A standardized approach through participation in a national collaborative aims to reduce oral feeding delays and improve outcomes.

Ethan Schwartz of Asheville, North Carolina, was born six weeks early with hypoplastic left heart syndrome (HLHS), a severe congenital heart defect in which the left ventricle is underdeveloped and unable to perform its primary function—pumping oxygenated blood into the aorta and throughout the body. However, in a three-stage surgical palliation starting with the Norwood procedure soon after birth, the right side of the heart takes over that function. In the interstage period before the next procedure, the bidirectional Glenn at 4 to 6 months of age, achieving adequate growth often requires supplemental or total nutrition through an enteral tube, a gastrostomy (G) or nasogastric (NG) tube. Many of these infants and their parents struggle with weaning off a tube, however, which may have a significant impact on development and growth.

"Between the Norwood and the Glenn, Ethan did great, he tolerated his feedings," says Chelsey Schwartz, Ethan's mother. "For some reason, after the Glenn Ethan struggled with his feedings and getting up to the volumes Kristi recommended for his size and age. He was still gaining weight, but he was vomiting several times a day."

Kristi is Kristi Fogg, pediatric cardiology dietitian at MUSC Shawn Jenkins Children's Hospital. Much of her focus is on guiding and supporting parents like Schwartz, too many of whom she says feel lost in feeding and weaning their newborn. Part of the problem, Fogg explains, has been the lack of a standardized approach for parents in weaning children off a feeding tube and optimizing oral feeding to grow.

"Parents have had to shop around and explain why their baby has different needs—they were floundering around on their own looking for a weaning program that works," says Fogg. "We realized parents just weren't prepared enough. We could do better through partnering with parents to have a smoother transition after the second surgery."

That led to Fogg and the single ventricle team's participation in the development of a tube weaning toolkit by the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC). The goal of the toolkit, released in January 2022, is to increase the percentage of patients successfully weaned from feeding tubes and eating oral foods by their first birthday, from the current 54 percent to 80 percent nationally by 2024. How? Through a standardized approach that engages a multidisciplinary team and empowers parents in the tube weaning process or, in Fogg's words, "gives them ownership."

Under the toolkit's guidelines, developed from surveys with NPC-QIC's 69 member hospitals and best practices literature reviews (Progress in Pediatric Cardiology, Vol. 62, Sept. 2021), tube weaning is broken down into three phases. The first is preparing families and clinicians for the weaning process, which includes evaluating not just the child's growth but strength to tolerate coming off the feeding tube, and how and when to manipulate feeding regimens and trouble shoot feeding intolerance. The second phase focuses on ways to encourage hunger in combination with developing oral feeding, while the follow-up phase focuses on transition for parents once tube weaning is complete.

When patients are discharged from MUSC Shawn Jenkins Children's Hospital, they are followed-up weekly, at times daily, by Fogg and interstage nurse practitioner Frances Woodard, PNP, members of the hospital's interstage home-monitoring program. Adoption of interstage home monitoring practices has been associated with significantly improved morbidity and mortality (Journal of the American Heart Association. Aug 11, 2020).

A third member of the team, cardiac intensivist Sinai Zyblewski, M.D., assesses infants' hemodynamic readiness to feed by mouth and tolerate transient decreases in fluid intake or mild electrolyte disturbances. "Otherwise," Zyblewski notes, "Kristi and the therapists drive the actual G-tube weaning and transition to oral feeding."

Ethan's mom says she felt well supported by the team, noting that Fogg and Woodard were always available for any questions. Nonetheless, at first Schwartz found weaning Ethan at home extremely stressful. One major hurdle was inducing hunger in an infant tube fed since birth, who in her mind was not familiar with the feeling of hunger.

"Oral feeding was never a part of Ethan's life, so when we took away a feeding at first [to encourage hunger], he didn't respond," says Schwartz. "It was very, very challenging—it was scary. I basically felt like I was starving my child."

It took some time but through ongoing teaching and trouble-shooting sessions with Fogg, Schwartz learned to trust the toolkit's weaning formula and, more importantly, herself.

"Once I really buckled down and listened to Kristi and followed the ‘take a tube feed away and replace it with oral food,' he did it pretty quickly," says Schwartz. "He had learned how to eat and drink, he was ready. It was a four-month learning curve in which Kristi was supportive the whole time."

"We always try to promote positive feeding experiences, always coaching parents so they are ready to jump in the feeding stage," says Fogg.

Given Schwartz's success, Fogg recruited her as a mentor mom—another aspect of the toolkit—to support and help guide other single-ventricle parents facing the same weaning challenges at home. Schwartz, a trauma nurse herself, was eager to take on the role.

"It's really easy to want to help someone else going through something that was so hard," says Schwartz. "But the weaning process is surely not cut and dry—what worked for Ethan wouldn't necessarily work for another child. It's nice to brainstorm different ideas."

In ways, MUSC Shawn Jenkins Children's Hospital has advanced care significantly for infants with single ventricle anatomy since 2012, when it signed on as an NPC-QIC member hospital. Before then, Woodard explains, there was no interstage monitoring program, no dietician and little teaching for parents ready to go home, putting their babies at greater risk of failure to thrive.

"A lot of them had oral aversion because no one taught them how to transition from tube feeding to oral feeding," says Woodard. "Since we were able to start the interstage monitoring program with a dietitian, the process has been much smoother with our goal of getting most of these children off their G-tube close to one year of age."

"With tube dependency, the longer you wait the harder it is to get off the tube—now it's a lot easier and seamless," adds Fogg, who as a co-lead in the collaborative organized and authored much of the material in the toolkit. "We've had so many successful weans now that we can better help parents through the process and alleviate the stress."

Ethan's mom would agree, noting that his G-tube was removed in June 2021 and now he's eating 4 meals and 2 snacks a day.

 Progressnotes Summer 2022