Hypoplastic Left Heart Syndrome (HLHS)
For many expecting moms, the 20-week check up is an exciting time. Getting to see your baby (sometimes for the first time) and perhaps even learning whether it will be a little boy or girl are the usual expectations. However, for Iliana Lopez, her 20-week appointment was anything but normal. The 2020 COVID-19 pandemic had just hit and Iliana answered questions about her family’s medical history alone since her partner was not allowed in the appointment. Unsure of why she was being asked these questions, she was soon told that her baby looked to have some heart defects. After a referral to Stanford for a follow-up ultrasound, it was confirmed that her baby had Hypoplastic Left Heart Syndrome (HLHS), a Congenital Heart Defect that occurs when part of the heart does not develop correctly as it should during the first 8 weeks of pregnancy.
“I remember going back to my car and just crying because I didn’t understand why any of this was happening,” Iliana recalls. “I just knew we had a tough road ahead of us.”
Her prediction proved true. The next 17 weeks included weekly check-ups and revolved around the fact that Iliana’s baby would need heart surgery within days of birth. Iliana and her partner were encouraged to move closer to Stanford Children’s Hospital, they found an apartment and packed their car. Then, on moving day, Iliana went in for her regular check-up where doctors discovered that her blood pressure was high and that she would need to be hospitalized. A few days later, Emma was born at five pounds and with declining blood pressure, Iliana’s fragile daughter was rushed away to the NICU and at just three days old, Emma had her first surgery.
Doctors performed the Norwood Procedure, a type of open-heart surgery for babies born with hypoplastic left heart syndrome that lets the right ventricle pump blood to the body. The surgery took about nine hours and shortly after surgery, Emma began having seizures due to some bleeding in the brain. Emma and her family spent the first four weeks of her life in the Cardiovascular Intensive Care Unit (CVICU). When Emma was just shy of a month old, Iliana held her precious daughter for the first time. They were finally planning to go home, when Emma began having some strange breathing. Another side effect of the surgery, doctors discovered her airway was closing and Emma was intubated for another three weeks. Finally, Emma and family came home in November 2020.
“I was so nervous to bring her home because she was high risk and everything was new to me,” Iliana recalls. “But as we were being discharged, we were told an agency called Coastal Kids Home Care would be providing an in-home nurse to support us. I remember still being nervous but feeling a sense of relief because it made me feel better knowing I wasn’t going to do this alone.”
Upon arriving at home, Iliana was contacted by Coastal Kids nurse, Sarah, who explained she would be coming every week and that she was there to support mom in any way she needed. Sarah visited Emma each week until her second heart surgery (which was rescheduled five times due to limited ICU beds) was completed. At this time, Sarah went out on maternity leave and nurse Ivanna was introduced to Emma and her family. According to mom, “Even when Sarah came back, I requested to have both because they both went above and beyond for us and Emma loved them equally.”
In March 2021, doctors performed the Glenn Procedure, a type of open-heart surgery for babies who are 4–6 months old that sends blood from the upper body directly to the lungs. This way, the single ventricle only has to pump blood to the body (and not to the lungs), so it doesn’t have to work as hard. Although the procedure seemed to go well and Emma was home within a week, her mom soon noticed that Emma’s head was getting bigger. Doctors discovered that Emma had fluid in her brain and scheduled a surgery. In May 2021, Emma went in for her third surgery, this time for a ventriculoperitoneal (VP) shunt, a thin plastic tube that helps drain extra cerebrospinal fluid (CSF) from the brain. Although a quick surgery that seemed to be another success, Emma began throwing up and having seizures. Doctors realized the VP was draining too much too fast and Emma was kept in the hospital for another two weeks.
“Each time we have gone home from the hospital it feels so scary,” Iliana explains. “But then I feel a comfort in my heart knowing that Coastal Kids will be there again. We have been in and out of the hospital but each time our nurses would go out of their way to text to see how Emma was doing. It makes you feel good to know that you come home to a team that cares for your daughter and that if you need something they will be there–nurses, office staff, whoever.”
Emma is now 18 months old, stable and developing a sweet personality. She will need future surgeries, but for now she is just enjoying being a little girl who loves to dance with her dad and go for walks with her mom. She still receives weekly visits from her Coastal Kids nurses and has recently started massage and music therapy. According to mom, Emma loves the new additions to her Coastal Kids team.
“I wasn’t sure how she would react to more new faces in the home but she loves Brenda (massage therapist) and Daniel (music therapist) just as much as Sarah and Ivanna,” says mom. “To know I don’t have to take her away from the house/her safe space and that the services are instead coming to her makes it so much better. I am just so happy your agency exists because you make parents feel safe at home.”