He Had Trouble Breathing, and Inhalers Didn’t Help. What Was Going On?

Plastic bronchitis is most common in children who have surgery to correct certain structural problems in the heart. But it can also be seen in adults, either after heart surgery or in a handful of disease processes, particularly inflammatory diseases of the lungs, like cystic fibrosis or severe allergic reactions.

When Sims reported her findings to her friend, she acknowledged that she wasn’t sure what caused his plastic bronchitis — if that’s what it was. She’d heard about a doctor at the University of Pennsylvania, in Philadelphia, who was familiar with the rare disease. He should see Dr. John Hansen-Flaschen. He might be the doctor who could finally figure this out.

Hansen-Flaschen is an emeritus professor of medicine at Penn, which means he is years past the usual retirement age. He primarily sees patients with diseases that others have not been able to diagnose. The doctor spent two hours with the man, getting every detail of his story. On examination, the patient was wheezing, and at the base of each lung, he could hear a crackling noise, like the sound made when Bubble Wrap is crushed. Hansen-Flaschen, like Sims, thought that the man probably did have plastic bronchitis. The question was why. He had never had surgery on his heart. And he didn’t really fit the pattern for many of the other diseases. It sounded as if he’d had this all his life.

Hansen-Flaschen postulated that the man could have been born with some abnormality in his lymphatic system — a narrowing or blockage that caused the lymph fluid to back up and overflow into his lungs. He had seen this disorder only a few times, but he had been fascinated by these patients and by the handful of other similar cases he’d read about.

Hansen-Flaschen knew an interventional radiologist at Penn, Maxim Itkin, who had developed a way to visualize the lymphatic system. By injecting contrast dye into the lymph vessels, he would be able to see any leaks or blockages in the system.

The patient went to see Itkin a few weeks later. Using an ultrasound probe, the doctor located the lymph nodes in the man’s groin. He then injected contrast dye into the nodes. He used a type of X-ray known as fluoroscopy to follow the dyed lymph as it traveled through the abdomen into the chest. Sure enough, he could see the fluid leak into the lungs. And at the very top of the lymphatic system in the chest, where the contents were supposed to be flushed into the blood stream, the vessel was narrowed and nearly completely obstructed.

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