Bio Blog Thingy

You may have heard of the Blue Fugates, or Blue People of Kentucky. This family was, as their nickname states, blue. While human skin color certainly has a wide range, blue is one that is obviously rather abnormal, which is what led to the Fugate family getting such attention and stupefying the medical community.

The Fugates lived in an isolated spot in the Appalachian Mountains, and thus, marriages between relatives were common. This led to the blue skin trait being kept mostly in the family and not getting spread to others. However, though inbreeding can cause many problems, it wasn’t actually the cause of this odd trait. Martin Fugate, a French immigrant, and his wife, Elizabeth Smith, were both carriers of a recessive gene that caused the family’s blue skin called methemoglobinemia.

Hemoglobin is a protein in your blood that is responsible for picking up oxygen from your lungs and transporting it to the rest of your body. Hemoglobin can’t do this alone though, and it needs the help of iron. A good analogy for this is oxygen is a dog, iron is the dog’s leash, and hemoglobin is the owner walking the dog. Hemoglobin needs iron to keep the oxygen tethered to it so it doesn’t “run away”, and can be transported where it needs to be (like one end of a park to another, if we are continuing with a dog analogy). However, there are actually two types of iron in your body: ferrous and ferric. Ferrous iron is really strong: think of this as a heavy-duty leash that you would use on a large, strong dog. It holds the oxygen tightly to prevent it from running away. This is what the hemoglobin in your body tends to use. Ferric iron, on the other hand, isn’t very strong. Think of this as a weak leash attached to a loose collar, something a dog could easily escape from. Hemoglobin with ferric iron (a loose collar) is called methemoglobin.

Your cells have a protein in them that converts ferric iron into ferrous iron (tightening the collar and reinforcing the leash), but in the case of the Fugates, this protein was dysfunctional. This can lead to a buildup of methemoglobin in your body as ferric iron can’t turn into ferrous iron, and oxygen escapes. Since blood’s red color comes from oxygen, without having it transported through your body blood turns a bluish color, a condition called methemoglobinemia.

Methemoglobinemia can potentially be dangerous if methemoglobin accounts for around 20% or more of your blood cells, but if it’s less than that, skin generally just turns blue and the condition is not dangerous. This was the case for the Fugate family, and since, as stated previously, they would often marry within the family, the condition was kept contained.

This condition can be treated by a chemical called methylene blue, which was what the Fugate family used to treat their methemoglobinemia. Methylene blue is capable of turning ferric iron to ferrous iron, therefore reducing the amount of methemoglobin in the body. Once being injected with methylene blue, the Fugate family’s color disappeared within minutes. Now, the Fugate family is no longer blue, and live normal, healthy lives.