Volume-assured pressure support mode for noninvasive ventilation: can it improve overnight adherence in children with neuromuscular disease?

PURPOSE: Volume-assured pressure support in noninvasive ventilation (VAPS-NIV) is a newer mode providing automatic pressure support adjustment to ensure a constant alveolar ventilation. Previous studies have shown that NIV effectiveness depends on patient adherence and tolerance. The aim of this study was to determine the adherence and efficacy of VAPS-NIV compared to spontaneous-time (S/T) mode in pediatric patients with neuromuscular disease (NMD). METHODS: This was a prospective observational study. Children with NMD who utilized NIV at home for ≥ 3 months were recruited from the Long-term ventilation clinic at The Hospital for Sick Children, Toronto, Canada, from July 1, 2015, to July 1, 2019. Baseline characteristics, date of initiation of NIV, and pulmonary function tests were recorded. Polysomnogram (PSG) data and adherence were recorded and analyzed comparing VAPS and S/T modes. RESULTS: Twenty children with NMD (17 male, 85%) were enrolled. The mean (SD) age at initiation of NIV was 11.6 ± 4.6 years. The median (IQR) duration of ventilation was 1.36 (0.80-2.98) years. The mean average daily usage and the median daily usage for VAPS mode and S/T mode were 8.4 ± 1.6 versus 7.2 ± 2.5 h (p = 0.012) and 8.6 ± 1.4 versus 7.8 ± 2.1 h (p = 0.022), respectively. There was no difference in sleep architecture, gas exchange, or parent proxy report of NIV tolerance between S/T and VAPS modes. CONCLUSION: VAPS was associated with an improvement in adherence to therapy in children with NMD compared to S/T mode. Longitudinal studies are required to evaluate long-term clinical outcomes using VAPS mode in children with NMD.

Intelligent volume-assured pressured support (iVAPS) for the treatment of congenital central hypoventilation syndrome.

PURPOSE: Congenital central hypoventilation syndrome (CCHS) is characterized by ventilatory insensitivity to hypercapnia and hypoxemia during sleep and/or wakefulness. Management of CCHS includes a long-term ventilation. However, ventilation can be challenging given differences in the control of breathing during different sleep stages. Intelligent volume-assured pressure support (iVAPS) is a mode of Bi-level positive airway pressure (BPAP) ventilation in which the pressure support is modulated to ensure a constant alveolar ventilation. The aim of this study was to determine if BPAP with iVAPS mode is more effective at controlling hypercapnia than BPAP with spontaneous/timed (S/T) mode. METHODS: A retrospective chart review of CCHS patients who underwent both a titration polysomnogram (PSG) with standard BPAP S/T mode and a consecutive follow-up study with BPAP iVAPS mode at The Hospital for Sick Children, Toronto, Canada, between January 1, 2013 and September 30, 2015 were included. Comparisons were made between S/T mode and iVAPS mode. RESULTS: Eight (four males) children with CCHS were included. The median (IQR) age at the time of PSG using Bi-level ventilation with S/T mode for study participants was 10.0 (IQR 8.4, 11.6) years followed by PSGs with iVAPS mode, median age 10.6 (IQR 9.1, 12.5) years. The non-rapid eye movement (NREM) peak transcutaneous CO2 (tcCO2) median (IQR) for iVAPS was 43.0 (40.0-46.0-) mmHg versus 46.5 (45.0-48.0) mmHg for S/T mode, (p value <0.05). CONCLUSION: iVAPS was associated with a reduction in the maximum tcCO2 during NREM sleep as compared to traditional S/T mode. Prospective, longitudinal studies are needed to evaluate the benefits of BPAP therapy iVAPS mode for the treatment of pediatric CCHS.

An Adverse Reaction in the Pediatric Sleep Laboratory.

We present a case of a 15-month-old boy with Cornelia de Lange Syndrome (NIPBL gene mutation). On a PSG, central sleep apnea (central apnea-hypopnea index of 19/hour) and nocturnal hypoventilation (transcutaneous CO2 > 50 mmHg for 53% of the night) were found. A positive pressure initiation study was aborted because the patient developed a serious adverse reaction. The differential diagnosis included a skin fragility condition versus an allergic contact dermatitis to the interface; this could be from the povidone-iodine solution used to clean the NiPPV interface or from the plastic of the interface itself. A skin biopsy was performed which was normal. The reaction was likely secondary to an allergic contact dermatitis from the povidone-iodine solution used to clean the NiPPV interface. The patient is currently tolerating NiPPV.