Premature newborns with bronchopulmonary dysplasia (BPD) may require continuous mechanical ventilation to support their lungs. But babies with BPD aren’t just newborns with underdeveloped lungs. Each has a unique presentation. And every part of their care matters, with the ultimate goal of enabling them to grow, develop, and return home to their families.
The Division of Neonatal Medicine at Boston Children’s Hospital achieves success through a personalized, long-term, whole-baby approach honed through outcome data, quality improvement initiatives, and recognition that each infant’s lung disease has unique characteristics.
“Our strategy is pro-growth,” says Kristen Leeman, MDassociate medical director of Boston Children’s NICU. “Our goal is to optimize care by focusing not only on the lungs, but also on the growth and neurological development of the baby.”
Nutrition is a key part of care, supporting lung and baby growth. An individualized approach to respiratory support is also important because lung physiology can change.
“We want to prevent ventilator respiratory injury,” says Jonathan Levin, MDwho has appointments in both neonatal medicine and Pulmonary medicine. “But at some point in development, we may need to change the strategy to adequately support the baby.”
Another quality improvement effort is to optimize tracheostomy timing. “Delaying tracheostomy can increase length of stay and harm neurodevelopment,” says Leeman. “We’re trying to recognize earlier when infants need long-term respiratory support so we can get them home sooner.”
Xavien: A Case Study
The case of Xavien Velazquez is illustrative. Born at 26 weeks gestation, he arrived at the Boston Children’s NICU in October 2021, two months after he was born. He had had two to three daily “coding” events at his home hospital in which he had stopped breathing.
“Xavien had very underdeveloped lungs and needed respiratory support, but there were elements of his borderline personality disorder that weren’t typical,” says Levin. “He had episodes of apnea, desaturation and bradycardia where his heart rate dropped very quickly. He should be re-intubated and undergo chest compressions. His team suspected something was wrong with his airway.
Arriving at the Boston Children’s at 33 weeks post-menstrual, Xavien had a dynamic bronchoscopy to look into his airway as he breathes and see the impact of real-time ventilator pressure adjustments. Bronchoscopy was performed with Xavien actively breathing. “We wanted to mimic real life as much as possible,” says Levin.
Bronchoscopy showed that Xavien’s borderline personality disorder was complicated by tracheomalaciaor almost complete collapse of his airways during exhalation, mainly in the tracheal region.
“Malacia can be a complication of chronic ventilatory support,” says Levin. “A weakening of the cartilage in the airways can also be accompanied by underdevelopment of the lungs or be caused in part by steroids. Once we identified the disease, we were able to adjust Xavien’s ventilator settings to prevent him from having these life-threatening episodes. We also recognized that he was a candidate for surgical repair of his tracheomalacia.
Many NICUs are uncomfortable with high pressures when ventilating newborns. But the team felt that high positive expiratory pressure (PEEP) would allow Xavien to grow and thrive until he was big enough to undergo surgery. The team therefore increased its PEEP to 18 cm H2O.
“He’d only been on PEEP 8 before, so every time he got upset, his airway would collapse like a pancake and he couldn’t breathe,” Levin says. “We found a ventilator setting that allowed him to grow, participate in physical therapy, and interact more with his family.”
Along with more ventilatory support, Xavien also needed less sedation.
“While the sedation kept him from panicking and hyperventilating, we thought too much might interfere with his development,” says Levin. “He had a relatively smooth time in NICU once he was on the right settings.”
Maximize Xavien Development
On November 12, about a month after Xavien arrived, his team performed a tracheostomy to provide safer ventilatory support and keep Xavien stable until he was old enough for surgery. Equally important, it freed up his mouth and face, allowing him to develop oral-motor skills and, again, to interact more with his parents.
Recognizing early on that Xavien would benefit from a tracheotomy allowed him a stable period of time during which he made significant developmental progress. Her ventilator settings were too high to leave the hospital safely, but surgery to open her airway may allow her to do so.
In January 2022, four months after his birth and just after his due date, Xavien was ready for surgery: a tracheopexy to open his collapsing airways, performed by Benjamin Zendejas-Mummert, MDof the Esophagus and respiratory tract treatment program. The operation sutured the back part of Xavien’s trachea to his spine to provide structural support.
After recovering from his surgery, Xavien was able to bring his ventilator settings down to a range where he could be safely cared for at home by his family.
Normally, Xavien would have been sent to a rehabilitation hospital to recover from the tracheopexy. But the nursing team, led by Theresa Andrews, RN, felt that with Xavien’s improvement after surgery, he could skip the months of rehabilitation and go straight home. Being at home would help him thrive developmentally.
Xavien’s parents were already actively involved in his care, visiting him daily and taking care of much of his tracheostomy and G-tube care. Prior to Xavien’s discharge, they underwent additional intensive preparation under the guidance by Christina Cavanaugh, RRT, in the Chronic and neuromuscular ventilation program.
Each parent had a 24-hour “dress rehearsal” at the NICU, where they were responsible for all of Xavien’s care (with staff available as backup). Through simulations in the SIMPeds lab, they practiced handling emergency scenarios while NICU case manager Karen Cote, RN, coordinated equipment and home services for the family through the intermediary of several agencies.
Xavien was released in March 2022, followed closely through a mix of telehealth and in-person visits by Levin, Cavanaugh and Kathryn Malpocher, PCP, in the chronic and neuromuscular ventilation program. He is still on a ventilator 24/7, but on lighter settings. The plan is to wean him and possibly remove the tracheostomy tube.
The attention given to the development of Xavien has paid off. Although he had some delays, he scored high on social/emotional measures.
“None of us have a crystal ball,” says Levin, “but we’re as prepared as we can be to succeed.”