Wednesday, April 15, 2009

GUNTHER'S DISEASE


Gunther's Disease is an extremely rare, congenital variation of Porphyria. Porphyria diseases are manifested in the body's inability to generate the chemical heme, a substance found in the bone marrow, red blood cells and liver. In Gunther's Disease the lack of heme allows other naturally occuring chemicals, called porphyrins, to build up to toxic levels in the body.

The physical manifestations of Gunther's Disease include extreme sensitivity to sunlight (blistering and scarring) as well as increased hair growth on the forhead and face. The chemical changes in the body can also cause the teeth to turn red and glow, and the urine to take on a pinkish hue. Bacteria can form in the affected areas causing infection which can require amputation of fingers, noses, etc.

People with Gunther's Disease must avoid the sunlight and restrict their diets to avoid chemicals which might trigger porphyria attacks. In some cases of porphyria, regular bloodletting can help to reduce the level of Porphyrins in the blood, and provide symptom relief. There is no cure for the disease.

Gunther's Disease typically manifests in infants, but there have been cases of rapid adult onset. Despite the lack of a cure or effective treatment, most people with Gunther's Disease live to be between 40 and 60 years old.

Gunther's Disease is sometimes known as "Vampire Disease" because its symptoms so closely mimic the mythological symptoms associated with vampires.

I rate this sick a
2 on my "lethality scale" (1-10)
and a 6 on my "disturbing scale" (1-10)

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