Blue Fugates: the rare blood disorder that turned the ‘Blue People of Kentucky’ blue

Who were the Blue People of Kentucky?

The origins of the Blue People of Kentucky go back to 1820, when a French immigrant named Martin Fugate came to the United States. Settling at Troublesome Creek, he married an American woman, Elizabeth Smith, and together they had seven children. Imagine their shock, however, when four of them were born blue.

There were no obvious health concerns and, otherwise, they developed normally. Yet they remained head-to-toe indigo for the rest of their lives.

That could have been the end of the story, were it not for the remoteness of the region. It did not exactly attract a diverse wave of settlers willing to make the difficult journey and endure the inhospitality there once they did.

That meant that marriage and inbreeding between a small number of families became common practice. Considering the shallow gene pool, it might come as no surprise that each new generation welcomed more blue members.

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It was only in 1960 – 140 years after Martin Fugate came to the US – that the strange condition became the subject of medical investigation. That was thanks to the efforts of a young haematologist at the University of Kentucky, Dr Madison Cawein, and a nurse named Ruth Pendergrass.

The problem would be to find one of the Blue People, with Troublesome Creek many hours away from Cawein’s base at the university. Then one day out of the blue, as it were, two blue-skinned siblings, Rachel and Patrick Ritchie, just happened to walk into the heart clinic where Pendergrass worked.

Why were the Blue People of Kentucky blue?

Since the blue-tinged families had no health issues – and had relatives who had lived to ripe old ages – Cawein suspected a rare blood disorder called methemoglobinemia, which affects how oxygen gets around the body. Essentially, there is not enough of a particular enzyme that turns methaemoglobin into oxygen-carrying haemoglobin.

By the slimmest of chances, both Martin Fugate and Elizabeth Smith carried the recessive gene. The average person has less than one per cent of methaemoglobin in their blood; the Blue People of Kentucky had between 10 and 20 per cent.

While not enough to be harmful, the disorder turned their blood a deep brown colour and gave their skin its blueish hue. The decades of inbreeding then let the disorder flourish.

Oddly enough, the treatment that Cawein devised to get rid of the blue skin was to put blue dye into the body. Within a matter of minutes of injecting Methylene Blue, which balanced out the methaemoglobin, the Ritchies’ skin began to turn pink. Tablets were then sufficient to keep the blue tinge at bay.

Who was the last Blue Person of Kentucky?

Gradually, the Blue People clan diminished, although there was one last appearance in 1975 when Benjamin ‘Benjy’ Stacy’ was born with purplish skin.

The boy, doctors were relieved to find out, was a descendent of Martin Fugate, and his skin eventually turned to a regular colour.

That said, whenever Benjy got upset, angry or cold, his lips and digits would temporarily turn blue – a remaining link to his peculiar family heritage.