There have been no published reports of central respiratory control abnormalities in pediatric patients with UNC80 or KCNJ11 mutations which cause neurologic channelopathies. We describe an 8-year-old male with a pathogenic UNC80 mutation, intellectual disability, hypotonia and epilepsy with severe central sleep apnea (213.5 events/h) on polysomnography (PSG). We also describe a 20-month-old female with a KCNJ11 mutation, neonatal diabetes and developmental delay who had severe central sleep apnea (131.1 events/h). Both patients had irregular respiratory patterns during sleep and wakefulness and were placed on empiric bilevel positive airway pressure therapy, which was well tolerated with resolution of abnormal respiratory control and hypercapnia. Patients with UNC80 and KCNJ11 gene mutations may have abnormal respiratory rhythm during sleep and wakefulness, mirroring animal models. We recommend routine PSG tests and further investigation into the respiratory control of patients with pediatric channelopathies involved in chemoreceptor function or central integration of respiratory control.

Introduction

Live video visits for ambulatory encounters offer potential benefits, including access to remote subspecialty services, care coordination between providers, and improved convenience for patients. We aimed to increase the utilization of video visits for pediatric patients at our medical center using an iterative quality improvement process.

Methods

A multispecialty improvement team identified opportunities to increase video visit utilization and prioritized interventions using benefit-effort analyses. Interventions focused on 6 key drivers. The outcome measure was the percentage of ambulatory encounters conducted by video. The process measure was the percentage of ambulatory pediatricians conducting video visits. The balancing measure was the percentage of no-shows among scheduled video visits. All measures were analyzed using statistical process control.

Results

Interventions were associated with increases in our outcome and process measures from 0.1% to 1.2% and 0.6% to 6.3%, respectively, during the first 8 months. Subsequently, the novel coronavirus (COVID-19) pandemic was associated with further increases in these measures to 41.8% and 73.5%, respectively, over 3 months. The balancing measure increased from 0% at baseline to 14.7% with no special cause variation during the intervention period. The most impactful interventions included clinician training outreach, providing equipment, and streamlining MyChart patient enrollment.

Conclusions

This improvement project effectively increased pediatric ambulatory video visit utilization, although the most significant driver of utilization was the COVID-19 pandemic. Project interventions implemented before COVID-19 facilitated rapid video visit adoption during the pandemic. A similar improvement process may be beneficial for other medical centers aiming to improve video visit utilization.