After adopting two boys and getting pregnant with another boy in the same year, Kristina and husband Christopher Wessley of Daytona Beach were thrilled to learn they were expecting “their princess.” But at a routine ultrasound at 14 weeks, they got the news no parents-to-be wanted to hear.
“The ultrasound showed that our baby had a large, fluid-filled cyst on the back of her head, called a cystic hygroma, which is usually an indicator that something else is going on,” remembers Kristina. “When we went to our first appointment with a high-risk obstetrician at 18 weeks, we learned our baby would be born with hypoplastic left heart syndrome and had symptoms of a genetic disorder called Turner’s syndrome.”
Hypolastic left heart syndrome is a rare and complex condition in which the chambers and arteries on the left side of the heart are small and underdeveloped, with valves that don't work properly. Since the left side can't pump effectively, the right side of the heart must take on double the work and pump to both the lungs and the rest of the body. In Turner’s syndrome, a female is born with one X chromosome instead of two or she is missing part of one X chromosome. The syndrome usually causes multiple health issues, such as heart defects and thyroid problems.
Throughout the rest of Kristina’s pregnancy, her baby was monitored by pediatric cardiologist Stephanie Lacey, DO, who heads the Fetal Cardiac Care Clinic in Jacksonville. The clinic is a program of the UF Health Pediatric Cardiovascular Center and Wolfson Children’s Hospital in Jacksonville.
On November 15, 2013, baby Emerie was born at Baptist Medical Center Jacksonville and was in Wolfson Children’s Level III NICU for the first 10 days of her life. Her pediatric heart physicians knew they would have to act fast to repair her heart, but traditional surgical repair wasn’t a viable option. “Turner’s syndrome adds significant risk to heart surgery due to swelling,” explains pediatric cardiologist Robert English, MD. “But without intervention, Emerie would likely not survive infancy.”
Dr. English and UF pediatric cardiovascular surgeon Michael Shillingford, MD, decided to try a novel approach: a hybrid procedure in the cardiac cath lab at Wolfson Children’s Hospital.
Knowing the procedure had never been done in Jacksonville before and that there was a possibility Emerie wouldn’t survive it, the Wessleys were understandably anxious. “But you have to have faith and believe it’s going to work,”says Kristina. “We tried our best to stay calm and to trust Emerie’s doctors. We figured God had a plan for her, one that He knows and we don’t.”
On November 25, at Wolfson Children’s Hospital, Dr. Shillingford opened Emerie’s chest surgically to expose the blood vessels of her heart, allowing Dr. English access to the patent ductus arteriosus (PDA).
“We needed to put a stent into the PDA soon after birth in order to provide an alternate pathway to connect lung circulation with the rest of the body’s circulation,” says Dr. English. “This procedure is much more difficult because, unlike in open-heart surgery, we can’t stop the heart’s circulation. And the stent had to be placed in just the right spot, while the heart is still pumping.” Dr. Shillingford also did a banding procedure on Emerie’s pulmonary artery to slow down her blood flow and keep blood from flooding the lungs. Emerie spent seven weeks in the Pediatric Intensive Care Unit at Wolfson Children’s.
“Her heart is working better than they expected,” says Kristina. “Every time they do an echocardiogram, they see heart growth, so the only procedure she may need in the future is one to remove the banding and the stent.”
In the meantime, Emerie is closely monitored 10 minutes from her home, at the Wolfson Children’s Specialty Center in Daytona Beach. Dr. English and his fellow pediatric cardiologists travel from Jacksonville to Daytona to increase access to outpatient specialty care for area kids.
“The new Specialty Center in Daytona is very nice, and it’s really comforting to us that Emerie can be seen by the same doctors she sees in Jacksonville,” says Kristina. “They really care and put their hearts into what they do. We owe them everything.”
Kristina says Emerie, whose name means, “very sweet and loves the Lord,” is thriving at home under the watchful eye of three brothers who adore her.
Dr. English says Emerie still has a long road ahead of her, but is doing well. “She has great parents and she made it through the first big hurdle. This procedure is a good example of how we’re offering the latest options for parents with congenital heart conditions, even those as complex as Emerie’s. Being able to see our patients in their own community is rewarding.”