Jase is all smiles and breathing better after battling bronchopulmonary dysplasia and surfactant dysfunction.
“Your baby won’t make it—it’s time to say your goodbyes.” Those are the last words a mother of a premature baby wants to hear from her doctors. Hailey Arledge, a mother of two from Valley Springs, decided to listen to her baby instead. She knew that her 25-week-old premature son, Jase, was going to live, despite what doctors at her hometown hospital said.
“If I hadn’t transferred Jase to Stanford Medicine Children’s Health, I know he would have died,” said Hailey.
Despite the excellence of her local hospital, her doctors were at a loss over how to help Jase, who was struggling to breathe after being born very prematurely. They didn’t believe he could be saved, so they advised against seeking a second opinion or transferring him to another hospital. They suggested that Hailey and her husband, Sam, take Jase off his ventilator and let him go peacefully. But Hailey was steadfast.
“I told Sam that until Jase shows us that he can’t go on, we are not going to do anything. I took it upon myself to call Stanford Medicine Children’s Neonatal Intensive Care Unit (NICU). I cried and explained that Stanford was our last shot,” said Hailey.
The NICU at Lucile Packard Children’s Hospital Stanford asked Hailey to have her local hospital send Jase’s medical records, saying they’d get back to her in a few days. To Hailey, waiting a few days seemed impossible, considering that Jase was quickly losing ground.
“Less than an hour later, they called me back and said Stanford Medicine Children’s would take him. A weight lifted off me, and I knew it was where Jase needed to be,” said Hailey.
When Jase arrived, he was so swollen that his eyes were barely visible. His body was reacting to a strong steroid that was meant to be temporary. His lungs were scarred from the mechanical ventilator that kept him alive. He was diagnosed with bronchopulmonary dysplasia (BPD) and with a rare genetic disorder called surfactant dysfunction. Stanford Medicine Children’s gathered its team of neonatal and pulmonology experts to figure out how to best help him.
“Having bronchopulmonary dysplasia and a surfactant disorder is very rare, and it’s remarkable that he survived,” said Michael Tracy MD, a pediatric pulmonologist at Stanford Medicine Children’s and an expert in lung disease in preterm infants, especially BPD.
Stanford doctors were the first to define bronchopulmonary dysplasia, in 1967, and has been at the forefront of treating the condition ever since. Today, the health system offers a bronchopulmonary dysplasia program for infants, bringing together a care team of pulmonologists, neonatologists, cardiologists, respiratory therapists, registered dietitians, social workers and care coordinators to support patients and their families. This team meets regularly, along with Hailey and Sam, to discuss the best treatment for Jase.
“Everyone comes into treatment meetings with their own points of view, and even if I don’t always agree with them, I love that everyone weighs in on options,” said Hailey. “They decide on an approach, get my OK, then stick to the plan. No one changes the plan without input. It’s what has gotten Jase to where he is today.”
At birth, Jase weighed 1 pound, 10 ounces, the weight of about three grapefruits. Had he just been an extremely preterm baby with bronchopulmonary dysplasia who developed lungs that could produce surfactant—a mixture of fats and proteins that line the lungs and allow the tiny air sacs called alveoli to fill with air—he might be breathing on his own without oxygen by now. But surfactant disorder is a game changer. Some babies born with a surfactant protein deficiency don’t survive. A few get a lung transplant. Others are diagnosed later in childhood.
“There are several types of surfactant disorders, with a range of symptoms and a variable prognosis. It’s a group of extremely rare genetic disorders that currently has no cure. We are working to create a database with pediatric hospitals nationwide to follow these babies to better understand how to treat them,” said Dr. Tracy.
As a national leader in pulmonary pediatrics, Stanford Children’s Health sees more babies with surfactant disorder than most hospitals, but treatment is still experimental until clinical trials can be conducted. While statistics on the prevalence of Jase’s type of surfactant deficiency—ABCA3—are unknown, another type occurs in just one in a million newborns worldwide, according to the National Institutes of Health.
Yet Jase doesn’t seem to care about odds. He just slowly improves with each passing day.
“Jase decides his fate, and he has decided he wants to be here. I think he will keep surprising us,” said Hailey.
After learning about Jase’s disorder, both Hailey and Sam received genetic testing. Both came out positive as carriers for mutations of the ABCA3 gene, despite no known family histories of the mutation. Fortunately, their older son, Sammie, isn’t affected.
Jase arrived at Packard Children’s NICU in July 2018. Most babies in his state would never expect to leave the NICU. Yet he continued to improve, graduating to the pediatric ICU, the pulmonary floor and then, finally, home.
“He spent over 300 days in the hospital, but he’s gradually shown improvement, and that’s remarkable,” said Dr. Tracy.
Over the course of his stay, Jase has moved from a ventilator and breathing tube to only needing oxygen via a nasal cannula during the day and a BiPAP breathing machine at night. He still receives medications to treat his surfactant disorder. So far, the team has been able to avoid a tracheostomy—an incision in his windpipe to help him breathe with a mechanical ventilator. As a last resort, he might need a lung transplant in the future. While it’s nothing that anyone hopes for, Packard Children’s is well equipped as the only pediatric lung transplant center on the West Coast.
“I was told early on that Jase would need a tracheostomy and be dependent on a machine for the rest of his life, so for him to be breathing on his own with oxygen is such a big deal,” said Hailey.
Dr. Tracy heartily agrees: “As long as we can protect Jase’s lungs, we believe his bronchopulmonary dysplasia will continue to improve. We knew kids with bronchopulmonary dysplasia could grow new lung tissue in the first few years. Now, researchers are finding they can grow new lung tissue into their teen years. His surfactant disorder is an unknown, but we are hopeful.”
Today, at close to 13 pounds, Jase puts all his weight into life: smiling, laughing at jokes, sitting up and clapping his hands. He babbles constantly. The other day he made the “m” sound, which Hailey hopes was the start of saying “Mama.”
“Everyone says he is beyond where he should be developmentally for a micro preemie, which is amazing because he has been behind for some time. He’s smart. He loves certain movies and cracks up at the same scenes every time. He’s always happy, and nothing seems to bother him,” said Hailey.
Jase just celebrated his first birthday on April 18, something no one could imagine during his first weeks of life.
“My birthday wish for him was to come home, live a happy life, and hopefully get off oxygen someday, but if not, that’s OK,” Hailey concluded. “We are just happy to have him in our lives.”
Learn more about managing bronchopulmonary dysplasia at Stanford Medicine Children’s Health >
