Rage Against the Dying of the Light (excerpt)

Tales of Those Living with Progressive Vision Loss

On a clear, crisp spring morning, 11-year-old Ava Rose Bullis and her brother, sister, parents, grandparents, aunt, uncle and cousins pile into six cars and drive to The Lanes at Seagirt Bowling Center in Belmar, New Jersey. When they arrive at noon, the place is already packed with weekend revelers; The air is full of the smell of chili hotdogs; Every now and then, a sharp clamor echoes across the cavernous space as a bowling ball smashes into a line of bowling pins.

Ava wears a neon-colored flat-rimmed cap, backwards, a black leather jacket over her navy blue T-shirt, and gray leggings that hug her calves. At the front desk, she changes into a pair of size four red-and-black bowling shoes before bending down to help her cousin Ethan with his laces. And while the four younger kids gambol around the room, she perches herself on one of the swivel chairs at the back of the room beside her father and grandmother, her bright hazel eyes darting restlessly, drinking in her surroundings. When she tilts her head, two tiny pieces of plastic hooked to the rim of her ears peek through her thin veil of blonde hair.

At first sight, they look like earrings, but Ava’s mom, Carly Fredricks, explains that these are cochlear implants — electrical devices designed to help Ava’s hearing.

Born with Usher Syndrome, Ava is not only profoundly deaf but also gradually going blind. Right now, the size of her visual field has narrowed to about 40 percent of a regular person’s, and once the sun goes down, she becomes completely blind. These symptoms are typical for patients with Ushers Syndrome, whose night vision and peripheral vision are the first to go.

According to Fredricks, when Ava was around three years old, they started to notice something wrong with her eyes. “When we read to her at night, she wouldn’t notice that we had left the room,” she said. “She would call out, reach out for us…”

But Ava’s disability doesn’t stop her from participating in a wide range of sports,

including basketball, soccer and karate. When it is her turn to bowl, she picks up a 9-pound dark turquoise ball from the rack and sends it sailing down the lane with a powerful thrust, knocking over 6 of the 10 pins. She holds that dramatic, half-kneeling pose for almost five full seconds, savoring her triumph.

“I’m losing my sight, but that doesn’t mean that I’m losing my ability,” she says.

Ava’s case is an example of how people in the blind community are often misunderstood by society. Even the term “blindness” can be misleading. It suggests total darkness, when in reality, vision loss is measured across a wide spectrum. Apart from those who were born into darkness, there are also many people who, like Ava, lose their sight steadily over a period of time. Currently, it is estimated that 25 million Americans are blind or visually impaired, and over 50% had gone through, or are suffering from, some form of progressive vision loss. For them, blindness is an ongoing process, an impending threat which grows closer and more real with each passing day, and this provides a set of unique challenges for both young and old.

While people bowled, a man in a bright yellow golf shirt weaves through the crowd, taking photos and clapping people on their backs. His name is Michael Benson, and he is the founder of the Visual Experience Foundation, a nonprofit organization dedicated to helping those who are losing their vision. Today’s event, Bowling for Vision, is not only a fundraiser, but also his way of educating people about progressive vision loss and reminding them that there is still life after blindness.

Benson, 57, has thinning gray hair and electric blue eyes that sparkle under the beam of the fluorescent lights. Born on December 16th, 1961, he was the youngest baby at that time to undergo glaucoma surgery. Shortly after his birth, the nurses noticed his abnormally large eyes, and less than a week later, he was brought to Dr. Adolph Posner of Manhattan Eye and Ear Hospital for surgery.

Glaucoma is a group of diseases that cause progressive damage to the eye’s optic nerves and may result in irreversible vision loss or blindness. It develops when the fluid inside the eyes does not flow properly out of the anterior chamber. This leads to a build-up of fluid and a rise in eye pressure, until it reaches a level that is harmful to one’s optic nerves. Due to the fact that glaucoma usually develops slowly and without symptoms, it is often referred to as “the silent thief of sight.”

Fortunately for Benson, his initial surgery was a success, and while growing up, his vision

was as normal as any other kid’s. He even played sports like football and wrestling, which were sometimes considered dangerous for people with delicate eye conditions. It wasn’t until he reached 16, when his eye pressures started building up again, that the fear of losing his sight truly began to sink in.

On a sweltering summer’s day in 1980, while he sat in stands of the packed Yankee Stadium, surrounded by roaring crowds and the sweet smell of popcorn, a stranger suddenly turned and said to him: “Wow, you’ve got really beautiful eyes.” Benson forced a smile and muttered “thank you,” but when he turned to face the ballfield again, his mind was reeling.

It wasn’t the first time strangers had complimented his eyes, but whenever it happened, all the fear and anxiety that he kept bottled up inside would bubble to the surface, like a snake rearing its head. When he looked down at the field again, his heart pounded. He couldn’t concentrate. All he could think of was the date of his next doctor’s appointment, and what might happen then. If his eye pressure was high again, he would be put in another surgery; and if the surgery went wrong, he might go blind once and for all; and if he went blind, what would he do? Who would ever love him?

This nightmarish train of thought went round and round his mind, and by the time he snapped out of his trance, he was shocked to find that three innings of the game had already passed. “This was how I felt for most of my life,” Benson recalled 37 years later. “I wasn’t living in the present, but instead in some made-up future in my head. And it wasn’t until I got older that I realized that my eye disease had stolen three innings from me.”

Progressive vision loss can be caused by a number of incurable eye disorders, including Glaucoma, Retinitis Pigmentosa, Macular Degeneration, Stargardt’s disease and Diabetic Retinopathy. Patients diagnosed with these diseases may be on their way to losing their sight permanently, but there is no guarantee as to how long the process would take, or the degree of vision loss it may lead to, since symptoms vary from person to person. Special eye drops can be used to control and delay progression of the disease, but such treatments are not foolproof, and in most cases, any vision lost in the process cannot be recovered. Like Alzheimer’s, Parkinson’s Disease and other progressive diseases, those faced with unstoppable vision loss often find themselves both haunted by what lies ahead and feeling an urgent need to preserve and make use of the faculties they still have.