Implementation of Spinal Muscular Atrophy Pathway Reduces Length of Stay

Background: Patients with spinal muscular atrophy (SMA) have compromised respiratory muscle function which results in impaired cough, hypoventilation during sleep, and chest wall deformity. We hypothesized that a multidisciplinary pathway to standardize the management of SMA patients with acute respiratory failure would assist in standardizing care and reduce length of stay. Methods: In 2017, a multidisciplinary team consisting of respiratory therapists, nurses, critical care physicians, and pulmonologists developed a pathway for the management of SMA patients with acute respiratory failure admitted to the pediatric intensive care unit (PICU). This comprehensive pathway included ventilator strategies, weaning practices, airway clearance regimens, nutrition guidance, and sedation recommendations. Interventions and clinical supports were utilized to increase pathway use including thorough formal pathway training, order set deployment, newsletter updates, and presentations at meetings held by all disciplines. Chart review of patients admitted with SMA to the PICU was completed from 2016-2021, including length of stay. The pathway was reviewed, and minimal updates were added in 2019. The project was exempt by the local Institutional Review Board. Results: Since pathway initiation, 90 patients with SMA were admitted to the PICU. 47/90 (52%) were treated via the pathway as determined by order set usage. Time periods for PICU length of stay pre and post pathway initiation were compared for patients who qualified for the pathway due to respiratory failure. The pathway did reduce LOS by 1.157 d (P = 0.853) but the difference was not significantly significant. Conclusions: Utilization of a pathway to standardize and streamline the management of patients with SMA in the PICU minimally reduces length of stay. The PICU will need to increase utilization of the pathway and need a larger patient pool to determine if there is a significant impact of pathway utilization on LOS.