Cecilia Faye Severs was brought into the world on August 29, 2024 at 33 weeks gestation via a smooth cesarean delivery after Anna began feeling contractions the night before. We knew she would be born premature after Anna's water broke two and a half months before our due date, and the hospital was already planning to induce her at the 34-week mark. But Baby Cici was too excited and couldn't wait any longer to make her grand entrance!
A few hours after her birth, while we were in Anna's recovery room, we received a phone call that would forever change our lives. There was a problem with Cici’s heart, and she needed to be transferred to Rady Children's Hospital immediately.
During her examination in the NICU, doctors determined that Cici had been born with a congenital heart defect (CHD) called truncus arteriosus. Both of us had no knowledge of CHD prior to this happening to our family. We are both healthy adults with no apparent family history of heart disease and felt knocked off our feet when Cici received her diagnosis.
In the development of a normal, healthy heart, as the fetus develops during pregnancy, the heart starts with a single large blood vessel. As fetal development progresses, the blood vessel divides into two arteries that carry blood out of the heart:
• The pulmonary artery, connected to the bottom right chamber of the heart. This divides into two arteries carrying oxygen-poor ("blue") blood to each side of the lungs.
• The aorta, connected to the bottom left chamber of the heart. This carries oxygen-rich ("red") blood to the body.
For Cici, however, the single large blood vessel failed to divide during fetal development, and she was born with only one artery carrying blood out of her heart. Her blood was "mixing" inside her heart, causing her lungs to receive too much blood while the rest of her body struggled to get enough. Before she could be discharged from the hospital, she would need major open-heart surgery to "rearrange the plumbing" in her heart. But because Cici was born premature, the medical team wanted to wait until she was full-term to perform the surgery.
The first month of Cici’s life was spent with the medical team administering various medications, blood transfusions, blood thinners, and other temporary measures to buy time for her to grow. When she was two weeks old, an intervention via cardiac catheterization was attempted where temporary "flow restrictors" were placed inside her pulmonary arteries to reroute more blood to her body. This was a new and relatively untested procedure, as our cardiologist estimated that maybe 100 kids worldwide had ever undergone it.
Unfortunately, the flow restrictors didn’t significantly improve Cici's condition, as she still needed frequent doses of sedative and paralytic medicine as the only way for her organs to get some of the necessary blood flow. Before the intervention, the doctors had hoped to get Cici healthy enough to remove her breathing tube and feed on breast milk, but she never reached that point. Just a few weeks after the cath lab intervention, Cici went in for an MRI which revealed that she had five times more blood flowing to her lungs than her body. The normal ratio is 1:1. This was worse than the doctors expected, and they told us she needed her major heart surgery the next day to make repairs.
Although the surgeon anticipated a six-hour surgery, it ended up being closer to 13 hours. After wheeling her down to the operating room at 9 a.m., we wouldn’t get to see her again until 11:30 p.m. As relieved as we were to be with her after surgery, she was a tough sight to see. Our cute little baby had swelled considerably. Her chest was still open after surgery, as it was too swollen to be sewn closed. We could see her heart beating, and she had tubes running throughout her body that pumped blood to and from a heart-lung bypass machine called ECMO. This acted as life support while her heart recovered from the surgery.
In the days that followed, her condition began deteriorating. She swelled so much that she couldn't even open her eyes, and her limbs became very dark and discolored. Her kidneys went into "shock" and stopped working, so the doctors surgically installed a drain in her stomach to remove fluid, placing her on dialysis from that point on. She started having seizures in her brain. Ultimately, her heart wasn't "waking up" on the timeline the doctors had hoped for. Although ECMO was intended for two to three days after surgery, she still wasn't healthy enough to come off after a full week.
Any apparent progress was accompanied by even steeper regressions. One night, a clot in the ECMO machine shut down the circuit, sending Cici into cardiac arrest. The medical team performed CPR for 10 minutes until they could restart her heart. After her first surgery, we learned that she had a genetic blood-clotting disorder called Factor V Leiden. When coupled with her heart condition, this put her at an even higher risk of developing clots. She also showed signs of developing heparin-induced thrombocytopenia, an allergic reaction to the blood thinner she was on. The surgeon attempted two more surgeries to repair her heart and take her off bypass, but the complications and blood clots continued to pile up.
The morning before Cici’s third and final surgery, we had been told by the night nurses she slightly opened her eyes around 3 a.m. That morning, we delicately tried to stir her awake, but Cici continued to sleep. We went through our normal pre-surgery routine, reading and singing to her while the team prepped to transfer her to the OR, but she kept sleeping. When the anesthesia team arrived and began administering meds, we worried we had missed our chance. But five minutes before she was wheeled down to the OR, Cici eyelids opened and she peeked through to give Mom and Dad the softest and sweetest glance of her eyes for the first time in three days.
Cici's heart never was strong enough to come off ECMO, while blood clots increased through her tiny little body. On October 8th, after consulting with the medical team, she was taken off life support. Surrounded by parents and grandparents, she breathed her last breath. The glance she gave the day before was her little way of saying goodbye.
Throughout our journey, we were truly amazed by the level of knowledge, collaboration, and compassion of the Rady Children's medical team. These doctors and nurses are the best of the best, and we wouldn't have wanted any other team to care for Cici. But as one of the doctors told us, "medicine is incredibly humbling," and although treatment for congenital heart disease has improved drastically over the last 50 years, the science is still relatively new, and there are still kids like Cici who manage to puzzle and challenge the doctors. Some procedures, like her flow restrictor placement, are still developing rapidly (there are currently no devices designed for babies and children, and the surgeons must manipulate equipment designed for adults). During Cici's journey, we had multiple discussions with doctors where we were told her situation, specifically being a congenital heart defect coupled with a blood-clotting disorder, didn't have a clear-cut solution based on the latest medical research and evidence.
For this reason, we have decided to honor Cici by fundraising for The Children's Heart Foundation. Their mission is simple: to advance the diagnosis, treatment, and prevention of congenital heart defects by funding the most promising research. They have funded over 160 research projects to date, contributing nearly $18 million toward CHD research and scientific collaborations. CHD is the most common birth defect in the United States, affecting nearly 1 in 110 babies. Despite this, it receives less public awareness than many other children's diseases and disorders. We sincerely hope you’ll join us in donating to this amazing cause. Please consider using the “Donate Now” button to leave a contribution today. Together, we can spread awareness and improve treatment outcomes for babies, kids, and adults with congenital heart disease.