This is the day Johnson undergoes brain surgery for Parkinson's disease. It's almost 8 a.m., on a Wednesday in early December, and he's wearing a thin, white gown and sitting in a wheelchair in the Neuroradiology Department in the basement of Johns Hopkins Hospital. He's surrounded by well-wishers: his wife, Grace; two of his children, Maria and Michelle; and his minister, Terry Schoener.
"Someone's coming for you in a few minutes," a nurse tells Ejner. A few minutes later, another nurse says, "They'll be ready for you in just a minute." After a half hour, at the first hint of footsteps, Maria jokes, "Here they come!"
They can kid around because the wait is almost over. They're confident that a procedure called a pallidotomy will do for Johnson what it's done for other Parkinson's patients: Relieve some of their symptoms. Allow them some freedom. Give back part of the dignity that the disease has slowly taken from them.
The Johnsons learned about pallidotomy last year while watching one of the TV news magazines. It is an old surgery that has been fine-tuned and reintroduced in recent years. It could enhance the benefits of the medicine Johnson has been taking every day for 10 years. One of the disease's many cruelties is that the most effective antiparkinson drug, Levodopa, becomes less effective the longer a patient takes it. Yet the TV showed doctors burning a tiny hole in the brain of a man with Parkinson's and making the shaking stop. Afterward, he not only got up and walked around, but also ran down the hall to show off for the cameras. He even danced. But no one knew how long the improvement would last. "If it lasts 18 months, it's the best thing in the world," the man on TV said. "If it lasts three hours, it's the best thing in the world."
Johnson, 64, has been waiting for a pallidotomy for over a year. A former political reporter, Maryland commissioner of motor vehicles, and chief of staff to the governor, he took early retirement several years back. He knew there was no stopping Parkinson's. But pallidotomy has given him hope.
He sits outside a room where a metal frame will be attached to his skull in preparation for surgery. That's the worst part of the day, other pallidotomy patients have told him. After that, you won't feel a thing.
Johnson has a long, narrow face, metal glasses, white tousled hair, and a furrowed brow. He couldn't sleep last night. This morning, he's antsy to get things under way.
When Vincent Lerie, a radiology technologist, arrives for Ejner, Grace leans over and kisses her husband on the head. "We want to see you dancing down the halls," she says.
Another kiss, this one from Michelle, 32. And one more from Maria, 39, who assures him, "You'll do great."
"I'll give it my best shot," he says.
As Lerie pushes the wheelchair toward the door, Grace tells her husband a second time, "We're going to watch you dance."
IN 1817, LONDON PHYSICIAN James Parkinson first described the symptoms of what became known as Parkinson's disease in a short book titled An Essay on the Shaking Palsy. Today, much about the disease remains a mystery: Doctors still don't know what causes it and don't have a cure.
If you include undiagnosed cases, over a million people in the United States have the disease, estimates Stephen Reich, the director of the Parkinson's Disease Center at Hopkins. Most are elderly; among people over 65, Reich says, as many as one in 100 have it. Harry Truman, Mao Tse-tung, and Adolf Hitler had it. Janet Reno and Billy Graham have it now. Muhammad Ali has Parkinson's, or a disorder closely resembling it.
Parkinson's disease is a chronic nerve disorder with a number of possible symptoms. The most common is slowed movement, called bradykinesia, accompanied by tremor or muscle rigidity. Patients may also experience slurring of speech; depression; insomnia; loss of balance; a soft, monotone voice; an expressionless face; problems swallowing; and temporary freezing, when they cannot walk through a doorway or step off a curb. The disease is progressive, so the symptoms gradually get worse. In its most advanced stage, it is crippling.
Pallidotomy is a treatment for the disease, the most effective treatment to come along in the last 30 years, according to some doctors. But it isn't a cure. No matter how often Reich and the doctors at Hopkins remind patients and their families of this distinction, many go right on hoping that the operation will cure them.
Johnson doesn't have to have the surgery. He isn't dying. He could go on waiting for a cure, what he calls "the silver bullet." But there's a good chance the procedure will alleviate some of his symptoms for several years.
So he's willing to undergo an operation in which Hopkins neurosurgeon Fred Lenz will delicately thread a needle-like probe into his brain and destroy a small area known as the pallidum, or globus pallidus. Located on each side of the brain, just above the ear, the pallidum is part of the basal ganglia, a cluster of nerve cells that helps control movement. In Parkinson's patients, the pallidum becomes overactive and acts like a brake, slowing down the motor system. By destroying part of the pallidum with a lesion, or burn, Lenz releases the brake and restores normal movement.
The challenge is finding and destroying a target approximately
eight millimeters long and four millimeters wide--roughly the
size of a Tic Tac--that lies deep in the brain. There's little
room for error: Directly beneath the pallidum is the optic tract,
which transmits visual information; behind the pallidum is the
internal capsule, the major pathway by which the brain controls
movement; and within the pallidum itself, just above the target--
the internal segment--is the external segment, which also plays a
vital role in movement. Burn one of these surrounding areas and
the patient could suffer paralysis or loss of peripheral
While many doctors rely solely on brain scans and electrical stimulation to locate the target, Lenz and his team of neurophysiologists map the brain with electrophysiologic recording before making the lesion. While this prolongs surgery, it makes the procedure more reliable. Using a microelectrode inserted in the brain, they record the electrical activity generated when an individual nerve cell fires a signal. By recognizing the distinct firing patterns of cells in different areas, they close in on the target.
Throughout the procedure, which ordinarily takes about three hours, the patient is awake. The doctors ask him what he sees and what he feels. This helps them identify the areas of the brain they're exploring.
If Lenz doesn't feel absolutely sure that he's found the part of the pallidum he needs and doesn't make a lesion, the patient is no worse off, but also no better. The pallidotomy team at Hopkins usually finds the target, though. In fact, they have in all but two of the nearly 60 operations performed prior to this one, going back two years.
Ejner Johnson would just as soon sleep through the pallidotomy and wake up after it's over and done with. "I'm not the best of patients," he said as the surgery drew near. "I get uptight around doctors."
WHEN LENZ STROLLS into the scan room, he's wearing bright green surgical scrubs instead of the long white coat, button-down shirt, and tie Johnson is accustomed to seeing at the doctor's office. But the short, quick steps, the light brown frames of his glasses and the confident, matter-of-fact manner are the same. Lenz is the only neurosurgeon at Hopkins who performs pallidotomies. "I like to think I'm good at this," he says. "This type of surgery is my specialty."
He removes Johnson's glasses and shaves a small area on top of his head, just left of center, where he plans to drill a small hole for the operation. Since Johnson's tremor is on the right side of his body, Lenz is performing the pallidotomy on the left side of his brain.
As he works, he asks Johnson about the name Ejner, which he pronounces "Edge-ner." It's Swedish, Johnson tells him. Pronounced "A-ner."
Lenz places a stereotactic frame on Johnson's head, a three-sided metal frame with coordinates to help position the probe. During the operation, it holds the long electrode in place as it passes through the brain. Lenz doesn't expose large sections of the cerebrum and physically separate them, the way people think of brain surgery. This is a blind procedure; he never actually sees the area of the brain on which he's operating.
"You're passing a needle into a melon, and you can't see if you're hitting the right spot," Lenz explains. That's why his team does the extensive mapping and recording. "If we don't feel we've found the target, we don't do it. You don't burn someone's brain without knowing exactly where you are."
Before drilling four shallow holes in the skull and securing the frame in place with fiberglass pins, he tightens the clamps so it won't budge. The pressure is excruciating.
"This is the most painful part, right?" Johnson asks, grimacing.
"That's right," Lenz says. "After this, the rest of the day is pretty boring. You just lay there."
The drilling itself is usually quick, loud, and painless, because Lenz numbs the scalp in advance. On the last hole, however, Johnson shouts, "Whoa! Whoa!"
Lenz stops. Lerie and another doctor bracing Johnson relax. "I could feel that one," Johnson says, his eyes squeezed shut. "It's burning."
Sometimes, the numbing takes longer. Lenz waits a few moments. "We're so close," he says. "Let's try it one more time."
The drilling still hurts, but once the frame is in place and Lenz loosens the clamps, Johnson feels relieved. The worst is over.
A COUPLE OF WEEKS EARLIER, Johnson was sitting in his green recliner in Severna Park, south of Baltimore, the latest Tom Clancy novel on the table by his side, a plastic pill box poking out of his shirt pocket. He wore white sneakers with Velcro straps. He used a quad cane--with four feet instead of one--to get around, and he walked with a limp, the result of two strokes. Pointing to the dented metal guard in front of the fireplace, he said, "That's a by-product of Parkinson's. I fell in the fireplace. I don't know what happened. I just fell in. A couple of months ago, I fell through my wife's cosmetic table."
Grace Johnson helps her husband in countless ways. She ties the laces of his dress shoes and cuts his dinner. Every night, she fills one pill box marked "7am, 8am, 10am, 1pm, 2pm, 4pm, 7pm" and a second marked "8pm, 10pm, 11pm." Most of that is medicine for Parkinson's. Throughout the day, she reminds Ejner to take his pills. If he forgets, they might spend an extra half hour in church or a restaurant, waiting until the freezing spells pass and he can stand. Grace, who runs her own travel agency, is the driver in the family now. She keeps a wheelchair in the trunk of the Mercedes.
"The only thing predictable about Parkinson's is that it's unpredictable," says Paula Goldberg, the nurse coordinator at Hopkins's Parkinson's Disease Center, which treats over 1,000 patients a year. Reich, along with neurologists Katrin Andreasson, Ted Dawson, Elizabeth O'Hearn, and Thomas Preziosi, offers medical, physical, occupational, and speech therapy.
In advanced stages of the disease, sometimes the drugs (the main one is Sinemet, which contains Levodopa) work, and sometimes they don't. That's a typical reaction. Doctors aren't sure if the disease or the drugs are to blame. But the more medicine patients take to control their symptoms, the more they experience side-effects, such as involuntary movements called dyskinesias. It's a vicious cycle--being unable to move, then unable to stop moving. Reich likens it to the blood-sugar fluctuations in diabetics.
Johnson's dyskinesias occur nearly every day, and they strike at random, for an hour or several hours at a time. They start slowly, his face contorting and twisting, then the activity spreads to his arms and his legs. His whole body squirms as if it has a mind of its own. He slides out of the chair onto the floor. Once, he accidentally pulled the tablecloth off the table at dinner.
"It's like a python slipped in your nervous system," Ejner says.
BEFORE ACTUALLY ENTERING the brain, Lenz needs a good picture of the target area, so Lerie, the radiology technologist, performs a series of CT scans. Johnson lies strapped to a table, his head just inside the massive scanner that records thin cross-sections of his brain. As the computerized images appear on a screen in the adjacent room, Lenz locates the anterior and posterior commissures--his landmarks. He writes down their coordinates in order to calculate the location of the pallidum.
Because Johnson is off his medicine for the operation, his right hand shakes. When the tremor increases, the entire table, extended like a diving board, bounces up and down, and the CT image looks fuzzy and wrinkled. To keep him still, Lerie tapes the patient's arm to the table.
The pallidum doesn't appear in many images of the brain. In none of the 17 CTs taken today, in fact, and in only five of the 200 MRIs taken a few weeks ago. Because of its particular anatomy and chemistry, the pallidum doesn't distinguish itself well in magnetic resonance imaging.
Neuroradiologist Tim Eckel studies an MRI film on the light board. The brain looks like a hazy, grayish cluster of shadows or storm clouds, nothing like the recognizable features of, say, a chest x-ray.
"This little brown dot here, this is the globus pallidus," Eckel says, pointing to an area the size of a drop of water. "It's surrounded by dark here, dark here, and dark here. It's the lighter gray area in the middle of that little black triangle. That's it."
That's the pallidum in Johnson's brain. That's Lenz's target.
BACK IN THE 1940S, PALLIDOTOMY wasn't as precise. Sometimes doctors burned the pallidum, and sometimes they burned another part of the basal ganglia, the nerve cluster where the pallidum is located. "It was a shot in the dark," says Lenz.
There were patients who improved dramatically, some who didn't change at all, and, in rare cases, some who died after surgery. In the 1960s, doctors largely abandoned the operation and turned to a new drug: Levodopa.
In Parkinson's patients, certain cells in the brain die, cells that produce dopamine, a chemical messenger vital for neurons to transmit signals to one another. Doctors still don't know why they die. "That's the million-dollar question," says Dawson, a Hopkins neurologist researching the disease. By the time Parkinson's patients begin exhibiting symptoms, they have lost about 80 percent of their dopamine-producing cells.
Levodopa, or L-dopa, appeared to solve this problem by replacing the lost dopamine and alleviating movement problems. But the solution was only temporary. Doctors discovered that after about five years, the benefits waned and the side effects worsened. That's why the rediscovery of pallidotomy was such an exciting breakthrough.
For years, researchers had been slowly unraveling the mystery of how Parkinson's affects the complex circuitry of the basal ganglia, where neurons transmit signals that coordinate movement. One of these researchers was Hopkins neurologist Mahlon DeLong, who is now at Emory University. In test monkeys given a toxin and made parkinsonian, DeLong and his colleagues discovered excessive neural activity in the internal segment of the pallidum. This meant that for the first time they understood why a pallidotomy should work. Destroy the internal segment and you destroy the misfiring neurons that disrupt movement. And using sophisticated electrophysiological recording, doctors could consistently locate this precise target.
His findings coincided with the work of other researchers in the early 1990s, when the medical community began to give pallidotomy a second look. The procedure worked on monkeys in the lab, but would it work on humans? The answer came in 1992. Lauri Laitinen, a doctor in Sweden, was among the first to report that by targeting the same area, the internal segment, he relieved Parkinson's symptoms in his patients.
Because of DeLong's ties to Hopkins, Lenz and Reich were keeping close tabs on the promising treatment. Initially, Reich was skeptical. Brain surgery was a last resort. But when DeLong and others began seeing positive results, Reich and Lenz were convinced. In December 1994, Lenz performed the procedure for the first time at Hopkins. Early on, the mapping took as long as eight hours, almost three times what it does now. "The more you do, the better you get at recognizing the electrical signature of the internal segment," says Lenz. "We know exactly what we're looking for."
OPERATING ROOM 4 IS LIKE the crowded helm of a ship on the morning of a voyage, the eager crew methodically going over various maps, notations, and monitors. They are anxious to set out, to find the pallidum deep in the ocean of Ejner Johnson's brain.
The doctors all look alike, wearing the same green scrubs, masks, and caps. Raised eyebrows, knitted brows, and crinkled crow's feet around the eyes are all you can see of their facial expressions.
Lance Rowland, a neurosurgery research technician, is hunched over several anatomical diagrams and a graph on which he's charted the day's first probe, based on the CTs and MRIs. Neurophysiologist Pat Dougherty mans the hydraulic microdriver, the machine that advances and retracts the probe. Between them is Ali Zirh, a Turkish neurosurgeon learning the procedure so that he can perform it in his country. His responsibility is to write down the depth of the electrode and the type of cells recorded.
Lenz stands at the head of the hospital bed behind the patient. It's been nearly three hours since the drilling, three hours with Johnson's head in the frame. So far, no complaints.
At the foot of the bed is a wall of monitors that flash and beep, telling the anesthesiologist Johnson's pulse, blood pressure, and the amount of oxygen in his blood. On the left wall, stacked like stereo equipment, are the instruments that amplify and record Johnson's brain activity. Behind the hospital bed is a small board, the kind you'd see in a classroom. Written in black marker is a chart with the letters x, y, and z, and the numbers 120, 95, and 120. These coordinates correspond to the area where they expect to find the internal segment.
At 10:52, Lenz makes a short incision and clears an area of skull. Next, he drills a burr hole, a dime-size opening, and gently cuts through the dura mater, the thick covering of the brain.
"Is it a normal-size brain?" Johnson asks. "My wife sometimes tells me it's the size of a pea."
That gets a big laugh.
When Lenz carefully inserts the long thin electrode through the frame and into Johnson's brain, the journey is under way.
"You can relax during this part," Mark Nicholson, the nurse, tells Johnson. "Some patients have actually fallen asleep during the procedure."
Before making the lesion, Lenz and the others will locate the optic tract, the internal capsule, and the internal and external segments of the globus pallidus, known as the GPi and GPe. Then, they'll define the specific dimensions of the target to avoid destroying cells in the surrounding areas.
When the electrode is next to a neuron, the probe's tiny tip-- one-fifth the size of a red blood cell--records the electrical activity. Brain cell activity is not continuous like a radio signal. Rather, the cells fire an action potential, which is a single event, like a heartbeat. The electricity generated by that event is amplified over a small speaker, and the doctors listen to the firing pattern to tell what kind of cell or cells are there. The axons in the optic tract make a whooshing sound when activated. The GPe neurons sound like intermittent rain on a window, and the ones in GPi, the target, sound like bees buzzing. During a probe, Nicholson turns out the lights in the O.R. because they interfere with the recording. The doctors also study the wave activity of these firing patterns on the small screen of an oscilloscope.
"How are we doing?" Johnson asks.
"Everything's fine," Lenz says. "Everything's going well."
As Dougherty rotates the microdriver's wheel with his thumb, the probe plunges deeper into the brain. The doctors study the squiggly waves. They listen to the crackling static. It looks and sounds as if Dougherty is trying to tune in a station on the radio.
Lenz leans over Johnson and flickers a flashlight in his eyes. This makes the neurons fire.
"I hear something," Rowland says.
The others hear it, too, a subtle change in the static. Whoosh. The optic tract.
They want to be certain, so they conduct another test. Dougherty hits the space bar on his computer keyboard, which produces an electrical pulse through the tip of the probe. It should activate the neurons. They call this microstimulating.
"Close your eyes and tell us if you see any sparkly lights," Dougherty tells Johnson.
Dougherty hits the space bar again. "They're little lights off to the side," he says, speaking slowly, as if Johnson doesn't understand. "Little purple lights. They flash real quick. Do you see them? Do you see the lights?"
"Are you sure you don't see anything?"
Dougherty looks at Rowland. They take a few notes and move on.
After a while, Johnson speaks up. "We about done here?"
"No," Dougherty says. "We've still got a ways to go."
It's 12:15, about a third of the way through the operation.
Ordinarily, the doctors would have already identified the target by now. But they haven't recorded the neural activity they expect in the GPi. They write a new set of coordinates on the board and keep looking.
WHEN PALLIDOTOMIES PROVED EFFECTIVE, word spread throughout the medical community as well as the network of Parkinson's patients and family members. In most cases, the disease debilitates patients' bodies, not their minds. They stay informed through the Internet and the World Wide Web. They lobby Congress for research money.
After the dramatic TV reports in 1995, the demand and hope for pallidotomies was so fierce that The New York Times and The Wall Street Journal ran front-page stories that year about the hysteria and controversy surrounding the operation. Among neurologists and neurosurgeons, there was debate about the procedure's effects and safety. Were the benefits exaggerated? Would they last? What about the people who didn't improve or wound up worse off? At that time, most insurance companies considered the procedure experimental and wouldn't cover it.
Parkinson's patients were willing to take the risk. As one man told The Wall Street Journal, "Would I fly in a shuttle that had a 92 percent reliability of getting me some distance away from Hell on Earth, 8 percent of doing me some harm, and well under 1 percent of killing me?"
What happens when a new procedure comes along, says Lenz, is that first people overestimate the benefits, then they overestimate the risks. The demand for pallidotomy made it one of the fastest growing--if not the fastest--areas of neurosurgery.
Back in 1994, he and Reich didn't know who would benefit most from pallidotomy. They soon found out that not everyone with Parkinson's did. In fact, only about 20 percent of Parkinson's patients are good candidates, Reich estimates.
Screening has become a significant part of the process. Those who benefit the most have moderately advanced Parkinson's and experience dyskinesias and "on-off" periods, when their reaction to the medicine is unpredictable. Those who experience other complications, or don't respond to the medicine, are less likely to benefit.
The procedure doesn't replace L-dopa. What it does is smooth out the patient's response to the medicine, reducing the frequency and severity of "off" times, when the drug is ineffective, and also alleviating the side-efffects, the dyskinesias. So instead of one hour without movement problems, patients may have three or more, maybe the whole day.
"What I tell patients is, it's a way to set the clock back," Reich says. "If you imagine the way you were three to five years ago, that's the way you'll be after surgery. But the clock continues to tick."
No one knows how long the benefits from pallidotomy last. Emory University School of Medicine has been following a group of patients for over two years now, Hopkins for almost as long. Periodically, Goldberg, the center's nurse coordinator, examines those 25 patients for 42 different symptoms, such as the frequency of their tremor. Already, Reich says, their improvement has declined slightly.
Ejner Johnson doesn't care if the operation will help him dance. He'd rather ski. For years, he and Grace rented a cabin in West Virginia's Canaan Valley, and the whole family came. The last time Ejner--"Pop-pop," the grandkids call him--skied, he couldn't get up when he fell.
Because of Grace's travel agency, she and Ejner have been all over the world, to too many countries to count. But the pictures that fill their home are not of South Pacific sunsets, or Kenyan safaris, or the Great Wall of China. They're of children and grandchildren, smiling, hugging, and mugging for the camera. There are 52 photos in the living room and 19 more in the hallway. And on a windowsill in the kitchen, there are 15 miniature wooden ducks in a row, a name painted on each, from Pop-pop to 6-month-old Douglas, the youngest grandchild.
"I'd like to do what everybody else does, within reason," Johnson said before the operation. "I want to swim and ski with my grandchildren. I want to be able to get up and go to the bathroom at night with some degree of confidence that I'm not going to fall on my wife's make-up table. I want to go to church in something other than a wheelchair. That's what I want out of this."
AS THE DOCTORS PAINSTAKINGLY
SEARCH his brain, often a millimeter
at a time, Johnson lies awake, wondering what they're up to,
what's taking so long. Right now, 15 minutes feels like an hour
to him, his hand tapping uncontrollably against his chest or his
leg. His back aches. He can't move his head. Not with a steel
needle lodged in his brain.|
"Try to relax, Mr. Johnson," Nicholson says.
"I can't," Johnson says. "I can't relax. I'm tired."
"Hold on," Lenz tells him.
"I can't hold on anymore. I'm tired....You're not listening to me."
Nicholson drapes a wet cloth across Johnson's forehead and tells him, "Think of the benefits."
Johnson doesn't answer right away. "I'm trying to think of the benefits, but I can't think of any."
Exasperated, Rowland stares at his graph, his head in his hands. Zirh rests his forehead on the back of a chair, looking at the floor. They're so close. They've found the areas surrounding the target that they want to avoid burning. Now if they could only find the target, the GPi. It has to be within a couple of millimeters of the probe, but they don't hear the familiar firing pattern they should. They don't hear the sound of bees buzzing.
Johnson, meanwhile, is wearing down. He tries to roll his head to one side.
"No!" Dougherty and Lenz tell him. "DON'T MOVE YOUR HEAD!"
"You're treating me like a kid."
The tests are ongoing and disorienting--the flashlight in his eyes, the questions about purple sparklies, the requests to hold his arms up, and stick out his tongue. He doesn't recognize the doctors behind their masks. The one with the flashlight has glasses. He looks familiar, but Johnson can't place him.
The longer he lies in bed, the more he's bothered by the unfamiliar sights and sounds of the operating room. The constant static. The occasional beep that sounds like an alarm. "What was that?" he asks. When the doctors turn out the lights, he worries they won't come back on.
"She isn't here," says Nicholson, who doesn't leave his side. From now on, someone else gets the lights. "She's in the waiting room. I just talked to her a little while ago. Are you asking about her because you want to let her know how you're doing?"
"I'm asking in desperation."
"Mr. Johnson, I know this is hard but when you've gone this far, you don't want to give up, do you?"
"I'm ready to collapse."
After each probe, the doctors confer and set a new course, adjusting the angle of the electrode. There are four sets of coordinates on the board already. Four shots at the bull's eye. Pretty soon, they will have to decide whether or not to make a lesion at all. As they prepare the next probe, their backs to Johnson, Dougherty tells Rowland, "This guy's not going to last."
A little later, his voice becoming hoarse, Johnson says, "I want to give up. I can't make it."
"Don't say that," Dougherty tells him. "Tell yourself you can do it."
"But I can't. That's a lie."
"Well, lie then. Because that's what you need to hear right now."
AS FAR AS EJNER JOHNSON KNOWS, he has had Parkinson's for over 10 years. He can't remember exactly when his hand started trembling. He ignored it at first, thinking the movement would go away. Then one day in 1986, Maryland Governor Harry Hughes noticed his chief of staff's hand shaking.
It was a tremor, replied Johnson. Nothing more.
"Well, you should have that checked out," Hughes told him.
The family doctor in Severna Park referred him to a neurologist in Annapolis. Several years later, Johnson was referred to Reich, a specialist in movement disorders.
"It was incessant," Johnson says of the disease. "I knew it was coming. It was going to get worse, and it did."
His voice grew unsteady, and he stopped testifying at the Statehouse. His handwriting, normally flowing, looked scrunched and unfamiliar. Where work had once been the focus of his life, the disease took over.
Growing up in Brooklyn, N.Y., he had been quite an athlete, a sure-handed third baseman and a point guard with a good outside shot. Now his body was betraying him. He couldn't take the simplest things, like standing or walking, for granted.
Last year, he asked Reich about a pallidotomy. Reich said he might be a good candidate after they had exhausted the antiparkinson drugs. When Johnson was finally eligible, he and Grace talked to other patients who had had the surgery. They met a man from Pennsylvania whose dyskinesias had crippled him so badly he couldn't drive. The day the Johnsons saw him, the man had driven himself to Baltimore. He was running his own business again.
No, the procedure wasn't a miracle, but it was a proven treatment; Medicare agreed to cover the $10,000 cost. To the Johnsons, pallidotomy offered the hope of some relief--but no guarantee.
THE NEXT TIME EJNER JOHNSON CALLS out for Grace, Nicholson dials the waiting room and puts Lenz on the phone. He explains to Grace that her husband has become agitated. Would she come talk to him?
When she enters the operating room, she's wearing a white gown over her clothes, a surgical mask, and a cap. Her eyes are huge. She's been in hospitals before, but this room looks different-- crowded with more doctors and more monitors than she expected, and dark. She can hear the frustration in the doctors' voices. Taking care not to touch the sterile green towels covered wtih surgical instruments, she approaches the bed. Ejner looks miserable.
He tells her he's had enough, that he wants to go home. Grace tells him, "They're trying to help you, Ejner." She holds his hand, the one that's shaking, and assures him it's going to be OK.
"Thanks for coming," Lenz tells her. It isn't often that a family member comes into the O.R.
After several minutes, Ejner seems calmer, less agitated, but also confused. He says something about pearly gates. "You can't follow me through the gates," he tells Grace. This scares her. Suddenly, she has difficulty breathing with the mask over her mouth. She decides to go get Terry Schoener, Ejner's minister. Maybe he can help.
Lenz readies the next probe, the sixth of the day. This one, he says, has to be perfect.
At 1:56, after three hours of searching in vain for the target, he aborts the procedure. The journey is over. Lenz and his team couldn't confirm the exact location of the GPi, so they can't make the lesion. They're almost certain the probe passed through the right area, but for some reason the activity wasn't there.
The electrode is removed and put away, the instruments are switched off, unplugged, and wheeled from the room. Lenz stitches Johnson back up. The O.R. is somber.
"It's unfortunate," Rowland says, "because you'd like to try and help everyone."
Outside the waiting room, Nicholson finds Grace Johnson and Terry Schoener. We won't be needing you, Nicholson tells the minister. The operation is over.
Grace breaks down and cries. She's mad--"mad at God, mad at the world." She wants to know, "Who's going to tell my husband?"
THE FOLLOWING MONDAY MORNING, Lenz and his team return to the O.R. and perform another pallidotomy. This time, they find the target with ease and make the lesion. It takes three probes. A textbook case.
Monday afternoon, back in Severna Park, Ejner and Grace Johnson have company. Melissa has dropped by to check on her father, and brought along her 3-year-old daughter, Jamie. One of Ejner's college friends and his wife are there, too. Johnson sits in his recliner by a roaring fire, munching on pretzels and sipping a diet soda. His hand shakes slightly in his lap. When the phone rings, it's his old boss, Harry Hughes, wishing him well.
Ejner was devastated when he learned the morning after the operation that the pallidotomy wasn't completed. Lenz broke the news to him, but it didn't register right away. He was still groggy. So Grace told him again. This time, he understood. I have to go through that again? he thought.
Several days later, the whole experience is still pretty fuzzy. He remembers bits and pieces. Melissa tells him what he said following the surgery: "I've got to call them and apologize because I was such a bastard in there."
"I did?" he says.
A little later, Grace says, "I think we ought to sit down and write a thank-you letter to Dr. Lenz."
Ejner agrees. "I want to assure the doctor that he made the right decision. He doesn't need that reassurance but I think it would help. He's not just a doctor, you know."
Wednesday night, following the surgery, Lenz telephoned Grace at home. He said he'd consulted with his colleagues and that her husband could wait two months and try a pallidotomy again.
Grace thinks he should. So do his children.
Ejner Johnson hasn't made up his mind.
Charles Salter Jr. is a freelance writer living in Baltimore.
RETURN TO FEBRUARY 1997 TABLE OF CONTENTS.