by Lisa Sanders
He offered a diagnosis and some reassurance.
“I don’t think the fever and the back pain are related,” he told them. “I think the back and leg pain is sciatica. And the fever—who knows? Some virus, probably.” He gave Gayle some ibuprofen and a muscle relaxer for her back. When Kathy—angered at the breezy exam and unconvinced by his diagnosis—brought up the possibility of Lyme disease (“We’ve been camping, for God’s sake”), he dutifully wrote out a prescription for doxycycline, the antibiotic of choice for this disease.
Kathy was worried—she was a physical therapist. She had seen lots of sciatica but none this bad. And this fever? Hard to believe they weren’t related. Gayle, on the other hand, was relieved by the reassuring diagnoses. She had never been sick and wasn’t ready to start now. After leaving the hospital they drove until dawn, then checked into a roadside motel and caught up on the sleep they’d missed. They slept soundly—Gayle with the help of the ibuprofen, the muscle relaxer, and, at Kathy’s insistence, the doxycycline. When they awoke it was late afternoon.
Gayle sat up. She felt a little better, though her legs were strangely heavy as she swung them to the floor. When she tried to stand, they buckled beneath her and she fell back, helpless, onto the bed.
“My legs aren’t working, Kathy. I can’t walk.” Gayle’s voice was high-pitched and terrified. “I can’t walk,” she repeated.
Kathy’s heart began to race. She knew it. There really was something wrong. They weren’t far from Baltimore—maybe there? No, Gayle insisted. She wanted to go home.
They were at least five or six hours from the small Connecticut city they lived in. Kathy drove as fast as she could directly to their local hospital. “It was the longest five hours of my life,” she told me later.
“Stay here,” she instructed her partner and disappeared into the emergency room. She returned a few minutes later with a couple of EMTs—emergency medical technicians—and a wheelchair. The three of them helped the now crippled woman out of the car and hurried her into the ER.
Dr. Parvin Zawahir, a first-year resident, was the doctor on call that night. She quickly reviewed the thin chart that documented the patient’s time in the ER. A fever of 101. Weakness. The blood work already done didn’t show much—the white blood cell count wasn’t elevated. Chemistry was normal. Liver—normal.
She found the patient’s curtained-off cubicle, introduced herself, and began the familiar process of taking a history. It had started five days ago, Gayle told her. She had a stomachache and some diarrhea. She figured it was a touch of food poisoning and didn’t think much of it. Two days later she’d developed a rash on her neck. It didn’t itch or hurt and she hadn’t even noticed it until Kathy pointed it out. She thought at first it might have been a spot rubbed raw by the strap of her bicycling helmet, but the next day it had spread to her legs and stomach. Then yesterday, she’d felt tired after shooting a few baskets—not her normal stamina. But she hadn’t actually felt sick until that pain woke her up almost twenty-four hours ago.
Any bites? Zawahir asked. Gayle nodded. Lots. She’d gotten plenty of mosquito bites. Didn’t recall any tick bites. She hadn’t been around anyone who was sick. No pets. She didn’t smoke—never had. She didn’t drink or use drugs.
The young doctor looked closely at the rash. It was faint but covered much of her body. It was made up of dozens of small, slightly raised, slightly red bumps.
Her back looked normal enough and had no tenderness. The rest of the exam was unremarkable until she got to the patient’s legs. Gayle was able to wiggle her toes and move her feet forward and backward. But she couldn’t lift her legs—at least not the left one. Zawahir sat down at the desk and started on her admission note. How was she to put all this together? Was this a problem of the muscles? That was the only part of the exam that was abnormal. Or was it the nerves that empowered the muscles? The kind of pain the patient described—with the electric charges down her leg—certainly sounded a lot like the sciatica the Maryland ER doctor had thought it was. But Zawahir couldn’t believe that the fever and pain were separate problems. That didn’t make sense. They started at the same time. No, they had to be linked.
Infection seemed most likely. Being outdoors for all that time, she was a perfect candidate for Lyme disease. On the other hand, the patient had been in Colorado and West Virginia and a dozen points between—was there Lyme disease in these places? What about Rocky Mountain spotted fever? That was also carried by ticks and characterized by a fever and a rash. And it could be deadly.
Could it be a mosquito-borne illness? In Connecticut, every summer there was a big scare for Eastern equine encephalitis. Though she didn’t know how many cases of this disease there were in a year, she’d read that it was frequently fatal. What other viruses could do this? Could this be West Nile virus? Herpes encephalitis? She wasn’t sure. She’d never seen any of these illnesses.
She would need to do a spinal tap to see if the lab could find any bacteria or evidence of infection in the fluid. And she would send off for more blood tests as well. An MRI would show if there was an infection in or near the spinal cord. She would start her on high-dose antibiotics—one that would cover both Lyme and Rocky Mountain spotted fever. And she’d like to get an infectious disease consult. Maybe a specialist could help her figure this case out.
Although she’d taken care of sicker patients, the intern was worried about the near paralysis of the patient’s legs. If you catch a neurological injury early enough you can sometimes reverse the damage. If not, this youthful, active woman could be crippled for life.
After rounds the next morning, Zawahir sought out Dr. Majid Sadigh, an infectious disease expert in the hospital and one of the smartest doctors she knew. Every doctor knows someone like this—the guy you go to when you’re stumped. Or worried. Or scared. In every hospital or community of physicians, there is always that one doctor whose clinical acumen and breadth of knowledge seem far greater than anyone else’s. There is no list of such names or awards given for this honor. It’s simply word of mouth among physicians. In central Connecticut, Sadigh was one of those doctors.
Majid Sadigh had trained in infectious disease in his homeland of Iran. In 1979, not long after Sadigh had completed his training, Mohammed Reza Pahlavi, the U.S.-supported monarch (known here as the Shah of Iran), was overthrown in a religious revolution and Sadigh and his family were forced to flee. He ended up in Waterbury, Connecticut. In order to practice medicine in this country, all foreign-trained physicians have to complete a residency here, regardless of their previous experience. The program Sadigh was accepted into was small but widely respected for the high quality of its teaching. Sadigh’s skills were so impressive that by the end of the first year of what is normally a three-year program, he was made chief resident. The following year, he joined the faculty at Yale Medical School and has been there ever since.
From the first days of his residency, Sadigh realized that he had a skill almost unknown in this country: he understood the techniques and the value of the physical examination. In Iran even simple tests are often unavailable. In this setting a physician must rely on the patient’s story and physical exam to make a diagnosis. “The body is there, filled with so much, so much to tell you. But if you do not speak the language, you will be deaf to its secrets. My job,” he told me, “is to teach our residents this important language.”
Zawahir briefly laid out the case for Sadigh, then took him to the patient. The young doctor watched with interest as Sadigh spoke to Gayle and Kathy. He sat down next to the bed and began to question the two women about what had happened. Then he carefully examined Gayle, paying special attention to the affected left leg. He elevated both heels, cupping them in his palms a couple of inches above the sheets.
“Lift your right leg,” he instructed. As she struggled to raise the weakened right leg, the paralyzed left leg sank a bit, but not low enough to touch the sheets.
“Now lift the left.” Gayle bit her lip as she s
trained to elevate the partially paralyzed leg. As she worked, the right heel sank down to the bed as she recruited the strength in her hips to raise the leg. The left leg never budged. Replacing her legs on the bed, he tested the strength in her lower legs.
“Push against my hand with your feet like you are stepping on the gas.” The right foot flexed forward; the left barely moved. He touched her gently on both legs.
“Can you feel this?” She nodded. “Is it the same on both legs?” Again she nodded. He worked his way up her legs. Sensation was normal. He lifted her left knee with one hand and struck it with a rubber arrowhead hammer. Nothing. He repeated the move on the right. The leg jerked and swung upward. He tried again on the left and again there was no response at all.
He stared at the left leg, then called Zawahir over. “Look at this,” he said, pointing to the patient’s leg. Tiny patches of skin on Gayle’s leg appeared to be moving, jerking, twisting. There was no movement of the leg itself—just the skin and the muscles of the thigh. Small groups of muscles were contracting spontaneously, independently. It looked as if there were little worms inching along under the skin.
“Fasciculations,” said Sadigh in his soft accented voice; little uncoordinated bursts of activity from a group of muscle fibers powered by a single nerve fiber. He knew he had found an important clue.
Outside the room, Sadigh reviewed what he thought were the important characteristics of the patient and her illness: First, she had been very healthy until now and had spent a lot of time outdoors. She had a profound weakness that affected both legs, but one much more than the other. It was only the thigh and hip muscles that were involved—the muscles of the lower leg and upper body were spared. Only the nerves that power the muscles were affected. Sensation, which is carried on different nerve fibers and connects to a different part of the spinal cord, was normal. And she had fasciculations. Those little muscle jerks were the clincher. The fasciculations and the sparing of sensation suggested that a single type of cell in the spinal cord was affected: the cells that control the muscles of the body, known as the anterior horn cells—a description based on where they are located in the spinal cord.
“I’ve seen this before—but not so much in this country. This is what polio looks like,” he said—then added: “But I do not think this is polio.” There is another disease, he explained, a disease new to this country. A disease that can look just like polio. A disease that can cause the same devastating paralysis. He paused. “I think she has the West Nile virus.”
West Nile had burst into the news four years earlier in the summer of 1999, when it ravaged a small community in Queens, New York. It was a disease well known in Africa, where it originated, and localized epidemics had been reported throughout Europe and parts of Russia, but until that summer, it had never been seen in the United States. The distinctive presentation of the disease—with its poliolike paralysis and its preference for those over fifty—had helped the Health Department doctors in New York recognize it as a new entity and move rapidly and aggressively to contain the epidemic. Nevertheless, sixty-two people were hospitalized with the virus that summer; seven of them—all over fifty—had died. Despite aggressive measures to wipe out the mosquitoes that spread the disease, by 2003 cases had been reported in every state in the continental United States.
Sadigh remembered the events of the summer of 1999 clearly. The poliolike quality of the disease had been much discussed at the time. Seeing Delacroix, Sadigh was certain this is what she had. A sample of Gayle Delacroix’s spinal fluid had to be sent to the state lab in Hartford to confirm the diagnosis. It would be days—maybe weeks—before the results would be available. In the meantime they would make sure that it wasn’t some other entity that they would need to treat.
After discussing the likelihood of West Nile virus with Dr. Sadigh, Zawahir returned to the patient’s bedside to tell her the news. Gayle and Kathy had heard about West Nile virus. Who in Connecticut had not? But they didn’t know much about it. Zawahir made the parallel to polio that Sadigh had made. When she heard that, the patient’s eyes filled with tears. The very word brought up images of children in iron lungs or walking with metal braces and crutches. Was that her future? Zawahir tried to reassure her but she didn’t know. This was one of the first cases seen in the state. They’d simply have to wait and see what happened.
“The hardest part was not knowing what was going on or where this would take me,” Gayle told me. The diagnosis of West Nile virus wasn’t reassuring, but for someone relatively young and exceptionally healthy it was survivable. She and her partner found themselves in a whole new world. It wasn’t where they wanted to be, but it was where they were, and so they threw themselves into the work of learning a new language, mastering a new landscape.
Kathy read up on West Nile virus and polio, hungry for strategies to help her partner fight back. By her third day in the hospital, though still febrile and weak, Gayle insisted on trying to get out of bed and stand. She did it, though she needed help. By the end of the week she had taken a few unsteady steps braced with a walker and monitored by the physical therapist. Meanwhile, the test results slowly trickled in. It wasn’t Lyme; it wasn’t Rocky Mountain spotted fever. It wasn’t tuberculosis, sarcoidosis, syphilis, or HIV. Antibiotics given in the hope of a treatable infection were stopped. Finally they received the confirmation of what they already knew. She had been infected with the West Nile virus.
“We hoped against hope that it wasn’t West Nile, but the doctors seemed pretty sure right from the start,” Gayle told me. Just knowing what she was up against—as scary as it was—was unexpectedly comforting and gave her a direction to focus her considerable energy to get well.
No Time for a Physical
In the case of Gayle Delacroix and the West Nile virus, the physical exam led directly to an extraordinary diagnosis. More commonly, the physical exam can provide not a diagnosis but an essential clue to direct further testing—a shortcut to the right answer. Ordering a slew of studies to evaluate a patient might get you the answer eventually, but time is often short in the care of a very sick patient. In many cases a careful exam can focus the search and help the physician find the problem faster. Where such an advantage would be most helpful, naturally, is among those patients who are critically ill. But even here—maybe especially here—the physical exam is becoming as obsolete as the doctor’s black bag.
The sicker the patient, the greater the temptation to skip the fundamentals—like the physical examination—and to rely on the available technology to provide us with answers. It’s a temptation that can sometimes prove fatal—as Charlie Jackson almost discovered.
For most of his adult life Charlie Jackson didn’t go to doctors. That changed when he had a massive stroke at age sixty-two. The stroke rendered his right leg and arm nearly motionless, his face crooked, and his speech slurred. Still, his beautiful cockeyed smile and gallant manner—he frequently showed up for his appointments toting a basket of peaches or a bag of pecans from back home in his native Carolina—made him a favorite at our office. He had been doing well, so I was shocked when I got a call from the staff saying that Charlie was dying.
He’d come to the office for a regular follow-up appointment with Sue, our nurse-practitioner. As soon as she saw him that morning, she knew that there was something very wrong. His walk, always a little ungainly after his stroke, was barely a shuffle. His slender frame was bent over his walker as if he couldn’t hold himself up.
“What’s the matter, Charlie?” she asked as she hurried to his side. “I … can’t … walk.” He choked out the words. His voice was strange in a new way, too—as if he were speaking in slow motion. She reached down and felt his pulse. It was slow—very slow. Too slow to keep even this slender reed of a man alive. She didn’t do any more of an examination. She knew he needed to be in a hospital.
The EMT team burst through the emergency room doors, pushing Charlie into the throng of the crowded room. The triage nurse directed t
hem straight into an empty cubicle as they barked out what they knew. “Sixty-four-year-old man … history of a stroke … complaints of weakness and belly pain.” His heart was slow, they reported; his blood pressure too low to be measured. The monitor showed a heart rate in the twenties—normal is over sixty. Dr. Ralph Warner strode in and quickly assessed the situation. “Get me an amp of atropine,” he snapped, calling for the medicine used to speed up the heart.
After injecting the medicine, he watched as the monitor continued its flat yellow line, broken far too rarely by the spike indicating another heartbeat. But slowly the patient’s heart rate and blood pressure began to rise.
With the usual chaos of the emergency room boiling around them, Warner forced himself to sit and focus as Charlie described his symptoms. It had started the night before, he told the doctor in his new, strange slur. He felt weak, could barely move. That morning his stomach began to ache. Any chest pain? Warner broke in. Shortness of breath? Fever or chills? Vomiting? The patient shook his head no. He was taking medications to lower his blood pressure and cholesterol. He had not smoked or drunk alcohol since his stroke. A brief exam showed Warner the results of the stroke but he saw nothing else.
Why was his heart beating so slowly? the doctor wondered. Had he taken too much of one of his medications? Had he suffered a heart attack that affected the natural pacemaker in his heart? The EKG, although abnormal, didn’t suggest a heart attack. Warner called the cardiologist, who rushed in to place a temporary pacemaker. Charlie was being prepped for this potentially lifesaving treatment when the lab called with part of the answer.
Blood work done in the emergency room showed that the patient’s kidneys weren’t working. And his potassium—an essential element in body chemistry, regulated by the kidneys—was dangerously high. Potassium controls how easily a cell responds to the body’s commands. Too little potassium, and the cells overreact to any stimulation; too much, and the body slows down. If the elevated potassium was slowing his heart, then getting rid of the mineral would allow his heart to pump at a normal rate. The patient was given a medicine to get the potassium out of his system and then transferred to the ICU for monitoring.