Degenerative eye disease may claim woman's sight, but won't take her positive attitude

By Richard J. Alley, Memphis Commercial Appeal

Lynette Herman stares into the middle distance while listening to a conversation, occasionally offering up information, correcting her husband's memory or erupting with a sharp burst of laughter.

There is levity in those steel grey eyes, and something else: a malfunction that reaches to the cells lining the back inside wall of the eyes. The mutation of a gene reaching back to at least the turn of the last century.

She suffers from retinitis pigmentosa, a disease causing the degeneration of the retina and eventual blindness in some 100,000 Americans. Suffering, though, is not the way Lynette, or her husband, Dr. Martin Herman, would characterize it.

While RP robs its carriers of eyesight gradually, usually taking until the age of 40 or so before rendering them legally blind, Lynette is anything but a victim, refusing to lose her positive attitude along with her vision.

One can't help but notice the beautiful pool table just beyond the living room at the Herman house. Martin says Lynette never played. She, however, insists she did. "I just found out blind people can play golf, too," she says.

"We're going to give it a shot," said her husband.

RP is hereditary and can be traced back in her family, originally from Tupelo, Miss., to her great-grandmother, passed to her grandmother and then four of her grandmother's children, including Lynette's father. Her sister, three aunts and uncles and five cousins also have RP.

Family reunions are full of people who know to touch when they approach to talk, never to move a chair and to not put a glass of Coke on a dark tablecloth. "I've known about it my whole life and wasn't just hit over the head with it," she said.

Diagnosed with the autosomal dominant variation of RP, she was born with a 50/50 chance of having the mutated gene. Lynette, 56, first noticed her fading eyesight as a child with trouble seeing at night, a typical first symptom, eventually needing a pronounced contrast

to distinguish objects in her environment as an adult. Her frontal vision is almost totally gone and she can see people and shapes better through her peripheral vision.

She continued with life as normal until she had to stop driving in her 40s, which, she admits, was probably pushing it. "But," she said, "it didn't affect my life until then. I've had a full life."

Lynette has been married to Martin, a pediatrics emergency specialist with Le Bonheur Children's Medical Center, for 25 years. The two have one daughter who, at 24, shows no sign of RP.

The husband and wife banter, joke and pick at each other's stories. He's helpful, but is in no way a crutch. She doesn't need one, nor would she accept one if offered. Though they walk the neighborhood together; a walking stick is never used, as she is wary of drawing attention to herself.

Martin walks ahead with their dog and calls out only if there is a break in the path or some other obstruction, such as the new street signs the city of Memphis placed in the middle of the sidewalks.

Eating out, Martin says, is the most difficult activity, especially any place with mood lighting. "I load up two plates at a buffet. I hope people are understanding."

The Hermans and Lynette's sister are all on the board of the Mid-South Chapter of Foundation Fighting Blindness (blindness.org), an organization that, according to their mission statement, "drives the research that will provide prevention, treatments and cures for people affected by retinitis pigmentosa, macular degeneration, Usher Syndrome, and the entire spectrum of retinal degenerative diseases."

Dr. Stephen Rose, a molecular biologist and the foundation's chief research officer, is excited by the prospects of gene therapy and the possibilities of "turning off the bad copy of the gene and getting rid of the toxic product being made." He sees progress at institutions around the country, specifically at the Children's Hospital of Philadelphia. There three subjects in their late teens and early 20s received gene therapy for Leber congenital amaurosis, an inherited disease causing blindness at birth. Afterward, they were able to read three lines on an eye chart and navigate an obstacle course.

The Hermans, naturally, are hopeful for a cure, or even a halt to the progression into blindness. But the issue isn't dwelt upon. Lynette fills her days with trading stocks online with the help of a Microsoft feature that switches an Internet browser's page to a high contrast, white characters on a black screen. She could read print up until last year and now downloads books into a digital recorder through a free program with the Library of Congress. She goes through two or three of these per week.

She watches DVDs with a special feature that describes a scene aloud as a narrator in a novel might. "I like watching those movies myself," Martin chimes in. She does miss going to the movies, however.

She cooks, she gardens and she researches products that might make her life easier, such as the Cobolt Speechmaster, a device which, when held against fabric such as a shirt or jacket, speaks the color of that item out loud. There are companies, too, working on cell phones with a bar code scanner that will tell the blind all of the information recorded in that code.

But as helpful as these items are and will be, a cure and good care are the true passions of the Hermans. "Anyone who has eyesight problems should be getting creditable, reliable help," Martin says. "We don't want anyone to have false hopes through unsanctioned, unsafe practices."

Until a cure comes along, she relies on her knowledge and familiarity with her condition, as well as her sense of humor and her husband, who attributes their long marriage to her blindness: "In her mind I'm still 35, with a head full of hair and no gray."