A Home for Moriah's Heart
Not long after Victoria and Justin Nelson became overjoyed first-time parents, they received the most terrifying news. The former high school sweethearts had known since before her birth that daughter Moriah would be born with a complex congenital heart defect called tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries (MAPCAs). But now, when Moriah was just a few months old, doctors at their Southern California hospital told them her condition was inoperable.Take your child home and love her until her heart gives out, the parents were told.
Instead, they started researching.
Thankfully, the Nelsons discovered that Moriah's heart defect wasn't inoperable at all. Again and again they found references to pediatric cardiothoracic surgeon Frank Hanley, MD, at Lucile Packard Children's Hospital, pioneer of an innovative surgical procedure called unifocalization.
Unifocalization often is a grueling, 10- to 12-hour operating room epic that, before Hanley began perfecting the technique in the early 1990s, other surgeons thought just couldn’t be done. Instead, children like Moriah would face the trauma and danger of multiple heart and lung surgeries over time.
In Moriah's case, Hanley’s unifocalization had the potential to correct her pulmonary artery abnormalities in one fell swoop. After review, Dr. Hanley agreed that Moriah was a good candidate, and in November 2008 the Nelsons made the drive up to Palo Alto.
"We were all there, with all our family members," Justin says, recalling the day of the procedure. "Finally at 9:30 at night, after twelve and a half hours of surgery, Dr. Hanley comes out to see us. He said, 'Guys, I was able to do a complete repair on her heart.'"
Victoria can't resist jumping in to help recount that moment:
"I still remember him saying, 'Her saturations are 100, like yours and mine.' That's one line I will never forget," she says. "I burst into tears. I couldn't believe it. I was so thankful."Given the initial prognosis, Victoria likened Hanley’s unifocalization and complete repair surgery to "a new beginning." But Moriah's situation turned out to be more complex than anyone had suspected. Though her heart defect had been repaired, a variety of separate issues necessitated a stay at the hospital's Cardiovascular Intensive Care Unit (CVICU). In January 2009, doctors found that Moriah's right diaphragm was paralyzed, and she required another surgery. Then there were infections related to an underlying immune system disorder. For most children, a visit to the CVICU post-surgery might last days or a few weeks. Moriah would spend an astonishing eleven months there.
"It essentially became this little girl's home, and the family's home away from home," says Stephen Roth, MD, MPH, Medical Director of the CVICU. "In that time there were two failed attempts to repatriate her back to Southern California, to be near the Nelsons’ home. But eventually the family decided the best thing to do would be to move to the Bay Area, so she could receive care here at Packard."
It was only at Packard Children's, the Nelsons say, that they felt fully cared for. At times this boiled down to something as simple as just being heard. Justin describes a period of intensely painful abdominal pain for Moriah -- pain that doctors elsewhere dismissed as colic.
"But at Packard they were so concerned about Moriah that they really listened to us when we'd describe what was going on. And that ended up making a big difference," he says.
Indeed, Moriah's team eventually came to identify her pain as abdominal migraine, a rare but extremely disruptive gastrointestinal condition. It was CVICU nurse practitioner Jana Norris, RN, PNP, who came to the rescue.
"At that point the abdominal pain was the thing that was most challenging for the family. Their hour-to-hour life was so impacted by it -- watching Moriah have these cycles of pain was horrible for them. She was retching and gagging, and had diarrhea, and there'd be desaturation and her oxygen levels would drop. It was hard to get her enough nutrition, too," Norris says.
Norris had successfully treated similar symptoms with a chronic pain medication. She did some research and communicated the idea to Moriah's doctors. They decided to give it a try.
"Within days the pain was almost entirely gone and she could sleep at night like another kid," Victoria recalls. "It changed our lives."
Finally, after giving her a tracheostomy, Moriah's doctors said she could leave the CVICU. In October 2009 she was moved into the Children's Recovery Center in Campbell, CA, where today she receives ventilator therapy to help her lungs work properly. Victoria and Justin found an apartment right across the street.
 Justin and Victoria Nelson with Dr. Stephen Roth |
Meanwhile, the Nelsons say they've also come to live in the present, and be grateful for the incredible progress their daughter makes every day.
"A child like Moriah forces you to live the way you'd want to live," Justin says. "You're embracing every little moment. If Moriah's good today, then I'm good. That's the way we live now. And we're just so thankful for the care she's gotten, and that she's progressing well."