Born in New York with hypoplastic left heart syndrome (HLHS), coarctation of the aorta and tricuspid regurgitation, James underwent three open heart surgeries by the time he was 4 years old. After the Fontan operation – the last procedure in staged reconstruction heart surgery – James began to experience a buildup of fluid (effusion) between the thin membranes surrounding the lungs and chest cavity (called the pleura). James was readmitted to the family’s New York hospital, where he was ultimately diagnosed with chylothorax, a rare lymphatic disorder.

Chylothorax is a condition in which chyle – a fluid made of lymph and fat that is produced in the small intestine during digestion – leaks into the space between the lungs and chest. Chylothorax can cause a severe cough, chest pain and difficulty breathing. James’ doctors placed chest tubes to drain the fluid and put James on a low-fat diet to reduce the production of chyle. But when viral season began, the 4-year-old suffered from a series of viruses that left him with a chronic cough and low oxygen levels.

On Thanksgiving morning, James was readmitted to the hospital with multiple viruses, including RSV. His pleural effusion had returned, and he was swelling with lymphatic fluid. Although his New York medical team treated his viral symptoms, they knew it was time for James to be transferred to a hospital with lymphatic expertise. “They said CHOP’s program was the best,” says James’ dad, Michael. 

Experts in lymphatic disorders

James was transferred by ambulance to Children’s Hospital of Philadelphia (CHOP), where he was cared for by experts in the Jill and Mark Fishman Center for Lymphatic Disorders, an international leader in the treatment of rare lymphatic disorders. The first of its kind to manage these chronic conditions, CHOP’s Center for Lymphatic Disorders provides highly specialized care for children and adults with lymphatic leaks and flow disorders.

The team determined that James needed a percutaneous lymphatic intervention, a minimally invasive, image-guided procedure designed to seal leaking lymphatic vessels and stop lymphatic fluid from spilling into the chest cavity. 

“They told us that James would be the youngest patient to undergo thoracic duct decompression,” says Casey. “It was scary, but we were willing to do whatever we needed to in order to help him.”

First, however, the team needed to address the amount of fluid currently built up in James’ chest cavity. Repeated pleural effusions had left thick pockets of fluid trapped around James’ lungs. Doctors had to administer a clot-dissolving medication through James’ chest tubes to break up the tissue and allow the fluid to drain. 

James spent two weeks at CHOP before he was finally healthy enough to undergo lymphatic imaging and intervention – a CHOP-developed imaging technique called dynamic contrast MR lymphangiography (DCMRL), in which a special dye, or MRI contrast agent, is injected directly into the lymphatic system, providing clear images of its anatomy and flow so lymphatic interventionalists can find and seal the leaks.

The DCMRL and intervention went well; however, James’ heart and breathing became unstable overnight. His team discovered that blood clots had formed throughout his body, including in his Fontan conduit – a pathway created during heart surgery to help blood flow to his lungs. This blockage kept his heart from circulating blood effectively. 

“We were told there was a good chance he wouldn’t survive,” says Michael.

James was swiftly transferred to the Cardiac Catheterization Laboratory, where a cardiac interventionalist was thankfully able to remove the clots, saving James’ life. The little boy was placed on ECMO to allow his lungs to fully rest while the remaining fluid drained.

A little ‘trooper’

On Jan. 2, James was finally able to undergo a thoracic duct decompression. He recovered quickly, and after rehab, was able to be discharged at the end of the month, which was sooner than expected. Although he needed a nasogastric (NG) tube to supplement fluids and extra calories, James was also able to eat solid food. He was placed on a blood-thinner, which he will need for life to prevent future clots. 

“He was such a trooper through all of this,” says Casey. His family decided to celebrate his return home with a trip to Disney World. 

“We had a magical time,” Casey remembers. “After all he had been through, there was nothing better than seeing him light up.”

James was doing so well at his follow-up visits, that doctors reduced his medication and diuretics. However, James soon came down with a severe stomach bug that threw off his fluid balance. James became so sick that he was admitted to the New York hospital and ultimately life-flighted back to CHOP, where he was placed on round-the-clock diuretics to restore fluid balance. 

After discharge, James underwent another cardiac catheterization to assess the condition of his heart. Unfortunately, his tricuspid regurgitation had grown so severe that doctors determined no further surgical repair was possible, and James will eventually need a new heart.

Now 6, James will soon begin the transplant evaluation process. In the meantime, he continues to take the diuretic and blood thinner; he’s able to eat without the NG tube and is finally gaining weight. In the fall, he’ll start first grade.

Every day, his parents remind him that he is “smart, courageous and confident.” He is a good friend and a good big brother to his sister, Sofia. 

“He’s living life to the fullest,” says Casey. “He’s our daily reminder not to take a single moment for granted.”