Plastic Bronchitis and Lymphatic Intervention: Jameson's Story

Jameson Finley’s parents thought the difficult times were finally behind them. Jameson had undergone the Fontan procedure, the last of the three open heart surgeries required to treat hypoplastic left heart syndrome, at a hospital near his home in Texas shortly before his fourth birthday.

Jameson Patient Story Photo After a challenging recovery, he was finally home – and his parents, Laura-Marie and Todd, were hopeful that his health would continue to improve. 

But a little over a year later, in November 2012, Jameson came down with pneumonia – twice.

“At that point, my ‘mommy brain’ kicked in and I thought, ‘This isn’t normal,’” says Laura-Marie.

Looking for a diagnosis: Plastic bronchitis?

Desperate for information that might help her son, Laura-Marie spent hours online, poring over medical research. She soon found information about plastic bronchitis, a serious disorder that occurs in 5 to 10 percent of patients following the Fontan surgery.

Plastic bronchitis occurs when lymphatic fluid and inflammatory cells leak into the airway and harden into large, rubbery “casts” that block the airway and cause severe breathing problems. If children are not able to cough out the casts, they can suffocate (asphyxiate).

A few weeks later, Jameson began coughing up pieces of mucus that resembled the casts Laura-Marie had seen online – and the Finleys’ search for answers intensified.

Searching for answers

The Finleys soon came across published work by Maxim Itkin, MD.

Intrigued, the Finleys emailed Dr. Itkin. Less than an hour later, he wrote back, encouraging the Finleys to contact him with additional questions.

we were shocked. not only was it a response, it was a response on a sunday evening at 10 p.m. it was like a small blessing come true.

The next day, the cardiologist at the Finleys’ home hospital diagnosed Jameson with plastic bronchitis and started him on breathing treatments to ease his symptoms.

While researching their son’s options, the Finleys spoke by phone with Dr. Itkin and his collaborators Yoav Dori, MD, PhD, an interventional cardiologist at The Children’s Hospital of Philadelphia (CHOP), and Marc Keller, MD, an interventional radiologist at CHOP. The three doctors had been working to develop new imaging and lymphatic interventional procedures that would help them better understand and solve lymphatic-related complications in children with heart disease. Insights from their work had led them to the discovery of a lymphatic abnormality in patients with plastic bronchitis – and to a new potential treatment for patients like Jameson.

Several weeks later, the Finleys were on their way to Philadelphia.

Finding hope at Children's Hospital of Philadelphia

At CHOP, Jameson underwent a series of advanced lymphatic imaging tests, including dynamic contrast MR lymphangiography, a test developed by Drs. Itkin and Dori. The tests allowed the clinicians to “map” the anatomy of Jameson’s lymphatic system and his lymphatic flow, and were the key to discovering how lymphatic fluid was leaking into his airway.

Laura-Marie and Todd also met with Drs. Itkin, Dori and Keller, as well as Jack Rychik, MD, director of CHOP’s Single Ventricle Survivorship Clinic, part of the Fontan Rehabilitation, Wellness, Activity and Resilience Development (FORWARD) Program, to discuss Jameson’s options, which included a trial of medical therapy, a heart transplant or surgical thoracic duct ligation.

They also discussed minimally invasive selective lymphatic embolization, a new treatment, pioneered at CHOP, that is more precise and targeted than a thoracic duct embolization or ligation. In a selective lymphatic embolization, doctors use bonding agents, inserted through a tiny tube (catheter), to seal leaks in the lymphatic system. Because lymphangiography allows clinicians to identify the source of the lymphatic leak, they can target their intervention to the affected area. This helps preserve the thoracic duct – an approach that experts at CHOP believe is important to the procedure’s success.

Hoping to spare their son additional hospital stays – “he had already been through so much,” says Todd – the Finleys decided to continue with medical therapy for the time being. The CHOP team adjusted Jameson’s medication regimen, and the Finleys returned to Texas. 

But over the next several months, Jameson’s condition worsened. “His casts were getting bigger and more frequent,” says Laura-Marie. “They looked like a huge tree, with a trunk and branches.” By summertime, he was coughing up casts, many of which were larger than a half-dollar, as often as three or four times a day.

“He was so tired and sick. He could barely make it 50 yards without one of us holding him,” says Laura-Marie. “One night while he was getting his breathing treatment, he looked at me and said, ‘I’m ready to go to Philadelphia and get better.’ It was heartbreaking.”

It was time, the Finleys agreed, to return to CHOP.

A life-changing procedure

On August 7, 2013, Jameson underwent minimally invasive selective lymphatic embolization in a special catheterization suite in CHOP’s Cardiac Operative and Imaging Complex. The procedure went well – and just three days later, he and his family were on their way home. In the weeks after the procedure, Jameson was able to go off his respiratory medications and breathing treatments.

he's been asymptomatic ever since. he's playing soccer. he can run. it's just been amazing.

Jameson continues to see his cardiologist in Texas, who works with the team at CHOP to manage his care. He also comes to CHOP once a year for follow-up with Dr. Rychik and the Single Ventricle Survivorship Clinic and Dr. Dori and other clinicians from the Hospital’s Jill and Mark Fishman Center for Lymphatic Disorders.

Originally posted: November 2014

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