Last-chance surgery can mean enormous risks ... then again, it can inspire even higher hopes
By Jessica Snyder Sachs
(originally published in Longevity}
Like a shy schoolgirl, Donna Lavender curls her legs and feet together under her chair as she waits to see her doctor. This petite woman of 32 first met Jerrold Vitek, M.D., a year before. At the time, the 44-year-old Emory University neurologist advised against brain surgery. The risks far outweighed her pain and disability, he had said. And though the risks hadn't changed much since then, Lavender's pain and disability had.
The inward curl of Lavender's legs and feet is not for girlish effect; it's a symptom of dystonia, a brain disorder that causes severe and uncontrollable muscle spasms. In her case, the disease has locked her calves, ankles and feet in an excruciating, twisting cramp. "It's like a great charley horse that never ends," she explains in a rich Southern drawl.
In its various forms, dystonia affects some 300,000 people in North America alone. Its cause is not known, though there are many theories. Some dystonias start in childhood, and these may be related to gene defects. Others arise in adulthood and stem from injury. That may be the case with Lavender, who had been hit in the back and knocked unconscious by a runaway oil drum six years earlier while unloading an "18 wheeler" she and her husband had been driving. Two weeks after the accident, she lost all feeling in her legs and became bedridden. But the spunky young woman had fought back, progressing from bed to wheelchair to walker to unassisted walking. Then, six months after the accident, the dystonia began.
It came as a bad nighttime cramp in her right leg. "When I tried to get up in the morning, I couldn't stand," she remembers. "The cramping came with a burning pain like I'd never felt before." So severe was the pain that some mornings Lavender woke with her heart racing at 220 beats per minute--about three times the normal rate. Her doctors worried about heart damage. Her marriage ended in divorce. "My husband didn't want a cripple for a wife," she says, turning to hide the tears.
In 1994, Lavender married again, this time to a childhood sweetheart who had always been there for her. They settled in the small town of Millegeville, in the central hills of Georgia. But that same year, Lavender's dystonia spread to her left leg, with cramping even more painful than that in the right. An orthopedic surgeon tried to relieve the twisting of Lavender's feet by splitting and rearranging the ligaments in her anklesbut without success. So Lavender hobbled through the day on the outside edges of her curled-under feet, until the deep, burning pain would force her to lie down.
Her family doctor recommended exploring a type of brain surgery sometimes used to relieve severe case of dystonia. So she had met with Vitek at Emory. But early on in the conversation, they agreed there were too many risks involved in brain surgery including the risk of paralysis. "I knew what it was like being in a wheelchair," Lavender says. "I wasn't willing to take that chance." So she returned home to manage as best she could with bed rest and painkillers.
Unfortunately, the dystonia worsened. The spasming, which had originally come in "spells" lasting a few hours, became constant. Even worse, it began to spread to Lavender's thighs. She came to rely on narcotics to dull the pain. But the drugs dulled her mind as well, and made her depressed. She worried about the emotional toll she must be taking on her 12-year-old daughter and her new husband. The decision no longer seemed so clear cut.
"In my heart, I knew I had to [have the surgery]," Lavender tells a reporter on the day when she and her doctor are to meet to reassess their original decision. For his part, Vitek wants to make sure she understood the difficulties and risks involved, and had the resolve to face them out.
DAY OF DECISION
Despite her pain, Lavender flashes a broad smile when this man, who offers "one last hope," bounds into the room. A wiry, dark-haired researcher with an eager grin, Vitek is a pioneer in the mapping of the thalamus, a knob of gray matter the size of a walnut buried deep inside the brain. One of its functions is to serve as a relay station for movement and sensory messages traveling from the body to the cerebral cortex. The root of Lavender's dystonia may lie here, he explains, making a quick sketch.
In structure, a portion of the thalamus is like an onion, with each successive layer associated with a different body part: foot, leg, arm, shoulder, face, etc.
"At one time this are was your friend," Vitek says, pointing to the area associated with the leg. "But something has gone wrong; the cells there are now firing erratically, jamming other brain circuits and causing problems."
The "last hope" Vitek offers is a thalamotomy, an operation in which the problematic part of Lavender's thalamus would be lesionedburned away with an electrode. Thalamotomies had been performed in the 1970s, he explains, but the results were inconsistent. About half the patients with dystonia got some benefit, with a small subset getting full relief. But others came away from the surgery with weak or paralyzed limbs, slurred speech and thinking problems.
And Vitek thinks he knows why. He leans forward now, his hands spread, his eyebrows jumping in excitement. "In part, it may be that some surgeons were missing their mark," he says. The thalamus may be shaped differently in each person, and it's easy to get lost. Lesion the wrong area and ...
But Vitek has high hopes of improving thalamotomy's accuracy. He has spent five years studying the organization and cell-activity patterns of the normal thalamus in animal models. Building on this familiarity, he uses microelectrodes to probe the patient's thalamus and create a customized "map." Then, once he has isolated the problematic area, he steps aside, allowing a neurosurgeon to destroy it.
"So far we've had good results," he tells Lavender. "but we're on a learning curve." Should Lavender go through with the operation, she would be part of a study Vitek is conducting involving microelectrode-guided thalamotomies for 10 to 12 dystonia patients.
To make the decision thornier, Lavender's case will be especially difficult. The layer of the thalamus associated with the legs lies against a brain capsule filled with nerve fibers that control movement. So the surgery must be superbly precise--destroying as much of the leg-associated thalamus area as possible, without harming the adjacent fibers and possibly causing paralysis.
Vitek proposes that the team lesion only the leg area of Lavender's right thalamus. This should relieve the more painful dystonia in her left leg. And if the operation proves successful, they can later perform an operation to lesion the leg area of the left thalamus, relieving the right leg dystonia.
Despite all the risks and caveats, Donna is determined. "I know that even if this surgery doesn't help me, what you learn will help someone else in the future," she says. It's the answer that every medical researcher wants to hear. But Vitek needs to be sure. "It's going to be a tough day," he says.
While the "standard" thalamotomy can be done in as little as an hour or two, guiding it by microelectrode mapping can take the better part of a day. "And you'll have to stay awake throughout, telling me what you feel as I stimulate different areas.
"We'll have to move your leg," he continues, gently lifting Lavender's knee a fraction of an inch. She blanches with the pain, and he apologizes. "We'll try to give you medication to decrease the pain to a tolerable level, but if we sedate you too much, the cells will change their pattern of [electrical] activity, and we won't be able to map accurately." Lavender nods and smiles, and asks how much of her hair will need to be shaved. Vitek assures her that only a patch will be involved, and the surgery is set for the following Wednesday.
As she leaves the room, Vitek worries about Lavender's ability to cooperate during the operation. "She's a brave woman," he says. "But I have a feeling that pain's going to be a problem."
At home, Donna alternates between peaceful resolve and frightened doubt. But her decision is reinforced by her husband and a concerned circle of friends from the local church, who have educated themselves about Lavender's dystonia and the proposed surgery.
DAY OF DETERMINATION
Wednesday morning, 2 A.M. Lavender and her husband prepare for the drive to Atlanta. They are joined by their pastor and his wife. Together they pray, riding down the highway into dawn.
6 A.M. Lavender arrives at Emory University Hospital and is fitted with a "halo," a metal ring riveted to her skull with sharp posts. Technologists make a series of CT scans of her brain.
10 A.M. Lavender is wheeled into the operating room. The nurses cover her body with balloonlike "quilt" filled with a gentle flow of warm air. The rest of the room is kept at a chilly 63 degrees to minimize the risk of infection once Lavender's brain is exposed.
Enter neurosurgeon Roy Bakay, M.D., a no-nonsense bear of a man who takes complete command of the operating room. He positions Lavender's head in a second, calibrated ring, carefully matching its position to readings from the CT scans. "Scrub up," he directs Vitek and his neurology team. If all goes smoothly, they'll be finished in four or five hours.
11 A.M. Smooth is not to be. As Bakay attempts to inject a local anesthetic into Lavender's scalp, she jumps and writhes in pain, twisting her head inside the metal frame. "It hurts," she cries. "Hold still!" Bakay yells. Two nurses jump to restrain her. "Donna, you have to hold still," they plead. Lavender, groggy from painkillers and a sleepless night, moans with fright and discomfort.
Noon. Bakay has cut a triangle-shaped opening in Lavender's skull. Through it he has lowered the long, thin microelectrode that Vitek now uses to send and receive electrical pulses through Lavender's brain. The room fills with the amplified crackle and buzz of brain-cell activity. Green lines jump and wiggle across two monitors on a seven-foot-tall tower of electronic equipment. "Fiber at 52.5," reports Vitek to a team of assistants, who plot his readings on tables and graphs.
Vitek watches the green lines dance across the monitor, then closes his eyes to concentrate on the distinctive sound of each click, crackle and buzz. Drawing in his years of study, he lets the sounds guide him. "An injury here, not classic thalamus," he reports.
He brushes his hands softly across Lavender's left leg. He lifts it and shakes it, creating a storm of activity across the monitors. He sends a small current through the electrode and quizzes, "Donna, can you feel this?" A long pause, then a quiet murmur. "No."
2 P.M. After nearly two hours of mapping the neurology team confers in the corner of he operating room, trying to fit their graph of Lavender's brain-cell activity over an anatomical map of a "typical" brain. Angling their color-coded points this way and that, they try to make a fit that will reveal their exact location. They step back in puzzlement.
Vitek and Bakay confer. Lavender's writhing at the start of the surgery may have changed the position of her head in the surgeon's frame. Instead of hitting the thalamus, the electrode may be dead-center in the motor capsule. If so, lesioning here would prove disastrous. Hands on hips, Bakay shakes his head in frustration, then strides to the head of the operating table. He must expand the hold in Lavender's skull to move the electrode farther back in her brain. Lavender cries quietly as Bakay drills. Nurses hold her hands and whisper reassurances.
2:30 P.M. The team starts remapping from scratch. "Hear it? Hear it?" On the electrode's third pass through Lavender's brain, Vitek can hear the distinctive sound of thalamic cell activity. "This could be hip related," he says, rolling Lavender's leg to produce a burst of static. "I don't think we're quite there yet, but ..."
Bakay paces in the background. A nurse clutches a hot water bottle and drapes a blanket over her shoulders to ward off the operating room chill.
"There's the great toe; there's the ankle; there's the deep ankle," Vitek calls over several bursts of static. The mapping continues.
3:40 P.M. From the operating table, a weak, hoarse voice: "I want to stop."
Nearly six hours after being wheeled into the operating room, Lavender begs to move, to go home. Vitek stops, stunned, then steps close. "Donna, I know it hurts. I know you're tired. But we're close." He touches her left hand reassuringly. "I can't," she groans. Vitek steps back, confers with Bakay and returns. "Donna, we can give you something to help you relax," he says. "Let's just try."
4:15 P.M. The increased sedation has quieted the patient, but she lapses unresponsive. "Donna, do you feel this? Do you feel this?" Vitek asks, increasing the intensity of the stimulation to her brain. No answer.
4:50 P.M. The mapping continues, based more on electrical readings than on Lavender's feedback. But the crackle and buzz of brain activity is heard less often. Lavender's thalamus, like the rest of her, is flagging under the sedation. A small mercy: Lavender's leg and foot have visibly relaxed, uncurling for the first time in months. "We often see that during surgery," says Vitek. "But it won't last unless we lesion."
5 P.M. The nursing shift changes for the night. Vitek has yet to sit down.
6:15 P.M. The neurology team has pinpointed the "motor outflow tract" associated with Lavender's left leg. The mapping becomes more detailed, with Vitek stimulating Lavender's brain with smaller and smaller micro-voltages. "Can you feel this? Where do you feel this?" Lavender struggles to murmur her answer. "My my my ... foot."
7 P.M. The neurology team is confident they have found the area to be destroyed. But they advise against a large lesion: too risky, given Lavender's sedated responses. One more pass with the microelectrode to make sure of their bearings.
7:45 P.M. Lavender has been on the table more than nine hours. But now cooperation is absolutely crucial. Bakay begins to burn away brain tissue with a strong current through the electrode. Vitek grasps Lavender's hand and leans close. "Donna, this is it. I need to have you with me!" He asks her to smile broadly, purse her lips, stick out her tongue. Her responses tell him that no unwanted damage is occurring. "Now say the days of the week out loud. Come on, louder. Can you feel my cold fingers on your leg? Good, you can do it. Just five more minutes ..."
8 P.M. Bakay retracts the electrode. "Sedate her!" he calls, and the anesthesiologist puts Lavender into a mercifully deep sleep. The neurology staff stumbles out of the operating room. Bakay and his assisting surgeon cover the hold in Lavender's skull with a plastic plate.
Outside the surgical suite, the neurology team is cautious, but still hopeful. "A little disappointing," Vitek admits. "We'd hoped to be more aggressive [in destroying the problematic areas of the thalamus]. But the last thing we wanted to do is leave her with more problems."
DAYS OF WAITING
Some good news comes within a day. An MRI scan on Lavender's brain shows that the lesion is well placed. Recovering in her room, Lavender spends the morning moving her left leg and foot for the pure enjoyment of it. "I've got my leg back," she says.
But she hasn't forgotten the operating table. "It was so hard," she groans. "It hurt!"
Both Lavender and Vitek are cautious about considering further operations, either to enlarge the lesion if the dystonia in the left leg returns, or to restore the right leg if it doesn't. But as it turns out, a second surgery may not be necessary.
Seven weeks after surgery, Lavendar has recovered function and gained pain relief not only in her left leg and foot, but in the right ones as well. "I've got my life back!" she rejoices, as she makes plans to go to nursing school. "It was worth it."
Vitek can only guess at the reason for such a miraculous recovery on the right side. One possibility: Some neural "cross projections" from the right thalamus (affecting the left side of the body) had crossed into the left thalamus, where the team burned away cells. So the surgery may have ended up destroying the brain cells affecting both legs.
Alternatively, the pain and cramping Donna had been experiencing before the surgery may have been so severe as to produce a sort of negative feedback loop. The elimination of pain and cramping on one side may have been sufficient to break the feedback circuit and provide overall relief.
From past experience, surgeons and neurologists say that if the relief persists for three months after surgery, it's likely to be permanent. At press time, Donna Lavender was more than halfway home.
Jessica Snyder Sachs is the author of GOOD GERMS, BAD GERMS: Health & Survival in a Bacterial World (Hill & Wang/FSG October 2007).