About 170,000 vehicles use the each day, passing by Larry Misrok’s apartment building, which sits at the entrance to one of the ramps. Crossing the street there might be a daunting task for anyone, but for Misrok, an 81-year old lawyer, it could be deadly. Misrok is virtually blind.
He navigates New York’s busy streets and broken sidewalks with high-tech glasses that have a camera attached to the side, made by a company called Aira. Misrok calls up an app on his telephone, which puts him in touch with one of Aira’s hundreds of workers around the country, who then commandeer the camera on his glasses and narrate his surroundings for him. “Watch out, there’s a curb coming,” or “There’s a cyclist coming up on your right,” the Aira helper might say. It’s not like having 20/20 sight, but it’s enough to get him to the grocery store half a block away—provided he turns away from the bridge ramp and not toward it.
Misrok’s troubles began in the 1970s, when he started seeing floaters in his eyes. “I thought it was a result of getting my head banged with a basketball the year before,” he says. He went to a local eye doctor but was told his problem was beyond their expertise. After trips to the head of ophthalmology at Montefiore hospital, and then a stream of experts at Mt. Sinai, New York University, Johns Hopkins, and the National Institute of Health in Bethesda, Maryland, he learned he had a rare autoimmune disease that caused inflammation in his eyes, destroying a layer of his retina.
The condition, known technically as , causes severe, progressive inflammation of the retina and the choroid, the vascular layer of the eye. It’s named “birdshot” for the small light-colored fundus spots that appear on the retina, which are scattered in a pattern like birdshot from a shotgun. “Right now, I can’t see you very well,” he says as I sit two feet away from him. “I know someone’s there, but I can’t tell if you’re smiling or frowning.”
Not without Aira, anyway. I went to visit him one Saturday afternoon to learn how the technology worked. When we’re ready to take a walk, Misrok puts on the glasses and headset with earphones, calls up the , and dials into an agent. Carl in Los Angeles picks up.
“I’m Larry Misrok. We’re going to go for a walk out of my apartment. Is the visual working well? I know you have audio,” he says as we step out of his apartment.
“The visual seems to be working,” the agent replies.
With his cell phone in one hand, a white cane in the other, Larry and I walk down the hallway. By the time we reach the elevator, we lose the connection, and the agent is gone.
About 1.3 million Americans are legally blind. Another 2.9 million have a visual impairment, defined as having sight worse than 20/40 in a person’s better eye, according to the (NEI), part of the National Institutes of Health. That’s 4.2 million people who have limited or no sight, a jump from 3.3 million people recorded in 2000. That figure is expected to grow to about 7.2 million in 2030 and 13 million by 2050.
Not all who can’t see are born blind. A spectrum of disorders exists that can cause people to lose some or all of their sight over the course of decades. Deficits usually occur in the two photoreceptors involved in sight: rods, which handle dim light and night vision and seeing in black and white, and cones, which handle daylight and bright vision and seeing in color. These photoreceptors are located in the retina, a thin layer in the back of the eye. If you think of the eye as a camera, the retina would be the film. The retina also contains the nerves that tell the brain what the photoreceptors are "seeing."
The most common serious eye problems are retinal degenerative diseases such as age-related macular degeneration, a disease that causes a progressive loss of central vision, and retinitis pigmentosa (RP), a group of genetic conditions that usually begin with night blindness but often evolve into tunnel vision. There’s also Stargardt disease, an inherited form of macular degeneration caused by the death of photoreceptor cells in the central portion of the retina called the macula, and Usher syndrome, an inherited condition characterized by hearing impairment and progressive vision loss.
As the number of those afflicted has grown, so has the number of tools to help them navigate. Many are readily available to anyone with a smart phone, like the , which tells users which buttons they’re pressing, or GPS apps that let users know where they’re standing in the middle of a city, can announce bus and train stops, and vibrate every time a street is crossed.
There’s a popular app called , that, like Aira, puts the user in touch with sighted workers who commandeer the user’s cell phone camera to help them see prescription labels, thermostat temperatures or how much money they’re holding in their hand. Richard Faubion, a development director for the Foundation Fighting Blindness, who is severely visually impaired himself, says he’ll call a Be My Eyes worker to tell him if his tie matches his shirt, whether he’s holding a can of peas or green beans, or if his suitcase is about to go by at an airport luggage carousel.
For computer work, there’s JAWS (Job Access With Speech) and Microsoft’s NVDA (Non-Visual Desktop Access) software that reads aloud the text on a screen and tells the user what they are typing as they type. SeeingAI, developed by Microsoft, combines many tools into one app, using artificial intelligence to describe people, text, and objects.
“Technology can be an extremely powerful tool for someone who is visually impaired if it is used along with a person’s other skills and abilities,” says Ryan Jones, a legally blind program manager with VFO Group, which helps companies become more accessible to users with disabilities. “These types of programs allow equal access to opportunities and careers for people who are visually impaired. They’re in just about every type of profession you can think of—except maybe airline pilot.”
Misrok and I walk through the lobby of his building and head toward the street, to see if the internet connection is better outside. He extends his cane out in front of him as he enters the revolving door. When he pops out the other side, he heads toward the driveway in front of his building but stops under some scaffolding, which probably doesn’t help with the internet but I’m not sure he even knows it’s there. Holding the cane under one arm, he uses both hands to hold the phone and double-tap the app. Every now and again, he puts his ear toward the phone to make sure he’s not missing any vital instructions.
The 120-some-odd paid agents across the country are told in an initial “onboarding” session what the user’s preferences are: the type of information they want, how detailed, and how they want it transmitted: Do they want details described as being to the left or right, or do they prefer degrees or hands on a clock?
Users then pay from $99 a month for 120 minutes to $199 a month for 300 minutes. Aira used to provide the glasses free of charge but now sells them. Misrok got his about two years ago.
Misrok says he finds the glasses very helpful in Massachusetts, where he has a home in the Berkshires. “I can walk down paths alone through the woods, and they keep me on the road, not walking off into the bushes,” he says. “I don’t need them for going to work. I have good staff.”
“These are some of the problems with technology, and some of the frustrations when you’re visually handicapped,” he says, as we stand waiting for the traffic light to change.
Is this why you don’t want to rely on it?, I ask.
“I don’t want to rely on anything. I want to rely on myself as much as possible,” he says.
Alex Bindel avoids gadgets altogether. He’s holding out for the technology that will give him all of his sight back. He’s been waiting a long time. Now 51, Bindel (which is not his real name—he requested a pseudonym out of concern that going public with his condition would affect his business) was diagnosed with RP at the age of 12, and at this point, about 99 percent of his rods are nonfunctional.
He remembers the heartache he felt when he first felt the burden of impairment. One night he went to a bonfire with an older cousin with whom he was close but who didn’t yet know about his condition. While he could see the bonfire, he could see little else in the dark, and his cousin, Robert, kept saying, “Go sit over there.”
“I was like, ‘Where?’ And he kept saying, “Over there!’” Bindel says. Unable to see, he just sat down where he was and wound up sitting on top of someone, embarrassing his cousin, who yelled at him in front of everyone.
“To this day, it's my biggest nightmare to be in a situation at night in the woods,” he says.
Being night blind, Bindel never went out to bars or clubs, and he preferred to only take women out on dates only during the day, because if he went out for a drink or to the movies, the lights would be too dim to hide his disease.
It’s not been all bad. His disease compelled him to leave a computer consulting job in Los Angeles, sell everything he owned, buy a motorcycle and travel across the country. A few years later, he packed up again to see the world, traveling on and off for three years, seeing 40 countries, hitchhiking around Europe, and taking on odd jobs. He milked cows in Norway, picked grapes in France, taught English in Italy. And he met a woman and moved with her to Namibia, where he lives now.
“I feared I wouldn’t be able to see it all before I went blind,” he says.
He’s lost his peripheral vision and has an even harder time seeing at night now. Like many with poor vision, he makes up for his handicap by using his memory, making mental notes of his surroundings, counting the number of steps until he reaches the floor, an approach that works until he stumbles upon something unexpected, like a chair someone has left in the middle of the hallway.
And yet, outside of enlarging the font on his computer, Bindel has always spurned technology for the visually impaired. He only recalls buying two gadgets. One was a night-vision scope he purchased in college. It was a military-grade hand-held telescope so strong that he needed a letter from his doctor to buy it. You close one eye and look through it with the other, and you can basically see in pitch black, he says.
The other was a really bright light that was rechargeable and would attach to a video camera. He purchased it at a conference for the visually impaired but realized after buying it that it was absolutely useless because anything bright enough for him to use in, say, a dark place like a movie theater was a floodlight to the seeing population.
“It’s a freaking bright light. It doesn't make social sense to use that, you know?” he says. “I tried it once. I went to the movies and turned it on and everyone was like, ‘Hey, what the hell man? What are you doing?!’ It was horrible.” He put it in a box and never took it out again.
“I didn't feel comfortable using it,” he says. “I never wanted people to know. I still don't. I never wanted people to treat me differently.”
Instead of loading up on gadgets, Bindel has been waiting for a genetic cure.
“I always said I was born at the exact right moment in time because the technology and the research may be there to help me by the time I need it. And I still feel that,” he says.
He may be right. Researchers are currently trying to fix the broken genes that cause vision loss in the first place. One growing area of research involves replacing the problematic gene with a copy of a functioning one. The new gene is transported into the relevant area by a viral vector, which experts liken to a taxi cab. The new gene is the passenger in the cab. The viral vector transfects the cell—it injects it with the new gene, rather than viral material.
The farthest along is voretigene neparvovec (or ), which received regulatory approval from the FDA in December 2017. Created by Spark Therapeutics, the treatment is for people with vision loss from mutations in the gene RPE65 leading to Leber congenital amaurosis (LCA) and RP and some other conditions. Nearly all patients with RPE65 mutations go virtually blind.
“Gene therapies have several outcomes: slowing, halting, or fi,” says Daniel Chung, clinical ophthalmic and medical strategy lead at Spark Therapeutics. “With Luxturna, we’ve seen increased light sensitivity. For others, their success may be just halting the disease or slowing its progression.”
Nacuity Pharmaceuticals is developing a molecule, known as N-acetylcysteine-amide (), designed to slow vision loss in RP patients by protecting retinal cells from oxidative stress, a process that accelerates degeneration in many inherited retinal conditions. So far, studies show NACA can slow retinal degeneration in rodents.
There’s a clinical trial for a drug called ALK-001, a modified Vitamin A, to address the genetic defect associated with Stargardt disease, which is caused by a lack of a protein that helps process Vitamin A. Photoreceptors need vitamin A to function but without the protein, fatty vitamin A byproducts can linger. ALK-001 reduces the likelihood vitamin A will bind to itself and form a toxic byproduct.
Applied Genetic Technologies has clinical trials underway to test the safety and efficacy of gene therapy products meant to address achromatopsia, or ACHM, an inherited condition resulting from mutations in the CNGB3 or CNGA3 genes. Most people with ACHM are legally blind, have extreme light sensitivity resulting in daytime blindness, and are color blind. The company has tested their replacement genes on dogs and sheep, where they put drops in the animals’ eyes to temporarily bring on symptoms of ACHM. They have the animals move through a maze. The animals are then treated with the drug and put in the maze again. Both dogs and sheep were able to get to the end of the maze more quickly.
Nightstar, a gene therapy company based in the U.K., has been undergoing clinical trials to address choroideremia, a mutation in the CHM gene that leads to degeneration of the choroid (Misrok’s vision is affected by choroid damage). In the first trials, patients received injections under their retina with a liquid that delivered healthy copies of the gene encoding the REP-1 protein to affected cells. In studies, more than 90 percent of participants reported that their visual acuity was maintained or improved a year later.
While those treatments involve swapping out the bad gene and replacing it with a good one, another burgeoning field of therapy involves leaving the broken gene in place but simply fi it. “Clustered regularly interspaced short palindromic repeats,” or CRISPR—which you likely first heard about last year when an uproar in the bio-ethical community ensued after a Chinese scientist announced he’d created the first babies with CRISPR-edited genes—involves swapping the mutated portion of the gene with a healthy piece of DNA. One researcher likened it to replacing the battery or the starter in a car rather than buying a new one.
CRISPR is a gigantic breakthrough in genetics, says Stephen Daiger, a professor of environmental and genetic sciences and ophthalmology at the University of Texas Houston Health Science Center. “It’s relatively inexpensive and conceptually easy to do, but we’re still at the very early stages of doing this with humans and working on the retina,” Daiger says. “If I had to bet on where the big breakthroughs are going to be over the next (decade), I’d have to say its gene editing.”
On the plus side, CRISPR may be the only option for diseases caused by genes that are too large to fit in a viral carrier (taxi cab). Some also see CRISPR and the idea of repairing rather than replacing a gene as a simpler approach to treating disease. The downside? Repairing rather than replacing doesn’t always work well enough to restore vision. It’s also unclear whether tampering with DNA will affect other otherwise healthy genes in the body.
Regardless, people are trying it. There are CRISPR projects at Johns Hopkins University aimed at RP caused by the P23H mutation in a gene called RHO. At Columbia University, researchers are looking at RP caused by the D190N mutation in RHO. At the Massachusetts Eye and Ear Infirmary, they’re studying RP caused by a mutation in RP1. And the University of California, Los Angeles, has focused on Usher syndrome 1B caused by a mutation in MYO7A.
But before someone can fix a gene, scientists have to determine which one is broken. Daiger’s lab at the University of Texas has identified a number of genes associated with various retinal diseases. Faubion, for instance, has X-linked RP, which is an inherited condition caused by mutations in the RPGR gene. He’s fortunate that his specific mutation is one currently being studied in clinical trials around the country. For many mutations, there are no trials yet.
When I go back to Misrok’s apartment a few weeks later, I find him sitting at his kitchen table with the glasses already on. He dials, and is soon talking to Noah in Minnesota through the earpiece that sat on his temples. Noah describes to Misrok everything that was in front of him as Misrok’s camera surveys the room.
I ask if I could try the glasses. With my eyes open, I listen to Noah describe everything in front of me. The kidney shaped coffee table to my right. The bookshelf to the left. The doorway to the kitchen in front of me. Walking through the kitchen, Noah describes the refrigerator and countertops, as I run my hand along them.
But I quickly see pitfalls. He fails to describe the area rug under my foot, and I almost trip on it. And the directions are coming so fast, countertop colors, tabletop patterns, I imagine that if I couldn’t see, I would have trouble deciphering what was relevant. Doing it outside on a Manhattan street, with all the cars, buses, traffic lights, people, strollers and bicycles, would feel like an onslaught.
I give the glasses back to Misrok, and we make our way to Whole Foods, this time with the help of Ashley in Sacramento. The store is only half a block away so many things could go wrong. At one point, we stand in the middle of a bike lane. We then have to cross Second Avenue and its six lanes of heavy traffic. I’m relieved when we finally get to the store.
When we get back to his apartment, I try the glasses again—this time, with my eyes closed. I quickly get tangled in a corner of the living room near what was initially described as a bedroom door but turns out to be a glass door to a balcony. Holding Misrok’s cane, I keep tapping the walls of the corner, bouncing between the shelf and the chair like a Pong cursor. I finally open my eyes to free myself from the corner and then close them again to head into the living room.
When I’m done, I tell Misrok, “I cheated a couple of times. I opened my eyes.”
Misrok laughs. “I wish I could cheat.”
Unlike at other conferences, the tables and chairs in all of the rooms at the “” annual conference of the Foundation Fighting Blindness’ annual conference, “Visions,” are arranged to maximize the walking space. Food is organized on the dinner plates like a clock, with, say, mashed potatoes at 3 o’clock and meat at 6 o’clock. And there’s a pen outside the conference area with a big sign that reads, “Guide Dog Relief Area.”
Although the conference is prized for its educational and networking opportunities, it becomes clear as the day progresses that despite the nice hotel, the boat trip and tour, the schmoozing with like-minded individuals, people are primarily there to find clinical trials that fit their specific disease.
In a panel on stem-cell-related research, attendees are told that while stem cells transplanted into humans may be used to cure retinal disease in the future, there’s still a major flaw: Once they’re transplanted in the body, scientists lose track of them.
“We can find out where they’re going in the animal, but we can’t find out where they’re going in people. We need better methods of imaging,” says Dennis Clegg, founder of the University of California/Santa BarbaraCenter for Stem Cell Biology and Engineering.
Replacing the whole retina also has its issues. There are apparently 1.2 million wires that take information from the retina and deposit it in a one-to-one correlation with the cells from the brain. If you disconnect the retina, you have to deal with that, say researchers.
“You can actually take an eye from one rat and put it into another, but the retinal cells weren’t connected to the brain,” Clegg says. “They said, okay, we’ll figure that one out later.”
After sitting in on some of the panels, I go into the exposition hall, where there are rows and rows of products to help people navigate their surroundings. There’s a smart cane that clips on to a traditional cane and enables users to not just feel what’s on the ground in front of them but to identify objects above the waist. When it senses an obstruction from knee to head height, it begins to vibrate. The user can then stop, wave the cane in the air to find the obstruction and avert it. The range is three meters, and the vibration speeds up as the user gets closer to the object.
A few rows down is a prosthetic retina, called the Argus II Retinal Prosthesis, which can restore some vision to people with RP and other retinal conditions. Developed by Second Sight Medical Products, it has already received market approval from the FDA and is now being used by more than 100 people around the world.
“This is the glasses part, and then there is an implant, surgically embedded in the eye of someone with retinitis pigmentosa,” says Mickey Damelio, senior vision rehabilitation manager at Second Sight.
He’s holding up a rubber model of an eye with little red lines all over the eyeball that look like wires. The implant provides electrical stimulation of the retina to induce visual perception. A miniature video camera housed in the patient's glasses captures a scene. The video is sent to a small computer that the patient wears that processes and transforms the electrical signals into instructions transmitted wirelessly to an antenna in the retinal implant. The implant contains an electrode array that emits small pulses of electricity. These pulses bypass the individual’s damaged photoreceptors and stimulate the retina's remaining cells, which then transmit the visual information along the optic nerve to the brain, creating the perception of patterns of light. Patients learn to interpret these visual patterns over time.
“There’s a lot of people here with RP, which shrinks people’s field of vision over time,” Damelio says. “When that field is gone or almost gone, we can install this on their eye and return some light sensitivity to them.”
When I put on the glasses, I see a blurry mess. I can’t imagine considering this product a gift. Damelio shows me a video of a man wearing the technology.
“He’s totally blind, but when he wears the glasses with this implant, he can discriminate between things,” Damelio says. “The patients will report that it’s talking to them in flashes or sparkles, and then from that, they can learn some fairly functional skills. But going and seeing a sunset? Not happening really,” he says.
In a booth around the corner was IrisVision, whose founders have created software for hardware that already exists: Samsung’s headsets for virtual reality gaming. The product sells for $2,500 and involves a camera that projects on a screen. In between your eyes and the screen are magnification lenses that make what the user sees look like a big screen TV up close, according to Zarak Afridi, director of strategic partnerships for IrisVision. The product can magnify images by one to 12 times, depending on what the wearer needs at that moment. Some situations may warrant six while others, like reading, may require 10.
Afridi says he once showed the product to a woman with RP, who like all people with RP, had lost her peripheral vision and her central vision had become reduced, like she was looking through a straw. She really wanted to see her daughter, he says.
“We spent almost an hour with her wearing the (glasses), and she found the spot where she still had some vision left, and she saw her.”
Tom Perski, who handles professional and consumer outreach for IrisVision, says he has Stargardt and his vision is 20 over 400. With his company’s device, he can see 20 over 40. And it has the added advantage of having a larger field of view than that of his competitors. Typically, the more you zoom in the camera, the smaller your field of view. With IrisVision, the camera is doing only part of the work, while the software and the magnifying lenses are doing the rest. The less the camera has to zoom in, the more field of view one can see, he says.
The booth of most interest to me was the Aira glasses. The second morning of the conference they perform a demonstration. Amy Bernal, vice president, customer experience at Aira, wears the glasses while a man named Kyle used Amy’s camera to guide her across the room.
“It’s like having a personal assistant available to you at any time,” Bernal says, noting that the person on the phone can call Uber or Lyft for you, and because of the data points that come up during the call, can know whether you’re allergic to certain foods.
What happens if you lose the cell signal, I ask an Aira representative, who says the product is not meant as a safety device. The user must still use a cane and dog.
“Think of it as a phone. What would you do if you didn’t have cell service?” the rep says.
After the presentation, I approach Sujeeth Kanuganti, the company’s chief technology officer, who points out that Misrok has an older generation of the product, which supported only a WiFi connection while the newer version, its fifth generation, called Horizon, uses a cellular connection. To get a cell or 4G LTE connection, the older versions had to use a device called a MIFI, which takes in WiFi connections and routes the data to a 4G network.
“So before, we had the glasses, the MIFI and you then had a connection from the MIFI to the 4G LTE network. With the multiple hops, it’s tough to maintain the connection,” Kanuganti says, noting that the tall buildings in big cities like New York don’t help. “The signal is bouncing everywhere, so getting a connection is a problem.”
The company simplified the glasses by having them connect directly to the 4G LTE network. The glasses now have a cable coming in from the back that goes directly into a Samsung device that gives direct 4G LTE service.
“It provides good connectivity with the cellular without any intermediate hops,” he says.
When Larry Misrok told Aira about his connectivity problems, they sent him new glasses with a new MIFI device, but it was still the old technology. Apparently, the company hadn’t made enough of its new Horizon model to fill all the orders it had.
I met Misrok at his office on Long Island a couple of months after our last visit, and when I arrived, he showed me his Aira alternative: the Be My Eyes app, which he uses when the Wi-Fi signal isn’t working. Be My Eyes uses the phone’s own camera so it only needs a cell phone signal. He used it recently when he went for a walk near his home in the Berkshires because the Wi-Fi signal was too spotty to get Aira.
He speaks to a person named Jennie from Georgia, and holds the phone up to his law library and asks her if she can read the titles on the binders of the books. She says she can’t, that they were too blurry. I then hold the camera closer to the books and at just the right angle—because I could see what that angle should be—and Jennie begins to reel off the titles of the books.
When I left him in his conference room to go catch a train, the secretaries in his law office had just brought back food for their lunch and are sitting down at the conference table to eat.
“Larry, we got you a salad,” says the assistant who had worked there the longest. Misrok walks into the conference and uses the aids to which he’s most accustomed: the kindness of those who love him.