Plastic Bronchitis: Matthew’s Story
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Matthew's parents thought the worst was behind them. Their son was born with a complex heart problem and had undergone four open heart surgeries to reconfigure his heart and circulatory system. But when Matthew was 6 years old, he developed symptoms of pneumonia, and while at a local hospital he coughed up a rubbery, caulk-like plug called a cast, a sign lymphatic fluid was leaking inside his lungs. He needed expert care — and his family knew just where to turn: Children's Hospital of Philadelphia (CHOP).
Matthew was diagnosed with plastic bronchitis, a lymphatic flow disorder that causes severe respiratory issues. In children with plastic bronchitis, lymphatic fluid builds up in the airways and forms casts which block the airways, making it difficult to breathe.
Though Matthew and his parents, Marie and Michael, live in Connecticut, and his doctors were located in New York City, everyone agreed Matthew would get the expert care he needed at the Jill and Mark Fishman Center for Lymphatic Disorders at CHOP. The center has treated hundreds of adults and children with lymphatic leaks and lymphatic flow disorders, making it the largest and most active treatment program of its kind in the world.
Early intervention
The family had traveled to CHOP twice before to consult with Jack Rychik, MD, Director of the Fetal Heart Program and the Single Ventricle Monitoring Clinic, part of the Fontan Rehabilitation, Wellness, Activity and Resilience Development (FORWARD) Program, and Kathryn M. Dodds, MSN, CRNP, RN.
Matthew was born in 2008 with a single ventricle heart defect, a complex heart problem in which one of the two pumping chambers isn’t strong enough to pump effectively. He had the first two surgeries of staged reconstruction, a series of three procedures to reconfigure the heart and circulatory system, in a New York City hospital. But Matthew's heart pressures were too high when it was time for the third operation, called the Fontan procedure. His family consulted with other pediatric cardiology specialists and brought Matthew, then 3 years old, to CHOP.
Dr. Rychik recommended adding two medications to help his pressures improve, and Matthew was able to have his Fontan as scheduled.
Three years later, when Matthew was 6, his health declined. His skin had a blueish hue and his legs were weak. Marie and Michael consulted with the team at CHOP for another second opinion. This time the recommendation was to revise his most recent heart surgery.
Lymphatic system treatment
Four months after Matthew's second Fontan procedure — and his fourth open heart surgery — Matthew was diagnosed with plastic bronchitis at his hospital in New York City.
At CHOP, Dr. Rychik and Kathryn Dodds coordinated Matthew’s care with Yoav Dori, MD, PhD, Director of the Jill and Mark Fishman Center for Lymphatic Disorders. The team initially recommended treating Matthew’s plastic bronchitis with medication and a nebulizer, and collaborated with Matthew’s medical team in New York to ensure continuity of care.
“If it wasn’t for CHOP, I don’t know what we would have done," Marie says.
“Dr. Rychik and Katie Dodds are incredible and supportive.”
An innovative procedure
Three years later, Matthew was admitted to the hospital with increased casts, and his family learned he was a good candidate for a procedure — pioneered by Dr. Dori — to locate and close leaks in the lymphatic system.
The center uses dynamic contrast MR lymphangiography (DCMRL), an advanced lymphatic imaging test, to map out the anatomy of the central lymphatic system and determine the location of the leaks. Doctors then perform a minimally invasive procedure to reach the sources of the leaks and seal them with a special glue. When the leaks are closed, the lymphatic system responds by opening up new pathways for the lymphatic fluid to return back into the venous system.
Matthew was successfully treated, and hasn’t had any major medical complications since the procedure. He has continued to grow stronger, and his health has improved to the point where all respiratory medications and treatments are no longer needed.
“We are so extremely grateful to Dr. Dori and that this treatment is available. I am excited that there are people like him who are so brilliant, thinking outside the box, and looking for solutions.”
Now 10, Matthew is a very active and outgoing boy. His passion is sports, both watching and playing. Matthew and his family are active in fundraising for cardiac research and are strong advocates for raising awareness about congenital heart defects. One day, Matthew hopes to help even more children like himself.
“I want to be a doctor when I grow up. Maybe do procedures like Dr. Dori,” says Matthew.
