After surgery for heart condition tetralogy of Fallot, James is all joy

Warriors come in all shapes and sizes. Some even smile. In the Irvine family, the lead warrior is a happy one: 6-month-old James, whose cheeriness hides the difficult medical journey he had in the first few months of life.

“I have been told by many people that James is an inspiration,” says his mom, Heidi. 

Heidi and her husband, Chris, are thrilled their warrior is now healthy. They intend to cherish every second they have with their first child, the big milestones and the little moments. There is always a camera ready to record it all. “He always gives me the biggest smile. It’s the best way to start our days,” Heidi says of her daily “photo shoots” with James.

A complicated delivery and difficult diagnosis

A prenatal ultrasound revealed James had tetralogy of Fallot (ToF), a group of four serious congenital heart defects (CHDs) that limit blood flow to the lungs. He also had pulmonary stenosis, a narrowing of the heart’s pulmonary valve. A cardiologist drew a picture of a normal heart and another of James’ heart. “That’s when we knew it was going to be bad,” Heidi says. “Who wants to hear that about their child? I wanted to run out of the room. It was awful.”

The family was referred to Boston Children’s Maternal Fetal Care Center (MFCC), in affiliation with Brigham and Women’s Hospital. The staff counseled the family on what to expect from a prenatal ToF diagnosis and what care would follow after delivery. After James was born at Brigham and Women’s Hospital, he needed treatment for a collapsed lung. Because of his worsening health, surgery to repair James’ ToF was scheduled at Boston Children’s Benderson Family Heart Center two months after his birth, sooner than initially expected.

In the meantime, he went home, put on weight, and got to know his family and their dog Kodi. Yet, despite the joys of bonding with James, Heidi worried, especially when his skin color turned blue. “I was constantly checking on him and making sure he was breathing.” 

Pushing through heart surgery ‘like a warrior’

During an appointment just a few days before surgery, James’ care team noticed he was having what is known as a tet spell. It’s when regular activities can change pressure in a heart with a serious form of ToF and increase the output of oxygen-poor blood. It can happen when children are bathing, crying, or feeding; a symptom is blueish skin color. James’ cardiologist, Dr. Matan Setton, and cardiac surgeon, Dr. Luis Quinonez, decided surgery couldn’t wait.

“I met Dr. Quinonez after we learned James was being admitted into the Cardiac Intensive Care Unit, where he would receive care before surgery,” Heidi recalls. “I was crying, and he was so sweet. He had a warm, calming presence. He was very informative.” Dr. Quinonez and his team successfully completed a full surgical repair of the four CHDs behind James’ ToF, leading to an emotional meeting afterward with the Irvines. “I gave him a hug,” Heidi says, “and I said, ‘Thank you for saving his life.’” 

Chris and Heidi were elated to see James’ health improve almost immediately after surgery. “He pushed through like a warrior,” she says. “We are so proud of him.” Heidi says she and Chris were also impressed with his care teams. “We knew he was in good hands.”

Days of discovery and smiles

The Irvine family is now enjoying James’ good health. After dizzying months of concern and worry, life feels settled.

When James isn’t posing for photos, he likes listening to music, discovering new touch-and-feel books, and hanging out with Kodi. “James has this way about him,” Heidi says. “He’s just so sweet: the kindness in his eyes and a smile that lights up a room.”

Heidi recently thanked Dr. Quinonez again, this time in a card. She wrote: “Thank you for saving his life. Now we get to hear his first laugh; watch him take his first steps. I didn’t know if we would be given that opportunity, and I’m so grateful we did.”

Learn more about the Benderson Family Heart Center or make an appointment.