After a lifetime of severe asthma and allergies, this felt different -- and far worse.
Cars sped past the 51-year-old man as he trundled along the grass-flanked highway outside Peekskill, N.Y., on his industrial-size riding tractor-mower. The early-summer sun beat down, and sweat stung his eyes. The air was dense with fragments of cut grass that dotted his clothes and face. Suddenly he couldn't breathe. There was just no room in his chest for a new breath, because the old air wouldn't come out. Panic seized him. He tightened the muscles in his stomach and chest to carefully push the used air out. He felt as if he were blowing through a narrow, crimped straw. Too much pressure only made it worse. He had allergies and bad asthma and worried that something he'd mowed was triggering everything. He forced himself to breathe slowly and struggled to calm his racing heart.
"Something bad happened," he told his wife when he reached her on his phone. She could hear the suppressed fear in his voice. "Did you use your inhaler?" she asked, her voice deliberately calm. In his panic, he hadn't. He did now, but it didn't do much, and he struggled to get the mower back to the storage facility. As soon as he got home, he called his pulmonologist. It was probably his asthma, the doctor told him in his office the next day and started him on a five-day course of prednisone and an antibiotic. When that didn't help, he tried another round. When that failed, the man decided to look for answers beyond his allergies and asthma. He had these problems his entire life but never felt like this before. This, he knew, was different.
Whistle on the Exhale
He saw his primary-care doctor. Although he still felt short of breath, the man's oxygen level was normal. He wasn't wheezing, so it wasn't his asthma. Was it his heart? He was at an age when the risk of heart disease begins to climb. And esophageal spasm, from reflux, can cause a feeling of chest tightness often mistaken for a heart attack. But the endoscopy to check for spasm was normal. Not reflux. His new cardiologist ordered an EKG, an echocardiogram and a stress test. Normal, normal, normal. When the cardiologist called to tell the patient the results, he asked if he had ever seen an ear, nose and throat specialist.
It was mid-December by the time he got in to see an E.N.T. in Poughkeepsie. Hearing the man's story, the doctor asked him to exhale forcefully. The air whistled as it came out. "I'd like to look at your airway," the doctor told him. The patient agreed and the E.N.T. placed a slender tube into his throat, then watched the images as the scope advanced. The doctor didn't reveal a diagnosis but recommended he see a surgeon who specialized in disorders of the trachea.
The patient drove to Lenox Hill Hospital in New York City in mid-February to meet Dr. Matthew Inra, a thoracic surgeon who specialized in the treatment of diseases of the lungs and trachea. Inra explained to the man that the E.N.T. was looking for some kind of mass in his trachea, a cancer perhaps, that was blocking his airways, giving him this sense of constricted breathing. That doctor was able to see as far as the vocal cords, which separate the mouth and throat from the trachea. Although he couldn't really see the windpipe, he suspected there was some kind of obstruction farther down.
Inra asked the patient to breathe in and out as hard as he could. The breath in was normal; the breath out had a sustained musical tone -- like a whistle. The trachea is a rigid tube, kept open and stable by C-shaped rings of cartilage stacked one on top of another. Normally, breath moves smoothly and quietly through this tube. A fixed obstruction would disrupt air flow and cause a noise when the air moved either way. But this patient only made noise when he breathed out, suggesting an intermittent obstruction. Inra suspected that the patient might have tracheobronchomalacia -- a weakness in the cartilage that allows the trachea to move or even collapse with heavy breathing.
Tracheobronchomalacia (TBM) is uncommon, most often seen in infants whose cartilage has not completely formed and is frequently outgrown by age 2. It's unclear how common TBM may be in the adult population. Studies suggest that 13 to 27 percent of those with chronic obstructive airway diseases such as emphysema or asthma develop this disorder. The thinking is that the inflammation associated with these diseases causes the breakdown of the cartilage rings. Without these supporting structures, the windpipes move with every breath. This loss of structure causes frequent respiratory infections. It can cause a whistling noise during breathing known as stridor. When severe, TBM can completely obstruct the airway, especially during forceful expiration, as it had in this man.
If his windpipe collapsed so that 90 percent of the opening was blocked, the patient would need surgery. To find this out, Inra explained, they would need to see his windpipe from the inside and while he was awake. It was an uncomfortable procedure, but the information it provided would give the answers they sought. The man agreed. He would do anything to find out how to get his breath back. "Whatever it takes, doc."
A Brief Gagging Sensation
He lay on his back on an exam table. Lidocaine was sprayed into his nose and mouth and after a few minutes he could feel nothing. A thin flexible scope was inserted through his nose and down his throat. A nurse stood next to him, giving him a play by play of what was going on. When it gets to the back of your throat, she told him, you will feel like gagging, but as soon as they get past that spot, the feeling will stop. Sure enough the man felt an overwhelming gagging sensation -- but it lasted only seconds. Inra skillfully advanced the camera through the vocal cords and into the trachea. The top of the trachea looked normal, with the ribs of cartilage clearly visible beneath the taut tissue. But as he moved the camera farther down the windpipe, the ribbing became smooth, less well-defined, as if it were melting into the surrounding tissue. By the time he got to the point where the one tube divided into two, to bring air to each lung, the cylinder had lost its precise ovoid shape and shifted with each breath.
Take a deep breath and hold it, Inra told the patient. Now breathe out as hard as you can. In a forced exhalation, the pressure outside the windpipe is greater than that within the tube. Normally the cartilage would hold the airway open. But his airway was far from normal. Inra watched as the man tried to force his air out. Almost immediately, the trachea snapped shut around the scope, trapping all the air inside his lungs. Inra moved the monitor so the patient could see the images, then had him exhale again. As he worked to do so, he saw what he was up against -- his windpipe closed completely. It was tracheobronchomalacia, as severe as Inra had ever seen it.
Six weeks later the man returned to Lenox Hill for his surgery. It was to be done using a robot so that the surgery could be performed through the smallest incisions, between the upper ribs. The trachea and the tubes leading into the lungs, known as bronchi, were stabilized by sewing them onto a mesh that is secured to the surrounding tissues. Once everything was sewn into place, the patient was awakened. As the surgeons watched, the patient was instructed to exhale forcefully. This would be the moment of truth. He took a deep breath and then forced the air out as hard as he could. The windpipe and bronchi remained in place; his breath moved unobstructed through the wide-open airway.
Recovery was tough, but within weeks the man was back to an almost-forgotten level of activity. He hadn't even noticed how much his life had slowed down even before that morning outside Peekskill. The patient is certain that he has had this problem since birth. He had severe asthma that laid him flat for weeks every fall his entire life. Was it possible that it wasn't really asthma but TBM? His surgeon isn't sure. Some children born with TBM can be undiagnosed until adulthood. It happens, but it's rare. The patient wonders whether it's truly rare -- or just rarely diagnosed.