Blue people of Kentucky: Story of people with the mysterious blue gene

Medical experts will tell you that having blue skin is an alarming symptom of heart or lung disease. The blue people of Kentucky are a different matter. For years, these people were a medical mystery until Madison Cawein III, a haematologist, made his accurate diagnosis.

For 200 years, the Fugate family, popularly known as the blue family of Kentucky were a small race in Kentucky, located in the U.S. state of West Virginia. Their glaring blue colour meant that they were isolated.

So, who are the blue people of Kentucky? Where are they now? They still exist and to know more about these peculiar people, continue reading.

Table of Content hide 1Who are the blue people of Kentucky? 2What happened to the blue people of Kentucky? 3The blue people of Kentucky now 4Conclusion

Who are the blue people of Kentucky?

The Fugates, also known as the blue people of Kentucky, is a family who has been living in the hills of West Virginia since the 19th century. This family has an ultra-rare blood disorder known as methemoglobinemia, which turns their skin blue.

The origin of the blue people of Kentucky can be traced back to a man named Martin Fugate, a French orphan, who had married Elizabeth Smith and settled in Troublesome Creek, a settlement near Hazard, Kentucky, around 1820. The couple were carriers of the recessive methemoglobinemia (met-H) gene. They had seven children and four of them inherited the blue skin from the met-H blood disorder.

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There were no railroads, which made it difficult to get out. So, the Fugates married their cousins in nearby settlements and there was inbreeding. This means that the met-H gene was passed from one generation to another.

Methemoglobinemia or met-H is a blood disorder which is caused by an abnormal amount of methemoglobin – a form of haemoglobin. Haemoglobin is responsible for distributing oxygen to the body. Without oxygen, the heart, brain and muscles will stop working, leading to death.

People with methemoglobinemia often have purple lips and blue skin and their blood is “chocolate coloured” because it is not oxygenated, according to Dr. Ayalew Tefferi, a haematologist from Minnesota’s Mayo Clinic. The disorder can either be inherited – as in the case of the Fugates – or caused by exposure to certain drugs and chemicals, such as anaesthetic drugs like benzocaine and xylocaine. Also, certain antibiotics, including dapsone and chloroquine, as well as meat additives like the carcinogen benzene and nitrites, can cause blue skin.

What happened to the blue people of Kentucky?

Although having blue skin is a result of a genetic disorder, most of the blue people of Kentucky lived long and healthy lives. The ” bluest” of the Fugates, Luna Stacy, had 13 children and lived to age 84. Descendants Martin Fugate with blue skin continued to live in Troublesome Creek, into the 20th century.

The story of the blue people of Kentucky remained a medical history until the 1960s when Dr. Cawein made the breakthrough discovery. The haematologist, who worked at the University of Kentucky’s Lexington Medical Clinic at the time, first heard the rumours and went looking for the blue people. He found a nurse, Ruth Pendergrass at an American Heart Association clinic in the town of Hazard. Pendergrass told him about 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,” Pendergrass said.

“It like scared me to death. She looked like she was having a heart attack. I just knew that the patient would die right there in the health department, but she wasn’t at all alarmed. She told me that her family was the blue Combses living on Ball Creek. She was a sister to one of the Fugate women.”

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Both Cawein and Pendergrass found more families with blue skin and mapped a family tree and took blood samples. According to a report on ABC News, Dr. Cawein had suspected methemoglobinemia as the cause of the blue skin. He concluded after uncovering a 1960 report in the Journal of Clinical Investigation by Dr. E. M. Scott. Dr. Scott, who worked in public health at the Arctic Research Center in Anchorage, had written about seeing a recessive genetic trait among Alaskans that turned their skin blue. The report suggested that there was inbreeding because a person would have to inherit two genes – one from each parent – to get the disorder.

Scott also speculated that the blue people lacked the enzyme diaphorase in their red blood cells. All the Fugates that Cawein tested had the enzyme deficiency, just like the Alaskans Scott had observed. Their blood had accumulated so much of the blue molecule that it overpowered the red haemoglobin that turn most Caucasians pink.

The blue people of Kentucky now

Coal mining arrived in Kentucky in 1912. So, the Fugates were able to leave Troublesome Creek and settle in other places. They began to marry other people and “the strain of inherited blue began to disappear as the recessive gene spread to families where it was unlikely to be paired with a similar gene”, the Science journal article explains.

The last known Fugate was Benjamin “Benjy” Stacy but he lost the “blue” colour by the age of seven, which suggests that he received his “blue” genes from just one parent.

According to Daily Mail, another surviving member of the Fugates’ said that the ailment persists in the family line to this day after interbreeding in the 19th century and early 20th century helped keep it alive. Hazel Fugate says that her husband, 69-year-old Gary, a descendant of Martin’s, suffers from methemoglobinemia and that ‘some days, he’s bluer than others’. Their son also had methemoglobinemia at birth, but he grew out of it by the age of five while their granddaughter also had it for just a few months.

Conclusion

This is the story of the blue people of Kentucky. These are normal people except that they had blue skin. Based on the story, they would have been normal-coloured had there not been inbreeding since their condition was a result of a recessive genetic defect.

While having blue skin is usually a sign of a medical emergency, it is not a problem for the Fugates. They have and continue to live for long, just like everyone else.

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