Living in the Dark

Aug. 1, 2006 — -- Can you imagine what life would be like if you could only come out at night?

If ultraviolet rays -- even from a light bulb -- could be dangerous, perhaps fatal?

Kasey Knauff, 5, of Bellefonte, Pa., faces this reality.

Her strange symptoms began the day she was born: April 10, 2001.

She was rushed to the neonatal intensive care unit and placed under special bilirubin lights to treat what doctors suspected was jaundice. Instead of getting better, Kasey's condition worsened.

A dermatologist was called in to examine her.

Howard Pride of the Geisinger Medical Center in Danville, Pa., removed Kasey's heart monitor patches and was shocked by what he found. The skin underneath the patches was white; the skin around them, red.

The bilirubin lights had caused a full body burn.

The dermatologist now knew that Kasey's skin was sensitive to light. It was another symptom, though, that would lead to a diagnosis -- the color of Kasey's urine was red.

That proved that Kasey was suffering from CEP -- Congenital Erythropoietic Porphyria -- a condition in which the body has trouble producing heme, an essential component of hemoglobin in the blood.

This causes light-activated chemicals called porphyrins to build up, turning bones and urine red, and teeth, a dark brown or purple.