Sleep disordered breathing assessment in patient with slowly progressive neuromuscular disease.

BACKGROUND: Sleep-disordered breathing (SDB) is common in patients with neuromuscular diseases (NMD). Focusing on hypercapnia may lead to the neglect of other SDB such as obstructive and/or central sleep apnea syndrome (SAS). Our objectives were to assess the risk of inappropriate SDB management according to different screening strategies and to evaluate the prevalence and determinants of isolated and overlapping sleep apnea in patients with slowly progressive NMD. METHODS: This monocentric, cross-sectional, retrospective study analyzed medical records of adult NMD patients referred to a sleep department. Diagnostic strategies, including respiratory polygraphy (RP), nocturnal transcutaneous capnography (tcCO2), and blood gases (BG), were assessed for their performance in diagnosing SDB. Demographics and pulmonary function test results were compared between patients with or without SDB to identify predictors. RESULTS: Among the 149 patients who underwent a full diagnostic panel (RP + tcCO2 + BG), 109 were diagnosed with SDB. Of these, 33% had isolated SAS, and central apneas were predominant. Using single diagnostic strategies would lead to inappropriate SDB management in two thirds of patients. A combination of 2 diagnostic tools resulted respectively in 21.1, 22.9 and 42.2 % of inappropriate SDB management for RP + tcCO2, RP + BG and tcCO2 + BG. CONCLUSION: The significant prevalence of sleep apnea syndrome in patients with slowly progressive NMD highlights the need for increased awareness among clinicians. Improved diagnostics involve a systematic approach addressing both sleep apnea and diurnal and nocturnal alveolar hypoventilation to avoid inappropriate management and limit the consequences of SDB.

Adherence to continuous positive airway pressure therapy in patients with spinal cord injury and obstructive sleep apnea: trajectories and predictors.

PURPOSE: To identify specific determinants of non-adherence or cessation of continuous positive airway pressure (CPAP) therapy in a population of patients with spinal cord injuries (SCI). METHODS: Retrospective analysis of data from patients with SCI who underwent a full night supervised polysomnography between 2015 and 2021 and presented with moderate to severe obstructive sleep apnea (OSA) and for whom CPAP was indicated. Adherence was studied at 1, 6, and 12 months. Univariate and multivariate analyses were performed to identify factors associated with non-adherence (< 4 h per night or CPAP cessation). Factors studied were demographic and disease-related data and both subjective and objective sleep parameters. RESULTS: A total of 60 patients were included (40% cervical SCI). In univariate analysis, the only predictive parameters of non-adherence observed at 1, 6, and 12 months were the average use of CPAP on the 1st night (p = 0.02) and over the 1st week (p ≤ 0.001). A complete lesion (AIS-A) was predictive of non-adherence at 1 and 6 months (p = 0.02 at 6 months), while mask leakage was associated with non-adherence at 12 months (p = 0.02). Upper limb autonomy and the presence of family caregivers did not appear to be protective. In multivariate analysis, only the average use in the first week remained predictive of adherence (> 4 h) in the short, medium and long term. CONCLUSION: In patients with SCI and OSA, the 1st week of CPAP treatment seems to be determinant of short-, medium-, and long-term CPAP adherence. Support for SCI patients from the start of treatment is essential and may help avoid treatment failures.

Expiratory obstruction in patients with Duchenne muscular dystrophy under non-invasive ventilation: A step-by-step analysis of a new obstructive pattern.

PURPOSE: Non-invasive ventilation (NIV) is the reference standard for managing chronic hypoventilation in patients with Duchenne muscular dystrophy (DMD). In these patients, upper airway obstruction under NIV may compromise efficacy and adherence. We aim to describe a novel pattern of expiratory obstructive events occurring during nocturnal barometric NIV. METHODS: We retrospectively included all patients with DMD who underwent full-night polygraphy during NIV as part of their usual follow-up between May 2018 and July 2019. RESULTS: We provide a step-by-step description of this previously undescribed pattern of obstruction. Expiratory obstructions lead to end-inspiratory breath-holding and impossibility to take another inspiratory breath with a barometric mode until expiration occurs. These events were observed in 4 (36%) of 11 DMD patients under barometric NIV. CONCLUSION: Expiratory obstructions may be common in DMD patients receiving NIV and should be sought out routinely. This previously undescribed variant of obstructive event must be identified.

Can the analysis of built-in software of CPAP devices replace polygraphy in children?

OBJECTIVES: Polysomnography (PSG) is the gold standard for the scoring of residual respiratory events during continuous positive airway pressure (CPAP). Studies comparing PSG scoring with automatic scoring by the built-in software of CPAP devices have reported acceptable agreements except for the hypopnea index (HI) in adult patients, but no study has yet been conducted in children. The aim of the present study was to compare the automatic scoring by CPAP device and manual scoring using the software tracings of the CPAP device integrating pulse oximetry (SpO2) with in-lab polygraphy (PG). METHODS: Consecutive clinically stable children treated with constant CPAP (ResMed) for at least one month and scheduled for a nocturnal PG were recruited. A pulse oximeter was connected to the CPAP device. The PG apnea-hypopnea index (AHIPG), scored according to modified AASM guidelines, was compared with the automatic AHI reported by the CPAP device (AHIA CPAP) and the manual scoring of the AHI on the CPAP software (AHIM CPAP). RESULTS: Fifteen children (1.5-18.6 years) were included. Mean residual AHIPG was 0.9 ± 1.2/hour (0.0-4.6/hour) vs. AHIA CPAP of 3.6 ± 3.6/hour (0.5-14.7/hour) (p < 0.001), and AHIM CPAP of 1.2 ± 1.6/hour (0.0-5.1/hour) (p = 0.01). Correlation between AHIPG and AHIA CPAP was good (r = 0.667; p = 0.007), and improved when considering AHIM CPAP (r = 0.933; p < 0.001). Strong correlations were also observed between the PG apnea index (AI) and HI, and the manually scored AI and HI on CPAP, respectively. CONCLUSIONS: Manual scoring of respiratory events on the built-in software tracings of CPAP devices integrating SpO2 signal may be helpful. These results have to be confirmed in patients with higher AHI.

Evaluation of ventilators for mouthpiece ventilation in neuromuscular disease.

BACKGROUND: Daytime mouthpiece ventilation is a useful adjunct to nocturnal noninvasive ventilation (NIV) in patients with neuromuscular disease. The aims of the study were to analyze the practice of mouthpiece ventilation and to evaluate the performance of ventilators for mouthpiece ventilation. METHODS: Practice of mouthpiece ventilation was assessed by a questionnaire, and the performance of 6 home ventilators with mouthpiece ventilation was assessed in a bench test using 24 different conditions per ventilator: 3 mouthpieces, a child and an adult patient profile, and 4 ventilatory modes. RESULTS: Questionnaires were obtained from 30 subjects (mean age 33 ± 11 y) using NIV for 12 ± 7 y. Fifteen subjects used NIV for > 20 h/day, and 11 were totally ventilator-dependent. The subject-reported benefits of mouthpiece ventilation were a reduction in dyspnea (73%) and fatigue (93%) and an improvement in speech (43%) and eating (27%). The bench study showed that none of the ventilators, even those with mouthpiece ventilation software, were able to deliver mouthpiece ventilation without alarms and/or autotriggering in each condition. Alarms and/or ineffective triggering or autotriggering were observed in 135 of the 198 conditions. The occurrence of alarms was more common with a large mouthpiece without a filter compared to a small mouthpiece with a filter (P < .001), but it was not related to the patient profile, the ventilatory mode, or the type of ventilator. CONCLUSIONS: Subjects are satisfied with mouthpiece ventilation. Alarms are common with home ventilators, although less common in those with mouthpiece ventilation software. Improvements in home ventilators are needed to facilitate the expansion of mouthpiece ventilation.

Continuous positive airway pressure and noninvasive ventilation adherence in children.

BACKGROUND: Adherence to continuous positive airway pressure (CPAP) and noninvasive ventilation (NIV) is crucial for the successful treatment of sleep-disordered breathing. The aim of our study was to analyze the adherence of children to long-term home CPAP/NIV treatment. METHODS: We analyzed data from all consecutive patients older than the age of 2years, in whom CPAP/NIV treatment was initiated in a specialized pediatric NIV and in those who were receiving CPAP/NIV treatment at home for at least 1month. Data of the memory cards of the ventilators and nocturnal gas exchange were analyzed during a routine CPAP/NIV overnight control in the hospital. CPAP/NIV adherence during the previous month was analyzed according to patient's age, ventilatory mode, type of interface, nocturnal gas exchange, and duration of treatment. RESULTS: The data of 62 children (mean age, 10±5years) with obstructive sleep apnea (n=51) treated with CPAP and neuromuscular disease (n=6) or lung diseases (n=5) treated with NIV were analyzed. Mean adherence was 8:17±2:30h:min per night, and the results did not significantly differ between CPAP and NIV adherence. Seventy-two percent of the patients used their device >8h per night. The mean number of nights of CPAP/NIV use during the last month was 26±5 nights per month. Treatment adherence was not correlated to age, the type of underlying disease, the type of interface (nasal, facial mask, or nasal cannula), nocturnal gas exchange, and duration of CPAP/NIV treatment. CONCLUSION: Long-term CPAP/NIV adherence at home was extremely high in this group of children followed in a pediatric NIV unit. This finding may explain the lack of effect of the interface, nocturnal gas exchange, and duration of CPAP/NIV treatment.

Harms of unintentional leaks during volume targeted pressure support ventilation.

BACKGROUND: Volume targeted pressure support ventilation (VT-PSV) is a hybrid mode increasingly used to maintain a minimal tidal volume (VT) by automatically adjusting the level of inspiratory pressure. The objective of the study was to determine the ability of home ventilators to maintain the preset minimal VT during unintentional leaks in a VT-PSV mode. METHODS: Seven ventilators were tested on a lung bench with different circuit configurations and with different levels of unintentional leaks. Unintentional leaks were generated using calibrated holes. RESULTS: All the studied ventilators with a single-limb circuit with intentional leak (n = 5) were able to maintain the minimal preset VT during unintentional leaks. One ventilator overcompensated VT during unintentional leaks of high intensity. In contrast, all studied ventilators with a single circuit with an expiratory valve (n = 2) or a double-circuit (n = 3) but one failed to maintain the minimal VT during unintentional leaks. Unintentional leaks generated a decrease in inspiratory pressure, which was responsible for the fall in VT. CONCLUSIONS: Most of the studied ventilators with a single-limb circuit with intentional leak correctly estimate the expiratory VT and therefore successfully maintain the preset minimal VT during unintentional leaks, in contrast to most of the studied ventilators with a double-circuit, which paradoxically are not able to directly measure expiratory VT. Importantly, the VT-PSV mode, when used with most ventilators with expiratory valve or double-circuit, can paradoxically exacerbate the VT drop during unintentional leaks.

Medical hypnosis as a tool to acclimatize children to noninvasive positive pressure ventilation: a pilot study.

BACKGROUND: Patient cooperation is crucial for the success of noninvasive positive pressure ventilation (NPPV). This study evaluated the efficacy of medical hypnosis to reduce anticipatory anxiety and acclimatization time in children who are candidates for long-term NPPV. METHODS: Medical hypnosis was performed by a trained nurse. The acclimatization time and long-term compliance with NPPV were evaluated. RESULTS: Hypnosis was performed in nine children aged 2 to 15 years. Seven children had a high level of anticipatory anxiety because of a tracheotomy since birth (n=2), a history of maxillofacial surgery (n=2), severe dyspnea because of lung disease (n=2), and morbid obesity and depression (n=1), and two children with obstructive sleep apnea failed standard NPPV initiation. The hypnosis techniques were based on distraction in the youngest patient and indirect or direct hypnotic suggestions in the older children to obtain a progressive psychocorporal relaxation. All patients accepted the interface and the NPPV after the first hypnosis session. A median of three sessions was needed for overnight (>6 h) NPPV acceptance. The 6-month compliance with NPPV was excellent, with a median use of 7.5 h per night. CONCLUSION: Medical hypnosis is an effective, safe, noninvasive, and inexpensive tool for reducing the anticipatory distress and acclimatization time for NPPV. This therapy is particularly useful in children with traumatic experiences, such as a tracheotomy or facial surgical procedures.

Nocturnal mouthpiece ventilation and medical hypnosis to treat severe obstructive sleep apnea in a child with cherubism.

A 4-year old boy presented severe obstructive sleep apnoea due to complete nasal obstruction secondary to cherubism. Because of anticipatory anxiety due to numerous surgical interventions, medical hypnosis was proposed to facilitate non-invasive continuous positive pressure ventilation (CPAP) acceptance. CPAP by means of an oral interface was completely accepted after three hypnosis sessions and resulted in the correction of his obstructive sleep apnea (OSA) syndrome. This report highlights the benefit of medical hypnosis in facilitating CPAP acceptance as well as the efficacy of mouthpiece ventilation in a severe form of cherubism with complete nasal obstruction.

Assessment of sleep quality by pulse wave amplitude and actigraphy in children with sleep-disordered breathing: evaluation at diagnosis and under non-invasive ventilation.

PURPOSE: The aims of this study were to assess the interest of pulse wave amplitude (PWA) and actigraphy for characterizing sleep in children with sleep-disordered breathing and to evaluate PWA and actigraphy to assess the efficacy of non-invasive positive pressure ventilation (NPPV). METHODS: We performed a retrospective analysis of children with sleep-disordered breathing. Patients were classified to upper airway obstructive disease (UAO) group or non-obstructive disease (non-UAO) group. Pulse oximetry (SpO2) and PWA were measured by photoplethysmography. Autonomic micro-arousals (AA) and AA related to SpO2 desaturations above 4 % (AA + DS4%) were quantified. The fragmentation index, sleep efficiency, sleep duration, and sleep latency were measured with actigraphy. Transcutaneous carbon dioxide (PtcCO2) was monitored. NPPV was started in case of severe OSA. RESULTS: AA + DS4% were more common in the UAO (n = 15) than the non-UAO group (n = 13) (p < 0.001). All nocturnal gas exchange parameters were worse in the UAO group. Eight children required NPPV. AA + DS4%, maximal PtcCO2, percent of time with PtcCO2 > 50 mmHg, and percent of time with SpO2 < 90 % decreased significantly after 1 month of NPPV. CONCLUSIONS: The analysis of AA + DS4% is very informative for the grading of the severity of OSA and for the efficacy of NPPV in children with sleep-disordered breathing.

Interfaces for long-term noninvasive positive pressure ventilation in children.

OBJECTIVE: The aim of the study was to report the type and tolerance of the interface chosen for long-term noninvasive positive pressure ventilation (NPPV) in children. METHODS: This was a descriptive study carried out in the clinical setting of a pediatric university hospital in which all children started on long-term NPPV over a 18-month period were included. RESULTS: NPPV was started in 97 children with neuromuscular disease or thoracic scoliosis (n = 35), obstructive sleep apnea with (n = 32) or without (n = 21) maxillofacial deformity, or lung disease (n = 9). All 25 children ≤ 2 years of age, as well as four older children, were fitted with custom-made nasal masks; all other children were fitted with an industrial nasal mask (50%), a facial mask (16%), or nasal prongs (2%). Industrial masks with and without manufactured leaks were used in 33 (34%) and 35 (36%) children, respectively. All patients with obstructive sleep apnea used interfaces with manufactured leaks, whereas all patients with neuromuscular disease or thoracic scoliosis used interfaces without manufactured leaks. Both types of interfaces were used in patients with lung disease. The interface had to be changed in 20 patients because of discomfort (n = 16), leaks (n = 4), facial growth (n = 3), skin injury (n = 2), or change of the ventilatory mode (n = 2). A second or third mask change was necessary in nine and four patients, respectively. CONCLUSION: The choice of the interface for NPPV in children is determined by the patient's age and the underlying disease. Discomfort is the main reason for mask change.