His unusual wheezing was getting worse. what was that?
One of the first things she noticed was that she had to keep clearing her throat. Everyone does it from time to time, but for her, a healthy woman in her early 70s, it had become a constant. Her husband never complained. He was a surgeon, and when the throat clearing started, he showed her some breathing exercises. It was sometimes useful, but she ended up having to start again every few minutes. Even more annoying was that any effort could trigger an odd, raspy wheeze. Even when she was on the phone, she would often mute herself so her friends and family wouldn’t worry.
His attending physician was not worried. His lungs were clear; his oxygen saturation was good. She saw a cardiologist, who said her heart was in good shape after a vigorous exercise test.
Despite the comfort, she noticed that she was getting out of breath more easily. In Europe with her granddaughter, she took 20,000 steps a day on the mostly flat streets of Paris, but the rolling cobblestones of Montmartre left her breathless. She knew she had to understand that. But when she got home to Cupertino, Calif., Covid hit and it all came to a halt.
During this time, the stairs in her house have become her measure. For decades, she walked up and down these steps several times a day with no problem. She had gotten used to the wheezing the stairs seemed to trigger, but now she felt out of breath by the time she reached the top. Then she had to stop halfway up. Then after only a few steps.
Finally, when the pandemic subsided after a terrible year and a half, she saw her GP and then a group of specialists. His lungs appeared clear and a chest X-ray was normal. Was it asthma or some kind of allergy? A variety of inhalers and an antihistamine were ineffective; an examination of his nose and throat with a small telescope found nothing. A scan of her lungs wasn’t quite normal: she had a few small nodules, and so seven months later she had another scan to see if any of the tiny dots had changed. They had no – probably just scars from a past infection. It was disheartening to hear that everything was fine and at the same time to know that it was not.
The doctors didn’t know what else to do, and neither did the patient. Her husband asked his colleagues. He called an old friend, Dr. James Wolfe, in nearby San Jose. Wolfe was a pulmonologist as well as an allergy specialist. Even if the antihistamines didn’t help, maybe allergies played a role.
Something other than wheezing
Weeks later, the patient and her husband sat in Wolfe’s exam room. While they were waiting for the specialist, the husband said to his wife: Can you jump up and down a few times so the doctor can hear what you’re doing when you’re a little out of breath?
It worked. As Wolfe greeted his old friend, he noticed the patient’s heavy breathing. But it was obvious to him that it wasn’t typical wheezing. These usually occur during exhalation. This woman’s breath was loudest when she inhaled – a type of wheezing known as stridor. This is an important observation because the causes of stridor are different from other types of wheezing. Stridor is usually caused by blockages in the upper airways – due to malfunctioning vocal cords or swollen tissues in the nose or throat. It was confusing; his upper airways had already been examined. They were fine.
Wolfe asked the patient to do a second breath test when she arrived. The first, performed a year earlier, was completely normal. This one was not. The changes were subtle but real. The amount of air she could push out on forced exhalation was less than when she was tested the previous year.
Could it be a difficult form of asthma, since the usual medications hadn’t helped? Or was it some kind of slowly growing lung infection? There is a bacterium, a distant cousin of tuberculosis, called mycobacterium avium complex (MAC), which can cause coughing, shortness of breath and the production of phlegm. It is rare but is seen most often in older women. It is thought to be caused, at least in part, by a woman’s reluctance to cough and clear mucus and other secretions from her lungs and airways. This is called Lady Windermere Syndrome, named after a character in an Oscar Wilde play. Lady Windermere is a very decent young Victorian woman who would probably be too well-behaved to cough or show other signs of illness. The nodules in the patient’s lungs that showed up on her CT scans could be the first sign of such an infection.
Wolfe ordered a series of tests to look for each of these disorders. He also ordered another CT scan of his lungs – his third – to see if the nodules had changed in the months since his last scan.
Browse CT images
Dr. Emily Tsai, a radiologist specializing in chest imaging at Stanford University School of Medicine, sat in a darkened room looking through the more than 300 images from the patient’s new CT scan. Although you can look at each image separately, it’s often more useful to view them sequentially, like a flipbook in which the drawings turn into moving images. This way, the radiologist can take a three-dimensional tour through the examined chest, following the blood vessels and airways as they appear, progress and terminate in this animated spectacle.
Tsai had developed her own system: First, she looked through the big picture, looking for obvious anomalies and getting the lay of the land. She compared the most recent views with the previous images. Then she focused on the part of the lung where there were reported or expected abnormalities. In this woman’s case, she looked at where the reported nodules were. There was a small scar – where the narrow, tree-like branches of the airways stretched and swelled into what was called bronchiectasis. This could certainly go hand in hand with a diagnosis of MAC infection. Then she took another careful look at all the other parts of the chest. In images like these, filled with so much information, a radiologist must examine the images as closely as possible. Nobody can see everything. Maybe artificial intelligence will get there one day. But she tried to see what was there.
As she scrolled to the top of the picture, she saw something that looked a little abnormal. The trachea, the breathing tube that connects the upper airways of the nose and mouth to the lower airways of the lungs, looked oddly narrow near the top. The narrowing was less than a centimeter long before widening to normal diameter. Tsai found the same narrowing in the other scans and reviewed the reports to see what previous radiologists had made of this finding. Neither of them mentioned it, perhaps because it looked like a small puddle of secretions. The key was that it was the same in all three exams. Secretions circulate. This shrinkage, whatever the cause, did not occur. Tsai wasn’t sure what to make of it, but in her report she suggested it might be contributing to the patient’s symptoms.
When Wolfe saw the radiologist’s report, he realized that this narrowing of the trachea could be the cause of all of the patient’s symptoms. How had it happened? She had never needed a breathing tube placed in her trachea during surgery or serious illness – it was the most common cause of this type of unusual finding. Wolfe ordered further tests to look for possible infections or inflammatory causes of the stricture. Not all of them were revealing. It wasn’t MAC or any of the other causes Wolfe could think of or test. Eliminating everything gave her her diagnosis: she had idiopathic subglottic stenosis. Idiopathic meant the cause was unknown. Subglottic identified location in the trachea, just below the vocal cords. It is a rare and poorly understood disorder seen almost exclusively in middle-aged women. Because her shrinkage made her breathless, the shrinkage had to be opened.
Wolfe sent her to a surgeon who used a balloon to expand the narrowed tract. The patient told me that she could feel the difference as soon as she woke up. And within eight months of her surgery, she regained everything she had lost. Within days, she was able to walk up and down the stairs in her hallway once again.
Lisa Sanders, MD, is a contributing editor at the magazine. His latest book is “Diagnosis: Solving the Most Baffling Medical Mysteries”. If you have a solved case to share, email her at [email protected].
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