VisionAware

By Mary D’Apice

A Diagnosis of Retinitis Pigmentosa

When Kathy was diagnosed with retinitis pigmentosa (RP) back in 1974 there was no medical treatment for this hereditary disease that affects roughly 100,000 Americans. At age 23, Kathy brushed away concerns that her vision would deteriorate over time and could ultimately lead to total blindness. “I figured in 20 years, which seemed a lifetime then, there would be a cure.” Indeed, the very first medical treatment was on the horizon, but it wouldn’t be until 2007. Kathy herself would become one of 15 patients around the country to participate in the first clinical trial of the Argus II Retinal Prosthesis System, also known as the bionic eye or retinal implant.

In the years following her diagnosis, Kathy’s RP progressed rapidly and within 15 years her vision was reduced to light perception only. Though she had to leave her job as a machinist, she was happy and fulfilled as a stay-at-home mom of three daughters. When her youngest left for college, she was ready to get back to work and realized that mobility training would be key to her plan. Inspired by her brother who also had RP and benefited from a guide dog, Kathy got her first dog, indeed her first pet, at Guide Dogs for the Blind in San Rafael, California in 1996. Vision rehabilitation specialists taught her new skills including braille and after some employment training, Kathy got a job as a receptionist.

Participating in a Clinical Trial

Successful in her job and independent, Kathy was enjoying a good quality of life when her orientation and mobility instructor suggested she’d be a strong candidate for a revolutionary device that could restore some functional vision in people with severe to profound retinitis pigmentosa. RP damages the photoreceptors in the retina, which renders the eye unable to transform light into images. The Argus II works by converting images into pulses of electricity that the brain perceives as patterns of light. A 3-part system, the Argus II consists of a camera mounted on glasses, a video processing unit and a prosthesis made up of an antenna, an electronics case and an electrode array implanted in the eye. Kathy felt she had nothing to lose and agreed to move forward in the hopes she could further the research. “What really motivated me was knowing that this is a hereditary disease. My daughters were getting married and I was worried about my grandchildren. “

Kathy had to meet the medical criteria for surgery, which included a functioning optic nerve (transmits visual information from the retina to the brain) but a psychological screening was also necessary to ensure she understood the risks and the degree to which her vision could be restored. While the media is quick to tout the Argus II as a medical miracle or a futuristic device out of Star Trek that “makes the blind see,” patients must embrace the hope it offers while recognizing the limitations of this nascent technology. The scientists at Second Sight Medical Products, Inc. told Kathy that their device was not capable of helping her to see color or detail but predicted she would be able to make out the outlines of objects due to the contrast of light and dark around the edges. For example she might see doorways or detect the presence of obstacles in her path. Kathy stresses that the Argus II is for people who have become blind from RP. “If you still have a pinhole of detail vision you are going to want to keep that. But if you have nothing, then this (vision) is something.”

Kathy’s resolve to pioneer the new technology was unshakable until she saw the 20-page consent form. “Of course they have to tell you what could possibly go wrong and it was a major commitment.” Nevertheless she agreed to spend the next three years commuting to The Doheny Eye Institute in Los Angeles where she would spend 3 hours in the lab each week. Kathy did not pay anything out of pocket for the surgery, but honored her commitments to the research as if it were a part-time job.

Surgery and Learning to See Again

Kathy reports that the 6-hour surgery was surprisingly easy and done on an outpatient basis. “There was a lot of hardware in my eye but I couldn’t tell there was anything in it.” Her eyes were a bit irritated because of the sutures but it wasn’t particularly painful. “My eyes healed in eight weeks and looked the same as they did before.”

Since the implant is just one part of the whole Argus II system, Kathy did not experience any change in her vision until she was able to go back to the clinic and begin training on the complete device. “My vision is manufactured so it is something you have to learn to interpret. You’re getting these flashes of white anytime there is contrast from light to dark. If I look straight ahead and someone walks by me, I can see flash, flash, flash. I can tell what direction they are going in.” Kathy identifies the moving figure as a person, not solely because of her vision but because she integrates the visual image with her other senses. “I can hear their footsteps,” she explains. Kathy describes how the Argus II helped in her work as a receptionist. Typically, she would call out “Can I help you?” in response to the sound of automatic doors opening. But if she was on the phone, she might not hear a person come in. “The Argus II could help me make out that a person was standing at the counter.” The Argus II, Kathy explains, is an assistive device, much like a magnifier, in that it is one tool that can be used with other tools or other senses to accomplish tasks.

Kathy worked with researchers in the lab for 3 months before she was able to take the Argus II home. “I could hardly wait just to take a walk. I got my dog and off we went to the greenbelt near my house. There was so much contrast there because of the shades of green that I kept telling my dog to halt every 10 feet!” She could see the edge of the sidewalk and was pretty sure that the object in front of her was a small bush. Realizing she was confusing and possibly annoying the dog she took him back home and returned with her cane to marvel at her surroundings through the new device.

It is impossible to promise how much vision the Argus II can restore because each patient has such varied experiences. In some cases, patients have been able to make out letters in one inch print. Kathy’s ability to read is very limited and during lab tests she found that she could trace a letter on the computer monitor only if it was at least 6 inches tall. Kathy has reported, however, that once she was able to identify a sign for a restroom. Somehow it is easier for her to read letters comprised of lines than those that are more curved.

As research continued, scientists were curious to know what Kathy was able to do with her vision and a breakthrough came one day when she realized she could sort laundry. “There was enough contrast on my beige carpet to know whether the item went to the white, light or dark pile. Before the surgery, my husband helped me sort laundry.” Kathy learned she could use the Argus II to find objects, providing there is enough contrast. For example, Kathy might turn her head to scan a counter and look for a flash that might indicate where her keys are. “I can scan a place setting in the dining room and know where the silverware is because I get a flash of light. I will reach for the glass, not because I can see it is a glass for sure, but I can reach for a shadowy outline that is in the place where a glass is usually located.” It seems Kathy sees best when a familiar object is in a predictable location.

Life with the Argus II System

The Argus II continues to enhance Kathy’s experiences but she relies on her dog guide for safety. For example, in a residential area she uses her vision to see the white lines in a crosswalk but if there are eight lanes of traffic she depends on the dog to guide her. Kathy states that she might see a parked car extending from a driveway into the sidewalk but since she has no depth perception it’s hard to estimate distance. Nevertheless, Kathy is glad she had the surgery. “I was very pleased with outcome because I wasn’t expecting perfect vision.”

Now that Kathy has retired she has been spending much of her time babysitting her four granddaughters. I asked how caring for her toddlers with the Argus II compared to caring for her own daughters years ago. Kathy laughs, “I don’t dare wear the Argus II with the babies. I can imagine them looking at me and pulling the cord.” Kathy says that she rarely uses the device in the house. But because she is independent without vision, Kathy says that she never required that the device to change the way she carries out everyday tasks.

Kathy is quick to point out that the Argus II has contributed to her enjoyment of life. “I remember the first time my husband told me there was a full moon. I went out in the yard, and I could point to where it was in sky.” She describes her first experiences with restored sight with wonder and awe. “Once we went on a cruise to a glacier near Alaska and I was told that the clinking noise I was hearing was from the ice around us. When I put my glasses on, I could see the edges of the ice flashing against the dark water.” Kathy also likes to wear the Argus II in the car. “On long trips I can see the trucks passing or I might ask my husband what something is and he’ll say it’s a billboard.”

Availability and Cost

Now after more than twenty-five years of development, the hard work of Second Sight researchers, and courageous volunteers like Kathy have born fruit. In February 2013, the Food and Drug Administration (FDA) approved the use of the Argus II and there are now 12 implantation centers around the country. The complete Argus II system costs just over $140,000. Beginning October 1, Medicare has agreed to issue full payment for the system. Each of the implantation centers will work with individual insurance providers. It is expected that most insurers will provide full coverage and the patient with be responsible for a relatively small co-pay.

Kathy would recommend the Argus II and in fact has encouraged her brother to consider the next generation of the device. She is glad she had the implant. “I was very impressed with how brilliant these people are who are figuring this all out. It’s really amazing. I know they are going to do really good things with this.”

Read a review on AccessWorld