By George N. True II
This is the second part of my series on unusual diseases.
In 1897, Irish author Bram Stoker gave us one of his most enduring characters. In that year, Count Dracula made his first appearance in Stoker’s Gothic horror novel “Dracula” which lay the groundwork for the many vampire stories that followed.
At that time, Stoker was the business manager of the Lyceum Theatre in London. To augment his income, he wrote several sensational novels on the side. But his most famous work was “Dracula” which eventually inspired many theatrical, film, and movie adaptations.
In Stoker’s novel, the count travels from Transylvania to England to find new victims and create more vampires until his plans are foiled by Professor Abraham Van Helsing. More than just horror fiction, “Dracula” is also considered invasion literature, a literary genre first recognized in the United Kingdom that explores the fear of foreign forces arriving in England without being opposed.
Vampire invasion
The notion of strange creatures out to destroy life on Earth was popular to readers throughout the 1880s and was explored by other writers, notably H. G. Wells. While “Dracula” is a good adventure yarn, it was not until the late 20th century when the novel achieved its peak popularity as soon as the movie versions arrived.
Unknown to many, however, real “vampires” live among us today. Like Stoker’s famous creation, they stay in the dark and are afraid of sunlight. But these people aren’t cursed like Dracula. They suffer from a rare blood disorder called porphyria.
According to the Mayo Clinic, porphyria is the name for a group of disorders that result from the buildup of natural chemicals that make porphyrin in the body. The latter allows hemoglobin (a protein in red blood cells) to function well and carry oxygen to organs and tissues, but high levels can cause a lot of trouble.
Of the two types, cutaneous porphyrias affect the skin because they make patients sensitive to sunlight like the fictional vampires who lurk in the dark. The most common type in this group is porphyria cutanea tarda (PCT). The US National Institute of Diabetes and Digestive and Kidney Diseases said this type of porphyria affects men and usually appears after the age of 40. One out of 10,000 to 25,000 people has PCT which is also referred to as vampire disease.
What happens in cutaneous porphyria?
Tom Peck of CNN said some famous victims of porphyria include King George III of England, Mary Queen of Scots, Dutch painter Vincent Van Gogh, and King Nebuchadnezzar of Babylon.
Because they are sensitive to sunlight and artificial light, the Mayo Clinic said people with PCT have the following symptoms:
- Blisters on the exposed skin, especially the hands, face, and arms
- Crusting and scarring of the skin
- Dark or reddish-brown urine
- Excessive hair growth on the face
- Hyperpigmentation (dark patches on the face, hands, and neck)
- Painful red, swollen, or itchy skin
- Peeling of the skin
- Scars that appear on the skin when the blisters heal
- Skin sores that develop with minor injuries
- Thin or fragile skin
Porphyria can be inherited from one or both parents. Most carriers have latent porphyria and don’t have symptoms. But they can pass on the disease to their children. The Mayo Clinic said PCT is usually acquired and can be triggered by the following:
- Excessive alcohol consumption (15 or more drinks per week for men, and 8 or more for women)
- Viral infections like hepatitis C and HIV
- Liver disease
- Smoking
- Taking estrogen found in birth control pills and hormone replacement therapy
- Too much iron in the body
Lifestyle changes for porphyria
Untreated, PCT can damage the skin, liver, and increase the risk of cirrhosis and liver cancer. The blisters can become infected and leave scars. The skin may have an abnormal color and appearance. The only way to prevent symptoms is to avoid the triggers or things that are causing PCT. This means patients should:
- Avoid alcohol
- Avoid injuring the skin
- Never take estrogens
- Stay away from the sun
- Stop smoking
- Wear sunscreen, long sleeves, and a hat to reduce sun exposure
For treatment, the doctor may prescribe:
- Drugs to treat malaria like chloroquine or hydroxychloroquine. These will help the body get rid of excess porphyrins. They are given to people who can’t tolerate phlebotomy (removing blood to reduce iron in the body)
- Pain medications
- Phlebotomy (drawing a pint of blood every 2 weeks to reduce iron levels in the body)
- Vitamin D supplements to treat deficiencies caused by lack of sunlight
These steps will ensure that patients don’t end up like Stoker’s popular character.
For some people “exotic” food means dirt, paper, and feces. Learn why people with pica eat them this Friday. Don’t miss it!
For questions, email georgenavatrue@yahoo.com.
