PORPHYRIA and the VAMPIRE LEGEND

Legend tells us that vampires come out at night. They are night creatures because the sun can hurt and even kill them. They come out at night to seek fresh blood because without it they will suffer agonizing pain and will die. Their bodies dry up due to lack of blood, and new blood refreshes their bodies and gives them energy and certain powers.

It has been long believed that the condition associated with vampire legends is porphyria. Vampire characteristics are similar to those of porphyrics and this may have led to the misconception in the early 1400-1600's that porphyria sufferers were vampires. Vampire legends are in every country and porphyria is also found throughout the world. Porphyria comes from the Greek word meaning purple.

Of course, we now know that porphyria patients are NOT vampires, but porphyria might have contributed to the origin of the vampire legends.

EXPLAINING PORPHYRIA

Porphyria is a group of disorders caused by the abnormal production of heme which is the base material responsible for making hemoglobin and chlorophyll. Most types of porphyria are inherited. A child needs to inherit the defective gene from only one parent to develop the disease.

Heme is a substance found in all body tissues. The largest amounts of heme are found in the blood and bone marrow, and heme is also found in the liver and red blood cells. Multiple enzymes are required for the body to convert chemical compounds called porphyrins into heme. If any of the enzymes are abnormal, the process is disturbed and cannot continue. This allows the porphyrins to build up in the body.

Excessive porphyrin in the body causes photosensitivity which is oversensitivity to sunlight. When porphyrins are exposed to light and oxygen, they generate a charged, unstable form of oxygen that can damage the skin. Nerve damage, pain and paralysis can occur in some porphyrias. Sometimes an attack is so severe it can also lead to respiratory paralysis and the patient is unable to speak, breath or swallow. At times, this if fatal.

Symptoms of porphyria depend on which enzymes are affected, but most often the nervous system and the skin are involved. There are at least eight types of porphyria but the two major types have the following signs and symptoms:

Acute porphyria: Acute porphyria involves the nervous system. The symptoms appear intermittently and can include pain in the abdomen, chest, legs, arms, and back, muscle numbness, tingling, paralysis, cramping, constipation, red urine, sensitivity to sunlight, vomiting, personality changes and mental disorders.

Cutaneous porphyria: People suffering from cutaneous porphyria have many skin problems. The skin becomes fragile and can blister and crack. Sunlight can also cause the sufferer to have itching and swelling of the skin.

Diagnosing porphyria is confirmed by blood, urine or stool tests that measure enzyme levels. There is no cure for the disease. Attacks of porphyria can develop over hours or days and can last for days or weeks. Things that can trigger an attack are drugs (barbiturates, tranquilizers, birth control pills, sedatives), chemicals, fasting, smoking, drinking alcohol, infections, emotional and physical stress, menstrual hormones and exposure to the sun.

Treatment depends on the type of porphyria and can include heme therapy and medications to manage the symptoms. Severe attacks may require hospitalization.

Porphyria is a life-long, intermittent illness, but with good long-term treatment and management the patient can have long, problem-free periods.