As a nurse in the Steven & Alexandra Cohen Foundation Newborn and Infant Critical Care Unit (NICCU) at Children’s Hospital Los Angeles (CHLA), Alisa Nelson, RN, was familiar with medically fragile babies. Caring for them was her job. But there was one diagnosis she didn’t see coming.
At 20 weeks pregnant, a routine anatomy scan ultrasound showed something that wasn’t right in the baby they would call Charlie: the left side of his heart was underdeveloped, and blood flow was significantly impaired.
Alisa and her husband, Matthew, met Jay Pruetz, M.D., director of CHLA’s fetal cardiology program who told them Charlie had hypoplastic left heart syndrome (HLHS). About 1,000 children in the United States are born each year with HLHS, which is fatal unless corrected by surgery.
He added that children with HLHS must have three open-heart surgeries in the first three years of life to reroute the heart’s blood flow and make the right ventricle the main pumping chamber. These surgeries don’t cure the condition, but they help to restore heart function.
Alisa and Matthew were devastated. Already parents of two healthy children, they had no reason to suspect a third pregnancy would lead to complications. What’s more, because of Alisa’s 15 years of experience as a nurse in the NICCU, she knew how trying major surgery for an infant could be and how taxing the ordeal would be on her family.
“Once we had a chance to digest what was happening, we agreed to make the best decision each day with the information we had,” she says. “We always said we’d let Charlie be our guide and show us and tell us what we needed to do.”
Early treatment
CHLA doctors closely monitored Charlie’s development during the last few months of Alisa’s pregnancy. "I found myself in the position I dreaded more than any other,” she says. “How odd that a caregiver could suddenly become a patient? Until the day he was born, I was the one with the medical record number and the chart, coming in for numerous appointments with cardiology and a multitude of other specialists. It felt odd to see my own name in the computer system and to continue caring for sick babies when I knew it was only a matter of time until my child lay in his own hospital bed.”
Charlie was born May 30, 2018, and immediately transported to the cardiothoracic intensive care unit at CHLA. Vaughn Starnes, M.D., co-director of the Heart Institute and head of the division of cardiothoracic surgery at CHLA, operated on Charlie four days later. The surgery lasted three long, nerve-wracking hours.
“As a NICCU nurse, I spent years supporting mothers as they watched their own babies fight for their lives,” Alisa says. "Now it was my turn to be in those shoes. I had to sit on my hands and watch as nurses, doctors, therapists and so many others cared for my child. Had it not been for my years as a nurse I don’t know that I could have handled this past year as gracefully as I like to think that I have.”
When surgeons were finished, Charlie was so swollen that he spent the first two days of recovery with his chest wide open, covered with gauze. “I literally could see his heart beating inside his chest,” Matthew says. “It was one of the scariest things I’ve ever seen.”
Charlie spent more than two weeks in the hospital, with his parents by his side. When Alisa and Matthew were able to take him back to their Burbank home, they had to follow a rigorous course of monitoring—weighing the baby once a day, recording the volume of every feeding and checking the percent of oxygen in his blood multiple times a day. They also had to be vigilant about his contact with their 7-year-old daughter, Edee, and 2-year-old son, Sammy, since any infection could put him in danger.
Despite this constant oversight, Charlie was readmitted to CHLA three times between surgeries, including twice because his oxygen levels were low. "Being the mother of a child with an incurable disease is the most heart-wrenching and difficult challenge I have ever faced,” Alisa says. “But after having served all those years as a nurse, there was no doubt in my mind Charlie was in the best place and getting the best care possible. Although I surely didn’t want to ever have to, I have witnessed the excellence of CHLA from both sides of the bed. I have felt the support for my sick child, for my healthy children and for myself as a mother."
Corinne Goodman, a nurse on the ECMO team at CHLA, offered support while Charlie was an inpatient. “It’s not every day we see one of our colleagues become the parent of a patient,” Goodman says. “Whenever I could, I would go see Alisa and talk with her and listen. I wanted to make sure I was a friend to her and remind her she was not alone.”
The second phase
In late October, Charlie went back to CHLA for the second major surgery: the bi-directional Glenn shunt procedure. Charlie responded positively, and this surgery also included a pivotal step in his participation in a clinical trial for patients with HLHS.
The trial, a consortium led by the Mayo Clinic in Minnesota, was hoping to demonstrate that stem cells from a patient’s umbilical cord blood could stimulate new muscle to grow in the patient’s heart. Because many HLHS patients have heart failure later in life, this new muscle growth could help delay or even prevent future problems.
Though it’s too early to determine if the cells have had any impact on Charlie’s recovery, his parents and doctors are optimistic. “The hope of the study is that by injecting these cells, we can repopulate the right ventricle and strengthen it over time and prime it for being the primary pumping chamber for the body,” Pruetz says. “Any improvements could have a serious impact on the child’s life moving forward.”
Alisa agrees. “Part of me has a fear about it, but if there’s a chance it can help him, we have to try,” she says, noting that Charlie was the 16th child in the country to participate in the trial. “It’s something to give us hope.”
What’s next
There were no complications with the second surgery, so Charlie’s recovery period was significantly shorter than it was after the first. This means Matthew and Alisa no longer have to quarantine the baby or monitor him so vigilantly. It also means they have been able to take him out and about. “It feels like we’ve been let out of a cage,” Matthew says. “For the first time since his birth, it feels like we have the opportunity to be a family.”
Charlie is living life like any other 8-month-old: dirty diapers, daily naps and snuggles with Mom and Dad. Unless you see his scar, there are no hints of what he has been through. At some point in the next two years, however, Charlie will have the third major surgery of the HLHS trio: the Fontan procedure.
Surgeons have been performing this three-part HLHS surgical treatment since the 1980s and some of the original patients are now reaching their 40s. Pruetz sees no reason why Charlie couldn’t join this group. And because breakthroughs and better treatments are developing all the time, doctors today can’t say what the future holds for children like Charlie.
Alisa, who is now back at work at CHLA, says every moment with her infant son is a gift, and she vows to celebrate every milestone along the way. “His heart could fail tomorrow or could last until he’s 60; we try to focus on the positive and enjoy the time we have with him while he’s here,” she says. “I made a conscious choice I’m not going to live in fear anymore, I’m going to live in love. However long we get to have with him, we are happy.”
