Despite the months anticipating his arrival, there were two things that Pierce’s parents could not have planned for. One, that he would be born during a pandemic and two, that the first 30 days of his life would be spent over 50 miles from home fighting for his life in the neonatal intensive care unit (NICU) at St. Christopher’s Hospital for Children.
After he stopped moving in the womb, Pierce was delivered prematurely at 35 weeks by emergency caesarian delivery at a Southern New Jersey hospital and immediately required help with his breathing. He was born with hypoxic ischemic encephalopathy (HIE) due to an interrupted supply of blood flow and oxygen from the placenta. HIE can affect all organs, especially the brain. It requires that total body cooling therapy to be initiated soon after birth to limit the extent of brain injury and improve the long-term outcome. Pierce had an exceptional group of physicians at his birth hospital who managed his immediate care after delivery and initiated his transfer so that he could begin this time sensitive therapy. The St. Christopher’s Critical Transport Team immediately deployed and Pierce began receiving cooling therapy upon arrival.
At St. Christopher’s NICU, Pierce was working harder to breathe despite receiving non-invasive respiratory support. His lungs were not tolerating the transition from intrauterine life and he required help with a breathing tube as he quickly reached maximum medical therapy. Meanwhile, 90 minutes away at the referring hospital, Pierce’s mom Sarah was recovering from a caesarian delivery and spent two long nights away from her newborn.
“St. Christopher’s is great with communication. They called me whenever they had an update and I could call them with questions. It was hard understanding everything that was going on, but they were able to put it into simple language that anyone could understand.”
An echocardiogram showed severe pulmonary hypertension and abnormal thickening of Pierce’s heart muscle called ventricular hypertrophy. This meant that every time his heart beat, the thickened tissues in his left ventricle had difficulty pumping blood to the rest of his body. This condition, required specific medications to help his heart be able to pump enough oxygen-rich blood to the rest of his body.
Managing these conditions simultaneously was challenging and often required treatments that made one condition worse while making the other better. Despite intensive management in the NICU with constant monitoring and fine adjustment of medications, Pierce’s condition slowly worsened over a 24-hour period with slow buildup of acid in the blood and a decline in oxygen saturation levels. Pierce was also on various intravenous infusions to maintain a safe blood pressure.
“We were helping his brain, heart, and lungs, but it became obvious that this was a battle that would not be won without help from another vital member of the medical team. His best chance for survival was to support his lungs with ECMO (Extracorporeal Membrane Oxygenation), while resting his heart and continuing to protect and care for his brain,” said Dr. Swosti Joshi, ECMO Medical Team.
ECMO Coordinator Jillian Deacon, RN, arranged a multidisciplinary team meeting of neonatology, cardiology, surgery and ECMO specialists. “Pierce’s case was a challenging one because he had several heart and lung problems, which required two different priorities of ECMO treatment. His lungs and heart were failing despite maximum medical management and his NICU doctors felt that ECMO was his only chance.”
After getting clearance from her own doctor, Sarah and her husband Michael arrived at St. Christopher’s and met Pierce’s care team, who updated them on his condition.
“We were overwhelmed and scared,” says Sarah. “But everyone did a great job of communicating what was going on, what he needed and the risks. There were all kinds of alarms going off, I was asking questions and they would answer even though they were caring for him at the same time."
"The nurses are fabulous, doctors are wonderful – they drew pictures of what was going on – and everyone’s ability to explain things gave us some peace of mind.”
Pierce’s parents were appropriately concerned, yet they trusted the team as they explained physiology and discussed pros and cons for this invasive procedure that did not promise success, but simply provided the best possibility for surviving. “There were many people in the room coming together to make decisions,” Sarah recalls. “It was great there were so many involved, because everyone thinks about things differently and you’re not just relying on one doctor’s expertise. They all had constructive things to say, welcomed our questions and made us part of the conversation.”
“We felt Pierce was an excellent candidate for veno-venous (VV) ECMO,” said pediatric surgeon Harsh Grewal, MD, FACS, FAAP and Christopher Pennell, MD. “This approach places a cannula into the heart to put oxygen into the blood while the lungs recover. Using a combination of ultrasound, fluoroscopy, and echocardiography, we placed the cannula into a vein and directed it into his heart. Success with this approach requires specialists from NICU, ECMO, cardiology, radiology, and surgery to work in concert during the procedure and epitomizes the multidisciplinary care our ECMO team provides.”
This minimally invasive procedure and the technology available at St Christopher’s helps very critically ill groups of patients, especially neonates who struggle with severe oxygen deprivation.
“This was a great team effort,” said Boban Abraham, MD, Pediatric Cardiologist. “My area of expertise is percutaneous, minimally invasive procedures. Fluoroscopy and wire manipulation are skills required to assist surgeons and improve safety of the cannulation. As a cardiologist, I utilize echocardiogram to help optimize the final position of the cannula and direct outflow to the tricuspid valve.”
The ECMO procedure went without complication and made an immediate difference in Pierce’s clinical status and medications to support blood pressure were quickly weaned. Staying at a hotel nearby, Pierce’s parents were present every single day for rounds, which included the entire multidisciplinary team. Though they had two older children at home, they were an active part of Pierce’s care and it is clear that he inherited his strength, resilience, and will to live from them.
Pierce responded well to his 5-day period of rest on ECMO, allowing his heart to respond to the medication and pump effectively. He was decannulated from ECMO and within days tolerated the breathing tube to be removed as well.
“We celebrated each small victory with the family as Pierce grew closer to coming off ECMO,” said ECMO nurse Jillian Deacon. “I am so proud to be a part of this team that provides hope to families in some of their darkest times.”
After three weeks of staying in Philadelphia, Sarah and Michael felt confident enough leaving the hospital to make the 50-mile trip home to care for their two-year-old child who had been staying with relatives and to update their thirteen-year-old child in person who lives with his mother’s family. Sarah continued to drive to Philadelphia during the pandemic and be with Pierce during visiting hours while Michael stayed at home with their toddler. Over the course of his month-long stay in the NICU, Pierce learned to feed from a bottle, breathe on his own, breastfeed and smile.
“I truly feel honored that in what must have been the scariest moment of their lives as parents, Pierce’s mother and father chose to trust us that we would do everything in our power to give their son his best chance at life,” said Yanick Vibert, DO, Pediatric Neonatologist.
“Every baby comes into our lives to teach us a lesson. Pierce has taught and reinforced the power of teamwork and communication. And I am a better physician because of it!”
Pierce was discharged on 06/13/2020, weighing in at a healthy nine pounds, three ounces. He will continue his follow-up care with St. Christopher’s for a few months, then transition to his general pediatrician in Cape May.
“Very little in life brings me more joy and satisfaction than to send one of our patients home after being so sick in the ICU,” says Daniel Marino, RN, CCRN, ECLS Program Manager. “Our team of highly trained ECMO specialists not only provide high quality care to our patients but also spend time with the parents to assure a greater understanding of the technology and their child’s condition. I am proud of all the work that the ECMO team has done to bring Pierce to the point where we say goodbye.”
“I’m glad we ended up at St. Christopher’s,” Sarah said. “I feel like if he ended up somewhere else, I don’t think he’d be here.”