Virtual mask fitting in pediatric patients during COVID-19: A case series.

INTRODUCTION: The COVID-19 pandemic has been an unprecedented threat to our health care system. Clinicians had to pivot and develop creative and timely "virtual" solutions to provide clinical care. Our aim was to develop a standardized approach to virtual "mask fitting" for children who are either being initiated or are already on existing long-term ventilation (LTV) at a pediatric hospital. CASE AND OUTCOMES: We present three cases involving the care of children who required mask fitting for noninvasive ventilation (NIV). LTV team consultations were delivered via videoconference or phone. With the guidance of the respiratory therapist (RT), the family caregiver (FC) took measurements on their child using a standardized clinical approach (developed by the LTV RTs). Based on the measurements, an appropriate mask was selected. Successful mask fit was based on patient/FC reports, as well as objective leak data obtained from the NIV download data. DISCUSSION: Virtual clinics used for managing patients in our LTV program were feasible and efficient resulting in improved workflow for the RTs and convenience for patients and FCs. Patients and FCs had significantly less pressure to attend in-person clinics and expressed high satisfaction in terms of their experience and importantly, meeting respiratory care needs. Within the context of COVID-19, remote patient education and intervention can be delivered effectively, while reducing the risk of exposure from in-person visits to hospital. CONCLUSION: A virtual/telemedicine program to manage pediatric patients requiring mask fitting for LTV was a feasible option during COVID-19.

Sleep disordered breathing in infants identified through newborn screening with spinal muscular atrophy.

BACKGROUND: Spinal muscular atrophy (SMA) is a genetic disorder that may result in neuromuscular weakness and respiratory insufficiency. Gene replacement therapy has changed the trajectory of this condition, but long-term outcomes related to sleep disordered breathing are not known. METHODS: This was a retrospective review of infants with SMA identified via newborn screening who subsequently received onasemnogene abeparvovec at the Hospital for Sick Children (Ontario, Canada). Polysomnograms were conducted at the time of confirmed diagnosis as well as regularly thereafter. RESULTS: Eleven children (4 female) were identified via newborn screen (7 with 2 copies of the SMN2 gene and 4 with 3 copies of the SMN2 gene) and received onasemnogene abeparvovec at a median age of 3.6 weeks. All eleven infants met criteria for sleep disordered breathing based on their first completed polysomnograms but improved over time. Three infants required respiratory technology, including a premature infant who was prescribed nocturnal supplemental oxygen therapy for central sleep apnea and two symptomatic infants with neuromuscular weakness who required nocturnal noninvasive ventilation. We did not find a correlation between motor scores and polysomnogram parameters. CONCLUSION: Children treated with onasemnogene abeparvovec have reduced sleep disordered breathing over time. Polysomnograms revealed abnormal parameters in all children, but the clinical significance of these findings was unclear for children who were asymptomatic for sleep disordered breathing or neuromuscular weakness. These results highlight the need to evaluate both motor scores and respiratory symptoms to ensure a holistic evaluation of clinical status.

Tough transitions: Family caregiver experiences with a pediatric long-term ventilation discharge pathway.

OBJECTIVES: Discharging a child home on long-term ventilation (LTV) via tracheostomy is complex and involves multiple healthcare providers across healthcare sectors. To date, there has been a paucity of data with respect to the experiences of families transitioning a child home on LTV. Our objective was to explore the perceptions of family caregivers (FCs) who have completed a newly developed LTV discharge pathway as they transitioned home. METHODS: We conducted 11 semi-structured interviews with FCs. Interviews focused on FC's experience with the training process, perception of competency from a knowledge and skill perspective, and opportunities for improvement. Interviews were audiotaped, transcribed verbatim, coded, and analyzed using an inductive thematic analysis approach. RESULTS: Eight mothers and three fathers of ten children participated. Six primary themes were identified: (1) making an informed decision, (2) transitioning to rehabilitation, (3) building capacity for self-care, (4) coordinating case management, (5) readying for discharge home, and (6) experiencing home care. CONCLUSION: Overall, FCs felt that the preparation and transition support obtained through the application of a standardized LTV discharge pathway allowed successful attainment of new knowledge and skills necessary to care for their child with LTV at home.