Pulmonary Division Uses Multidisciplinary Technique to Treat Chronic Lung Disease

Treating chronic respiratory disease in children frequently presents significant tactical problems, particularly when they develop other complicating issues, such as progressive, diffuse bronchial stenosis. To date, it hasn’t been feasible to address this problem endoscopically in children. The smaller airways in pediatric patients don’t pair well with adult-sized endoscopic tools.

Through a multidisciplinary effort, Cincinnati Children’s developed a way to overcome this challenge. Together, pulmonologists, interventional radiologists and otolaryngologists improved an existing technique to potentially enhance outcomes for these patients who typically face a slow pulmonary decline. 

“We must try to open these very narrow airways. In children, there’s no tool designed to do it, and adult tools are too large for kids,” says pediatric pulmonologist Erik Hysinger, MD. “We had to find a way to circumvent that. To do it, we collaborated with interventional radiologists to use some of the really small tools they use in blood vessels to apply to the airways.”

A Patient in Need

Traditionally, most children with granulomatosis with polyangiitis (GPA) and progressive bronchial stenosis are successfully treated with corticosteroids and immunosuppressive therapy. If medical management fails, however, few therapeutic options exist.

That was the case for a 12-year-old male patient referred to Cincinnati Children’s in early 2022.

“This young man was a basketball player who was very active,” Hysinger says. “He began immunosuppressive therapy, but eventually developed severe shortness of breath. He was completely winded when he walked from one side of his house to the other and, eventually, he ended up in a wheelchair because he just couldn’t breathe.”

Pulmonary function testing showed that the patient’s lung function had plummeted from the expected 97% to 37% despite immunosuppressive therapy. A bronchoscopy showed diffuse bronchial stenosis that did not respond to medical management. A lung transplant was the next—and last—option. But that wasn’t feasible.

“I reached out to my colleagues in lung transplant, but it wasn’t going to work. His underlying autoimmune problem wasn’t fixed, so it would probably cause the same issue with new lungs,” he says. “Other than that, all we could offer was additional breathing support, recognizing that he eventually would succumb to his illness.” 

That outcome wasn’t acceptable to Hysinger and his colleagues. 

Developing an Answer

A balloon dilation to open the bronchial airways is typically the solution to this problem. However, the patient’s anatomy was too small to accommodate the endoscopic tools required to complete the job. It was an obstacle that seemed insurmountable.

Finding a workable answer would require creativity and ingenuity from Hysinger and his multidisciplinary team.

He reached out to Jason Woods, PhD, director of the Center for Pulmonary Imaging Research, and John Racadio, MD, director of interventional radiology, research and innovation. Together with Nicole Hilvert, RT, program manager of the Cincinnati Children’s hybrid operating room, and Carolyn Wallace, lead respiratory therapist for bronchoscopy, they brainstormed and developed a novel technique to treat pediatric bronchial stenosis.

The first step in the process was to identify which airways could be recaptured for improved lung function, Woods says. To do so, they took an innovative approach. They tracked inhaled hyperpolarized xenon with imaging to follow its path through the lungs, revealing areas that were primed for potential ventilation. 

“Xenon is a noble gas that doesn’t otherwise exist in the body, so we were able to follow it with MRI [magnetic resonance imaging],” he says. “We quickly visualized within 10 to 15 seconds where the gas traveled. That revealed which segments or subsegments of the lungs were ventilating well or poorly. In fact, we identified one airway where we could recapture nearly 25 percent of his lung function.”

Based on imaging results, the team pinpointed their targets. They then merged existing strategies—imaging guidance, biopsy needles, and angioplasty catheters and balloons—for their next step and tested it on a model in their hybrid operating room, Racadio says. The results were excellent.

To reach airways completely occluded due to scar tissue, the team used X-ray imaging to guide a transbronchial biopsy needle to the spot where it could most easily break through the blockage. They advanced an angiography guidewire through the biopsy needle, threaded the angioplasty balloon over the wire, and then inflated it to open the airway. Once the airway was patent, they injected steroids and performed cryotherapy to keep the lumen open. 

“We combined our catheters and guidewires to see what would fit inside the different-sized scopes. We had to figure out what was physically possible to reach the airways,” he says. “Getting to branches that were totally blocked off was something that had not even been previously considered possible.”

Initially, the patient had a repeat balloon dilation every week. Since February, he’s undergone 13 procedures, but they’re now spaced out to one a month.

The best news, Hysinger says, is that he’s back to playing basketball with his friends.

The Benefit of Multidisciplinary Partnerships

Multispecialty partnerships always have the potential to offer patients more advanced care options and better outcomes. In this case, though, the interdisciplinary approach conquered a significant industry shortcoming, Wallace says.

“One of our biggest issues is size. Interventional pulmonary techniques in the adult world are easier because the tools are larger. But our average patient is 2 years old, and manufacturers don’t make a lot of pediatric tools,” she says. “We had to get creative to adapt something made for a 50-year-old to work for a patient with airways less than half that size.”

Creating these types of teams is part of how Cincinnati Children’s is revolutionizing patient care, Woods says.

“The clinical-research interaction is part of what makes us unique. There’s a real push and responsibility for clinical translation to make research relevant for our patients. This novel technique is a direct demonstration of that applicability,” he says. “Our research, translation and clinical interaction are unparalleled. There’s no better place in the entire world to be a pediatric lung patient right now.”