Every Patient Tells a Story (20 page)

Additional admissions sent me scrambling down to the emergency room. I returned a couple of hours later to see how the patient was doing and what the attending physician had done. She’d been evaluated by the surgical resident, who wanted to take her to the OR. New labs suggested that there was dead tissue that needed to be removed.

Her family did not agree to the operation. She had already made her wishes known to them—no extraordinary measures, no surgery. They would control her pain, the family instructed, and see what happened. If she survived, so be it; if not, at least let her slip away peacefully. Her daughter would be in as soon as she could get there. I went in to see the patient before I left that morning. The room was quiet but now filled with light from what looked to be a glorious summer day beyond the window. She lay unmoving on the bed; her eyes remained closed but the muscles of her face were finally relaxed. The delicate pale skin of her face draped gracefully over her cheekbones, like a sleeping beauty never found by her prince.

Although there was nothing I could do for her, I dropped by to see Carlotta the next night, and the night after that. She never woke up when I called her name or touched her thin shoulder. The room slowly filled up with cards, colorful drawings, and flowers. “We love you Grammy,” neatly outlined in black and roughly crayoned in primary colors, was taped to the
wall across from her bed so that it would be the first thing she saw when and if she opened her eyes. Toys stored on the deep window ledge suggested at least one grandchild or great-grandchild was a regular.

When I came by the fourth night the room was empty. The cards and drawing were gone; the bed, crisply made, waited for its next occupant. Standing in that doorway, I said my own goodbyes to this woman. This is how every doctor learns, often by standing at the bedside of the patients she didn’t save. And this is how doctors pay their own private respects. I have diagnosed this disease and others similar to it, and every time I make the right call, I see Carlotta’s face once more.

Hand to Hand, Mind to Mind

Part of the romance, the appeal of the physical exam—at least for me—comes from the way it’s taught. I learned from the individual physicians who instructed me. They, in turn, had learned it from the physicians who taught them, creating a line of transmission that extends backward, like genealogy, to the originator. Emphasizing the personal nature of this transmission, the examination maneuvers or techniques often carry the name of the doctor or sometimes nurse who created them. Spurling’s sign, named for an early-twentieth-century American neurosurgeon, describes the maneuver Roy Glenwood Spurling developed to see if a pain in the arm or hand originated in the cervical spine. In this maneuver the head is tilted toward the side with the pain and then the physician presses straight down, compressing the soft discs between the bony vertebrae. If this reproduces the pain, reported Spurling in a paper published in 1944, the pain can be attributed to a pinched nerve in the neck, a useful tool in the days before MRI and still routinely taught as a way to evaluate arm pain.

Tinel’s sign was named after a French neurologist, Jules Tinel. He developed the test while caring for World War I soldiers with injuries due to gunshot wounds. Frequently, once the wounds were healed, sensation and strength would still be limited due to damaged nerves to the region. Tinel
would tap on the nerve just before it entered the injured extremity. If the patient felt tingling in the damaged area, said Tinel, the nerve was recovering and the soldier could expect to get back some sensation and use. These days, it’s commonly taught as a method for diagnosing carpal tunnel syndrome, an overuse injury of the median nerve that causes numbness or tingling in the thumb, first finger, or second finger. If a tap on the wrist reproduces these symptoms, the patient is said to have carpal tunnel syndrome.

Here’s the problem. Many of these maneuvers don’t work. Spurling’s sign is no more predictive of cervical disc disease than flipping a coin. Many people will have pain with this kind of maneuver, but the pain could have many causes: rheumatoid arthritis, osteoarthritis, bone metastases from cancer. And many with a pinched nerve in the neck will have no pain. Still, it keeps getting taught.

Tinel’s sign is just as worthless in diagnosing carpal tunnel syndrome. People who have carpal tunnel syndrome may have tingling when the nerve is tapped, but so will people with other problems. And many people with carpal tunnel syndrome won’t feel the diagnostic tingling when tapped. So it can’t reliably identify either those who have it or rule out those who don’t.

The individual components of the physical examination were developed when physicians had few other means of diagnosing problems. Any sign or symptom that was found useful at the time was welcomed into the fold. Unlike modern (and expensive) high-tech tests or medications, there was no requirement for any of these exam techniques to be evaluated. And often, when these techniques were developed, there was no way to tell if the tests were right or not except by surgery or autopsy. As technology improved, so did our ability to test our tests. But we’re only beginning to do that. In the meantime, doctors keep teaching them.

A colleague, Dr. Tom Duffy, told me about a test I’d never heard of, and about a patient for whom it made an important difference. Michael Crosby was a young man—healthy and active with no medical problems at all. Michael remembered clearly the moment he became aware that he was ill. It was his second day of teaching. A new job, a new school. He was giving the class a quiz and as the students worked he paced between their desks.
Their heads were down, pens in hand, eyes moving from the words on the board to their own papers as they worked their way through the first test of the year.

He was a substitute teacher. And that morning he felt strangely nervous. He could feel his heart pounding in his chest and hear himself breathing in short, deep gasps. He’d trained for five years to get here; done internships in some of the worst neighborhoods in urban upstate New York, and yet this middle-class ninth-grade Spanish class in rural Connecticut had him scared? His racing heart told him it was true.

But was this fear? All he knew was that it was hard to breathe. Really hard. And suddenly he was terrified. Breathing—the easiest, most natural thing in the world—all at once felt neither easy nor natural. He could feel himself go through the motion of breathing and yet the breath didn’t seem to make it to his lungs. He felt sweat beading coolly on his face. His tie felt too tight around his neck. He glanced at the clock. Could he make it to the end of the period? He sat behind the desk at the front of the room and tried to relax.

The bell finally rang. The students dropped their papers onto his desk and clotted at the door. Crosby was right behind them.

The hallway to the school nurse’s office seemed to stretch out into the distance. Every step was an effort. “I can’t breathe,” he croaked, once he finally made it to the tiny medical office. “I feel sick.” Pat Howard, the school nurse, led him to a bed. He could hear her asking him questions, trying to get more information, but it was hard to speak. He felt like he was drowning on dry land. She removed his tie, then placed a mask over his mouth and nose. The cool rush of oxygen brought some relief. He remembered being loaded into an ambulance. When he opened his eyes again, he was in the emergency room surrounded by unknown faces.

He was quickly diagnosed with a massive pulmonary embolus. A blood clot from somewhere in his body had broken free and been carried through the circulation into the heart, then lodged in his lungs. He was started on blood thinners and admitted to the ICU where he could be monitored closely. As soon as he was stable the doctors turned their attention to the clot itself: where had it come from and why did he have it? They needed to know because another assault like that could kill him.

Clotting is something our lives depend on. But like so much in the body, context is everything. In the right place, at the right time, a blood clot can save your life by preventing uncontrolled bleeding. In another setting, that same clot can kill. Clots normally form at the site of any injury to a blood vessel. They can also form when blood stops moving; that’s why anything that causes prolonged immobility, like traveling or being stuck in bed, increases the risk of a pathological clot. Pregnancy increases your risk. So do certain drugs and hormones. Some people have a genetic abnormality that makes their blood coagulate too readily. Finding the cause of a clot is crucial to estimating the risk of another.

So, his doctors looked: He had no clot in his legs—the most common source of abnormal blood clots. CT scans of his chest, abdomen, and pelvis likewise showed nothing. He hadn’t traveled recently, hadn’t been sick. He took no medicines and didn’t smoke. His doctors sent off studies of his blood to look for any evidence that his blood coagulated too eagerly. Normal. They could find no reason for this otherwise healthy young man to develop a clot. He was discharged from the hospital after two weeks and told that he would have to be on warfarin, a drug that prevents blood from clotting, for the rest of his life. Without it the risk that he would have another clot was just too high.

It’s difficult to be a patient with an illness that can’t be explained. What made that uncertainty even worse was the new certainty that accompanied it—that he would have to take a blood-thinning medicine forever. He was twenty-three years old, a jock with a sport for every season. The blood-thinning medicine would protect him from another pulmonary embolus but in return he would have to avoid anything that could cause bleeding—including the games he loved.

The patient searched for an alternative and found my friend Tom Duffy, a hematologist at Yale University with a reputation as a great diagnostician. He hoped that Duffy could figure out what caused this devastating pulmonary embolism and possibly get him off the warfarin.

Duffy is a slender, fit man in his sixties with round tortoiseshell glasses, a preference for bow ties, and a precise, studied manner of speaking. He listened to the patient’s story and then asked for a few more details: What
kind of physical activity had he been doing in the weeks before the clot? He was alternating three days of weightlifting with two days of swimming or running. Had he taken any performance-enhancing drugs? The young man admitted that he had when he was younger but he’d taken nothing for years.

As he listened to the patient, Duffy considered the possibilities. The first set of doctors had done the usual testing, so this was going to be one of the unusual causes of pulmonary embolus. The scans done when he was in the hospital hadn’t shown a clot in the vessels of his legs or trunk. A rare blood disease called paroxysmal nocturnal hemoglobinuria can cause blood clots in the liver, the spleen, or beneath the skin. The CT scan wouldn’t have shown that. Could he have this rarity? Or could he have a myxoma, a rare type of tumor that grows in heart muscle, which can cause a clot within the heart itself? The physical exam might give some clues if these diseases were involved.

As the patient undressed for the exam, Duffy was struck by the highly developed muscles of his upper body. “He looked like one of those young men in a fitness magazine,” he told me later. “It was quite striking.” Otherwise his exam was completely normal: there were no extra sounds in his heart suggesting a tumor or anything else obstructing the flow of blood. His abdominal exam revealed no tenderness or enlargement that would suggest a clot hidden there.

Duffy looked at the patient again. He remembered something he’d learned in medical school many years before. He lifted the patient’s arm until it was parallel to the floor. Carefully placing a finger over the pulse at the young man’s wrist, he moved the arm so that it was pointed just slightly behind the patient. Then he asked the patient to tilt his head up, turn his face away from the elevated arm, and take a deep breath. When he did that, the pulse disappeared. When the patient looked forward again, the pulse returned. He repeated the maneuver. Again, the pulse disappeared when the patient turned his head and took a breath. Immediately Duffy suspected what had caused the clot.

The vessels that carry the blood from the heart to and from the shoulders and arms have to travel underneath the clavicle and above the top of the rib cage—through a very narrow space. The presence of an extra rib or
hypertrophied muscles of the shoulder or neck can make this tight opening even tighter. This problem, known as thoracic outlet syndrome, is most commonly seen in young athletes who use their upper extremities extensively—baseball pitchers or weightlifters—or in workers who use their arms above the level of their shoulders—painters, wallpaper hangers, or teachers who write on a blackboard. For those with this condition, when the arm is elevated, the extra bone or muscle narrows the space between the two structures and the vessels that travel through them can be blocked. This patient was both a weightlifter and a teacher. He was a perfect setup.

Duffy set about to confirm his diagnosis and rule out any other cause of the clot. The blood work ruled out paroxysmal nocturnal hemoglobinuria. He got an MRI of the heart, which showed no tumor. An MRI taken while the patient lay with his arms above his head and his head turned away—the maneuver he’d done for Dr. Duffy—showed that one of the large veins carrying blood from the arms back to the heart was partially obstructed. Duffy was right. He referred the patient to a surgeon who had experience with this unusual and difficult surgery and the patient had his first rib removed from each side the following summer. The next winter he was able to stop taking the warfarin. That was four years ago. He’s been symptom-free ever since.

The value of any test or exam resides in its ability to reliably predict the presence or absence of disease. Many doctors wrote to me, after I published this story, to question the accuracy of the test Tom Duffy had performed, a maneuver known as Adson’s test. I searched the published literature, and these doctors were right—there was nothing on it. It simply hadn’t been studied. In other words, no one really knows how good the test is.