July 20, 2020History of Medicine
Nowadays, with our scientific knowledge of porphyria, instead of fearing these folks, we can love and care for them. Porphyria remains incurable, and treatment is mainly supportive: pain control, fluids and avoidance of drugs and chemicals that provoke acute attacks. Some success has been achieved with stem cell transplants.
The following is an edited excerpt from the book Of Plagues and Vampires: Believable Myths and Unbelievable Facts from Medical Practice by Michael Hefferon. https://theconversation.com/vampire-myths-originated-with-a-real-blood-disorder-140830
The concept of a vampire predates Bram Stoker's tales of Count Dracula - probably by several centuries. But did vampires ever really exist? In 1819, 80 years before the publication of Dracula, John Polidori, an Anglo-Italian physician, published a novel called The Vampire. Polidori was born and brought up in Dublin. He was a friend to Oscar Wilde and William Gladstone. Stoker's novel, however, became the benchmark for our descriptions of vampires. But how and where did this concept develop? It appears that the folklore surrounding the vampire phenomenon originated in that Balkan area where Stoker located his tale of Count Dracula.
Medical source of the myth
But where did the myth of vampires come from? Like many myths, it is based partly in fact. A blood disorder called porphyria, which has has been with us for millennia, became prevalent among the nobility and royalty of Eastern Europe. Porphyria is an inherited blood disorder that causes the body to produce less heme - a critical component of hemoglobin, the protein in red blood cells that carries oxygen from the lungs to the body tissues. It seems likely that this disorder is the origin of the vampire myth. In fact, porphyria is sometimes referred to as the vampyre disease.
Elements of vampire folklore correspond to symptoms of porphyria.
1. Sensitivity to sunlight: Extreme sensitivity to sunlight, leading to facial disfigurement, blackened skin and hair growth.
2. Fangs: In addition to facial disfigurement, repeated attacks of the disease cause the gums to recede, exposing the teeth, which then look like fangs.
3. Blood drinking: Because the urine of persons with porphyria is dark red, folklore surmised that they were drinking blood. In fact, some physicians had recommended that these patients drink blood to compensate for the defect in their red blood cells, but this recommendation was for animal blood. It is more likely that these patients, who only went out after dark, were judged to be looking for blood, and their fangs led to folk tales about vampires.
4. Aversion to garlic: The sulfur content of garlic could lead to an attack of porphyria, leading to very acute pain. Thus, the aversion to garlic.
5. Reflections not seen in mirrors: In the mythology, a vampire is not able to look in a mirror, or cannot see its reflection. The facial disfigurement caused by porphyria becomes worse with time. Poor oxygenation leads to destruction of facial tissues, and collapse of the facial structure. Patients understandably avoided mirrors.
6. Fear of the crucifix: During the Spanish Inquisition (1478-1834), 600 vampires were reportedly burned at the stake. Some of these accused vampires were innocent sufferers of porphyria. At that time, porphyria patients had good reason to fear the Christian faith and Christian symbols.
King George III and porphyria: a clinical re-examination of the historical evidence (Hist Psychiatry March 2020)
The diagnosis that George III suffered from acute porphyria has gained widespread acceptance, but re-examination of the evidence suggests it is unlikely that he had porphyria. The porphyria diagnosis was advanced by Ida Macalpine and Richard Hunter, whose clinical symptomatology and historical methodology were flawed. They highlighted selected symptoms, while ignoring, dismissing or suppressing counter-evidence. Their claims about peripheral neuropathy, cataracts, vocal hoarseness and abdominal pains are re-evaluated; and it is also demonstrated that evidence of discolored urine is exceedingly weak. Macalpine and Hunter believed that mental illnesses were primarily caused by physical diseases, and their diagnosis of George III formed part of a wider agenda to promote controversial views about past, contemporary and future methods in psychiatry.