Management of Critically Ill Patients With Spinal Muscular Atrophy Admitted With Acute Respiratory Failure.

BACKGROUND: Spinal muscular atrophy (SMA) is a neurodegenerative disease that results in progressive muscular atrophy and weakness. The primary cause of morbidity and mortality in these children is pulmonary disease due to poor airway clearance that leads to acute respiratory failure. There is a paucity of literature on the treatment of children with SMA and acute respiratory failure. METHODS: We performed a retrospective chart review of pediatric patients with SMA type I or II admitted to the PICU in a tertiary-care children's hospital with acute respiratory failure who required mechanical ventilation and/or aggressive airway clearance. RESULTS: The study included 300 unique encounters among 137 unique subjects. All the subjects received airway clearance at home before admission, and 257 encounters (85.7%) were supported with noninvasive ventilation (NIV) before admission. Sixty-eight subjects (49.6%) required endotracheal intubation on admission or at some point during their PICU stay. The median (interquartile range [IQR]) time to intubation was 0 (0-20) h, and the median (IQR) duration of mechanical ventilation was 2 (1-5) d on invasive mechanical ventilation with no statistical difference between type I and type II (P = .89). Of those, 65 (91.1%) were successfully extubated to NIV on the first attempt, whereas 4 subjects (5.8%) required multiple extubation attempts and 3 subjects (4.4%) required subsequent tracheostomy. For the subjects who were intubated, both PICU and hospital length of stay were longer (P < .001) when compared with the subjects managed by NIV alone. The subjects with SMA type I had a longer PICU length of stay, with a median (IQR) of 5 (3-11) d versus 4 (2-7) d (P = .002). The hospital length of stay and duration of invasive mechanical ventilation were not statistically different (P = .055 and P = .068, respectively). CONCLUSIONS: The subjects with SMA types I or II can be treated successfully with NIV and aggressive airway clearance during acute respiratory failure. Similarly, when intubation is required, successful extubation can be achieved with NIV transitional support combined with aggressive airway clearance maneuvers.

Effectiveness and Safety of Albuterol Solutions With and Without Benzalkonium Chloride.

INTRODUCTION: Continuous aerosolized ß2 agonist, namely albuterol, is the most commonly used therapy for critical asthma. Benzalkonium chloride is a preservative present in some formulations of aerosolized albuterol solutions that can induce bronchospasm. Recent studies have shown that inhalation of albuterol containing benzalkonium chloride might induce unintended bronchoconstriction and poor outcomes. This study aimed to investigate whether using albuterol solutions containing benzalkonium chloride results in prolonged hospital length of stay (LOS). METHODS: This was a retrospective cohort study of pediatric subjects admitted to the pediatric ICU (PICU) and treated with continuous albuterol. Data were collected and compared before and after a change to benzalkonium chloride-containing solutions. Subjects who were treated with preservative-free solutions were used as control. The primary outcome was PICU and hospital LOS; secondary outcomes included the duration of continuous albuterol and use of adjunctive therapies. RESULTS: A total of 266 admissions were included in the study. One hundred forty subjects (52.6%) were exposed to benzalkonium chloride. Median age and severity of illness scoring were similar between groups. The initial dose of continuous albuterol was significantly higher in the benzalkonium chloride group (median 15 interquartile range [IQR] 10-20 mg/h) compared to the preservative-free group (median 10 IQR 10-20 mg/h) (P < .001). PICU LOS was longer for the preservative-free group, 2.5 (IQR 1.4-4.6) d vs 1.8 (IQR 1.1-2.9) d for benzalkonium chloride group (P = .002). There was no significant difference in duration of continuous albuterol therapy (P = .16) or need for adjunctive respiratory support (heliox [P = .32], noninvasive ventilation [P = .81], and invasive mechanical ventilation [P = .57]). CONCLUSIONS: In contrast to published literature showing that benzalkonium chloride may be associated with a longer duration of continuous albuterol nebulization and hospital LOS, our study demonstrated that benzalkonium chloride-containing albuterol is safe for continuous nebulization in critically ill children and not associated with worse outcomes.

Feasibility of Aerosol Bronchodilators Delivery Through High-Flow Nasal Cannula in Pediatric Subjects With Respiratory Distress.

BACKGROUND: High-flow nasal cannula (HFNC) is commonly used to provide respiratory support to pediatric patients with respiratory failure. Although the use of bronchodilators via HFNC has been described, the feasibility and safety of aerosolized bronchodilator delivery via HFNC are controversial. In this study, we sought to evaluate whether the HFNC system can be used to deliver nebulized bronchodilators at lower gas flow of 2-4 L/min, increase patient comfort, and minimize respiratory therapist (RT) bedside time when compared to traditional interfaces. METHODS: A retrospective chart review of all pediatric subjects who were admitted to the pediatric ICU in a tertiary care children's hospital and required nebulized bronchodilators between December 2017 and June 2018. RESULTS: A total of 205 nebulizations were administered to 28 children; 31% of nebulized bronchodilators were given using a nebulization system integrated into the HFNC. Nebulized treatments resulted in an average increase in heart rate of 9.98 (95% CI 3.72-16.2) beats/min when HFNC was used and 0.64 (95% CI -1.65 to 2.93) beats/min when traditional interfaces were used, a difference of 9.34 (95% CI 2.30-16.4) beats/min (P < .001). RT bedside time was significantly longer for HFNC nebulized treatments (P = .031). Subjective level of comfort was not statically different when nebulized bronchodilators were delivered via HFNC or via traditional interfaces. Length of pediatric ICU stay was not statistically different between subjects who received some aerosol nebulized bronchodilators via HFNC versus those who received all bronchodilators through traditional interfaces (P = .11). CONCLUSIONS: Aerosol bronchodilator delivery using HFNC is feasible at low gas flow (ie, 2-4 L/min). However, the use of HFNC did not improve subjects' comfort, and it increased RT bedside time. Further prospective randomized studies are needed to determine the efficacy and efficiency of aerosol therapy delivered through HFNC and potential patient-oriented outcomes.