Every Patient Tells a Story

by Lisa Sanders

  This new generation of physicians rejected the dependence on the vagaries of the patient’s history. They argued that diseases could be classified based on changes that could be seen, felt, tasted, smelled, and heard by the doctor—changes that could be detected by an objective observation, independent of the patient’s subjective account.

  Laennec was a leader in this revolutionary reworking of the fundamental ideas of medicine. He used his new invention to find concrete, objective manifestations of disease. Others before him had developed some techniques that Laennec himself frequently made use of. But it was Laennec who made the greatest contribution to the radical new medicine, not only providing its first tool but making the link between what he was able to see and hear and the hidden dysfunction within the body.

  Laennec was in the perfect place to do this too. He was the director of Necker Hospital, a small institution on the outskirts of Paris. Because of his position, he was able to follow hospital patients and their examination over the course of an entire hospital stay. All too often he could then correlate what he found on examination with what was revealed at autopsy. Laennec pioneered the way to link the pathologic changes caused by disease within the body to clinical information—the physical exam—collected from outside the body. His work put the physical exam at the forefront of the modern approach to medicine. Using his eyes, his ears, his stethoscope, the doctor became a detective—deducing the pathology within from observations made from without. Using the clues provided by symptoms described by the patient and the signs elicited and observed by the physician, the doctor-detective was able to track down the villain—the morbid processes within the body.

  Laennec recorded each patient’s physical exam in his daily notes, carefully tracking how the exam changed over time and incorporating these findings into the cases he reported. When the patient died—a common occurrence for those sick enough to go to the hospital—Laennec could identify the cause of the disease and the symptoms that revealed it. Once Laennec had made this link between the findings on physical exam and those at autopsy, he was able to diagnose similar patients in life with a precision rarely seen in previous centuries of physicians. Many of the diseases we now routinely identify by physical exam were first described by Laennec.

  For example, Laennec was the first to diagnose emphysema. Others had seen the destructive nature of the disease on autopsy but Laennec linked the symptoms and physical findings to the pathological entity. The case involved a thirty-seven-year-old farmer who was admitted to the hospital in 1818 for worsening shortness of breath. Any exertion left him gasping for air. His hands, feet, and scrotum were hugely swollen and tinged with blue—cyanotic from a lack of oxygen. Laennec and his colleagues had seen these symptoms before. It was usually attributed to heart failure, where the heart becomes too weak to keep pumping out the quantity of blood sent back from the circulation, and fluid backs up—accumulating in lungs, abdomen, and extremities.

  The patient’s story of slowly worsening shortness of breath with any exertion combined with the clear evidence of this kind of overflow had the doctors at Necker convinced that the young farmer had heart failure. Laennec disagreed. He looked at the barrel chest of the farmer—and pictured the hyperinflated lungs he’d seen in cadavers with emphysema. He thumped the chest and heard it resonate—which suggested the lungs were filled with air—and yet noted that when he listened with his stethoscope, very little air could be heard moving in or out when the man breathed. Based on this, Laennec predicted that at autopsy the man would have a disease of the lungs, not of the heart.

  They didn’t have to wait long to find out. The farmer first came to Necker Hospital in May; he died just five months later—not of heart or lung disease but of smallpox. The autopsy showed, just as Laennec predicted, a normal heart. In the lungs, however, the delicate membranal lacework of the air exchange tissue had been ripped away, leaving large empty holes throughout—the now classic finding of emphysema.

  One of the heart sounds first described and understood by Laennec was mitral stenosis—a pathological narrowing of one of the valves of the heart. He tells the story of a strapping young man, Louis Ponsard, sixteen years old, a gardener, who came to Laennec’s hospital complaining of “oppression and palpitations.” He was a short man, muscular, and according to Laennec, “having all the appearance of splendid health.” Ponsard told the young doctor that two years earlier he “was occupied in carrying some soil on a wheelbarrow. He was forcibly stopped in the midst of his work by a violent beating of the heart, accompanied by oppression, spitting of blood and nasal hemorrhage, coming on without any preceding discomfort.” The symptoms resolved later that day, Laennec writes, “but they reappeared each time the patient attempted to take the slightest bit of exercise.”

  When Laennec examined the patient, he noted a subtle vibration of the chest, what’s called a thrill, between beats. This was accompanied by a murmur that Laennec describes as a “sound [like that] produced by a file rubbing on wood.” Based on these signs and symptoms Laennec postulated that the young man suffered from “ossification of the mitral valve,” what we now call mitral valve stenosis, or narrowing. When blood leaves the lungs, it passes through the mitral valve to enter the left ventricle on its way out into the body. In this disease, that passageway becomes narrowed and rigid. When there is a need for greater blood—during exertion—the normal valve is able to open wider to let the excess blood through. In this young man the valve was rigid, bonelike, and so couldn’t expand to allow the greater quantity of blood through.

  Understanding the problem this way allowed Laennec to treat the disease. If the problem was too much blood to make it through the narrowed valve, the available solution was to reduce the amount of blood. The young gardener was bled several times with a dramatic improvement in his symptoms.

  This was probably one of the very few diseases for which the commonly applied treatment of bleeding may have been effective. Of course, the treatment is only temporary. The young gardener had to return to Necker several times over the next several years to be bled. And ultimately he had to change jobs. He became the servant of a priest, and with this reduced workload his symptoms became much more manageable. Laennec never heard from him again. Perhaps he lived happily ever after, but given what we know about mitral stenosis now, it’s unlikely that he survived many years after his initial visits to Necker.

  I learned about mitral stenosis the way I’ve learned so much of medicine—from my own mistakes. In fact, Laennec’s discovery is what brought me to that makeshift classroom at the American College of Physicians conference. Like the dozen or so other doctors, I was there because I suddenly understood that despite years of training and practice, I still didn’t know how to perform an adequate examination of the heart. Just like the doctors in the studies I’d read, I couldn’t recognize some of the most basic abnormalities of the heart. I owe that discovery to Susan Sukhoo.

  Susan was a slender woman of Indian extraction who’d been born and raised in Guyana, then immigrated to Miami with her husband some twenty years ago. She became my patient when she moved to Connecticut to live near her sisters after finding out that her husband was supporting a mistress. She was fifty-eight, had a little hypertension that was well controlled on a single medication, and many of our early visits focused on the consequences of her grief and depression.

  Then she developed asthma.

  She came to my office one frigid December morning looking her usual self—dressed simply but with a quiet elegance in tidy jeans, colorful T-shirt, and blazer. A single strand of pearls hugged the contours of her clavicles, showing off a youthful neck. Her hair was swept up in a simple knot at the back of her head, its smooth darkness only beginning to show traces of white. She smiled shyly at me as I entered the room and greeted her. “I’m wheezeling,” she told me in the lilting inflections of her Guyanese-Indian accent. I wasn’t exactly sure what she meant. “When I walk, especially when it’s cold out, I start wheezeling,” she e
xplained, and then, like a caller on the radio show Car Talk, she began imitating the musical sound she heard when she breathed. She was wheezing.

  The “wheezeling” sometimes woke her up at night and she would have to sit up. A couple of nights she ended up sleeping in a chair because she felt like she couldn’t breathe lying down. She had no chest pain, but sometimes felt chest tightness when she took a deep breath. These episodes lasted only a few minutes. After they resolved, she told me, she felt fine. She had recently had an upper respiratory tract infection and with further questioning thought the wheeze might have started when she was sick.

  On exam, her blood pressure was normal. The amount of oxygen in her blood was fine. But there were diffuse wheezes throughout both lung fields. The breath came in with the normal whoosh of air flowing through a tube. But when exhaling, her chest was filled with a variety of musical sounds. This cacophony of different pitches and durations sounded like an orchestra of plastic horns warming up before a performance. Otherwise her exam was unremarkable.

  Wheezing is caused by a transient constriction of the airways. Asthma is the most common cause of wheezing but it would be unusual for a woman this age with no history of this disease to suddenly develop it. Some infections can cause the airways of the lungs to overreact to sudden changes in air temperature or flow and that can cause wheezing—especially when you go from the warmth of a well-heated room into the frigid winter air or take a sudden deep breath. I gave her an inhaler to dampen the overreacting airways and assured her that it probably wouldn’t last long. Wheezing and cough are common symptoms after a cold and usually resolve after a month or so. She’d had her cold several weeks earlier so I figured she must be on the tail end of the thing.

  When I saw her next, a couple of months later, I asked her about the wheezing. Oh yes, she told me, “the wheezeling was there every day.” She took a deep breath and let it out slowly. I could hear the wheeze from across the room. The inhaler was helpful, she added, and she used it almost every day. I wasn’t sure what to make of this. We learn in medical school that “all that wheezes isn’t asthma,” but what then? Was this emphysema? She had never smoked, but her husband had. Could this be a so-called cardiac wheeze, where a weak heart can’t pump all the blood that comes into it and so fluid gets backed up into the lungs, causing the wheeze? She hadn’t had any chest pain, and her only risk factor for a heart attack (which can give you a weak heart) was her high blood pressure, and that had always been well controlled. She was from an area where TB was common—could this be an unusual manifestation of tuberculosis?

  I got an EKG, which was normal. Reassured that she hadn’t had a hidden heart attack, I also tested her for tuberculosis. In addition, I ordered some tests to do over the next couple of weeks to try to identify the cause of the wheeze. Pulmonary function tests would help distinguish asthma from emphysema or heart disease. All the other possible causes seemed far too unlikely in this extremely healthy woman. I also gave her another inhaler, this one containing steroids to reduce the frequency of what I still assumed was an atypical asthma.

  She returned to the office a month later. “Did anyone call to tell you I was in the hospital?” she asked. I’d heard nothing about it. It is a chronic problem in the community where I work. When a patient goes to the hospital—especially the other hospital in town—the doctor is often the last to know.

  It happened in the middle of the night, she told me. She’d woken up drenched in sweat and gasping for air. A cough emerged from deep inside her chest. Her heart pounded so hard she felt the bed move with every beat. The chest tightness she’d felt when she’d first described her wheezing was back and much worse than it had ever been. She struggled to the phone—any exertion made her chest squeeze even tighter. She cried when she heard the siren, so grateful that help was close. In the ambulance and in the ER she’d been given albuterol, a medicine that relieves wheezing for patients with asthma. Normally it helped but that night it didn’t seem to do anything.

  An EKG showed she wasn’t having a heart attack. A chest X-ray showed fluid in her lungs and they gave her a shot of something they told her was a medicine to help her pee out the extra fluid. Within an hour of getting that shot she started to feel better.

  She stayed in the hospital for three days as her doctors tried to figure out why she had the fluid in her lungs. Dr. Eric Holmboe, an internist on the teaching faculty, had diagnosed her on examination. His residents had called to tell him about the fifty-eight-year-old woman with poorly controlled, newly diagnosed asthma, and even before he saw her he was creating a list of diseases that could cause an asthma-like presentation. Whatever it was, he told me, he would have laid out money that it wasn’t asthma.

  When he listened to her heart, he heard the murmur Laennec had described. It was a quiet sound and could easily have been overlooked in a noisy emergency room. He could really only hear it when the patient lay on her left side so that the mitral valve was closer to the surface of the chest. And yet when he heard it, he knew she had mitral stenosis.

  An ultrasound of her heart confirmed his diagnosis. The blood that would normally travel through that opening, to fill up the left ventricle—the main pumping chamber of the heart—couldn’t get through the now tiny orifice. The opening, normally the size of a half dollar, had shrunk down so that it was smaller than a dime. The circulating blood couldn’t all get through and so it backed up, flooding the lungs with fluid. “The doctor in the hospital asked me if I had ever had rheumatic fever as a kid,” Susan told me, “and I told him everybody in my family had it. But I hadn’t thought about it for years and years.”

  Rheumatic fever is an inflammatory complication of strep infection—often strep throat. Most often joints are the target. Days to weeks after an untreated case of strep throat, the patient will develop hot, swollen, and painful joints. It can be a single joint, multiple joints, or most strangely of all the inflammation can travel from one joint to another. The same inflammatory process can attack the heart as well. It is frequently undetected because it doesn’t cause any symptoms—not for years.

  In Susan’s case the damage done as a child had slowly eaten away at her valve and by the time she developed “asthma” the valve was nearly completely closed. She was scheduled to get a new mitral valve in a month, she told me that day.

  Mitral stenosis—why hadn’t I heard any evidence of this significant lesion during her heart exam? I placed my stethoscope on her chest, starting, as I had been taught, on the right, and worked my way to the left side of the sternum, then down to the middle of the rib cage, and then left again toward the edge. The lower left aspect of the chest is where this particular murmur is usually heard; it then travels to the far left side of the body. When I reached the lower left position I listened intently. I could barely hear—something. I had her lean forward, so that the heart would swing out, a little closer to the chest wall. There it was—a soft, low-pitched sound that came between heartbeats in diastole, rumbly and harsh and very, very quiet. I listened near the edge of the chest. I heard it there too. Now.

  In my earlier exams I had completely missed this. I checked my previous notes—no mention of a murmur. It was a quiet sound and I hadn’t done the kind of thorough exam I had been taught to do, so I hadn’t heard it. I finished up my visit; I told her to let me know when she was to go into the hospital and I’d come visit her there.

  Ultimately Susan’s problem was resolved at the source. The tiny opening was widened. She had her scarred mitral valve removed and a metallic valve was inserted. Her heart was as good as new.

  At home the night after I heard about Susan’s diagnosis, and for many nights thereafter, I thought about this missed diagnosis. All those months of “wheezeling” and shortness of breath and I’d been treating her as if she had asthma. She was getting worse right in front of my eyes as the aperture of the mitral valve approached a critical stage. It distressed me to know I could have figured it out too, if only I had done a proper exam. How many
other diagnoses have I missed because of an inadequate examination of the heart? And I’m not alone. How many diagnoses have we all missed, because most of us don’t have a clue about an adequate heart exam?

  Putting the Ear to the Test

  But what if it’s not our fault? If so few doctors can make this kind of diagnosis, maybe it’s not possible. Just how good is the heart exam at picking up these defects anyway? As practiced now, we know that it isn’t very good at all. Few of the doctors in practice and in training are able to use the heart exam to make a correct diagnosis. We’ve come instead to depend on technology to make this diagnosis for us.

  Echocardiography has been shown to be accurate in diagnosing many of the same diseases that the cardiac exam used to be good for. Small wonder then that the number of echocardiograms has increased so dramatically. The number of echos ordered almost doubled over a seven-year period—growing from 11 million a year in 1996 to 21 million a year in 2003. In one large multispecialty group in Boston the number of echos increased over 10 percent over one year alone, with 9 percent of all patients seen in the practice getting one. Is it simply that we no longer have any faith in our own ability to perform the exam, or is the exam fundamentally flawed and ready to be thrown over? Actually, studies show that the cardiac exam can be pretty darn good when done properly. In one study, five cardiologists were pitted against echocardiography in fifty-two patients with known valvular heart disease—one of the most difficult and important diagnoses we make when we examine the heart. The cardiologists had to correctly identify which of the four valves of the heart was affected and estimate the degree of damage. Each patient was also evaluated by echocardiography. How did the cardiologist do?

  As in so many of these contests, the machine won. The echo was correct 95 to 100 percent of the time. Yet the doctors put up a good fight. Their diagnoses were right between 70 and 90 percent of the time. Other studies have shown similar results. That’s certainly much better than the current crop of physicians if you believe Mangione’s studies. The question is—is it good enough? Doctors and patients alike would probably say no. The ear and the stethoscope cannot replace the echo for locating the source of an abnormal heart sound when it’s important.