THE ELEGANT EYEBALL by John Gamel
They aren’t what most people think they are. Human eyes, touted as ethereal objects by poets and novelists throughout history, are nothing more than white spheres, somewhat larger than your average marble, covered by a leather-like tissue known as sclera and filled with nature’s facsimile of Jell-O. Your beloved’s eyes may pierce your heart, but in all likelihood they closely resemble the eyes of every other person on the planet. At least I hope they do, for otherwise he or she suffers from severe myopia (near-sightedness), hyperopia (far-sightedness), or worse.
Such uniformity is essential: for an eye to focus properly, its length and optical system must match to within a fraction of a millimeter. When a man and woman toss their genes together to make a baby, nature sets the focal point (determined by the optical power of the cornea and crystalline lens) at a standard distance, then adjusts the length of the eyeball to that same distance – twenty-four millimeters, or about one inch, with a few millimeters of variation thrown in for good measure. Thus unlike livers and kidneys and hearts and brains – those ordinary, non-spherical organs – eyes tend to an impressive sameness all over the world. My spleen may be half again bigger than yours, intestines can vary by five feet in length from person to person, but, with rare and usually disastrous exceptions, eyes are like so many peas in a pod.
Trust me. I’ve handled hundreds of eyeballs, removed from their owners for a variety of unpleasant reasons. One of my jobs – that of the ophthalmic pathologist – is to slice these globes into wafer-thin strips, stain the strips with vivid colors, then examine the results under a microscope. Given these credentials, I can assure you that your lover’s eyes differ from those of your most despised enemy in only the most superficial ways – in the color and texture of the iris and in the size of its pupil. When we wax eloquent about “beautiful eyes,” we are usually moved more by the trimmings – the lids, the lashes, the prominence of the globe in its orbit – than by anything contained within the eye itself. The Japanese sometimes refer to Westerners as “big eyes,” an illusion produced by the lid position and orbital structure of Occidentals, while in truth Asians exhibit a collective tendency toward myopia that gives them on average slightly larger eyeballs.
* * *
A crisis came upon me during my fourth year of medical school. This was the crucial moment, the ultimate decision: to what specialty would I devote my life? Should I tend to phlegmy children who wriggle and scream and scratch my flesh when I thrust an otoscope into their ear? Should I slice open bellies, wander among livers and spleens and gallbladders, grope my way through greasy omental fat pads to explore coil after coil of diseased intestines? Or should I tend to the human heart, throbbing in its nest between the foamy pink lungs?
I flirted with cardiology, then settled on neurology. Nothing rivals the complexity of the human brain, I reasoned, and no goal is more noble than curing its various ailments. The ultimate philosophical dialectic: using the skilled synapses of my own brain, I would diagnose and cure the diseased brains of others. Fortunately, before it was too late, a six-week elective in neurology revealed the terrible truth: almost every neurology patient suffers from a stroke or a seizure or an incurable brain tumor, and they never – NEVER – get better. Worse yet, the rare patient with a curable lesion is snatched up by the neurosurgeons, arguably the most arrogant species on earth. By the end of the elective I felt like a zombie myself.
How about ophthalmology? Clean, precise, offering its own dialectic: with my intact eye I would diagnose and cure the diseased eyes of others. It didn’t take long, only one good look into the ocular depths through a dilated pupil, and my quest was finished. There before me lay a stunning image – a lacework of arteries and veins delicate as a spider’s web, spread on a burnt umber palate swirled and streaked with shades of ocher. Most spectacular of all was the retina, a transparent wafer that gleamed like polished glass under the light of my ophthalmoscope. In the center the optic nerve shone like a rising sun. I was in love.
* * *
Since every normal eye displays a clear cornea and a white scleral coat, any notion of special beauty attributed to the globe itself must derive from the iris, the dynamic membrane that contains the pupil and rests in front of the crystalline lens. The iris comes in many colors, but if one trusts the obsession of poets and novelists, the most beautiful irises are always blue: light-blue, velvety-blue, sloe-eyed, peacock, midnight, cobalt, ice-blue. Green gets an occasional nod – “she had jewel-bright emerald eyes, so lustrous and fetching they tore through my heart” – but most of the time blue runs the show: powder-blue and baby-blue, cerulean and aqua. Azure. Turquoise. The rankest discrimination. And a bit ironic, since blue irises contain no intrinsic pigment, showing only the raw color of the tissue itself. The pigment-secreting cells of Caucasians often remain inactive during the first few years of life, breaking the heart of many a parent when their blue-eyed special baby darkens over the years into brown-eyed mediocrity.
The texture of the iris is all but invisible to the unaided eye, but the ophthalmologist’s slit-lamp microscope discloses a panorama of crypts and valleys, diaphanous spokes, flecks and spots and strands that dance about with each twitch of the pupil. Dark irises tend toward a tight weave, while light irises fluff up like a shag rug. And then there’s the all-important pupil – squeezed into a dot by morphine and bright light; enlarged by fear, darkness, sexual arousal, and death. Yes, the coroner’s final measure, the mark of a departed soul – enormous black pupils that give nary a twitch to even the brightest light. Despite this morbid association, many cultures consider large pupils a sign of beauty. “Belladonna,” Spanish for “beautiful lady,” is also the name of a poisonous, pupil-dilating extract from the plant Atropa belladonna, more commonly known as deadly nightshade.
A note on cosmetics: under an ophthalmologist’s slit-lamp microscope, false lashes look like mutilated telephone poles, while mascara shows up as greasy black chunks that squiggle across the corneal tear film with every blink. For the efficiency-minded woman there is permanent eyeliner – a dark line tattooed along the lid margin. It works beautifully, provided styles don’t change, and provided the tattooist, working millimeters from the cornea, doesn’t inject ink into the eyeball.
About myopia – if you have it, be happy. As shown by numerous scientific studies, near-sighted men and women boast a higher average intelligence than their non-myopic cohorts. The precise mechanism of this association remains unknown, but it is tempting to postulate an effect of myopia on early childhood development. Most near-sighted kids wander around undiagnosed for years, and during this formative period – unable to see the baseballs, Frisbees, and rocks thrown at them by their playmates – they spend a lot of time indoors. The non-athletic myopes who take up reading to while away the hours get high scores on their SATs, while those who take up eating tweak our claustrophobia by overflowing the adjacent seat on airplanes. Myopia also exerts a compelling influence on career choice: the great majority of my fellow ophthalmologists wear either myopic contact lenses or thick myopic spectacles. Pathology breeds preoccupation.
* * *
However beautiful the human eye, it serves a more important purpose than romantic allure. Forty percent of the brain is devoted to vision, which provides us with more information than our other four senses combined. Our optic nerves transmit millions of impulses to the brain every second, impulses that specify the location, color, and intensity of light for all the points in our visual space. Better yet, thanks to a mysterious algorithm that fuses the slightly disparate images from each of our eyes, our visual cortex, via a neurological miracle known as depth perception, shows us the world in three dimensions. An impressive feat, since a video camera, arguably the benchmark of modern technology, can muster only two dimensions.
Certain ocular tissues stand on the pinnacle of evolution. How does nature, so crude in claw and fang, create a surface that brings light to a pinpoint focus? This surface must be perfectly curved, perfectly transparent, perfectly smooth. It must be – water! Which is to say, the cornea owes its optical perfection to a tear film whose dissolved salts, lipids and proteins maintain a flawless wetted surface. A man who has no tears stands on the brink of blindness. Worse yet, that man will writhe in agony: a dry cornea, thanks to the most exquisite pain threshold in the human body, responds to each blink with a tormenting jolt. Dry-eye victims compare the sensation to that caused by rubbing the eyeball with shards of glass.
Another challenge surmounted by evolution: for light to reach the retina unimpeded, the cornea and lens must remain transparent, and yet, like all living tissues, they must be nourished. More than 99.9 percent of human cells obtain their nourishment from capillary blood flow, but capillaries passing through the cornea and lens would veil our vision in a net of opaque strands. To remain crystal clear, the outer portion of the cornea must survive on oxygen absorbed from the surrounding air, while the lens and the inner cornea depend on the flow of aqueous, a transparent, oxygen-poor fluid that circulates through the inner chambers of the eye. This fluid derives from cells sheltered behind the lens and exits through trabeculated channels located near the base of the iris. Blockage of these channels causes nauseating glaucomatous pain, while a deficiency of aqueous flow collapses the eye into a spitball. Thus pain-free normal vision, the presumed birthright of every human on earth, demands an arrangement of tissues so wondrous and complex its subtleties should take our breath away.
* * *
Of all the ugly things in this world, I would argue that diseases top the list: cancer, syphilis, leprosy, gangrene, fungating ulcers. Even the pictures lying flat and odorless on the pages of a textbook bring a surge of nausea. And let us not forget elephantiasis, an infestation by microscopic worms that wriggle into the lymphatic system, causing an accumulation of lymph so massive it forces the victim to stumble about on legs the size of tree trunks, carrying his scrotum before him in a wheelbarrow.
But surely the eye, the most delicate human organ, is afflicted by only the subtlest diseases. Or so one might think. I soon discovered the fallacy of this logic. Indeed, some of the most grotesque diseases known to medicine are those that disfigure the eye. Ophthalmology did not prove the sanitary refuge I had hoped for. On the second day of my student elective in the Stanford Eye Clinic, I examined Justine Jewell, a slender diabetic in her late teens. She was accompanied by a tall mother who carried twice her daughter’s bulk. Justine complained, “My eyes are full of floaters.” Good, I thought. Floaters. No problem. Everything looked fine from the outside – white sclera, clear corneas, pale blue irises. Then I shined my ophthalmoscope through her pupils to examine the interior. “Excuse me,” I said, and stepped out of the room.
By that time in my career, I had seen the interior of ten or twelve eyes, each a breathtaking panorama of amber and brown, yellow and pink, shading through a delicate lacework of arteries and veins. But Justine’s eyes were filled with tangles of angry red spiders. Dark clots rose into the vitreous gel, trailing streamers of blood in all directions. I rushed into the hall to grab Doug Jacobson, the retinal specialist in clinic that morning. It took only an instant. Doug focused the beam of his ophthalmoscope on Justine’s right eye, then her left, removed the ophthalmoscope from his head and hung it on the wall.
“You have diabetic retinopathy,” he said. “And I’m sorry to say it’s very advanced.”
The mother burst into tears. “Oh, doctor,” she sobbed, “my grandmother, my cousin Ernest, this woman across the street – so many people I know went blind from diabetes! Can’t you do something?” Justine said nothing. Her eyes were dry, wide open, the blue irises stretched into pale rims around the blackness of her dilated pupils. Later, in private, Jacobson gave me her diagnosis in the vernacular – jungle-osis. Jungle-osis meant dense black clots, arching streamers of blood, a traction retinal detachment bound with scars so dense they defy the reparative efforts of even the most skillful surgeon. It meant blindness, both eyes, and soon – weeks, perhaps a month or two. Justine, not yet twenty years of age, was doomed to stumble through the remaining decades of her life with a white cane or a guide dog. Worse yet, she might develop absolute glaucoma, an intractable rise in pressure so painful and nauseating the victims often beg to have the eyes removed.
But – perhaps not. Justine’s only hope was a treatment so recently developed we had no proof that it worked, a treatment whose promise was based on the crudest evidence. For decades, ophthalmologists had noted a strange phenomenon: when one eye of a diabetic showed widespread retinal scars from an old injury or infection, that eye often retained vision long after diabetic hemorrhages had blinded the unscarred eye. Apparently, by a mechanism no one understood at the time, these scars protected the surviving retinal tissue from the ravages of diabetes. And so, by a logic that might impress a blacksmith or a witch doctor, the new treatment called for obliterating much of the nonessential peripheral retina in an effort to save the central portion that gives us our sharpest vision. Since we had no other option, Jacobson advised Justine – a young girl poised on the brink of blindness, dry-eyed and speechless with fear – to let us perform an experiment on her.
Just months before Justine’s arrival, our clinic had acquired the Coherent Radiation Model 800, one of the first lasers used to treat the human eye. Its console, six feet long and three feet high, looked like a coffin on legs. A glass tube buried deep within the bowels of its circuitry gave off a high-pitched whine. When activated by a foot switch, the tube emitted an eerie, bluish-green beam of light. Shone against a wall, the beam formed a circle of shimmering motes that scurried about like atoms in a nuclear furnace. A fabulous instrument, more precise than any razor, but now its tightly focused beam was to serve a crude purpose – destroying retinal tissue. By the hundreds, by the thousands, the laser emitted tiny flashes, each flash the space-age equivalent of a magnifying lens burning a hole in a leaf. When the treatment was complete, lifeless white scars obliterated more than half of the patient’s peripheral retina. Care was taken to avoid the central portion, assuring that, if the treatment proved successful, the patient might continue to read and drive.
Justine suffered. To dull the pain from those hundreds of burns, we injected Xylocaine deep behind the eye. The contact lens used to deliver the laser beam sometimes caused a painful corneal abrasion. For three or four days after every treatment, fluid leaking from the peripheral burns seeped into the central retina, blurring and distorting her vision. Her mother was always there, clutching an enormous purse against her chest, squeezing her eyes shut every time her daughter moaned under our long needle. But after six treatments the vitreous hemorrhages began to clear. The tangle of spiders melted away. Four months after Justine’s first visit, Jacobson announced, “That’s it. All the hemorrhage is gone.”
Justine’s mother burst into tears, dropped her purse on the floor and threw her arms around Jacobson. His face blushed fiery red as he struggled against her grip, muttering, “No, no . . . it’s too soon to tell . . . ” but he was a small man, an inch or two shorter and many pounds lighter than the joyful mother.
Four years later, during the last months of my residency, Justine’s vision was still 20/20 in both eyes. There was no trace of hemorrhage, nor of the spidery vessels that signal recurrent proliferative retinopathy. Over the next three decades, recoveries similar to Justine’s would number in the hundreds of thousands as laser treatment became the gold standard for diabetic retinopathy. A study published in 1976 showed a four-fold reduction in visual loss among treated patients, but modifications to the original method eventually reduced total blindness among diabetics to a tiny fraction of the original incidence. By the late 1990s, charitable institutions had delivered improved versions of the Coherent Model 800 Laser to developing nations across the globe. Unfortunately, there remains a dark side to this story: many diabetics slip through the system, seeking care only when rampant scars have obliterated all hope of treatment, while some patients suffer an attack of retinopathy so fulminant and destructive, even the most timely therapy cannot sustain the acuity needed to drive or read.
Despite these limitations, laser surgery for proliferative retinopathy has proved a medical triumph of the first order. Here is something crude in principle, simple to perform and easily learned, but it works. In the miracle that defeated jungle-osis, my role – treating thousands of patients with the laser, plus teaching the procedure to almost a hundred residents – has been the greatest privilege of my career.
* * *
Despite laser surgery and a host of other high-tech developments, the blind are still with us. If we live long enough, our eyes will always fail us. Most ten-year-olds can count the legs on an ant, while only the rare nonagenarian can see the ant itself. Sooner or later our eyes, along with our knees and our hearts and our hair, will surrender to the ravages of Mother Nature and Father Time. Before this vile duo sets upon their victim, the sclera is snowy white, the ocular media – composed of the cornea and lens and vitreous gel – remain crystal clear. Under a bright light, the retina shimmers like beaten silver. Indeed, the sparkle we see in children’s eyes is no illusion. But by our twenties, the shank of young adulthood, the luster has begun to fade, foreshadowing the greenish cataract and rheumy yellow sclera of senescence.
When Father Time lets fly his first cruel arrow, the target is often our crystalline lens, a lentil-shaped tissue that hangs behind the iris, suspended by a thousand translucent filaments. Tension on these filaments allows youngsters to focus their eyes from near to far with the ease and precision of a Nikon camera. But in order to sustain its marvelous clarity, the lens must survive without capillaries, nourished only by a clear, oxygen-poor aqueous fluid. Such metabolic tenuousness leaves the lens vulnerable to every biological assault. Like the canary in the mine, it is often the first tissue to fall victim when subtle toxins or the ravages of aging attack our body. Over the years, our lenses first lose the elasticity that allows them to alter their focus from distance to near, heaping upon us the curse of bifocals or reading glasses. Then, over the following decades, inexorable as an unloved season, cataracts appear, diffracting light into haloes, casting an odd tint on familiar objects, eventually drawing a dark veil over our world.
If granddaddy lives long enough, he won’t be able to read, but if he’s lucky – if cataracts are the only cause of his impairment – twenty minutes at the hands of a skilled surgeon will do the trick: voila, the opaque lump is gone, sucked out through a vibrating needle and replaced by an acrylic lens the size of a cornflake. The next morning granddaddy will pour over his morning newspaper as happily as he did in his twenties.
* * *
“It’s macular degeneration, isn’t it, doctor?”
“Yes, I’m afraid so.”
To see clearly, we need more than a clear cornea and a clear lens. Much more: a tissue that transforms light into nervous impulses (the retina), plus a cable to transmit these impulses to the brain (the optic nerve) and a sentient organ to receive and process them (the visual cortex). Of the tissues in this chain, arguably the most complex and vulnerable is the retina, a delicate, multi-layered, altogether wondrous membrane. Alas, against the retina, as against the lens, Mother Nature and Father Time marshal their terrible forces. Among those patients who live beyond their sixth decade, an ever-increasing proportion show degeneration of the macula, the central portion of the retina and the site of its most active, densely-packed neurons. First the macula begins to lose its silvery sheen, then it fades slowly into a ragged, lusterless wasteland known as dry macular degeneration – a progress akin to the inexorable wrinkles and liver spots that transform a baby’s face into the face of a crone. Year by year, line by line on the acuity chart, the victims lose their vision. Worse yet, year by year the dread disease expands its roster of victims until, among the few that reach the century mark, virtually none are spared its cruel devastation.
“Doctor, when I got up this morning there was a black spot in my right eye. It blocks out everything I look at.”
This was Sister Maria, an eighty-four-year-old nun. I have heard similar words from a sixty-nine-year-old railroad engineer and an eighty-six-year-old former prizefighter with a crooked nose. A colleague of mine, a seventy-three-year-old professor of pathology at the University of Louisville School of Medicine, spoke more succinctly. One afternoon he got up from his microscope, walked across the street and barged into my clinic. “God dammit, Gamel,” he said, “what the hell’s going on with my eye?”
These were the unlucky ones. Most patients with macular degeneration suffer the “dry” form, which steals vision slowly, year by year, but a small percent suffer an abrupt hemorrhage or leakage beneath the retina that marks the onset of “wet” macular degeneration. From that point on, every object they look at disappears into a black hole. Fate makes only one concession: though the blind spot tends to enlarge over time, and though it quickly destroys the ability to read or drive, it rarely obliterates all sight, allowing the great majority of sufferers to navigate a familiar environment.
“Doctor . . . please . . . tell me . . . is there a treatment?”
Dry-eyed or weeping, motionless or wringing their hands, clear-voiced or choked with fear, sooner or later every patient with macular degeneration will ask the same question. Theory offers two potential cures: transplanting the eye, or replacing the retina and its supporting structures. For the moment, both procedures remain well beyond the reach of science. The complexity of the retina rivals that of the brain itself. To transplant either organ, the surgeon must reconnect millions of axons – microscopic neural tubes so fragile the subtlest trauma destroys them forever. I suspect this achievement will elude the best surgeons for generations to come.
Thus at the moment we can boast only paltry progress against macular degeneration. For patients with the dry form, the only proven remedy is a regimen of vitamins and antioxidants that delays – but does not stop – the insidious loss of vision. On a more positive note, recent advances in molecular biology have given us a panoply of new treatments for wet macular degeneration. Though vastly more effective than the therapies available during my early career, these miracle molecules remain an imperfect cure: they must be injected repeatedly into the eye, they improve vision in only a small proportion of patients, and of these only a lucky few sustain the improvement for the remainder of their lives. Despite the triumphs of modern medicine, Father Time and Mother Nature will eventually have their way with us. Decay is written into our genes. It is our destiny.
* * *
Of all my patients with macular degeneration, Hans Bergerman proved the most astute observer. No surprise, given his curriculum vitae: Professor Emeritus, former Chairman of Stanford’s Department of Anthropology, editor of five books, author of two hundred academic publications. His bushy brows and bald, sun-darkened head gave him a gnomish look. Born in Brazil of German parents, he spoke with a crisp accent and sat stiff as a soldier in the exam chair. He never took his dark eyes off me for a moment.
“Let’s see how this matches yours,” he said, handing me a sketched outline of the dark blur he had noted in his right eye. I had just finished my exam and was drawing my own picture of the lesion that lurked beneath the macula of that eye. Both drawings resembled a childish doodle of a wolf’s head, but in my doodle the snout and ears were marked with a red pencil to show streaks of sub-retinal blood. The wolf’s bulbous jowl was formed by a tangle of vessels that threatened to hemorrhage at any moment, destroying forever Bergerman’s central vision. The diagnosis was crystal clear: wet macular degeneration. Several years before, the same disease had destroyed the central vision in the patient’s left eye.
Bergerman was lucky. Wet macular degeneration usually strikes precisely in the central portion of the retina, where laser therapy, the only option available back in the 1970s, would cause instant blindness. His lesion lay a fraction of a millimeter removed from the center. A shiver of anxiety ran down my spine. I had to treat the poor man by cauterizing the tangle of vessels with a laser beam. The zone that divided success from disaster was devilishly narrow, no greater than the width of a few human hairs. Doug Jacobson, the faculty attending who watched my every move through the viewing tube on the slit-lamp microscope, could not have guessed the quantity of sweat that soaked my armpits as I fired dozens of blue-green flashes into Bergerman’s eye.
Three months later, Bergerman said, “Thank you, doctor.” His vision was 20/30. The tangle of vessels had shrunk to a dry, flat scar. He thanked me again two years later, the day he awoke to find a huge black spot in the center of his vision. My treatment had failed. I knew it would fail – all laser treatments eventually failed, unless the patient died first – but the sight of that dark clot mounded beneath his retina broke my heart.
“Thank you very much,” he said. “Your laser allowed me to read for an extra two years.” On his way out of the exam room, he stopped to shake my hand and squeeze my shoulder. He gave me a rueful smile. “Why so sad, doctor? You look like you just lost your best friend. Who do you think you are – a magician, a god who turns old men into young men?”
* * *
Boris Osterhaus was a gray-haired farmer from Cecelia, Kentucky. His pot belly stretched the bib of his denim overalls tight as a drum. Minutes after arriving in my office, he pulled a pouch of Red Man Chewing Tobacco from the pocket of his denim shirt, then – remembering that this was neither the time nor the place to tuck a wad into his mouth – grimaced and stuffed the pouch back in the pocket. An optometrist in Cecelia had referred him to an ophthalmologist in Elizabethtown, the ophthalmologist had referred him to me, and now, after a hundred miles over back-country roads, Boris learned from my lips that he would never read or drive again. A dry, pock-marked wilderness had destroyed the macula in both of his eyes. When I finished my dismal spiel – a diplomatic version of “Mother Nature is a bitch, Father Time is a son of a bitch, and there’s not a damn thing we can do about it” – Boris leapt out of his chair to grip me in a knuckle-cracking handshake.
“Thank you, doctor, thank you so much. I just can’t tell you how good it is to finally hear the truth straight up and down. Now I can get that confounded woman” – he gestured toward the stern-faced daughter who had brought him – “to stop dragging me all over the county. She keeps saying nowadays you doctors can fix anything. What a load of rubbish! I’ve lived eighty-three hard-bit years, and ain’t nobody in my family been able to read much after they was seventy-five or eighty. That’s just the way it is. I knowed it all along, but she wouldn’t listen.”
* * *
Patients are more than the sum of their failing parts. The wisest among them know that life cannot be cured, but even they need someone to inform them, and – when healing fails – to accompany them on the lonely road to disability and death. I did not learn this truth in a book or a laboratory or a lecture hall. My patients taught it to me. They came in desperation, returned year after year to share their struggles, and, as the years passed, they died. One way or the other I always lost the battle, but they gave me many precious moments. The eye begins as a perfect thing, a miraculous organ, but its luster, mortal and doomed as life itself, fades with each passing year. I watched it all through my slit-lamp microscope. I watched my patients grow old, and slowly, decade by decade, they taught me how to do it.
John Gamel’s essays have appeared in Boulevard, The Antioch Review, and The Kenyon Review. This is his second appearance in Alaska Quarterly Review.