The obvious place to look isn’t always the right place.
“I’ve been coughing up blood,” the 59-year-old man confessed to his wife as they drove toward New York City from their home in Connecticut. It started the night before, he admitted when she asked. After 40 years of marriage, his wife, a nurse, was used to this kind of nonchalance from her husband, though it always carried a kind of punch. Take this exit, she instructed. They were near an urgent-care center in Brewster, N.Y. He needed to get this checked out.
It was quiet when the couple walked in to urgent care, so her husband was seen right away. He had a runny nose and a cough for the past couple of days, and a few times he’d seen streaks of blood in what he coughed up, he told the nurse. His chest hurt a little when he took a deep breath, but otherwise he felt just fine. His vital signs, however, told a different story. He didn’t have a fever, but his oxygen level was at 91 percent. Even with the worst cold, his oxygen should have been 98 to 100 percent. Did he feel short of breath? Not particularly, he said. Maybe when he was walking from the car, but sitting here now? Not at all. He needed a CT scan of his chest, the couple was told, and so he was transferred to the emergency department at Northern Westchester Hospital in Mount Kisco, N.Y.
The CT didn’t reveal any clots in the arteries of the lungs, so he didn’t have the feared pulmonary embolus, which could have caused his low oxygen and hemoptysis (coughing up blood) without other symptoms. Even so, the images were far from normal. There were patches of a light haze in areas that should be dark in both lungs. Maybe pneumonia? People usually feel sicker than this man when they have pneumonia, but how else could they explain the low oxygen level? He was started on antibiotics and admitted for observation.
Crisis in the Harbor
Dr. Tara Shapiro was the doctor assigned to his care that night. She was not at all sure the problem was in the man’s lungs. The CT scan also revealed a heart that was thicker and more muscular-looking than it should be. This kind of hypertrophy, as it’s called, is frequently seen in patients who have high blood pressure, when the heart has to work hard to squeeze its payload into the bloodstream. But this man didn’t have high blood pressure.
The patient had a full cardiac work-up a few months earlier that he said was normal. But Shapiro was still worried that it was his heart and not his lungs that was failing him. His oxygen level improved greatly in just the few hours he’d been in the hospital. It was far too early for the antibiotics to have done this. More likely it was from the powerful diuretic he’d already been given in case the haziness in his lungs was fluid rather than an infection. A muscular heart doesn’t pump as well as a normal heart and sometimes can’t keep up. When that happens, fluid can get backed up — right into the lungs.
Shapiro reached out to a cardiologist colleague, Dr. Ronald Wallach. He was one of the most knowledgeable doctors she knew. Wallach saw the patient the following day, just before he was discharged. The patient’s wife was reassured by the doctor’s white hair and air of quiet authority. Her husband, something of a hardhead, would certainly listen to this guy.
After hearing the man’s story, Wallach asked if he had been short of breath before. Well, maybe it had been going on for a while, the man acknowledged. How long? The man’s wife gave him a sharp look. Certainly for the past several months, at least since the summer, he said. That’s when he’d had some serious trouble breathing.
He was out one weekend on his motorboat with his wife and adult daughter. The women were on inner tubes in the cove just behind New Rochelle Harbor, enjoying the sun and calm waters. Then suddenly: “Throw me the rope,” he heard his wife shout. He looked up to see the two women moving rapidly away from his boat, caught in the oceanbound tide. He wrapped one end of a rope around his body and threw the other end out to his wife. It took a couple of tries, but she got it. He struggled to pull wife and daughter back to the side of the boat. As they clambered over the side, it was his wife who noticed his breathing. His face was red and shiny with sweat, and he was panting for breath. Are you OK? she asked. He nodded his head and held up a finger as if to say give me a minute. It took more than a minute — a lot more. It scared her. He was a tough guy, but it might have scared him too, because, though he refused to go to the emergency room then, he did see his primary-care doctor later that week.
That doctor immediately sent him to a pulmonologist and then a cardiologist. The lung doctor gave him a diagnosis of asthma. It’s unusual at this age, the doctor said, but it happens. He gave the patient an inhaler to use when he felt short of breath. It didn’t help. The cardiologist ordered a stress test. The patient lasted only a few minutes before he was too out of breath to continue. His EKG was normal throughout the test, so his cardiologist chalked it up to his asthma. He was an elevator mechanic and that meant that most days he had to climb stairs — sometimes lots of stairs — to fix broken machinery. The man noticed the stairs had become a little harder on him over the past year or so, but, he asked Wallach with shrug and a smile, what can you do?
Zigzag Fibers
It was the EKG done in the emergency department that provided Wallach with the last clue he needed to make his diagnosis. An EKG measures the electricity generated by the heart in order to make the muscles contract effectively. A thick, muscular heart will make an EKG tracing that is bigger, more exaggerated than normal. The more muscle present, the bigger the signal. But this man’s heart generated a signal that was smaller than normal. Less electricity could suggest less muscle. Was this man’s heart enlarged by something other than muscle?
There are diseases that can invade cardiac muscles to make them look bigger but be weaker. A disease like that could account for all the man’s symptoms — the thick-looking walls, the overflow into the lungs, the strange EKG, the shortness of breath, even the hemoptysis. “I think you might have something serious,” Wallach told the patient. A cardiac M.R.I. could give them the answer. The patient got that test a few days later. He wasn’t out of the scanner for more than 20 minutes when his phone rang. It was Wallach. The images told the story: The man had a disease known as amyloidosis.
Amyloidosis is the final result of many disease processes that ultimately cause zigzag-shaped fibers to accumulate in different parts of the body. Cardiac amyloidosis can be a result of a cancer known as multiple myeloma. In this cancer, a type of white cell called a plasma cell creates abnormal fibers that can break down and form the characteristic saw-toothed fibers of amyloidosis. These jagged fibers can also be a result of aging. In this version of the disease, carrier proteins known as transthyretins break down and take on the abnormal but characteristic irregular folds of amyloidosis. In both diseases, these serrated fibers travel through the body, invading and accumulating in muscle — often the heart muscle.
Tests on blood and urine quickly showed that his disease wasn’t due to myeloma. That was a relief; the prognosis for patients with cardiac amyloidosis from multiple myeloma is poor. They often die within a year of getting the diagnosis. A biopsy of the heart muscle proved that it was the form of amyloidosis associated with aging. This type of amyloidosis is also progressive but much more slowly. The patient was referred to a cardiothoracic surgeon at Columbia University. Sooner or later, he was going to need a heart transplant.
Three years passed before Wallach heard again from the patient. He wrote to let Wallach know he’d received his heart transplant and was doing well. He was writing to say thank you: “You saved my life.”
I asked Wallach how he could make this diagnosis when other doctors had not. He called it the Aunt Tilly Sign. “If I described Aunt Tilly to you and sent you out into a crowd to find her, you’d probably fail. But if you’d ever seen Aunt Tilly” — he snapped his fingers — “no problem. You’d find her in a second. It’s all about recognition.”
Lisa Sanders, M.D., is a contributing writer for the magazine. Her latest book is “Diagnosis: Solving the Most Baffling Medical Mysteries.” If you have a solved case to share with Dr. Sanders, write her at Lisa .Sandersmd@gmail.com.
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