Nothing can prepare you for the sight of your newborn baby lying limp with tubes and wires coming from an open incision in her chest. No one knows this better than Kaitlin Porter and Joel Breen of Lochaber, Nova Scotia, near Antigonish. Kaitlin and Joel’s second daughter, Everly, was born with several serious congenital defects of the heart and required emergency lifesaving surgery at the IWK Health Centre just days after her birth.
“She was doing so well her first day,” Kaitlin recalls. “She was breastfeeding, alert and bright, but the next day when I went to the neonatal intensive care unit to feed her, she was fussy and lethargic. I got a call just before midnight that night that she would have to have surgery the next day, if she made it through the night. And my whole world came crumbling down.”
This was not the first time Kaitlin and Joel and their families had felt the shock of some very unexpected bad news. They faced the same kind of terror the day after the 20-week prenatal ultrasound that revealed some of the problems with Everly’s very special heart.
“I got a call from our family doctor shortly after the ultrasound, asking us to come in to discuss the results the next day,” Kaitlin says. “My mind was racing, I couldn’t imagine what the doctor would have to say. The next day, she told us there was something wrong with the baby’s heart, and my world came crashing down.”
Two weeks later, Kaitlin and Joel made the trip to the IWK for a more detailed examination of the baby’s heart using ECHO technology. As the cardiologist, Dr. Santokh Dhillon, described the defects in Everly’s heart, the couple became increasingly dismayed.
First they learned that the baby’s aorta and pulmonary artery were each connecting to the wrong ventricle, a condition known as transposition of the great arteries that interferes with proper blood flow. There was also a ventricular-septal defect, which meant that part of the wall of tissue that separates the two lower chambers of the heart was missing, making one single chamber.
A second ECHO, at 32 weeks, found another problem. One of the valves in the heart was not working properly. If not repaired once the baby was born, the leaking valve would force the heart to work too hard and lead to heart failure.
It was a seemingly bleak scenario, but Dr. Dhillon reassured the parents that surgery would correct the problems.
At 37 weeks, Kaitlin re-located to Halifax to be close to the IWK “just in case.” She went into labour the next day and Everly was born by planned caesarean section. Two days later Everly had her first surgery, an emergency procedure to correct a newly diagnosed problem called PDA (patent ductus arteriosus). This is when the hole in the aorta that is there during pregnancy to allow the baby’s blood to skip the trip to the lungs for oxygen does not close quickly enough after the birth. Because Everly’s heart was already compromised in other ways, the PDA was very dangerous for her.
Fortunately for Everly, her parents, her big sister Ashlyn, grandparents and extended family, the procedure went perfectly. Everly is doing well and her doctors are now watching and waiting to give her as much time as possible to grow and get stronger before she needs the big operation to correct the transposition of the great arteries. This may also correct the leaking valve. In the meantime, Everly is on medication for high blood pressure and heart failure while she is waiting for the next operation.
“It’s a shock to encounter such serious problems with a baby, especially when our first child is so healthy,” Kaitlin says. “We’ve been surprised to learn that one in 100 babies is born with a congenital heart defect.”
Kaitlin and Joel are now working to raise awareness of congenital heart disease as well as money for heart research. They’ve thrown their support behind Dalhousie Medical Research Foundation’s 2019 Spring Molly Appeal, which is raising money to support a biobank that Dalhousie medical researchers will use to learn more about heart failure and other forms of heart disease.
“We will store clinical information and tissue samples from heart failure patients in the biobank,” says Dr. Thomas Pulinilkunnil, a researcher at Dalhousie Medicine New Brunswick who studies metabolic disorders and heart disease. “Information we collect about patients with congenital heart defects can help us create zebrafish models of infant heart failure, which can be used to learn more about heart failure in infants and how best to control it.”
No cause is known for the kind of heart defects that Everly was born with—although thankfully research has led to successful surgical methods of repairing them. “Everly’s heart was the size of a walnut when the first surgery was done,” Kaitlin says. “Heart disease and heart failure are not just diseases of people over 60, they affect people of all ages. We’re happy to support the Molly Appeal in helping researchers in the Maritimes lead the way to new treatments and cures.”