Reducing Mortality for Technology-Dependent Infants

BPD: A Common Complication of Prematurity

Bronchopulmonary dysplasia (BPD) remains the most common complication of premature birth.^1,2 Currently, the most accepted definition of BPD is the need for oxygen or respiratory support at 36 weeks’ postmenstrual age (PMA).

Patients with severe BPD often have severe complications, including pulmonary hypertension, poor growth, long-term pulmonary morbidity, and neurodevelopmental disability.³ The term BPD is often used interchangeably with chronic lung disease of prematurity, or CLD.

Establishing a Multidisciplinary CLD Team

There are a lack of published guidelines and national standards for treating infants with severe BPD/CLD. However, the Children’s Mercy Kansas City Neonatal Intensive Care Unit (NICU), an 84-bed Level IV regional neonatal referral center, saw the need to establish a dedicated multidisciplinary CLD team to care for these infants, and was one of the first in the nation to do so.

Launched in 2010, this team follows a standardized approach to the diagnosis and management of infants with BPD/CLD who do not respond to standard therapies.

Over the past decade, this team has grown to include dedicated neonatologists supported by pediatric pulmonology; cardiology; otolaryngology; a facilitator/coordinator; primary bedside NICU nurses; neonatal nurse practitioners; respiratory therapists; a tracheostomy coordinator; clinical pharmacology; dietary; social work; psychology; a parent advisor group; occupational, speech and language therapies; and members of the infant tracheostomy and home ventilator, pulmonary hypertension and palliative care teams. This group meets biweekly to discuss each case to determine acute and long-term management.

Patient Population Characteristics

To determine whether a multidisciplinary CLD team approach can improve outcomes for this complex infant population, the Children’s Mercy Multidisciplinary CLD team recently published its experience in the Journal of Perinatology.

A total of 267 patients hospitalized from 2008 to 2018 were identified for a retrospective analysis. Patients born from 2008 to 2009 with CLD served as a reference data point but not a comparison group prior to the establishment of the CLD Multidisciplinary team in 2010.

Characteristics of this patient population:

• 56% male; 44% female
• 62.2% Caucasian; 22.8% African American; 5.7% Hispanic; 0.7% Asian; 7.5% other
• 26 weeks median gestational age at birth; mean 28.5 weeks
• 0.85 kg median birth weight; mean birth weight 1.3 kg
• 44% primary respiratory conditions (non-BPD)
• 24% extremely preterm infants (born <28 weeks’ gestation) with severe BPD
• 14% genetic conditions
• 6% cardiac anomalies
• 4% neurologic conditions
• 8% other
• Over 75% of all patients in the cohort received diuretics, systemic and inhaled steroids, and bronchodilators. Pulmonary hypertension was diagnosed in 37.2% of the patient population; 39.7% were treated with inhaled nitric oxide, and <18% required PDE5 inhibitors.⁴

Can a Multidisciplinary CLD Team Improve Outcomes?

Since 2010, mortality has improved for infants with tracheostomy cared for by the dedicated Multidisciplinary CLD team at Children’s Mercy. Most patients were categorized as having primary pulmonary and airway conditions that evolved into what is broadly considered chronic lung disease of infancy (CLDI), while one-quarter of the cohort had the strict definition of severe BPD.

The overall survival rate reported for these infants was 88.8%, with 65% of them being ventilator-dependent through a tracheostomy at the time of NICU discharge. Tracheostomy was not associated with the odds of death prior to NICU discharge. In a cohort of infants with severe BPD/CLD, secondary pulmonary hypertension was strongly associated with the need for a tracheostomy, or death, or tracheostomy and death.

Though not addressed in this study, unique to Children’s Mercy is the continuum of care that extends beyond NICU discharge for these patients via the Infant Tracheostomy and Home Ventilator Program. This neonatology-driven program, led by Winston Manimtim, MD, Neonatologist and Medical Director, is the only one in the U.S. serving as a medical home for these patients in a multidisciplinary setting with other key subspecialists, including pulmonologists, gastroenterologists and otolaryngologists.

This team’s improved survival rates in preterm infants with tracheostomy have been highlighted in other recent studies.

Improving the Odds for Technology-Dependent Infants

A recent survey done by the Children’s Hospital Neonatal Consortium found that among 31 children’s hospital NICUs, 41% had a self-designated multidisciplinary BPD team (unpublished data), indicating that more and more NICUs are realizing the benefits of implementing a consistent, coordinated multidisciplinary approach to caring for these inpatients.

This paper includes resources, such as guidelines and quality improvement projects, to help other providers investigate and develop a multidisciplinary team approach to care for this complex infant population.

To access these supplementary materials, visit https://doi.org/10.1038/s41372-021-00974-2.

Learn more about the Children’s Mercy Multidisciplinary CLD Team

Winston Manimtim, MD, Neonatologist; Medical Director, Infant Tracheostomy and Home Ventilator Program

wmmanimtim@cmh.edu

(816) 302-3592

For consults, admissions or transport call: 1 (800) GO MERCY / 1 (800) 466-3729.

References:

1. Northway WH Jr., Rosan RC, Porter DY. Pulmonary disease following respiratory therapy of hyaline-membrane disease, Bronchopulmonary dysplasia. N Engl J Med. 1967;276:357–68.

2. Doyle LW, Anderson PJ. Long-term outcomes of bronchopulmonary dysplasia. Semin Fetal Neonatal Med. 2009;14:391–5.

3. Ehrenkranz RA, Walsh MC, Vohr BR, Jobe AH, Wright LL, Fanaroff AA, et al. Validation of the National Institutes of Health consensus definition of bronchopulmonary dysplasia. Pediatrics. 2005;116:1353–60.

4. Hansen TP, Noel-MacDonnell J, Kuckelman S, Norberg M, Truog W, Manimtim W. A multidisciplinary chronic lung disease team in a neonatal intensive care unit is associated with increased survival to discharge of infants with tracheostomy. Journal of Perinatology. https://doi.org/10.1038/s41372-021-00974-2.