THE PROVIDENCE SUNDAY JOURNAL

May 10, 1998

Surgery on Blind Man Pushes Medicine’s Frontiers

By Felice J. Freyer

Journal-Bulletin Medical Writer

BOSTON- Thrilled and apprehensive, Brian J. Hubbard waits in his doctor’s office, already wearing two layers of multicolored hospital johnnies and blue foam operating room slippers.

A black patch covers his right eye, which a week earlier had been paralyzed with a botulism injection in preparation for today’s surgery. His left eye seems to gaze so directly that it takes a moment to realize that it, too, is sightless.

Hubbard is thrilled because he believes that, at 51, he is about to make medical history. He’s apprehensive because his doctors, who have spent years preparing for this day, are counting on him to endure several hours in the operating room fully conscious.

Today, doctors at the Massachusetts Eye and Ear Infirmary will place inside Hubbard’s right eye a tinsel-thin metal chip bearing 100 miniscule electrodes. They want to find out whether electrical charges through the nerves behind his retina can produce meaningful light sensations in his brain - whether they can make a blind man "see." It worked in rabbits. Until now, it’s never been tried in a person.

Hubbard, a clinical social worker and author who lives in Newport, sits in a chair facing the doctor’s unoccupied desk, with only his fiancée, Sharron Mattson, at his side. His burly physique is tense with anticipation, his fair, clean-shaven face amiable, quick to smile.

A nurse comes in to say it’s time, and Hubbard climbs on a gurney for the trip down to the pre-operative area. Dr. Joseph Rizzo, co-director of the Retinal Implant Project, walks over to greet him. As he talks to Hubbard, Mattson hands Rizzo the little box-shaped microphone that transmits his voice to an FM receiver at Hubbard’s waist, and from there to the hearing aids nestled behind each ear. The congenital condition that caused his blindness also left Hubbard hearing impaired since birth.

Rizzo asks Hubbard how he’s doing, whether he was able to sleep at all last night.

For the past decade, Rizzo, who is both an ophthalmologist and a neurologist, has led a cooperative effort between Massachusetts Eye and Ear and the Massachusetts Institute of Technology that aims to bring useful vision to people whose retinas no longer work.

Hubbard met Rizzo a year ago after Hubbard wrote to the surgeon volunteering to help in any way he could. He didn’t know it at the time that Rizzo was looking for a patient in whom to test the new device. But he was hoping that maybe Rizzo could get him back enough vision, enough glimmers of light, so that he could ski again.

Ski? You can see why Rizzo chose Hubbard as his first test patient. It’s not just that he’s intelligent, articulate, enthusiastic. Hubbard’s a bit of a daredevil; He’s got that unstoppable spirit. When he had only 2 degrees of vision ( normal eyesight provides 180 degrees )Hubbard went helicopter skiing in New Zealand. He holds three gold medals from the U.S. Disabled Ski Team.

He would ski with a guide, peering down the dark tunnel of his blindness to the small field of sight, where the guide’s motions alerted him to moguls and turns ahead. Unable to see the trees flying past, all he knew of his speed was the intensity of the wind against his body. When the final circle of sight clouded over about five years ago, Hubbard gave up skiing.

Brian Hubbard accepts hi blindness, he accepts his deafness. But he sure would like to ski again.

Mattson and Hubbard separate now, as Hubbard is wheeled into the operating room and his fiancée joins a handful of people in an adjacent observation area with a glass wall. No sound penetrates the wall, but she can see the 11 people surrounding Hubbard, who is supine but awake on the operating table.

Dr. John Lowenstein, a retinal surgeon, and Rizzo take positions on either side of Hubbard’s head. John Wyatt of MIT, the electrical engineer who is co-director of the implant project, stands at a nearby table manipulating a box with an array of switches on top.

Rizzo wears a headset with a microphone at his mouth to communicate with Hubbard.

In the 11 months spent preparing for this day, Monday, April 27, Hubbard has become well versed in the technology and the possible outcomes. He knows that he might not see anything at all, but that doctors hope to test how well he can perceive different patterns of light as Wyatt flips the switches on the box.

Any he knows that even if it works beautifully, the doctors will remove the implant after the experiment because the electrode array has not yet been made biocompatible - safe to leave in the body. Even in the best of circumstances, it will take at least five years before the implant can be used as treatment.

Hubbard thinks he may suffer from Usher Syndrome, a genetic disorder characterized by hearing impairment at birth, followed by retinitis pigmentosa, a progressive form of blindness. ( His only child, a 27-year-old daughter, did not inherit his disability.)

Growing up in Lynn, Mass. Hubbard was fitted with a hearing aid at age 8, and with amplification he has been able to speak and understand well, using the telephone and even launching a career as a psychotherapist. (His clients wear clip-on microphones.) Despite the lifelong hearing impairment, Hubbard enunciates clearly, with just a trace of an indefinable accent that makes him sound exotic rather than deaf.

The first symptom of retinitis pigmentosa came with a severe night blindness that developed when Hubbard was 8. But no one recognized his illness until Hubbard was a teenager playing ice hockey. He was a top- notch player, except that he kept getting clobbered from the side.

Only after a head injury from a hockey accident landed him in the hospital did it become clear that Hubbard had virtually no peripheral vision. He was diagnosed with retinitis pigmentosa. He says no one told him point-blank that his field of vision would continue to narrow over the years until he could see nothing at all.

With an illness that progresses so slowly, with doctors who were less than forthcoming, with the pressures of adolescence, Hubbard says, "I had a lot of room for psychological denial." Only a stray comment from his sister, when he was a freshman in college, brought home for fact that he would go blind. He was telling her that an experimental treatment he’d been trying didn’t seem to be working, and she remarked that even if it didn’t restore any vision, wouldn’t it be great it if kept things from getting worse?

"Then all of a sudden," Hubbard recalls, "I get this flash of cold panic."

After nearly an hour of preparation in the operating room, the doctors are ready to cut. A large microscope with three eyepieces is maneuvered over Hubbard’s face. It also contains a camera that projects a magnified image of the surgery to a television screen visible from the observation area.

Mattson looks down at her lap as the scalpel penetrates the outer membrane of the eye. She says she can’t stand to watch. People in the operation room are laughing, and Patti, the head nurse, scribbles a message on a paper towel and holds it up to the glass: "Brian wants to know if Joe wants to trade seats with him because he is so comfortable."

Retinitis pigmentosa, the degeneration of the retina that rendered Brian Hubbard blind is the world’s leading cause of inherited blindness. Researchers are working on various types of retina implants in hope of helping people with RP, as well as those with another, more common form of blindness, age related macular degeneration, which affects 700,000 Americans.

Both conditions involve the rods and cones, cells at the back of the retina that act as photoreceptors, converting light to nerve signals that travel to the brain. In macular degeneration and retinitis pigmentosa, the rods and cones shrivel up, but the nerves that connect them to the brain remain unharmed. Theoretically, a retinal implant could substitute for the ruined cells.

In it’s final form. The retinal implant will involve more than the electrode array being implanted today, which picks up only electricity, no light. A tiny camera will be mounted on a pair of glasses. The camera will convert visual information into an electronic code that will be carried on a laser beam to the electrodes in the retina, and then through the nerves to the brain.

Mattson now has the courage to look at the video screen. She watches as two incisions are made for illumination and surgical tools, and a third through which to insert the implant.

Suddenly, she blurts out: "Brian is so brave to do this."

A divorced mother of three, Mattson met Hubbard through a mutual acquaintance, and in time became his sweetheart and his personal assistant. "We work well together," she says. "It’s like ‘Where have you been all my life?’"

When they met, Mattson had lost 80 percent of her hearing to otosclerosis, in which a tiny vibrating bone in the middle ear loses its ability to carry sound. Hubbard, who knew her hearing impairment was curable, led her to a surgeon in Worcester who corrected the problem, and now Mattson hears normally. It was that Worcester doctor who told Hubbard about Joe Rizzo’s work.

Now. The electrode array is coated with a viscous solution to ease its entry into the eye, and the surgeon slides in the implant, a square less than a tenth of an inch across, like a speck of glitter. Another paper towel message from nurse Patti: "They are fixing the electrodes now. Brian is doing well."

Doctors have several reasons to believe the implant might be able to bring vision, not normal eyesight, but useful images, to blind people whose optic nerves sill function. Their model is the cochlear implant used to treat deafness: it converts sound waves into electrical signals transmitted to the brains of deaf people, who learn to interpret them.

Doctors also know that electricity can produce the sensation of light; electrical impulses applied directly to the visual cortex in the brain, or in front of the retina, caused patients to see spots of light. Rizzo and Wyatt have placed their implants into the eyes of rabbits and produced a response in the rabbits’ visual cortex.

Btu, while researchers can measure the activity in rabbits’ brains, they can’t ask the rodents what it looks like. That’s where Brian Hubbard comes in. Today, Rizzo and Wyatt want to find out if the implant produces interpretable signals. Will Hubbard see merely a shapeless glow? Or will he see discrete dots of light in recognizable patterns?

Hubbard has spent a long time thinking about those patterns of light, what they’ll look like, what it will feel like.

Patti holds up another paper towel: "They’re adjusting the power levels good for Brian."

Several minutes pass, and it’s hard to tell what’s happening. Rizzo clasps his gloved hands tightly and converses with Wyatt, who leans his elbows on the table. Patti comes back into the observation area. "So far, he’s saying ‘no,’ except for one firework flash," she says.

Ten minutes later on the video screen, the implant is seen being removed from the eye. It looks like they’re closing the incision. Joe Rizzo is shaking his head.

Wyatt, the MIT engineer, leaves the operating room and walks around to the observation area. "Brian’s ok," he tells the small group gathered there. Still wearing his surgical mask, Wyatt reveals little emotion in his smooth forehead and arched eyebrows.

"We could not get a reliable response," Wyatt says. What little Hubbard saw was unrelated to the voltage. "It’s disappointing. We’ll just have to think about why, and keep trying."

Sharron Mattson is standing now, her face close to the glass, her eyes fixed on Hubbard as the operating room staff, businesslike, starts to remove surgical drapes. She has known Hubbard for 18 months, and on Sept 19, her 47^th birthday she will become his fourth wife.

IN RESEARCH, an experiment in which a hoped- for response does not occur is not necessarily a failure. It’s another piece of information, another step in the laborious process of trial and error.

A few minutes after surgery, Rizzo comes to Hubbard’s side in the recovery area, gently touching his shoulder as he speaks. "There was no damage to the eye," he tells him. "Everything was well-prepared. We wouldn’t do anything differently. To come through this without feeling there is anything we would have done differently is a victory. We still learned an awful lot from doing this."

A nurse, speaking in a warm brogue, asks Hubbard if he would like a painkiller.

"If you have one," he replies.

Brian Hubbard is an expert in traumatic loss, otherwise known as heartbreak. He knows how the human spirit rebels against it, and how happiness comes only from accepting one’s losses and making the most of what one has. That concept lies behind his self-published book, From Emptiness to Empowerment: Changing Physical and Other Losses into Strengths, and behind his career as a psychotherapist. For six years, he ran a counseling service to help disabled people achieve independence.

He remembers a turning point in his own journey through progressing blindness; the day in 1980 when someone told him to wear a hideous neon-orange bib with big black letters that said, BLIND SKIIER.

"There’s no way in hell," Hubbard responded, "that you’re going to get me to put that thing on."

The person urging this insult on Hubbard was a man who had lost his legs below the knees and had learned to ski on prostheses. Hubbard heard about him and tracked him down around the time that Hubbard was considering giving up skiing. The man, Paul DiBello, had a saying: Be a gimp or be a wimp. That is, have the courage to accept your limitations.

Hubbard put on the cursed bib, became a gimp, and headed for the slopes. "It really helped that situation," he recalls, laughing. "People would get out of my way." Hubbard went on to many more years of skiing and his gold medals.

Three days after surgery, Hubbard is back in his spare office in Middletown. Sharron Mattson at his side as always. He is chipper, despite the patch on his eye and the pain underneath.

Hubbard says that Rizzo told him the evening after surgery that he wanted to try moving the implant to a different part of the retina, but some bleeding was clouding the pupil; to avoid the risk of injury they decided to stop the procedure.

Hubbard says he is honor-bound not to discuss what he experienced during the procedure, because research protocols require Rizzo and Wyatt to first publish their findings in a medical journal.

But he’s upbeat about it; "The whole thing was very worthwhile," he says. "I was very pleased with myself getting through the actual procedure."

Ethical rules prohibit doing the experiment again on the same patient, but Hubbard says that that even though it hurt, even though he could, at times, feel the knife in his eye, he wants to talk them out of that rule.

"I would do it again," he says.

Brian Hubbard has a web site: http://www.brianjhubbard.com