Second Sight

The sun was rising over Gloucester Harbor, and Nick Fairfield, a silver-haired lobsterman and waterfowl carver from Annisquam, Mass. was eager to get out to his lobster boat. But his small outboard wouldn't start. After a few futile yanks on the chord, Fairfield looked up and blinked. The straight white clapboards on an old house near shore appeared to be wiggling.

"What's going on?" Fairfield murmured. Covering his right eye he found the problem - his left. Everywhere he looked, straight lines were wavy.

For two weeks, he tried to trick his eye into focusing. Finally Fairfield called his ophthalmologist, who took one look at his eye and sent him to Boston to see a specialist.

The diagnosis: age-related macular degeneration, a leading cause of blindness in the United States. The prognosis: irreversible vision loss in his left eye, within months or weeks, and a fifty-fifty chance of losing sight in his right eye as well. The cure: none.

"I was scared, really scared," says Fairfield, 60, a youthful man with an easy laugh and clear green eyes. "My wife and I lead such a vibrant life. All of a sudden I thought of her, helping me around, holding my elbow. And I thought, this isn't going to fly." Fairfield's shock and sadness hardened into grim resolve. "It wasn't a question of whether I would commit suicide," he says, "but how."

But one month later, at a follow-up visit with a Boston eye specialist, Jeffrey Heier, Fairfield got some heartening news. Heier was testing a drug that had shown promise in treating Fairfield's malady: "wet" age-related macular degeneration. The wet form of the disease - caused by leaky blood vessels under the retina - strikes only 10 percent of the estimated 9 million Americans who have or may be developing macular degeneration, but accounts for up to 90 percent of the blindness the disease causes.

"You're a perfect candidate," Heier told Fairfield, whose vision loss was fresh enough to be, in theory, reversible. "Would you be interested?"

He would be among a select number - only a few dozen in the world - to try rhuFab, a new drug developed by Genentech, a San Francisco-based biotechnology company. But he had to wait four agonizing months to enter the federally approved clinical trial, and with each day his sight was growing dimmer.

Fairfield's eye was sprouting abnormal blood vessels. In the center of his retina, a cluster of light-sensitive cells called the macula was being invaded by malformed capillaries. As they bled and leaked fluids, the vessels were literally lifting up Fairfield's macula, much like bubbling wallpaper in a damp room.

The macula controls straight-ahead vision, fine detail and color perception. People with wet macular degeneration often cannot read, write, drive, watch television, even recognize faces. Fairfield watched a growing black hole appear on the page as he tried to read a newspaper, and on the road as he tried to drive. A fully lit Christmas tree became a dark outline. He had to stop woodcarving, his main livelihood, because he could not trust his eyes to guide the fine cuts of his sharp tools.

Even the brightly lit navigational tower in the Annisquam River, which Fairfield could see from his kitchen window, vanished. "For 18 years, I could tell the humidity in the air, the cloudiness, just by the tint of that red light," he says. "But when I shut my right eye, it wasn't there."

For eye doctors, diagnosing and treating the disease is a race against time. "If you can get rid of the blister quickly, you have a better chance of keeping what vision you've got," says Steven D. Schwartz, chief of the retina division at UCLA's Jules Stein Eye Institute. "The damage can happen in a day, even in a matter of hours. Just a few drops of fluid in the right place ca ngive you large distortion in your central vision."

Laser therapy can slow down or stop deterioration for some patients. If not halted, the leakage forms a white fibrous scar under the macula. Cut off from food and an oxygen supply, the macular cells wither and die. Because this tissue consists of irreplaceable nerve cells, it cannot regenerate. "Once it's gone, we haven't had a way to bring it back," says Schwartz.

By late February, when Fairfield was scheduled to begin the experimental regimen, he was so desperate that the treatment - a needle, filled with serum, injected directly into his eyeball - was no longer daunting. Heier first numbed and dilated Fairfield's left eye with anesthetic drops, then injected a powerful local anesthetic into the eye. Moments later, Heier injected rhuFab into the vitreous gel that fills the eyeball.

"There was a prick, and definitely some uncomfortableness, but not huge pain," says Fairfield. (Most patients agree that the idea of being jabbed in the eye is far worse than the experience.) The effect was mesmerizing. "When that second needle went in, it was like the ocean washing over your eye," Fairfield says, "It was like being underwater." The drug was swimming through the vitreous fluid toward the retina lining the back wall of his eye.

RhuFab is one of several competing new drugs designed to stop macular degeneration before the scar forms. Called anti-neovasculars, they take aim at the body's prime stimulator of blood vessel growth, a protein called vascular endothelial growth factor. Ordinarily, VEGF is a healer, repairing injured tissue throughout the body. In wet macular degeneration VEGF goes haywire, triggering growth of the oozing blood vessels.

Eye Your Risk

The chances of developing age-related macular degeneration go up if you're over 60, a woman, Caucasian, have a family history of the disease, smoke or have high blood pressure. If you stop smoking and eat right your risk drops. Also:

Take vitamins. Research shows that people at risk for advanced AMD can cut their odds by 25 percent with a high-dose combination of vitamin C, vitamin E, beta carotene and zinc. But check with your doctor first.

Take an eye exam. Keep an Amsler grid - your doctor can give you one - taped to the bathroom mirror. If part of the grid is wavy or missing, call your doctor.

Two new drugs - Genentech's rhuFab and Eyetech's Macugen - scour the eye for free-floating VEGF, bind to it and neutralize it. "It's a potentionally spectacular strategy," says Schwartz, who is testing several of the new drugs. "It seems to stop the growth of these blood vessels, and the leakage."

According to preliminary reports, the drugs may go further. "The new blood vessels apparently disappear." says Frederick Ferris, clinical research director of the National Eye Institute, a branch of the National Institutes of Health. "It's really exciting if it's true, because it holds the possibility of not having to inject into a person's eye for the rest of their life. If you could do it three or four times and that's it - that would be remarkable."

Initial results have been encouraging. Of the 53 patients who've received four monthly injections of rhuFab, 14 have recovered much of their lost sight - three lines or more on an eye chart. Thirty-six others were able to stave off further vision loss. Three patients were not helped. The only side effects were redness and irritation in the treated eye, which lasts for a few days. (For more information on the progress of clinical trials with rhuFab, call 888-662-6728.) Macugen restored vision to one in four patients in a 36 person trial. Alcon is testing another anti-neovascular drug, a steroid called anecortave acetate. Injected behind the eye every 6 months, it has also shown promising results. (Alcon is seeking to enroll patients for clinical trials. The toll-free number is 866-692-5959, or go to www.alconinc.com, click on "USA," then "clinical studies.")

"We haven't seen anything like it before," says Schwartz. "Frankly, it gives me the chills to talk about the potential for meaningful restoration of central vision, because we've never been able to do that. But we still don't know if these drugs are helpful or harmful in the long run."

Experts warn that only a tiny number of patients were enrolled in the trials and their progress was followed for a year or less. Until larger and longer trials are completed in the next few years, scientists won't know if the drugs are truly safe or effective.

"The field is littered with treatments that seemed promising in the short term but in the long term don't seem to make a big difference," says Ferris. The potential risks associated with injections include retinal detachment and infection. No one really knows the lasting dangers of repeatedly poking a person in the eye with a needle.

New Cures For Blindness?

Look out baby boomers. The National Eye Institute reports that blindness in the United States, which now afflicts one million people, may double in coming years as the ranks of older Americans swell. One science fiction-like solution may be a bionic eye. Prototypes have been implanted in blind people, and have restored some sight. One version use three components: a miniature video camera mounted on an eyeglass fram, a signal processor that translates the image, and a brain implant that "sees" the outside world. All the models are still experimental, risky and expensive - as much as $100,000 - but researchers expect to fine-tune their inventions over the next few years. In the meantime, new treatments are emerging that target specific eye diseases.

Cataracts are the leading cause of blindness worldwide. Surgical advances are making cataract surgery safer, quicker and more effective. No-stitch procedures, tinier cuts and so-called phaco-chop - dissolving the cataract with an ultrasonic probe - have made for faster recoveries. The new lenses correct astigmatisms and provide bifocal vision.

Diabetic retinopathy affects one third of the 16 million Americans with diabetes. Retinal blood vessels break down, leak or cause swelling. Doctors think the same experimental drugs use to combat "wet" macular degeneration may help treat this disease. Right now, laser therapy and vitrectomy - removal and replacement of the jelly-like fluid of the eye - can prevent blindness for 90 percent of patients.

Advanced dry macular degeneration, the withering of retinal tissue, has no effective treatment. The disease causes severe vision loss, and millions are at risk. Under study is an experimental technique called rheopheresis, in which a patient's blood is siphoned from one arm, filtered to remove potentially damaging fats and proteins, and returned via the other. The controversial theory is that eliminating "macromolecules" may boost healthy blood flow to the retina.

But patients aren't expressing the same level of scientific caution. Ernest Hayeck, 78, a retired judge in Worcester, Mass., had to abandon reading when his vision deteriorated last fall. After three shots of rhuFab, his eyesight returned to 20/20 with his old glasses on; four months after his last treatment, he's reading the fine print in a textbook. "The cure was as rapid as the degeneration," Hayeck says. "It's a very good feeling to wake up in the morning and open your eyes and discover that you can still see."

Ed Nowak, 82, a photographer and writer in Needham, Mass., went from "a total eclipse of the sun" in his left eye to slight fuzziness. "it's like God opened the gates to heaven and I can see the other side," he says.

Eileen Russell, 77, also from Worcester, became legally blind suddenly last March. After seven rhuFab injections she's regained her 20/25 vision and plans to return to her part-time nursing job. "I got my life back," she says.

The new drugs haven't worked wonders for everyone. One retired physician in Wellesley, Mass., says the black spot is gone, but he still can't read a clock dial from across the room. He blames himself for not seeking help earlier. "Now I think I have too much scarring to reverse the problem."

And Nick Fairfield? He's one of the lucky ones. Within four days of that first injection, he could discern the blinking red light on the Annisquam River tower. Within a month, he could identify features on a television weather map. He's busy woodcarving again, carefully chiseling tiny tear ducts on the eyes of wooden loons.

The other night, Fairfield went outside to admire the full moon. "I saw the whole thing," he said, his voice husky with emotion. His eyesight is still a bit cloudy; he couldn't make out the Man in the Moon.

But Fairfield says he's profoundly grateful. "I'm seeing again," he says. "And that's just incredible."

Second Sight

For the millions of people who are going blind, dramatic new therapies may restore their vision

by Alexis Jetter
Originally published in Reader's Digest, December 2002 issue

Eye Your Risk

New Cures For Blindness?

Second Sight

For the millions of people who are going blind, dramatic new therapies may restore their vision

by Alexis Jetter Originally published in Reader's Digest, December 2002 issue

Eye Your Risk

New Cures For Blindness?

by Alexis Jetter
Originally published in Reader's Digest, December 2002 issue