Deep in the Kentucky woods, since the 1800s, a fiercely private family gave birth to blue-skinned children. The color of their skin was so shocking for normal people to see that they chose to stay hidden away from the rest of society in their small community. Very few people knew they even existed until today.
In 1820, French orphan Martin Fugate and his wife Elizabeth Smith moved onto the banks of Troublesome Creek, a beautiful area in Appalachian Kentucky. There is no official record documenting that Martin was actually blue, but he and his wife both carried a recessive gene that gave their son Zachariah Fugate a startling blue color. Martin and Elizabeth had seven children—four of them were blue. Since the gene causing their blue coloration is recessive, the family had a 25% chance of having a blue child with each pregnancy if Martin and Elizabeth were carriers. If Martin was blue, the odds would have increased to 50% for each child as Martin would have carried two copies of the recessive gene.
This is because of a condition called methemoglobinemia, which causes methemoglobin levels in the red blood cells to rise above 1 percent. It turns the skin blue, the lips purple, and the blood a chocolate brown. Methemoglobinemia can be triggered by exposure to particular chemicals (benzocaine and xylocaine, for example), but in this case, it was inherited, and the product of a faulty gene that most probably caused a deficiency in an enzyme called cytochrome-b5 methemoglobin reductase.
In the 1960s, Dr. Madison Cawein was able to test two Fugate descendants, Patrick and Rachel Ritchie. “They were bluer’n hell,” Cawein said. “They were really embarrassed about being blue. They wouldn’t come into the waiting room. You could tell how much it bothered them.” After conducting many blood tests, Cawein came to the conclusion that methemoglobinemia was the root of the problem, and determined that he could convert the methemoglobin back by simply injecting a missing enzyme to oxygenate the blood. Strangely, the easiest way to do this was with a chemical called methylene blue. Though the family thought the doctor was completely crazy—after all, how could injecting them with another blue substance make them less blue?—they allowed Cawein to give it a shot.
An Ironic Cure for Blue Skin
To convert the blue methemoglobin back into red hemoglobin, Dr. Cawein suggested the use of a dye called methylene blue. Ironically, this blue dye could change the blue color of the affected blood into a normal red color. Vitamin C (ascorbic acid) is another method of treating the condition.
Of course, it was a tad difficult to convince the blue people of Kentucky that a blue dye would cure the condition. Nevertheless, Patrick and Rachel volunteered to try the treatment.
A simple injection of the dye caused a stunning color change. Within minutes, Patrick changed from blue to pink. This, however, was a temporary fix. The dye couldn’t fix their enzyme deficiency, so Dr. Cawein left the people with a supply of methylene blue pills to be taken on a continual basis.
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