Honesty about vision loss paves a path to Hollywood
January 28, 2020
When author Nicole Kear decided to come clean about her degenerative vision loss, the world opened up for the funny, sassy New Yorker.
Nicole Kear’s memoir, Now I See You, took her on a wide-ranging book and speaking tour that finally uncloaked the truth about her retinitis pigmentosa—and changed her life forever.
Nicole Kear, author of the memoir, Now I See You, didn’t intend to hide the fact that she was going blind. She just neglected to mention her retinitis pigmentosa (RP) when meeting new people in her early 20s. Since she still had the bulk of her vision in those early years, the condition rarely felt relevant, except in occasional situations. By the time her vision really began to decline, however, she’d actually known some of those “new” people for years—and they had no idea she was going blind. She had inadvertently begun to live a lie.
A diagnosis of retinitis pigmentosa
Kear’s journey with inherited retinal disease (IRD) started when she was a sophomore at Yale. One night she was sitting on a Long Island beach with her then boyfriend and he began to point out the sky’s brightest stars. She couldn’t see any of them. Not Sirius. Not Arcturus. Nothing. “I can’t believe you can’t see them,” he said to her, pointing to star after star. “They’re right there. They are so bright!” The native New Yorker lived in a city where, thanks to the sheer wattage of the NYC nightlife—which obliterated starlight for everyone—the only visible stars were on Broadway. Kear had no experience with anything other than a black sky and thought it was perfectly normal.
Several months later at a routine ophthalmologist appointment Kear mentioned the incident to her eye care professional. More specialists and a few tests later, Kear was sitting by herself in a NYC retinal specialist’s office when he handed down the diagnosis—and prognosis. She had an IRD called retinitis pigmentosa and it would progress over the years until she was completely blind.
“I remember thinking, ‘Oh my God. My mother should be here. I should not be hearing this by myself, holding a Victoria’s Secret bag,” says Kear, remembering how shocked she was at the doctor’s words. “I had stopped at Victoria’s Secret on the way to the appointment, after lunch at a Chinese food restaurant. I had not been even the slightest bit worried about my eye appointment!”
When her ophthalmologist, registering the dismay on his patient’s face, added, “Don’t kill the messenger. I’m only the messenger,” the 19-year-old was taken aback. She still feels the sting of that tone-deaf moment, 23 years later. “I guess in medical school when they give you the bedside manners class, he overslept,” she says. Her ophthalmologist told her that she had 10 to 15 years of sight left, with no recourse, treatment, or cure. Dazed, she made her way home.
Advances in genetic testing meant they’d finally been able to locate the two genes responsible for her RP.
It wasn’t until she got to her parents’ house, however, that the gravity of the situation became real. Her father was a medical man, a cardiologist. When she walked into his home office, what she saw told her everything she needed to know about the precariousness of her situation. “He had this big wooden desk and all of these ponderous medical tomes with very, very tissue–thin pages,” says Kear. “I remember him sitting over his desk, pouring over those pages and crying. That’s when I realized, ‘Oh my God. This is really quite bad.’”
Early genetic testing
Kear’s parents found their daughter a renowned retinal specialist in Philadelphia who took the relatively unusual step—mind you it was the late 90s and the early days of retinal gene therapy research—of suggesting genetic testing. He was hopeful they could discover the mutation responsible for her retinitis pigmentosa. That information, he said, might not only help in the management of her disease but could also provide important information for accessing clinical trials should they become available. Philadelphia was at the epicenter of early retinal gene therapy research and he was deeply aware of its potential.
“It seemed a little science fictioney to me back then,” admits Kear, “but he said it would be a smart thing to do and I was like, ‘Sure. Okay. I trust you completely.” The specialist drew her blood and sent it off for testing. Weeks of waiting turned into months, and months into years. Kear eventually forgot about the whole thing. Nearly a decade later, however, she received a call “out of the blue” from her doctor’s office. He had the results of her blood work. In the decade since they’d sent her sample off, the genetic testing landscape had advanced and was providing the IRD community with actionable answers. The lab had finally been able to locate the two genes responsible for her RP.
No longer a naïve 19-year-old, the now 30-something mother understood the implications of identifying her mutations. Her primary concern was what the results could tell her about the risk to her children. “Having mutations on two different genes meant that my kids were far less likely to develop RP. It was a relief to have that clear and confirmed,” she says. Now Nicole and her ophthalmologist keep an eye on the rapidly changing landscape of retinal gene therapy. “I am armed with information, which is invaluable moving forward,” she says.
Nicole Kear and her family strike a pose, shades and all!
Carpe diem, baby
In the years between diagnosis and receiving her genetic testing results, Kear was very, very busy. She felt like she needed to live life to its fullest—while she could still see. “That’s when I really began this kind of carpe diem campaign. If I was on the fence about doing something, in the back of my mind I would think, ‘You know, I may not be able to see that in the years to come.’ So, I started saying ‘yes’ to adventure before my vision’s expiration date,” she says. She worked in theater until her vision loss forced a pivot to writing. She went to clown school. Really. She met and married her husband. They had three children.
Eventually, Kear decided to write a memoir about parenting. She struggled to generate any interest in the book. “The idea just wasn’t selling,” she says, “so I said to my husband, ‘What do you think it’s missing?’ And he’s like, ‘Well, it might be missing the fact that you’re losing your vision. When you write a memoir, you talk about important things like that.’”
It was Kear’s “aha” moment. “I was like, ‘You’re right, husband. Of course, my crappy little book won’t sell—because it’s a lie!’” And that’s when Kear’s life with retinitis pigmentosa came full circle. She poured her experiences and her truth into Now I See You and came clean about her accidental dishonesty. The book sold, and sold well—thanks in no small part to a self-deprecating, comedic style that endeared her to readers across the nation. The memoir also caught the eye of Hollywood and is currently in production with Emma Roberts slated to play the author.
From the sidelines, Kear happily follows the movie project her book has become while continuing to focus on writing, raising children, and sharing her story. “I have a new book that I’m really excited about coming out in April of 2020,” she says. “It’s a middle grade book called Foreverland.” Kear says her son actually helped come up with the idea for the storyline. “He wanted to know, ‘Am I going to get royalties?’” Kear chuckles as she remembers how hopeful he was. “I just said, ‘That’s really optimistic of you, kid.’”
It appears that Kear’s son gets his blue-sky sense of optimism from mom. Nicole Kear may accept the reality of her declining sight, but she does so with an eye to the future. She now knows that if a clinical trial begins that addresses her mutation, she has the genetic information she needs to raise her hand and say, “Count me in!”
Now I See You
Funny and irreverent, Nicole Kear shares the story of being diagnosed with retinitis pigmentosa at 19 and how she unintentionally started living a life of secrets, keeping her condition hidden from many of the people in her life. Until she couldn’t anymore.
Her story about facing truth and embracing every big, beautiful, and sometimes broken part of ourselves is an inspirational read. Hollywood thought so, too. Now I See You is currently in production and will feature Emma Roberts as Kear.
A New York native, Nicole received a BA from Yale, an MA from Columbia, and a red nose from the San Francisco School of Circus Arts. She is the author of the memoir, Now I See You, and a six-book children’s series The Fix-It Friends, and the co-author for The Startup Squad, a three-book middle-grade series. Up next? Another book for 8- to 12-year-olds called Foreverland. Nicole lives in Brooklyn with her husband and three children.