“The vision I have left”: One woman’s story of living with degenerative eye disease

Nicole C. Kear always thought it was normal when she’d bump into things – but when she turned 19, she soon realized it wasn’t just clumsiness.  She was diagnosed with a degenerative eye disease that would eventually leave her blind.

In her newly published memoir, “Now I See You,” Kear details the story of how the diagnosis taught her to embrace life.

Just after her sophomore year of college, Kear went to her ophthalmologist for a routine check-up. She offhandedly mentioned an incident at the beach a few months prior, when she realized she couldn’t see any of the stars.

“[My boyfriend at the time] was really shocked, because they were very bright,” Kear told FoxNews.com. “I never suspected it was an actual problem; we joked about it.”

Her ophthalmologist referred Kear to a specialist, who then used an electroretinogram to measure the electrical activity in her retinas in response to light. The device utilizes electrodes in the form of contact lenses, which measure the electrical responses of the eyes’ cells.

“Once he whipped that out, I had a suspicion things weren’t going very well,” Kear said.

The New York City native was diagnosed with retinitis pigmentosa, and she was told that she had 10 to 15 years of vision left.  Her doctor advised Kear to ultimately start preparing for going blind.

“That’s when it really soaked in: ‘This is bad; this is like old-school incurable,’” she said.

Retinitis pigmentosa is an inherited degenerative eye disease that causes the death of the eyes’ photoreceptor cells – known as the rods and the cones. The “rods,” are responsible for night vision and peripheral vision, while the day-seeing cells, or “cones,” deal with center vision. Symptoms of the disease include clumsiness, difficulty seeing at night and loss of side vision (having “tunnel vision”), because the rods begin to die first. Eventually, a patient will lose all vision from the sides and, when the cones die, from the center of the eye.

There are currently no treatments for the disease, though progress is being made with gene and stem cell therapy research. A National Institutes of Health (NIH) study found that 15,000 international units of vitamin A a day can reduce cell loss from 10 percent to 8 percent.

Retinitis pigmentosa is difficult to diagnose because symptoms are often unnoticed or confused with other disorders.

“The challenge of this is that it’s not so common – about 1.5 million people worldwide – but then most of my patients have seen four or five doctors before me,” Dr. Stephen Tsang, attending ophthalmologist at New York-Presbyterian Hospital/Columbia University Medical Center who has not treated Kear, told FoxNews.com. “Because of clumsiness and side vision problems, a lot have seen neurologists for brain scans.”

Although Kear’s doctor warned her that she should start preparing for blindness, she resisted and instead embarked on completing a bucket list. She traveled with her sister throughout Europe, threw herself into college life at Yale, joined a circus school in San Francisco for a summer, and became an actress.

But the most important priority was becoming a mother.

“The first thing that struck me, even though I was so far from having them: Does that mean I can’t have children?” she said. “I wanted to see my children when they were born.”

Kear married her husband, David, in 2003, and they now have three children, ages 2,7 and 9. The kids know their mom is losing her vision, and Kear is glad she’s been forthright with them, as they understand it and take it very matter-of-factly.

Fortunately, Kear’s vision and her management of the disease have held up well. She is legally blind, with only 10 degrees of vision, and she has developed cataracts, a common side effect of retinitis pigmentosa, which has worsened any acuity she had in the center of her vision.

However, she credits technological developments – such as being able to zoom in on her iPhone, or enlarging text on her Kindle – for helping her manage without giving up too much of her life.

Kear hasn’t learned Braille yet, but she has trained with a cane and expects she’ll have to use it more in the near future – especially at night and in crowded places.

“I’m glad I lived life the way I did, but the doctor was right: At a certain point, you have to prepare,” she said.

Kear’s biggest struggles have been telling people about her vision loss, which she’s been able to confront with the publishing of her book, but also the uncertainty of the future.

“It is hard; you can get dejected about it, but everybody has obstacles. We all have our challenges and hurdles,” Kear said. “This is mine, and it’s not the worst. There are ways through it.”