The Blue people from Kentucky *
The "Blue People of Kentucky" refers to the Fugate family, who lived in the Appalachian Mountains of eastern Kentucky and were known for a rare genetic condition called methemoglobinemia, which caused their skin to appear blue. This condition was caused by a recessive gene that reduced the oxygen-carrying capacity of their blood.
Background
The Fugate family settled in Troublesome Creek in the 19th century. Due to the isolation of the region and a limited gene pool, this recessive gene was passed down through generations, leading to a significant number of family members exhibiting blue-tinted skin.
The Science Behind It
Methemoglobinemia occurs when there is an excess of methemoglobin in the blood, a form of hemoglobin that cannot effectively release oxygen to body tissues. The condition is usually harmless but can cause the blue discoloration.
In the Fugates’ case, it was caused by a deficiency in an enzyme called cytochrome-b5 reductase, which is needed to convert methemoglobin back to normal hemoglobin.
Treatment
In the 20th century, Dr. Madison Cawein discovered that methylene blue, a dye used in medical treatments, could temporarily reverse the blue tint. Administering this compound helped many family members return to a more typical skin tone, at least temporarily.
Cultural Significance
The Blue People of Kentucky have become a fascinating topic in both medical history and folklore. Their story highlights the interplay between genetics, isolation, and community.