Extra Credit Paper
29 – November - 2011
Porphyria: The Vampire Disease
Porphyrias are a hereditary group of disorders in which heme is not properly made. There are many different forms of this disorder, with porphyria cutanea tarda being the most common type. Remember what happens to Edward Cullen from the Twilight series when he gets out in the sun? Yea, me neither. But in classic movies and early folklore, vampires were commonly depicted as having purplish, ruddy colored bloated skin. Fictional vampires are also known to fear the sunlight because the sun could damage their delicate skin or they could start sparkling. Porphyria has these same symptoms, minus the sparkling skin, which is why it is nicknamed The Vampire Disease.
The process of making heme is a multi-step process. Porphyrins are made during this process. People with porphyria lack certain enzymes which causes buildups of porphyrins in the body. It usually attacks the nervous system or skin or both. When exposed to sunlight, a person with cutaneous porphyria can develop blisters, itching, scaring, and swelling of the skin. Porphyria in the nervous system is call acute porphyria. Symptoms of acute porphyria include: chest or abdominal pain, vomiting, diarrhea, or constipation. Certain medications, sunlight, smoking, alcohol, stress, or infections can trigger attacks with acute porphyria. An attack can include muscle numbness or tingling, paralysis, cramping, and personality and mental changes. With many forms of porphyria, a person may experience red urine.
Porphyria is a rare disease. All the forms of the disease together affect less than 200,000 people in the US. The most common form, porphyria cutanea tarda (PCT), affects around 1 in 10,000 people, while only six reported cases of ALAD-deficiency porphyria have been documented. Erythropoietic protophyria is the most common form among children. Acute Prophyrias seem to occur more often in northern...