The Bottom of the Pit
Volume 23, P. 70
Mildred M., a receptionist and insurance clerk at the University of Louisville Eye Clinic, was a large, placid woman. She had a round face and a round body, with long dark hair that spread across her back like a tent. She was loyal, industrious, and trustworthy, but her face remained an unsmiling mask throughout the day, and her habit of chewing gum kept me in a state of distraction. She chewed slowly, incessantly, with a rhythmic side-to-side movement. Each and every time I asked her to schedule a patient for a return visit, she said, “I’ll see what I can do.” Twenty odd times a day, a hundred times a week, five thousand times a year – and without letting up on her chewing for an instant – she said, “I’ll see what I can do.”
Despite her perseverations and dour placidity, Mildred had one extraordinary talent – a photographic memory that allowed her to remember the name and birth date of every patient in our clinic. Before her arrival, we had often suffered the embarrassment of calling some elderly gentleman’s home to find out why he had missed his appointment or refused to pay his bill, only to discover that the poor fellow was lying in Cave Hill Cemetery. Thanks to Mildred, every week or two I would find a chart lying on my desk with DECEASED printed across the cover in Mildred’s perfect hand, block letters like those found in the workbooks of grade-school children. On most occasions, she taped beneath this label an obituary clipped from The Louisville Courier-Journal.
One patient in particular comes to mind – Michael Harvey, a handsome, broad-shouldered fellow who was under my care for ocular histoplasmosis. Since he was only forty-nine and, except for his eye disease, had seemed in good health during a visit some weeks before, I was startled to find his chart lying on my desk with DECEASED printed across the cover. The usual obituary was missing, so I asked Mildred if she knew what had happened.
“It was all over WHAS News last night,” she said, fixing me with her placid gaze. Her jaw maintained its rhythmic orbit. “They showed six patrol cars pulled up in Mr. Harvey’s driveway.”
“What happened?” I asked.
“His wife beat him to death with a pipe wrench.”
“Beat him to death with a pipe wrench! Why the hell did she do that?”
“’Cause he was a plumber,” she replied, her broad face showing no trace of a smile.
* * *
Ophthalmology is a dangerous specialty. If medical students try it once, if they spend even a single day amidst our marvelous array of gadgets, even the most jaded and worldly-wise among them might be lost forever. Yes, of course urologists and cardiologists have their own toys, and proctologists boast a fetching array of probes and endoscopes, but these are trifles compared to what one finds in the offices and operating rooms of ophthalmologists. We have slit-lamp microscopes, optical miracles made by Zeiss of Germany and Haag Streit of Switzerland, whose narrow beams slice deep into the eye’s glittering layers: corneal endothelium, lens capsule, iris crypts and ruffs, diaphanous vascular nets that weave their way through the retina and choroid and optic nerve. Our lasers flash pin-point bursts of energy, reshaping tissues to tolerances no wider than a human hair. Wafer-thin knives peel away scars, while instruments smaller than a pencil lead suck up blood and vitreous gel and emulsified lens material, clearing the visual axis and restoring sight to hundreds of thousands of patients every year.
Yet the blind are still with us – blind men, blind women, and blind children.
As an ophthalmologist who specializes in retinal diseases, I’ve lived happily among my gadgets for over three decades, caring for patients who stand on the threshold of blindness. My colleagues and I have pulled many back from that threshold, and yet, despite our skills and our marvelous tools, patients still go blind. To sustain my sanity, I have hardened my heart against their sorrows. Which is to say, I seldom weep with patients when they learn from my lips that they will never again read or drive, but beyond the usual maladies they suffer – diabetic retinopathy, macular degeneration, obstructed arteries and veins – my practice holds for me one special terror.
At long intervals, perhaps two or three times a year, that terror chases me down. Mildred hails me as I pass her desk, then whispers in my ear, “There’s a kid waiting for you in Lane One. It doesn’t look good.” Mildred’s perspicacity is uncanny. A glance at a note from the referring ophthalmologist or a conversation overheard in the waiting room is enough to set her curious mind aflame. In Lane One I find an innocent, smooth-skinned boy or girl. He may be five or six years old. She may be ten or twelve. Often the visit comes in late August or early September, during the first week of school – a classroom filled with unfamiliar objects.
The teacher has observed an odd behavior, an exceptional clumsiness that – coming on slowly, as these dreaded things always do – has escaped the parents’ notice. The child who stumbles is anxious, fearful of a strange doctor. The parent accompanying the child, almost always the mother, shows in her face and voice a deeper fear. The ophthalmologist who referred the child to me has already aroused the first stirrings of terror: “It could be . . . no, no, we’re not certain, of course . . . but it could be . . . ” This is not the common and hoped-for myopia or astigmatism, disorders easily remedied with spectacles.
Often the child is so small he must kneel in my exam chair to lift his tiny face up to my slit-lamp microscope. Under the beam from this mysterious gadget, his tissues are crystal clear – no trace of corneal opacity, no clouding of the anterior chamber, no hemorrhage in the vitreous jell. The problem lies deeper, far back in the eye, where the retina of a healthy child gleams and glitters like polished glass. But not in this child. Here there is only dullness, a terrible wasting that announces its onset with ragged black strands – a pattern textbooks describe as bone spicules. The normal pastel shades of coral and sienna and burnt umber have vanished, effaced by ugly tracks that ravage the retina, stealing, in the end, the last ray of light from the child’s visual field.
Retinitis pigmentosa. Mildred has scored another hit. I hold the child’s thin arm as he climbs down from the chair, send him to the play room, then explain to the mother: a fragment of genetic code has faltered, a defect beyond the most powerful microscope. Now strands of corrosive rot are consuming her son’s retinas. A molecular mistake, and now those delicate neural tissues – a hundred million rods and cones, a billion axons and dendrites and glial cells, joined in a matrix of wondrous complexity – have begun a downward spiral more destructive than the ravages brought on by a hundred years of aging. The child stumbles because his vision has shrunk to a tiny island in a sea of darkness. How long before he becomes totally blind? Well, it could be years. Three, maybe five. Or it could be . . .
* * *
When I was ten years old, the kids I ran with managed to dig their way into an abandoned septic tank. It gave off a terrific stench, but even worse were the sounds we heard when our shovel broke through the rusted cover – a bubbling, belching rumble as the noxious gas escaped. Our ultimate prize was the hideous black mess at the bottom of the pit. It was so awful we came back again and again to look. One night, flashlight in hand, we convinced ourselves we could see giant worms wriggling beneath the slime. For years afterward, the image sent a thrill of revulsion down my spine.
But the day I started medical school, revulsion lost its savor: the thought of dissecting a human cadaver terrified me, jumping my pulse up twenty beats a minute. I reassured myself with the neurotic’s classic mantra – “calm down you fool, things never turn out as badly as you fear” – but when the moment finally came, I found myself stumbling through a nightmare even more terrible than the one I had imagined. It was late afternoon in an ancient stone-walled building. A long dim corridor lined on both sides by tables, each table covered with a white plastic sheet whose tell-tale bumps betrayed the contour of a corpse laid upon its back. The reek of formaldehyde stung my eyes. My knees wobbled. My heart flopped wildly in my chest. A surge of nausea filled my throat with bile.
Medical students dissect cadavers in order to learn human anatomy, but this is at best a secondary purpose. The first law of medical decorum states that a doctor must not faint dead away when he encounters a gaping wound or a festering peri-rectal abscess, and pedagogy has yet to discover better assurance against such an event than a year spent peeling every fragment of tissue from a malodorous corpse. Formaldehyde turns muscles slate gray, while skin takes on the color of liverwurst. The fecal contents of the colon and small bowel retain their signature bouquet. Worse yet, embalming remains an imperfect science, allowing bacteria to putrify the glistening yellow fat that erupts from the abdominal cavity. Compared to the rancid cadaver I shared with my three anatomy partners, a peri-rectal abscess is a nosegay.
This gross-out treatment worked. After dissecting our way up the lower extremities and deep into the pelvic cavity, all four of us were battle-hardened veterans. We washed our hands before urinating rather than after, shed no tears to formaldehyde fumes, and worked through lunch with two kibitzers munching ham sandwiches while their partners exposed the vertebrae by peeling away long gray strips of paraspinous muscle. At the end of the first semester, one partner insisted that he could dine, twitching nary an eyebrow, from a plate laid in our cadaver’s now all but empty abdominal cavity.
The anatomy lab served its purpose. By the time we had stripped our cadaver down to a skeleton, a gangrenous appendix caused me no more distress than a fly squashed on a window pane. I did not faint or flee the ER when a gush of blood from a severed artery splashed on my shoes. In the operating room, if fumes escaped from a putrid colonic pocket, my stomach heaved and my brow grew damp with sweat, but I did not vomit.
After three years of medical school, I thought myself a tough character. Blood and gore caused no distress, but one night during my ER rotation, while I was watching a surgery resident sew up a split lip, my machismo collapsed. The patient, a tall, willowy high school student who wore her dark hair tied in a pony tail, had been beaten and raped. It wasn’t the wound itself that did me in, the tiny stitches or rivulets of blood oozing from each pierce of the needle, but the victim’s soft, rhythmic sobs – sobs that spoke of irreparable violation. One moment I was peering over the resident’s shoulder, puzzled by an inexplicable melting sensation in my thighs, the next moment I was sitting on the floor with my head tucked between my knees. The orderly standing behind me had eased my slumped body against a wall to keep me from cracking my head. The armpits of my blue scrub shirt were drenched. My heart had been hardened against wounds of the flesh, but nothing I had seen in the lab or the lecture hall could protect me from the revolting cruelty of forced penetration.
* * *
Retinitis pigmentosa: for me the words have a raspy, unpleasant feel on the tongue. They speak of ineluctable blindness, of a child’s world shrinking to a tiny vanishing point. And they offer a special shock to the mother who accompanies the child. Are you and the father blood relatives? If you are – if you succumbed to your cousin’s wealth or his olive skin or his sexy bedroom voice – then mark against yourself a charge of contributory negligence. Retinitis pigmentosa is often inherited as a recessive disorder; to suffer its ravages, the victim must receive a defective gene from both parents, an especially common disaster when that child is born of a consanguineous union. Thus the innocent must bear the curse of their own conception.
Worse still, on occasion retinitis pigmentosa brings a cousin of its own, the vile Usher syndrome, first described in 1858 by Albrecht von Gräfe, a pioneer of modern ophthalmology. Here a genetic defect erodes not only the child’s retinas but his cochleas as well, allowing deafness to ride upon the shoulders of blindness. Over time, the victim’s world vanishes beneath a black and silent sea, leaving behind only what he can touch or taste or smell.
* * *
Diseased tissues are the most hideous things on earth. There is a distance to be measured, measured in units of horror and revulsion, between the loveliness of a healthy eye and the blinding rot that destroys it – a distance that separates the azure sky of a summer day from the bottom of the pit. But, as I discovered near the end of my career in ophthalmology, the ultimate horror comes not from nature, even at her worse, but from the willful hand of man.
Lila Summers. The name still troubles me. Would that I had been spared Lila Summers, or – better yet – that she had been spared her fate. In clinic one afternoon, a few months before I was to assume the comfortable part-time role of professor emeritus, Mildred gave me her somber warning: “Doctor Gamel, I think we’ve got another one.” I wasn’t in the mood to listen, since her previous two warning had missed the mark: one child suffered from a benign retinopathy that resembled retinitis pigmentosa but had only a minimal effect on vision, while the other child was feigning blindness to lure his parents’ attention away from his new-born baby brother.
Lila’s mother was a pale, slender woman, so young the two seemed more like sisters than mother and daughter. Both wore simple white shifts trimmed at hem and sleeve with a double row of cornflowers. Lila carried her right arm in a cast slung about her shoulder. Her right ankle was bound with an elastic bandage. She limped as she entered my office and climbed into the exam chair.
The mother’s cheerful voice carried no hint of the anxiety I had come to expect in such cases, allowing the story she told to creep up on my blind side. She gazed steadily into my eyes. A smile flickered at the corners of her mouth, as though she hoped I would find her tale amusing. Only too late did I discover the truth: what I saw in that face was not humor, but the desperation of a woman-child begging me to laugh at her foolish and misguided fears.
“It’s so silly,” she said, patting Lila’s knee. “Ridiculous, if you ask me. Lila’s drama teacher said I should bring her to an eye doctor. She was there, she saw Lila fall. They were practicing the Nutcracker Suite with the lights turned down so these glow-in-the-dark sparkles on the wings of the sugarplum fairies would show up better. The other kids got around fine, but Lila fell in the orchestra pit.”
The mother leaned forward to touch my wrist. She smiled, giggled, shook her head from side to side to show her exasperation. Her smile said, How absurd. She crossed her legs and smoothed her skirt. She giggled again. Her smile pleaded, Tell me she’s wrong, doctor. Tell me the teacher’s wrong.
I examined Lila, and once again, there it was. My heart didn’t sink only because it had already sunk the instant I heard the story of Lila’s fall. Her retinas were almost gone, two tiny pink spots soon to vanish beneath a ragged black tide. I took a deep breath, forced a smile, sent Lila from the room. I needed more information before unloading this nightmare on the mother.
“Is your daughter hard of hearing?”
“Well . . . yes . . . maybe a little . . . ”
“Ok, ok,” I said, holding up my hand to stop her voice. At that moment, on that subject, I had all the information my reeling mind could handle. I scribbled a note in the chart. “We’ll refer Lila to an audiologist. My receptionist will take care if it. Just a few more questions. Are you and your husband related by blood?”
The mother hesitated. She looked at me. Her face – again, there was something I couldn’t read. Not a frown, certainly not a smile. Confusion? Anxiety?
“You and your husband,” I said, “are you first or second cousins, or something like that?”
“No,” she said at last. “We couldn’t be related. He’s from California, I met him when he was in basic training at Fort Knox. Nobody else in his family has ever come anywhere near Kentucky.”
Perhaps I had asked the wrong question, but never mind. I didn’t say, In five years your beautiful daughter will be blind as a stone, and maybe deaf to boot. No, I said, “We’ll need more tests to be sure, but I suspect your daughter’s vision is failing because of an inherited disease, a disease she was born with. It may take many years before . . . We can’t be sure when . . .”
As the truth dawned in the mother’s widening blue eyes, she clutched my arm and pressed the other hand against her mouth. I patted her shoulder, offered a box of tissues, waited through long, terrible minutes of choking sobs. Then came the usual litany – “But doctor, can’t you do something? . . . You mean there’s no treatment, nothing at all?” – repeated four or five times in a quavering voice, the tone incredulous one moment and angry the next. At last she fell silent. Her nose and cheeks were fiery red, but her eyes were dry. I walked her to the appointment desk with my arm across her narrow shoulders, then bucked myself up to examine my last ten or twelve patients. Finally – exhausted, dispirited, eager for my impending retirement – I strode through the waiting room and snatched open the door to the parking lot.
Mildred sat alone at her desk, stolid and mountainous as ever. I had passed without noticing her. She shouted across the empty room.
“Doctor Gamel, that woman, Lila Summer’s mother, she said I should tell you something.”
I propped the door open with my foot and turned around.
“What?” I shouted back.
“She didn’t want to tell you herself. Said she felt too nervous.” Mildred sounded nervous herself, her usual monotone rising to a loud quaver. Very annoying.
“Tell me what?”
“Her father – he was the father.”
“It wasn’t her husband, it was her father. He was the father.”
My god, what was that idiot going on about? The stack of charts clutched under my arm had to be dictated that very evening. The next day I would meet with our department’s new chairman, a reputed tyrant who might reject the retirement contract I had negotiated with the old chairman. The last thing I wanted to deal with was Mildred’s gibberish.
“Thanks,” I shouted, then let the door slam behind me. Half way home it struck me. I pulled off the road into a service station. My car was stopped at an odd angle, well away from the gas pumps. I nearly vomited. It took a long time for the waves of nausea to pass. The attendant came up to my car, wiping grease from his hands with a rag. I rolled down the window.
“You all right?” he said.
“Yea, I’ll be fine,” I said. “Just give me a few minutes.”
“If you don’t want gas, you better get the hell out of the way.” Now he was shouting. “Can’t you see – there’s a goddamn eighteen-wheeler blocked up behind you?”
* * *
Lila’s audiology tests revealed marginal values in the higher frequencies but no definite abnormality. Thus, with a little luck, she will someday hear the laughter of her grandchildren. At my retirement, soon after her first appointment, I turned her care over to our new chairman, a world-renowned retinal specialist whose skills, like mine, could offer not the slightest hope of extracting the cruel seed planted in every cell of Lila’s body. During the six years since my retirement, he has told me nothing about her progress, and I have chosen not to ask.