Premature newborns with bronchopulmonary dysplasia (BPD) may require ongoing 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 is important, with the ultimate goal of enabling them to grow, develop and go home to their families.
Boston Children’s Hospital’s Division of Newborn Medicine is achieving success with a tailored, long-term, whole-baby approach, honed by outcome data, quality improvement initiatives and the recognition that each child’s lung disease has unique characteristics.
“Our strategy is pro-growth,” says Kristen Leeman, MD, associate medical director of Boston Children’s NICU. “Our goal is to optimize care with a focus not only on the lungs, but also on the growth and neurodevelopment of the baby.”
Nutrition is an important part of care and supports the growth of both the lungs and the baby. An individualized approach to respiratory support is also important because lung physiology can change.
“We want to prevent respiratory injury to the lung,” says Jonathan Levin, MD, who has appointments in both Newborn Medicine and Pulmonary Medicine. “But at some point during development, we may have to shift the strategy to adequately support the baby.”
Another QI effort involves optimizing the timing of tracheostomy. “Delaying a tracheostomy can increase length of stay and impede neurodevelopment,” says Leeman. “We’re trying to recognize earlier when babies need long-term respiratory support so that we can get them home sooner.”
Xavien: A case study
The case of Xavien Velazquez is illustrative. Born at 26 weeks gestational age, he entered the Boston Children’s NICU in October 2021, two months after his birth. He’d had two to three daily “coding” events at his birth hospital where he stopped breathing.
“Xavien had very underdeveloped lungs and needed respiratory support, but there were elements of his BPD that weren’t typical,” Levin says. “He had apnea, desaturation and episodes of bradycardia where his heart rate would drop very quickly. He would have to be re-intubated and receive chest compressions. His team suspected something was wrong with his airways.”
Arriving at Boston Children’s at 33 weeks postmenstrual, Xavien had a dynamic bronchoscopy to look inside his airway as he breathed and to see the impact of real-time adjustments in breathing pressure. The bronchoscopy was done while Xavien was actively breathing. “We wanted to mimic real life as much as possible,” Levin says.
The bronchoscopy showed that Xavien’s BPD was complicated by tracheomalacia, or a near-complete collapse of his airway during exhalation, usually in the tracheal area.
“Malacia can be a complication of chronic ventilator support,” Levin says. “Weakening of the cartilage in the airways can also be associated with underdevelopment of the lungs or be partly caused by steroids. Once we identified malacia, we were able to titrate Xavien’s ventilator settings to prevent him from having these life-threatening episodes. We also recognized that he was a candidate to have his tracheomalacia surgically repaired.”
Rethink ventilation
Many NICUs feel uncomfortable using high pressure to ventilate newborns. But the team believed that high positive expiratory pressure (PEEP) would keep Xavien growing and thriving until he was big enough to have surgery. The team therefore increased its PEEP to 18 cm H2O.
“He had only taken PEEP 8 before, so every time he got upset his airways collapsed like a pancake and he couldn’t breathe,” Levin says. “We came up with a ventilator setting that allowed him to grow, participate in physical therapy and communicate more with his family.”
With more ventilator support, Xavien also needed less sedation.
“While sedation helped him panic and hyperventilate, we felt that too much could hinder his development,” Levin says. “He had a relatively smooth time in the NICU once he was on the right settings.”
Maximizing Xavien’s Development
On November 12, about a month after Xavien’s arrival, his team continued with a tracheostomy to provide better ventilator support and keep Xavien stable until he was big enough for surgery. Equally important, it cleared his mouth and face, allowing him to develop oral-motor skills and, again, communicate more with his parents.
Recognizing early on that Xavien would benefit from a tracheostomy gave him a stable period in which he made important developmental leaps. His ventilator settings were too high to safely leave the hospital, but surgery to open his airways could allow him to do so.
In January 2022, four months after birth and just past his due date, Xavien was ready for surgery: a tracheopexy to open his collapsing airway, performed by Benjamin Zendejas-Mummert, MD, of the Oesophageal and Airway Treatment Program. The surgery sutured the posterior portion of Xavien’s trachea to his spine to provide structural support.
After recovering from surgery, Xavien was able to reduce his ventilator settings to a level where he could be safely cared for by his family at home.
unloading Xavien
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 the improvement Xavien made after surgery, he could skip the months of rehab and go straight home. Being at home would help him to develop in his development.
Xavien’s parents already took an active part in his care, visiting daily and handling much of his tracheostomy and G-tube care. Prior to Xavien’s discharge, they underwent further intensive preparation under the guidance of Christina Cavanaugh, RRT, in the Chronic Ventilation and Neuromuscular Program.
Each parent had a 24-hour dress rehearsal at the NICU, where they were responsible for all of Xavien’s care (with backup staff). Through simulations in the SIMPeds lab, they practiced dealing with emergency scenarios, while NICU case manager Karen Cote, RN, coordinated the family’s home equipment and services through multiple agencies.
Xavien was fired in March 2022, followed closely by a mix of telecare and in-person visits by Levin, Cavanaugh and Kathryn Malpocher, PNP, on the Chronic Ventilation and Neuromuscular Program. He is still on a ventilator 24/7, but on lighter settings. The plan is to wean him and eventually remove the tracheostomy tube.
The attention paid to Xavien’s development has paid off. Although he has some delay, he scores high on social-emotional measures.
“None of us has a crystal ball,” Levin says, “but he is as prepared as possible for success.”
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