Chapter three

A ROLE in the BODY

AS A BOY GROWING UP IN INDIA, I idolized my missionary father, who responded to every human need he encountered. Only once did I see him hesitate to help: when I was seven, and three strange men trudged up the dirt path to our mountain home.

At first glance these three seemed like hundreds of other strangers who came to our home for medical treatment. Each man wore a breechcloth and turban, with a blanket draped over one shoulder. As they approached, however, I noticed telltale differences: a mottled quality to their skin, thickened foreheads and ears, feet bandaged with strips of blood-stained cloth. As they came closer, I noticed they also lacked fingers and one had no toes, his legs ending in rounded stumps.

Something ominous was happening, and I didn’t want to miss it. After calling my father I scrambled on hands and knees to a nearby vantage point. My heart pounded as I saw a look of uncertainty, almost fear, pass across my father’s face. I had never seen that expression on my father.

The three men prostrated themselves on the ground, a common Indian custom that my father disliked. “I am not God—he is the One you should worship,” he would usually say and lift the Indians to their feet. Not this time. He stood still. Finally, in a sad voice he said, “There’s not much we can do. I’m sorry. Wait where you are, don’t move. I’ll do what I can.”

He ran to the dispensary while the men squatted on the ground. Soon he returned with a roll of bandages, a can of salve, and a pair of surgical gloves he struggled to put on. This confused me, as he seldom wore gloves while treating a patient. Father washed the strangers’ feet, applied ointment to their sores, and bandaged them. Strangely, they did not wince or cry out as he cleaned and wrapped their sores.

Meanwhile, my mother had arranged a selection of fruit in a wicker basket. She set it on the ground beside the visitors, suggesting they take the basket. They took the fruit but left the basket, and as they disappeared over the ridge, I went to pick it up. “No, Paul!” Mother cried out. “Don’t touch it! And don’t go near that place where they sat.” I watched Father take the basket and burn it, then scrub his hands with hot water and soap. Then Mother bathed my sister and me, though we had had no direct contact with the visitors.

That incident was my first exposure to leprosy, the oldest recorded disease and one of the most dreaded. Although I would have recoiled from the suggestion as a boy of seven, I later felt called to spend my life as a doctor among leprosy patients. I have worked with them almost daily, in the process forming many intimate and lasting friendships among these misunderstood and courageous people.

Over those years, many of the fears and prejudices about leprosy have crumbled, at least in the medical profession. Partly because of effective drugs, it is now viewed as a controllable, barely infectious disease. Leprosy got its fearsome reputation because of the visible damage it does to the body. Even to those who are taking drugs, it remains a disease that can cause severe lesions, blindness, and loss of hands and feet. Only in my lifetime have we learned how this ancient disease produces such terrible effects.

As I studied patients in India, several findings pushed me toward a rather simple theory: perhaps the horrible effects of leprosy come about because leprosy patients have lost the sense of pain. The disease does not spread like flesh-eating bacteria; rather, it attacks a single type of cell, the nerve cell. When that nerve cell falls silent, it no longer warns of danger, and the painless person quite literally destroys his or her own body.

After years of testing and observation, I felt sure that the theory was sound. After many false starts, my team learned to track how the damage occurs. A person uses a hammer with a splintered handle, does not feel the pain, and an infection flares up. Another steps off a curb, spraining an ankle, and, oblivious, keeps walking. Another loses use of the nerve that triggers the eyelid to blink every few seconds for lubricating moisture; the eye dries out and the person becomes blind. Virtually all the devastating effects of leprosy trace back to a single source: one type of nerve cell that has fallen silent.

Solitary and Communal Cells

I remember the first time I saw a living cell under a microscope. I had snuck into a college lab early one morning with a teacup of brackish liquid I’d scooped from a pond. Bits of decomposing leaves were floating on top, emitting the musty odor of organic decay.

No sooner had I touched one drop of pond water to a glass slide under the microscope than a universe sprang to life. Hundreds of organisms crowded into view: delicate, single-celled globes of crystal unfurling and flitting sideways, excited by the warmth of my microscope light. I edged the slide a bit, glancing past the more lively organisms. Ah, there it was: an amoeba. A mere chip of translucent blue, barely visible to my naked eye, it revealed its inner workings through the microscope.

This simple, primordial creature performed all the basic functions of my human body. It breathed, digested, excreted, reproduced. In its own peculiar way it even moved, jutting a bit of itself forward and the rest following with a motion as effortless as a drop of oil spreading on a table. After one or two hours of such activity, the grainy, liquid blob would travel a third of an inch. Though in appearance a meager bit of gel, the amoeba manifested life, which differs profoundly from mere matter.

That busy, throbbing drop of water gave me a lasting image of the jungle of life and death we share, and beckoned me to further explore living cells.

Years later I am still observing cells, though as a physician I focus now on how they cooperate within the body. I have my own laboratory at a leprosy hospital on swampy ground in the bayou country of Louisiana. Again I enter the lab early, before anyone is stirring, and only the soft buzz of fluorescent lights breaks the quiet.

This morning I will examine a hibernating albino bat who sleeps in a box in my refrigerator. He helps me understand how the body responds to injury and infection. I lift him carefully, lay him on his back, and spread his wings in a cruciform posture. His face is weirdly human, like the shrunken heads in museums. I keep expecting him to open an eye and shriek at me, but he doesn’t. He sleeps.

As I place his wing under the microscope lens, again a new universe unfolds. The albinic skin under his wing is so limpid that I can look directly through his skin cells into the vessels underneath. I focus the microscope on one bluish capillary until I can see individual blood cells pushing and thrusting through it. The pulsing fluid is like a river stocked with living matter: a speck of blood the size of this letter o contains five million red cells and seven thousand white cells.

I am searching for white blood cells, the body’s elite special forces, which protect against invaders. Transparent, bristling with weapons and possessing a Houdini-like ability to slip between other cells, the white cells function as the body’s advance guard. Flattened on a microscope slide, they resemble fried eggs sprinkled with pepper.

As I stare, the white cells remind me of the amoeba I first saw as a student in England. Amorphous blobs of liquid, they roam through the bat’s body by extending a finger-like projection and hunching along to follow it. Sometimes they creep sideways on the walls of the veins; sometimes they let go and free-float in the bloodstream. To navigate smaller capillaries, the bulky white cells must elongate their shapes, while red blood cells jostle impatiently behind them.

An observer can’t help thinking white cells sluggish and ineffective at patrolling territory—until an attack occurs. I take a thin, steel needle and, without waking the bat, prick through its wing to puncture a fine capillary. Instantly, a silent alarm sounds. Muscle cells contract around the damaged capillary wall, damming up the loss of precious blood. Clotting agents halt the flow at the skin’s surface. The most dramatic change, though, occurs among the listless white cells.

As if they have a sense of smell, nearby white cells abruptly halt their aimless wandering. Like beagles on the scent of a rabbit, they home in from all directions toward the point of invasion. Their unique shape-changing qualities allow them to ooze between the overlapping cells of capillary walls. When they arrive, the battle begins.

Lennart Nilsson, the Swedish photographer renowned for his remarkable close-ups of activity inside the human body, has captured the battle on film as seen through an electron microscope. In the distance, a shapeless white cell, resembling science fiction’s creature “the Blob,” lumbers toward a cluster of luminous green bacterial spheres. Like a blanket pulled over a corpse, the cell assumes their shape; for a time the bacteria glow eerily inside the white cell. But those pepper-like dots inside the white cell are granules of chemical explosives, which soon detonate and destroy the invaders. In thirty seconds to a minute, only the bloated white cell remains.

Although the battle often results in the white cell’s demise, the death of a single cell has little significance. Besides the fifty billion active white cells prowling the adult human, a backup force one hundred times as large lies in reserve in the bone marrow. When an infection occurs, these reserves leap from the marshes of bone marrow, like beardless young recruits pressed into service. The body can thus mobilize a vast number of white cells; indeed, doctors use a count of them as a diagnostic test to judge the severity of infection.

Each day we live at the mercy of organisms one-trillionth our size. A drop of water may contain as many bacteria as there are humans on earth. Bacteria enshroud my body: when I wash my hands, I sluice five million of them from the folds of my skin. Immunologists share a little joke that they cite when asked how the body can possibly prepare every type of antibody required in our perilous world: GOD, they reply—an acronym for “generator of diversity.”

If the body has previously identified a known threat, as in a smallpox vaccination, it imprints certain white cells with a death wish to target that one danger. These cells spend their lives coursing through the bloodstream, alert, scouting. Often the summons to battle never sounds. If it does, however, they hold within them the power to disarm a foreign agent that could destroy every cell in the body.

Medical author Ronald J. Glasser concludes rather humbly, “No matter how we may wish to view ourselves, despite all our fantasies of grandeur and dominion, all our fragile human successes, the real struggle . . . has always been against bacteria and viruses, against adversaries never more than seven microns wide.” He describes the process as “a mixture of mystery and chemistry . . . a combination of physics and grace down at the molecular level.”

If we as doctors were forced to choose either (1) the human immune system alone or (2) all the resources and technology of science but with the loss of our immune system, without a moment’s hesitation we would choose the former. The disease AIDS exposes the helplessness of modern technology when a person’s immune system shuts down: pneumonia, cold sores, or even diarrhea can pose a mortal danger.

Specialization: Loss and Gain

Dig up a block of forest soil one foot square and one inch deep. According to the naturalist author Annie Dillard, that block contains an average of 1,356 living creatures, including 865 mites, 265 springtails, 22 millipedes, and 19 adult beetles. It would require an electron microscope to reveal the additional trillions of bacteria and the host of fungi and algae.

In a laboratory the scientist begins with our friend the amoeba and works up, classifying from the “lower” to the “higher.” What is this term lower? How can we trample a million creatures on a hike and return home guiltless? A strict vegan who gulps cold spring water imbibes a horde of creatures—animals!—without flinching. Why do we wince at a bloodied cat along the roadside but take no notice of the billions of tiny animals pulverized by the bulldozer scraping out a roadbed?

The key to our ranking is specialization: the process of cells dividing up labor and limiting their role to a single task. We recognize a more meaningful life in the cat, a higher animal consisting of many cells working together.

The amoeba on my microscope slide occupies the bottom of the zoological ladder. It moves, yes, but no more than a few inches per day. It may spend its lifetime confined to a tin can or the hollow of an old tire. Unlike humans, it will never tour Europe, visit the Taj Mahal, or climb the Rockies. In order to do that, one needs specialized muscle cells, rows and rows of them, aligned like stalks of wheat. The lower animals skitter, creep, or worm along, covering mere yards of turf. The higher ones hop and leap and gallop or, if winged, vault and soar and dive. Specialization makes the difference.

Consider just one product of specialized cells, the organ of sight. As the husband of an eye surgeon, I hear often about the wonders of the eyes, which take up a mere one percent of the weight of the head. An amoeba has some crude visual awareness: it moves toward light—and that is all. Specialization gives me the ability to gaze through the viewing end of the microscope, noting the subtleties of color in the near-senseless amoeba. The amoeba comprises one cell, whereas I peer at it with 127 million visual cells. Named for their shape, these rods and cones line up in rows to receive images and transmit them to the brain.

Rods, slender and graceful tentacles that extend toward light, outnumber the bulbous cones 120 million to 7 million. They are so sensitive that the smallest measurable unit of light, one photon, can excite them; under optimum conditions the human eye can detect a candle at a distance of fifteen miles. Yet with rods alone I would see only shades of gray, as on a moonlit night. Squeezed into the dense forest of rods, the larger cones give me more focal resolution and the ability to distinguish more than a million unique colors.

When it detects a designated wavelength of light, each rod or cone triggers an electrical response to the brain. The brain compiles all these yes-or-no binary messages from rods and cones and—voilà!—I get an image of an amoeba swimming on my microscope slide. The feat requires so much processing power that half of my brain is devoted directly or indirectly to vision. As I contemplate vision, I am most impressed by this fact: when I see, I remain totally unconscious of cells encoding data and firing, then decoding and reassembling it within the brain. The hospital chapel outside my window presents itself not as a series of dots and light flashes, but as a beloved building, whole and meaningful and evoking many fond memories.

Compared to the amoeba’s one-celled independence, the lives of my rods and cones may seem dull and stationary. But who among us would trade ends of the eyepiece? For specialization to work, the individual cell must lose all but one or two of its abilities. Although vision cells forgo an amoeba’s autonomy and movement, they enable a much “higher,” more significant achievement. A single rod or cone can provide me with the wavelength of light that completes my appreciation of a rainbow, a kingfisher plunging into a stream, or a subtle change of expression in the face of a dear friend. Or it may protect me from disaster by firing off a message to the brain when a rock falls from a hillside toward my approaching car.

José, Reunited

An encounter with a patient I’ll call José captures for me the importance of membership in the human body and what happens when damaged cells sever the body’s connections to the governing brain.

José’s body had suffered much damage from leprosy by the time he traveled from Puerto Rico to our leprosy hospital in Louisiana for treatment. By then, research had proved that leprosy does its damage by affecting nerve cells, thus making patients vulnerable to injury. José’s insensitivity was so great that, when blindfolded, he could not even detect whether someone was holding his hand. Touch cells and pain cells had fallen silent. As a result, scars and ulcers covered his hands, face, and feet, bearing mute witness to the abuse his body had suffered because it lacked the warning system of pain. Mere stubs on his hands marked where fingers used to be.

Since pain cells in his eyes no longer alerted him when to blink, José’s eyes gradually dried out. That condition, aggravated by severe cataracts and glaucoma, soon made him blind. My wife, Margaret, informed him that surgery might correct the cataract problem and restore some vision, but she could not operate until inflammation of the iris went away. Shortly after that, a terrible misfortune cut off José’s last link with the outside world. In a last-ditch attempt to arrest the sulfone-resistant leprosy, doctors tried treating him with a new drug, and José had a rare allergic reaction. In a final cruelty, he lost his hearing.

At the age of forty-five, José lost contact with the outside world. He could not see another person, nor hear if someone spoke. Unlike Helen Keller, he could not even use tactile sign language because leprosy had dulled his sense of touch. Even his sense of smell disappeared as the leprosy bacilli invaded the lining of his nose. All his sensory inlets, except taste, were now blocked. Weeks passed and we watched, helpless, as José began to accept the reality of total isolation.

José’s body responded with a pathetic mirroring of what was happening to his psyche: his limbs pulled inward toward his trunk and he spent his days curled into a fetal position on the bed. Unable to tell day from night, he would awake from sleep and forget where he was. When he spoke, he did not know if anyone heard or answered. Sometimes he would speak anyway, bellowing because he could not hear the volume, pouring out the inexpressible loneliness of a mind locked in solitary confinement.

In such a state thoughts incurve, stirring up fears and suspicions. José’s body coiled tighter and tighter on the bed, preparing for death in the same posture as his birth. Most of us on the staff would pass his room, pause for a moment at the door, shake our heads, and continue walking. What could we do?

Margaret faithfully visited José. Unwilling to watch him self-destruct, she felt she must attempt some kind of radical intervention to restore at least part of his sight. She waited anxiously for the infection in his eye to improve enough for her to schedule surgery.

In order to follow government rules, Margaret faced a nearly insurmountable problem. She must obtain “informed consent” forms for the surgery, but who would sign for José? No one could penetrate through his isolation to ask him for permission. After painstaking research, the hospital staff finally located a sister in Puerto Rico, and the police department there visited her with a surgery release form. The illiterate sister marked an X on a paper, and Margaret scheduled surgery at last, with faint hope of success.

José, of course, did not comprehend what was happening as he was moved to a stretcher and wheeled to the operating room. He lay passive throughout the eye surgery, feeling nothing. After a two-hour procedure, he was bandaged and sent back to his room to recover.

Margaret removed the bandages a few days later, an experience she will never forget. Although José had sensed some gross movement and had probably reasoned someone was trying to help him, nothing prepared him for the result. He got the use of one eye back and could see again. As his eye struggled against the bright light and slowly brought into focus the medical people gathered around the bed, the face that had not smiled in months cracked into a huge, toothless grin.

During that time of solitude, José’s brain had floated intact inside his skull, complete with memory, emotions, and instructions for directing his body. Suddenly human contact was restored. José made it known that he wanted his wheelchair parked at the door to his room all day long. He would sit there quietly, every few seconds glancing up and down the long corridors of the leprosarium. When he saw another person approaching, his face would break into that irrepressible smile.

José insists on coming to our small church every Sunday, even though he can hear nothing of the service. With stubby fingers, he can barely grasp the control knob of his electric wheelchair, and his narrow tunnel vision causes him to bump into objects up and down the hospital corridors. Other attenders have learned to greet him by stooping down, putting their faces directly in front of his, and waving. José’s wonderful smile breaks out, and sometimes his bellowing laugh. Although he cannot see well, and still cannot hear or feel, somehow he can sense the fellowship of the church. He has rejoined the community, and for him that is enough.