The Last Blue People: Birth Defect Causes Bright-Blue Skin

If you, like most Americans, grew up watching The Smurfs, you probably find the notion of blue people whimsical, or even amusing. While you may know that some people develop a bluish tint to their skin while experiencing severe heart problems or lung disease, you've probably never seen a healthy person who has had blue skin his entire life. As strange as it sounds, there have been dozens of recorded cases of hereditary methemoglobinemia-- a condition that causes people to be born with deep-blue or indigo skin.

The last person with this extremely rare condition, Benjy Stacy, was born in rural Kentucky in the early 1950s. Stacy, who was born with deep violet skin the color of a ripe plum, has only one gene for the condition. Stacy's great-grandmother, Luna Fugate, was as blue as a smurf, since she carried two genes for the condition-- inherited from her own parents, who were fourth cousins.

A report nearly thirty years ago described Ben Stacy's condition and family history. Ben Stacy was the last blue descendant of Martin Fugate, the patriarch of the family later to become known notoriously as the Blue Fugates of Kentucky. Fugate emigrated to rural Kentucky in 1820, in the wilderness surrounding Troublesome Creek on the Cumberland Plateau. It would be a century later before any roads or railways would enter this isolated pocket of land. In an area where few strangers ever ventured, Martin Fugate's descendants were left with little choice but to intermarry with one another-- thus perpetuating the rare, odd-looking trait of blue skin.

Of Martin Fugate's seven children, four were blue. Early in the history of the region, the gene spread like wildfire because of their isolation from the rest of the world. Bunched into tiny, isolated cabins along the riverside, the Fugates married into the few other families in the region: Combs, Smith, Ritchie, and Stacy families. But it only took a few generations before Fugates were routinely marrying other Fugates. First cousins frequently married; second cousins even moreso. After Martin Fugate's son Zachariah married his own aunt, he solidified the gene in the region's history.

For generations, the Blue Fugates survived and thrived among the oaks and hickories in the Kentucky forests. Luna Stacy (nee Fugate), the last of the smurf-blue family members, had thirteen children, all fathered by a man who was entirely unrelated to her. When none of the children showed any signs of blue skin, the trait was presumed dead. Luna Stacy's grandchildren all appeared to be free of the trait. Then, unexpectedly, Luna Stacy's grandson begot the indigo-blue baby Benjy. She died over ten years later, at the ripe old age of 84-- never having received medical care in her entire life.

Benjy Stacy's condition is relatively mild in the grand scheme of the blue-skin disease now known as hereditary methmeglobenemia. As someone carrying only one gene for the condition, he was born with extremely blue skin, but developed pink-white skin as he got older. When he was an adult, his mouth and extremeties would still turn an intense blue shade every time he became angry or felt cold. Benjy never had any blue children, and neither did any of his distant cousins riding the coattails of the famed Blue Fugates. Now, with incest rare and taboo, it is safe to declare the Blue Fugates of Kentucky extinct.

Hereditary methemoglobinemia, the condition affecting the Blue Fugates has only been recorded in two other families. One, a small Inuit population in Alaska, was last recorded in the mid-1900s. Like the Fugates, this group had lived in extreme isolation, so rare genes tended to appear and recur through generations. However, the blue Eskimos were short-lived, with the last of the family dying in the 1900s without any blue descendants. The other smurf-like family, native to a small Irish town, bore a pair of bright-blue identical twins. The twins, born in the late 1800s, experienced relief from their blue condition after taking large doses of vitamin C.

Today, we understand the cause and cure of hereditary methemoglobinemia. Although it is likely extinct, and no treatment for it will ever be needed again, we understand that the condition is caused by an inability do produce diaphorase-- the compound necessary for converting methemoglobin, which gives blood the blue coloration seen in oxygen-depleted veins under normal skin, back into hemoglobin, which gives blood its red color.

Normally, people turn blue because their blood is oxygen-depleted. Blue skin is seen in people with severe heart disease, because their body is not successfully replenishing oxygen to blood cells. It is also a symptom of suffocation or advanced lung disease. While someone with blue skin is usually dying or experiencing a major medical emergency, the Blue Fugates were unusually healthy. They routinely lived to be 80 to 90 years old-- far more than twice the average for a malnutrition-affected region far from medical care.

The world will probably never see another blue individual. Every day, medical advances and globalization further increase the already-minuscule chances of recessive traits like hereditary methemoglobinemia becoming apparent in the mainstream human population. Nevertheless, this strange mutation is one more stunning example of just how diverse human biology can be. While the majority of us range in color from pink-white to deep black, along with all the shades of golden, tan, brown and bronze in between, a person with blue-indigo skin is a rare anomaly indeed. The Blue Fugates of Kentucky may be extinct, but medical science has not forgotten them or their legacy.

This 1982 story tells the full history of the Blue Fugates of Kentucky and their impact on the culture and history of the region.