Losing sight of the stars was just the beginning


Kristen Cox remembers failing her eye test in the fifth grade. Though she was fittted for eyeglasses, she knew her vision -- and her world -- was changing. Because around the same time, Cox, a Republican selected this week to run for lieutenant governor with Gov. Robert L. Ehrlich Jr., noticed that when she would look up at the night sky, the stars would disappear.

She started to think that's what stars do -- vanish. But she and her family soon learned that she had Stargardt disease. It was her vision that was fading.

Stargardt, named after German ophthamologist, Karl Stargardt, who discovered it in 1901, is a type of juvenile macular degeneration. The macula is the center of the retina, the part of the eye that senses light and processes images to be sent to the brain.

"The eye is like a tennis ball, hollow on the inside," said Dr. Neal Adams, chief of Johns Hopkins' Division of Visual Physiology, part of the Wilmer Eye Institute.

The retina is a thin tissue lining the inside. Within the retina are two types of photoreceptors--rods and cones. People perceive dim light and peripheral vision through their rods, while cones detect colors and central objects. Stargardt affects the cones, causing bluriness or a decrease in the sharpness of vision and difficulty seeing colors.

Adams says about 25,000 Americans are affected by the disease, which is genetic. It occurs in about one in 10,000 children.

Discovering the trait can come as a surprise. The parents of 11-year-old Madeline Shertzer had never heard of the disease.

"Progressively we noticed her sitting closer and closer to the TV set, so we would tell her to move back," said her father, Ron Shertzer. An ophthalmologist believed the signs pointed to Stargardt, a diagnosis confirmed at Hopkins last November.

Since then, Maddie has been adjusting to her vision loss. A regional educational agency provides a closed-circuit television at school that enlarges books and tests on a projector. She also listens to books on tape.

Those affected have trouble reading and recognizing faces. "It's almost like having a boulder in the center of your vision," says Dr. Janet Sunness, medical director of Hoover Services for Low Vision and Blindness at Greater Baltimore Medical Center.

She works with nearly 100 patients, teaching them how to use optical aids and overcome their blind spots.

Vision loss usually begins in the pre-teens and worsens until the 20s. Cox said that hers faded gradually through her teen years, forcing her to give up skiing and soccer. She coped in the classroom by using magnifying glasses and large print books. She even had a 26-volume dictionary.

Now, she says, she is extremely light-sensitive. She has some peripheral vision in her left eye. "My right eye is pretty shot," Cox said yesterday.

There are no medical treatments for Stargardt. Hopkins scientists are looking at ways to use adult stem cells to replace degenerative photoreceptor cells.

Maddie rides her bike infrequently but still plays sports and volunteers at a public library.

"We're positive about the fact that she's got a lot of years to go," said her mother, Ann McGuire, who said they're hoping for "a cure by the time she's able to drive."

stephanie.beasley@baltsun.com jennifer.skalka@baltsun.com

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