics: Concepts and Applications, now in its 13th edition. version of him. “But if you marry your cousin, the odds become one in eight. The danger skyrockets if you share blood.”

When two strangers lost the genetic lottery

Martin Fugatti arrived on the uninhabited frontier of Kentucky in 1820. He was an orphan from France who knew nothing of his ancestry. Legend has it that Martin himself may have had a blue tint to his skin, but not the deep blue skin color of later Fugatti.

Martin married a red-haired American woman named Elizabeth Smith, and the two formed a home on the banks of Troublesome Creek near Hazard County, Kentucky. Elizabeth had pale white, almost translucent skin. What neither she nor Martin could know was that they both carried the recessive gene for a rare inherited blood disorder called methemoglobinemia.

“The beginning of this story is so wild because Martin moved to Kentucky from Europe and married a complete stranger, a non-relative who just happened to have the same mutation,” says Lewis. “This is crazy”.

Martin and Elizabeth had seven children, four of whom were “deep blue” according to Fugatti family tradition.

Since railroad tracks and paved roads did not reach Troublesome Creek for nearly a century, the residual blue gene was passed down through generations of Fugatti families and neighboring families, who became known as “the blue people of Kentucky.”

Cause her blood turned blue

Methemoglobinemia is a blood disorder, not a skin disorder. It has nothing to do with melanin, the amino acid that gives people dark skin. In people with methemoglobinemia, the skin appears blue because the veins under the skin leak dark blue blood.

From high school biology, you may remember that blood is red because red blood cells are full of proteins called hemoglobin. Hemoglobin gets its red color from a compound called heme that contains an iron atom. This iron atom binds oxygen, so red blood cells circulate oxygen throughout the body.

Oxygen or lack of oxygen is what turns blood from red to blue in people with methemoglobinemia. A mutated gene causes your body to make a rare form of hemoglobin called methemoglobin, which can’t bind oxygen. If enough blood is “contaminated” with this defective type of hemoglobin, the blood turns from red to a dark blue, almost purple.

For the Fugattis, family members expressed the gene to varying degrees. If their blood had a lower concentration of methemoglobin, they might blush blue only in the cold, whereas people with higher concentrations of methemoglobin would be a deep blue from head to toe.

Is there a cure for blue skin?

Methemoglobinemia is one of the rare genetic conditions that can be treated with a simple pill.

The man who discovered the cure for methemoglobinemia was Madison Cawein III, a hematologist (blood doctor) at the University of Kentucky, who heard stories about “blue people” and went looking for samples in the 1960s.

Cawein got lucky when two siblings named Patrick and Rachel Ritchie walked into a Hazard County clinic. “They were bluer than hell,” Cawein said in an interview with Science 82 in 1982. “I started asking them questions: ‘Do you have any relatives that are blue?’ Then I sat down and we started filming the family.” He recalled that the Ritchie brothers were “really ashamed of being blue.” However, the disorder did not appear to cause any particular health problems.

The condition was clearly genetic, but the key for Cawein was reading reports of hereditary methemoglobinemia among isolated Inuit populations in Alaska, where blood relatives often intermarried. He knew the same was true in this isolated corner of Appalachia.

In Inuit communities, scientists had identified the problem, a deficiency in an enzyme that converts methemoglobin to hemoglobin. Studying the problem, Cawein realized that he could convert methemoglobin to hemoglobin without the enzyme. All that was needed was a substance that could “donate” a free electron to methemoglobin, allowing it to bind oxygen.

The solution, surprisingly, was a widely used dye called methylene blue. He injected the Ritchie brothers with 100 milligrams of the blue dye and didn’t have to wait long to see the results.

“Within a few minutes, the blue color was gone from their skin,” Cawein said. “For the first time in their lives, they were pink. They were excited.”

What happened to the blue people?

When the young began moving away from the farms around Troublesome Creek in the mid-20th century, they took their remaining blue genes with them. Over time, fewer and fewer babies were born blue, and those who were were given a pill of methylene blue once a day to restore the pink color to their cheeks.

But there are other ways to get blue skin without inheriting it. Methemoglobinemia can also be caused by reactions to certain topical pain relievers such as benzocaine and xylocaine.

And in at least one famous case, a man turned his skin permanently blue from consuming too many colloidal silver supplements and smearing his skin with colloidal silver cream (the condition is called argyria, or silver poisoning).

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