Outcomes of Children With Severe Bronchopulmonary Dysplasia Who Were Ventilator Dependent at Home

Abstract

WHAT'S KNOWN ON THIS SUBJECT: Respiratory outcomes of patients with bronchopulmonary dysplasia (BPD) range from no oxygen requirement to chronic respiratory failure. Outcomes of least severe types of BPD are well described. Limited data exist on outcomes of patients with BPD-related chronic ventilator dependency. WHAT THIS STUDY ADDS:Along with a first estimation of the incidence of patients with severe BPD-related chronic respiratory failure who were dependent on positive pressure ventilation via tracheostomy at home, we describe their survival rate, liberation from positive pressure ventilation, and decannulation. abstract OBJECTIVE: To describe the incidence and outcomes of children with chronic respiratory failure secondary to severe bronchopulmonary dysplasia (BPD) on chronic positive pressure ventilation (PPV) via tracheostomy at home. METHODS:We retrospectively reviewed medical charts of patients with severe BPD who were PPV dependent at home and who were enrolled in a university-affiliated home ventilator program between 1984 and 2010. We excluded patients with other comorbidities that could contribute to the development of chronic respiratory failure. We reported the incidence of these children in Indiana and cumulative incidences of survival, liberation from PPV, and decannulation.RESULTS: Over 27 years, 628 children were cared for in our home ventilator program. Of these, 102 patients met inclusion criteria: 83 (81.4%) were alive and 19 (18.6%) were deceased. Sixty-nine patients (67.6%) were liberated from PPV, and 97.1% of them were weaned before their fifth birthday, with a median age at liberation of 24 months (interquartile range, 19-33). Similarly, 60 patients (58.8%) were decannulated, of which 96.7% completed this process before their sixth birthday, with a median age at decannulation of 37.5 months (interquartile range, 31.5-45). The incidence of children with chronic respiratory failure secondary to BPD who were PPVdependent at home in Indiana was 1.23 per 100 000 live births in 1984 and increased to 4.77 per 100 000 live births in 2010.CONCLUSIONS: Although extreme prematurity associated with severe BPD necessitating PPV at home carries significant risks of morbidity and mortality, successful liberation from mechanical ventilation and decannulation are likely to occur. Pediatrics 2013;132:e727-e734