the Mad Hatter of ‘Alice’, the Vampires and Wolves of Twilight, and the Na’vi of Avatar
After the frenzy of Match Week and in between trying to understand whether health reform would pass this weekend, I went to see the still #1 movie in the land Alice in Wonderland in 3D. This epic creation by part genius – part disturbed director Tim Burton features the wickedly talented method actor Johnny Depp as the ‘Mad Hatter.’ As I was watching Johnny Depp’s orange hair and freakish eyes, it occurred to me that some of the most popular fiction movies over the last several years have featured some notable legends and their medical problems. For example, the cult sensation Twilight franchise features Edward and the Cullens (a clan of friendly neighborhood vampires) and introduced us to wolves with the recent release of the New Moon (on DVD this weekend). Lastly, who could forgot the blue Na’vi people of Avatar earlier this year. Interestingly, these movies are all in some way linked to very rare medical conditions.
Mad Hatter- The Mad Hatter as played by Johnny Depp is clearly disturbed individual but comes to Alice’s rescue. The Hatter is “mad” due to chronic mercury poisoning. Hatters used to use mercury, an orange liquid, to make felt for hats. The liquid was often absorbed through the skin and could result in symptoms of mercury poisoning including confusion and confabulation (Korsakoff’s syndrome also seen with chronic alcohol use). Other symptoms could include nervousness, irritability, insomnia, tremors, weakness, skin discoloration and eye problems among others. The most common cause of mercury poisoning today is contaminated fish. Upon reading about mercury poisoning, it was Depp’s idea to use orange hair for the Mad Hatter. Burton loved the idea since orange hair is associated with some creepy fixtures of our imagination (who isn’t scared of clowns for example?) Interestingly, the original Mad Hatter is based on an eccentric furniture dealer and not someone with mercury poisoning.
Vampires Vampires, like Edward Cullen, are blood thirsty, pale, photophobic, and hate garlic, which are all symptoms associated with porphyria, a group of rare, largely hereditary blood diseases. Porphyria is a family of disorders of heme (necessary for hemoglobin) synthesis which leads to anemia (low blood count) and leads to pale skin. In some types of porphyria (cutanea tarda), the nonfunctional heme structures that cannot be made into hemoglobin, if hit by light, result in rashes, leading those individuals to want to avoid sunlight. The connection between vampires and porphyria went mainstream in 1985 when biochemist David Dolphin explored whether vampires may have suffered from porphyria. Unfortunately, this publicity has resulted in a lot of stigma for porphyria sufferers. Mary Queen of Scots and King George III are some of the famous sufferers of porphyria (the acute intermittent type).
Wolf-man or “werewolf” It is true that humans with congenital hypertrichosis lanuginosa look like wolves. Unlike Jacob, this unfortunate syndrome involves massive amounts of hair on the face and body, resulting in some of the people with this disorder to tour as circus performers. Interestingly, porphyria, more commonly associated with vampires, can also lead to hypertrichosis, leading some to link the disease to werewolves. However, another hypothesis is that werewolves and vampires actually suffer from rabies, which can also lead to similar symptoms (including the garlic!).
Na’vi or “blue people” Although blue skin is considered ‘alien’ in Avatar 3D, there are actually ‘blue people.’ Methemoglobinemia is a blood disorder in which blood cells can’t bind with oxygen which impairs the oxygen supply to parts of the body, resulting in cyanosis (blue skin). Fortunately, the treatment of methemoglobinemia is actually a blue dye, ‘methylene blue,’ which converts methemoglobin back to to hemoglobin. There is an acquired form and also a congenital form. The most famous carriers of this hereditary genetic error are the blue Fugates of Troublesome Creek from Kentucky who dated back to 1800. Their disorder was eventually diagnosed and treated in the 1960’s and the story is incredible.
With these movies, who says learning medicine can’t be fun?