According to Valerie, 17-month-old Addison doesn’t like to stick to plans. She does things her own way, even when it throws her parents — and her doctors — for a loop. But that tenacity of spirit has kept Addison, born with multiple heart defects, defying the odds, even when they’re stacked against her.
Valerie and her wife Tegan were at their first sonogram when their N.C. obstetrician, detecting a too-low heart rate, informed the parents that their unborn baby might not survive. Over the next two weeks, Addison tripled in size, and her heart rate normalized. By the time Tegan was 18 weeks pregnant, Addison appeared to be healthy and developing normally.
Because Tegan has diabetes, however, the family was referred to a specialist in maternal-fetal medicine. Addison’s heart issues then became apparent. Suspecting a ventricular septal defect (VSD) — an abnormal opening between the heart’s lower chambers — the obstetrician referred the family to a pediatric cardiologist for a fetal echocardiogram. Over the next several weeks, Addison was diagnosed with multiple VSDs and complete heart block, a condition in which the electrical signal that controls the heartbeat is blocked from reaching the ventricles. Once again, Valerie and Tegan were told that their daughter’s survival was unlikely.
‘All I felt was fear.’
In May 2019, Tegan began to have contractions. She was only 29 weeks pregnant, and she had preeclampsia. The couple had planned to deliver in their local children’s hospital, where Addison would be transferred to the operating room for artificial heart pacing immediately after she was born. With the baby’s heart rate barely detectable, however, Tegan was rushed into an emergency C-section.
“It was a blur,” says Valerie, recalling that Addison — at a mere 4 pounds — was intubated immediately after delivery. “Her little hands were purple. The only thing I remember feeling is fear.”
Within an hour after her birth, Addison was transported to the nearby children’s hospital where a temporary pacemaker was placed, in hopes of giving the baby time to grow large enough for a permanent replacement.
In the meantime, Valerie’s time was split between the two hospitals where her wife and daughter were recovering. Gradually, she began to learn more about her daughter’s condition. Addison was diagnosed with heterotaxy syndrome, a condition in which internal organs are abnormally arranged; double outlet right ventricle; unbalanced complete atrioventricular canal defect with a small or hypoplastic left ventricle (a variant of hypoplastic left heart syndrome); and congenital complete heart block.
Temporary pacemakers are typically expected to last approximately two weeks, and Addison’s doctors feared she would still be too small for a permanent pacemaker. Ever willful, Addison lasted 53 days on the temporary pacemaker, surviving her second operation, during which a permanent pacemaker was inserted.
Invested in patient care
By the time Addison was transferred to Children’s Hospital of Philadelphia (CHOP), she had spent over 100 days in her home hospital. At CHOP’s Cardiac Center, she underwent a full array of new tests, as CHOP’s world-leading cardiologists and surgeons — including Therese M. Giglia, MD; Jonathan M. Chen, MD; Jonathan J. Rome, MD; and Ari Gartenberg, MD — spent countless hours developing a plan that would give the family the best series of options and give Addison the best possible outcome.
“It was a whirlwind,” remembers Valerie. “We learned so much about her heart. The doctors were amazing — you could feel how invested they were in her care.”
When Addison was ready for discharge, the family returned home with clear next steps. They recently returned to the Cardiac Center for a sedated CT and lung perfusion scan, the outcome of which will determine Addison’s surgical path. She will most likely require multiple operations, and the Cardiac Center will continue to coordinate her ongoing, complex care across multiple specialties, including General Surgery, Pulmonary, Gastroenterology and Ear, Nose and Throat.
In the meantime, the 17-month-old remains undeterred by her heart condition. A happy, sassy child, Addison is crawling, chatting up a storm and seems determined to soon walk.
Though her future remains uncertain, Valerie and Tegan trust CHOP with their daughter’s care. “We’re involved in the plan, and we know what’s happening every step of the way,” says Valerie.
“CHOP has given us hope.”