ObjectivesTo describe the current clinical practice patterns of Canadian pediatric respirologists... more ObjectivesTo describe the current clinical practice patterns of Canadian pediatric respirologists at pediatric tertiary care institutions regarding chronic tracheostomy tube care and management of home invasive ventilation.MethodsA pediatric respirologist/pediatrician with expertise in tracheostomy tube care and home ventilation was identified at each Canadian pediatric tertiary care center to complete a 59‐item survey of multiple choice and short answer questions. Domains assessed included tracheostomy tube care, caregiver competency and home monitoring, speaking valves, medical management of tracheostomy complications, decannulation, and long‐term follow‐up.ResultsThe response rate was 100% (17/17) with all Canadian tertiary care pediatric centers represented and heterogeneity of practice was observed in all domains assessed. For example, though most centers employ Bivona™ (17/17) and Shiley™ (15/17) tracheostomy tubes, variability was observed around tube change, re‐use, and clea...
Obstructive sleep apnea syndrome is a major cause of morbidity in the Down syndrome population an... more Obstructive sleep apnea syndrome is a major cause of morbidity in the Down syndrome population and is commonly treated with adenoidectomy and/or tonsillectomy (AT). However, these children are at increased risk for perioperative respiratory adverse events (PRAEs). The objective of this study was to examine risk factors for major PRAEs requiring intervention in children with Down syndrome undergoing AT and to describe their postoperative monitoring environment. This retrospective study included all children with Down syndrome aged 0–18 years who underwent a preoperative polysomnogram followed by AT at a tertiary pediatric institution. Descriptive statistics were used to summarize baseline demographic and clinical characteristics. A multivariable model for prediction of PRAEs was constructed. A priori, it was decided that minimum oxygen saturation, apnea–hypopnea index, and average oxygen saturation asleep would be included, along with medical comorbidities associated with PRAEs at p < 0.2 in univariable analyses. Fifty-eight children were included in this study; twelve had a PRAE. Cardiac disease was associated with PRAEs on univariable analysis (p = 0.03). In multivariable analysis, average oxygen saturation asleep was associated with PRAEs (OR 1.50; 95% confidence interval 1.00, 2.41; p = 0.05). For all of the remaining variables, p > 0.15. Fifty-six children were admitted for monitoring overnight; four were admitted to the intensive care unit and fifty-two were admitted to the ward. Conclusions: A multivariable model found evidence that lower average oxygen saturation while asleep was associated with PRAEs requiring intervention in children with Down syndrome. This study highlights the difficulty in predicting complications in this population. What is known: • Obstructive sleep apnea syndrome is a major cause of morbidity in the Down syndrome population and is commonly treated with adenoidectomy and/or tonsillectomy. • However, children with Down syndrome are at increased risk for perioperative respiratory adverse events (PRAEs) following adenoidectomy and/or tonsillectomy. What is new: • We found that a lower average oxygen saturation asleep is associated with increased odds of PRAEs, adjusting for age, total apnea–hypopnea index, cardiac comorbidity, and minimum oxygen saturation. • This study highlights the difficulty in predicting complications in this population. What is known: • Obstructive sleep apnea syndrome is a major cause of morbidity in the Down syndrome population and is commonly treated with adenoidectomy and/or tonsillectomy. • However, children with Down syndrome are at increased risk for perioperative respiratory adverse events (PRAEs) following adenoidectomy and/or tonsillectomy. What is new: • We found that a lower average oxygen saturation asleep is associated with increased odds of PRAEs, adjusting for age, total apnea–hypopnea index, cardiac comorbidity, and minimum oxygen saturation. • This study highlights the difficulty in predicting complications in this population.
STUDY OBJECTIVES Despite the importance of treating sleep-disordered breathing (SDB), positive ai... more STUDY OBJECTIVES Despite the importance of treating sleep-disordered breathing (SDB), positive airway pressure (PAP) adherence rates in children are low. Identifying readily-available predictors of non-adherence would enable the development of targeted interventions and supports, but literature is limited. Our objective was to identify baseline clinical predictors of six-month PAP therapy non-adherence in children with SDB through a retrospective cohort study. METHODS This study evaluated children (8-17 years) prescribed PAP therapy for SDB between 2011-2017 at a single pediatric tertiary hospital. The primary outcome was non-adherence at six months, measured using both machine downloads and self-report. Candidate baseline predictors included demographics, comorbidities, and SDB characteristics. Relative risks (RR) and 95% confidence intervals (95%CI) were estimated using a modified Poisson regression. Missing data were imputed prior to analysis. RESULTS The study included 104 children. The independent predictors most strongly associated with greater non-adherence were older age (RR=1.08 for a one year increase, 95%CI=1.00-1.16) and higher oxygen saturation nadir (RR=1.03 for a 1% increase, 95%CI=1.00-1.05), while those most strongly associated with lower non-adherence were higher arousal index (RR=0.97 for a one event/hour increase, 95%CI=0.95-1.00), developmental delay (RR=0.58, 95%CI=0.30-1.13), and asthma (RR=0.72, 95%CI=0.44-1.17). CONCLUSIONS Overall, children who are older, have less severe SDB, or less disrupted sleep at baseline are more likely to be non-adherent to PAP therapy, and may benefit from additional supports to acclimatize to therapy. As clinical predictors were only weakly associated with non-adherence, non-clinical characteristics may play a larger role in predicting adherence.
STUDY OBJECTIVES Positional obstructive sleep apnea (POSA) is a phenotype of obstructive sleep ap... more STUDY OBJECTIVES Positional obstructive sleep apnea (POSA) is a phenotype of obstructive sleep apnea (OSA) where sleep-related obstructive events occur predominantly in supine position. Limited knowledge exists regarding the presence of POSA in children with obesity. The study objective was to determine the prevalence of POSA while identifying factors associated with POSA in children with obesity. METHODS This was a cross-sectional study of children with obesity, aged 8 to 18 years, with a diagnostic polysomnogram (PSG) between 2012 to 2019, who were referred for the evaluation of sleep-related breathing. POSA was defined as an overall obstructive apnea-hypopnea index (OAHI) ≥ 5 events per hour, and a supine OAHI to non-supine OAHI ratio of ≥2. Patient demographics, anthropometrics, and PSG data were recorded. RESULTS Of the 112 children with obesity with a diagnostic PSG, 43 (38%) had OSA. Amongst those with OSA, 25/43 (58%) had POSA (mean age 14.6±2.3 years, mean BMI 37.7±7.6 kg/m², 68% male) and 18/43 (42%) had no-POSA (mean age 13.9 ± 2.8 years, mean BMI 37.9 ± 7.2 kg/m², 78% male). Among those with POSA, 13/25 (52%) had mild OSA, 7/25 (28%) had moderate OSA and 5/25 (20%) had severe OSA. No significant differences were found in age, gender and anthropometric measures between POSA and no-POSA groups. Time spent in supine and non-supine sleep did not differ significantly between groups. CONCLUSIONS In children with obesity and OSA, POSA occurs frequently. Identifying POSA allows for potential targeted positional therapy for children with obesity.
(VA) and changes in rates of adolescent motor vehicle crashes and school night sleep duration. Cr... more (VA) and changes in rates of adolescent motor vehicle crashes and school night sleep duration. Crash rates in FC were also compared to those in the rest of the state during the same time period. Methods: VA Department of Motor Vehicles crash data (including overall crash rates, distraction, speed, and alcohol-related accidents) in 16-18 year old drivers between September and June of each year in Fairfax County versus the rest of the state were compared in the combined two year periods preceding (2013-14 and 2014-15; T1) and following (2015-16 and 2016-17; T2) school start time change in the fall of 2015. Average sleep duration was obtained from the Fairfax County Youth Survey (FCYS) a county-wide, anonymous, self-report survey administered annually to all 8th, 10th, and 12th-grade public school students. The results were also compared (pre-and post-school start time change). Results: The crash rate per 1000 in 16-18 year old licensed drivers in FC during T1 was significantly higher compared to T2, 31.63 vs 29.59 accidents per 1000 (95% CI, 1.0-1.14, OR 1.07, P¼0.03). In contrast, adolescent crash rates in the rest of Virginia were not statistically significantly different at T1 vs T2. With regards to subtypes of crashes, distractionrelated crashes were significantly higher per 1000 in FC at T1 compared to T2 at 7.01 vs 6.13 (95% CI, 0.99-1.31, OR 1.14, P¼0.05), but were not significantly different in the remainder of the state.In the T2 period in FC, 47% 12th-grade students endorsed sleeping 7 hours during school nights, which has increased by 5% (p¼0.001) after the implementation of the school start time change in the 2015-16. Conclusion: The results of this study suggests that school start time delay is associated with decreased adolescent motor vehicle crash risk, with significant implications for public health and safety.
Purpose of Review: Myotonic dystrophy type 1 (DM1) is a severe, progressive genetic disease that ... more Purpose of Review: Myotonic dystrophy type 1 (DM1) is a severe, progressive genetic disease that affects approximately 1 in 2,500 individuals globally [Ashizawa et al.: Neurol Clin Pract 2018;8(6):507–20]. In patients with DM1, respiratory muscle weakness frequently evolves, leading to respiratory failure as the main cause of death in this patient population, followed by cardiac complications [de Die-Smulders et al.: Brain 1998;121(Pt 8):1557–63], [Mathieu et al.: Neurology 1999;52(8):1658–62], [Groh et al.: Muscle Nerve 2011;43(5):648–51]. This paper provides a more detailed outline on the diagnostic and management protocols, which can guide pulmonologists who may not have experience with DM1 or who are not part of a neuromuscular multidisciplinary clinic. A group of neuromuscular experts in DM1 including pulmonologists, respiratory physiotherapists and sleep specialists discussed respiratory testing and management at baseline and during follow-up visits, based on their clinical ex...
Lung volume recruitment (LVR) is a common respiratory therapy for people living with restrictive ... more Lung volume recruitment (LVR) is a common respiratory therapy for people living with restrictive neuromuscular disease. LVR is important in acute respiratory compromise, during respiratory rehabilitation and as a preventative therapy. Cohort data associate LVR use with increased survival is diseases such as Duchenne Muscular Dystrophy.<sup>1</sup> While LVR is a commonly prescribed therapy, we have previously had to rely on self-report alone for any indication of adherence with prescribed therapy. This has significantly hampered both clinical decision making and research into effectiveness. In the paper from Canada and Australia that these data support, two large clinical services have collaborated to develop an objective LVR counter, to undertake extensive bench-testing, physiological testing, and to assess counter performance compared with diary self-report. The clinical validation was undertaken within a large, single-centre randomized controlled trial. The counter performed well and we believe the innovation will impact clinical care and research. These instructions, example output, and macro-enabled Excel spreadsheet are contined herein. 1. McKim DA, Katz SL, Barrowman N, et al. Lung Volume Recruitment Slows Pulmonary Function Decline in Duchenne Muscular Dystrophy. <em>Archives of Physical Medicine and Rehabilitation</em> 2012;93(7):1117-22.
Long-term non-invasive ventilation (NIV) is a common modality of breathing support used for a ran... more Long-term non-invasive ventilation (NIV) is a common modality of breathing support used for a range of sleep and respiratory disorders. The aim of this scoping review was to provide a summary of the literature relevant to long-term NIV use in children. We used systematic methodology to identify 11,581 studies with final inclusion of 289. We identified 76 terms referring to NIV; the most common term was NIV (22%). Study design characteristics were most often single center (84%), observational (63%), and retrospective (54%). NIV use was reported for 73 medical conditions with obstructive sleep apnea and spinal muscular atrophy as the most common conditions. Descriptive data, including NIV incidence (61%) and patient characteristics (51%), were most commonly reported. Outcomes from sleep studies were reported in 27% of studies followed by outcomes on reduction in respiratory morbidity in 19%. Adverse events and adherence were reported in 20% and 26% of articles respectively. Authors reported positive conclusions for 73% of articles. Long-term use of NIV has been documented in a large variety of pediatric patient groups with studies of lower methodological quality. While there are considerable data for the most common conditions, there are fewer data to support NIV use for many additional conditions.
Respiratory management of Duchenne muscular dystrophy (DMD) is not well studied and may vary acro... more Respiratory management of Duchenne muscular dystrophy (DMD) is not well studied and may vary across centers and practitioners. Our objective was to describe and compare the respiratory management practices of Canadian Pediatric Respirologists and Neuromuscular specialists for children with DMD. Methods: A web-based survey was sent to all 56 practicing Canadian Pediatric Respirologists and to all 24 members of the Canadian Pediatric Neuromuscular Group (CPNG) who follow children with neuromuscular diseases. The survey included 28 questions about timing and indications for respiratory consultation, sleep disordered breathing (SDB) assessments, and treatments. Results: Thirty eight (68%) pediatric respirologists and 17 (71%) CPNG members responded. Respirologists provide initial consultation after a patient's first admission to hospital with respiratory complications (14/38, 37%) and when symptoms of SDB are present (14/38, 37%). Half of the CPNG members request initial Respirology consultation at the time of DMD diagnosis. Both groups request routine pulmonary function tests. Ninety-six percent of respirologists use maximal inspiratory (MIP) and expiratory pressures (MEP) to assess respiratory muscle strength, whereas 82% of CPNG members additionally use peak cough flow. Assessment for SDB is requested by both groups when pulmonary function is abnormal or patients are symptomatic. Respirologists favor polysomnography, whereas CPNG members use overnight pulse oximetry. Nocturnal non-invasive ventilation and lung volume recruitment (LVR) are used in a minority of patients. Conclusions: Respirologists and CPNG members provide similar respiratory management of DMD patients, but differ in timing of consultation and choice of tests for pulmonary function and SDB. Canadian practices differ from the American Thoracic Society and Centre for Disease Control guidelines.
Increased neck circumference is a risk factor for obstructive sleep apnea in adults. With rising ... more Increased neck circumference is a risk factor for obstructive sleep apnea in adults. With rising obesity prevalence in children, it may be an important identifier of obstructive sleep apnea in children. The reliability of measuring neck circumference in children has not been systematically evaluated. To determine the inter- and intra-rater reliability of neck circumference measurements in children aged 2-16 years. Children aged 2-16 years with limb fractures were recruited. Neck circumference was measured by three investigators each using two separate unmarked paper tapes in the 2-5 year age group (N = 43), and three separate tapes in the 6-10 and 11-16 year age groups (N = 18 and 40). Neck circumference measurements showed excellent inter-rater reliability for children 6-10 and 11-16 years (ICC = 0.952 and 0.989). Substantial variation was observed for the 2-5 year age group (ICC = 0.701). Good intra-rater reliability was demonstrated for the three groups (ICC range: 0.776, 0.950). Repeatability coefficients were 2.5-3.4 cm in the youngest age group and were 1.2-1.4 cm in the 6-16 year age group. In children 6-16 years old, neck circumference shows very good inter and good intra-rater reliability. Multiple measurements are not required for precision and reliability.
Obese youth with sleep-disordered breathing are treated with positive airway pressure to improve ... more Obese youth with sleep-disordered breathing are treated with positive airway pressure to improve sleep and cardiovascular status. While improvements in sleep parameters have been confirmed, a study by Katz et al. showed no major improvement in ambulatory blood pressure. The aim of this ancillary study was to analyze short-term blood pressure variability, following positive airway pressure treatment, as a more sensitive marker of cardiovascular health. We analyzed 24-h blood pressure variability data in 17 children, taken at baseline and after 12 months of treatment. These data were derived from an already published prospective, multicenter cohort study conducted in 27 youth (8–16 years) with obesity who were prescribed 1-year of positive airway pressure for moderate–severe sleep-disordered breathing. Significant decreases were found in 24 h systolic blood pressure (p = 0.040) and nighttime diastolic blood pressure (p = 0.041) average real variability, and diastolic blood pressure (p = 0.035) weighted standard deviation. Significant decreases were noted in nighttime diastolic blood pressure time rate variability (p = 0.007). Positive airway pressure treatment resulted in a significant decrease in blood pressure variability, suggesting a clinically significant improvement of sympathetic nerve activity in youth with obesity and sleep-disordered breathing. Cardiovascular variability, as measured by blood pressure variability, is improved in children following positive airway pressure treatment. Our novel findings of improved blood pressure time rate variability are the first described in the pediatric literature. Future studies aimed at analyzing target organ damage in this patient population will allow for a better understanding as to whether alterations in blood pressure variability translate to decreasing target organ damage in children, as seen in adults. Cardiovascular variability, as measured by blood pressure variability, is improved in children following positive airway pressure treatment. Our novel findings of improved blood pressure time rate variability are the first described in the pediatric literature. Future studies aimed at analyzing target organ damage in this patient population will allow for a better understanding as to whether alterations in blood pressure variability translate to decreasing target organ damage in children, as seen in adults.
Objective: To determine the scope of sleep concerns, clinical features and polysomnography result... more Objective: To determine the scope of sleep concerns, clinical features and polysomnography results and to identify factors that predict obstructive sleep apnea (OSA) in a cohort of children with obesity. Methods: The study was a multi-center retrospective chart review. Data were collected from 3 pediatric sleep laboratories over a 2-year period for all children 8-16 years of age with a BMI ≥95 th centile undergoing polysomnography. Data sources included clinical charts and polysomnography results. Clinical and polysomnography factors were examined as predictors of OSA. Results: A total of 210 children met inclusion criteria and 205 had sufficient data for analysis. The mean age was 12.5 ± 2.7 years; 65% were male. Multiple sleep concerns and comorbidities were reported in most children (90% and 91% respectively). Polysomnography identified OSA in 44% of children; 28% of children demonstrated moderate/severe OSA. Mouth breathing/nasal congestion (OR 0.33, 95%CI 0.18-0.61), syndrome/multiple anomalies (OR 2.4, 95%CI 1.22-4.93) and family history of OSA (OR 2.7, 95% CI 1.2-5.8) or sleep problems (OR 12.4, 95% CI 1.5, 99.6) were the only factors predictive of OSA. Polysomnographic oxygen desaturation index <6 events/h showed an odds ratio of 4.96 (95% CI 2.27-10.86) for the absence of OSA. Conclusions: Children with obesity undergoing polysomnography are medically complex with multiple sleep concerns including a high burden of daytime symptoms; just under half demonstrate polysomnographic features of OSA. Earlier identification of OSA, recognition of non-OSA sleep concerns, and treatment strategies to improve sleep may contribute to overall health outcomes for children with obesity.
BACKGROUND Positive airway pressure (PAP) is used to treat children with concurrent obesity and s... more BACKGROUND Positive airway pressure (PAP) is used to treat children with concurrent obesity and sleep-disordered breathing (SDB), but achieving adherence remains challenging. We aimed to identify factors associated with PAP adherence in a prospective cohort of children with obesity prescribed PAP for newly diagnosed SDB. METHODS A questionnaire to assess factors related to PAP adherence was administered to participants and their parent ≥ 12 months after enrolment. Adherence (PAP use ≥ 4 hours/night on > 50% of nights), was measured with PAP machine downloads, diaries and physician assessments. Questionnaire responses were compared between adherent/non-adherent participants and between children/parents. Age, total and obstructive apnea-hypopnea index (OAHI), lowest oxygen saturation, and highest CO₂ were compared between adherent/non-adherent children with univariate differences of medians, with 95% confidence intervals. RESULTS Fourteen children (median age 14.3 years, 93% male; all with obstructive sleep apnea) were included. Eleven (79%) were adherent to PAP. SDB symptom improvement was reported in 9/14 (64%); 8/14 (57%) had positive experiences with PAP. Most children assumed an active role in PAP initiation and felt supported by the clinical team. Responses between adherent/non-adherent groups and between children/parents were similar. Oxygen saturation nadir (median difference between non-adherent and adherent groups 8.9% (95% CI 1.7, 16.1)), but not age, AHI, OAHI or maximum CO₂, was associated with PAP adherence. CONCLUSION Children with obesity-related SDB with lower nocturnal oxygen saturation nadir were more likely to adhere to PAP therapy. Ensuring adequate understanding of PAP therapy and medical team support are key factors in PAP success.
BackgroundDespite recommendations for regular lung volume recruitment (LVR) use in clinical pract... more BackgroundDespite recommendations for regular lung volume recruitment (LVR) use in clinical practice guidelines for children with neuromuscular disease, adherence to LVR is poor. We aimed to describe the experience of LVR by boys with Duchenne muscular dystrophy (DMD), their families, and healthcare providers (HCPs), as well as to identify the barriers and facilitators to LVR use.MethodsThis multicenter, qualitative study evaluated boys with DMD (n = 11) who used twice‐daily LVR as part of a randomized controlled trial, as well as their parents (n = 11), and HCPs involved in the clinical use of LVR (n = 9). Semistructured interviews were conducted to identify participants' understanding of LVR therapy and their beliefs, barriers and facilitators to its use. Thematic analysis was conducted using an inductive approach. A subanalysis compared adherent and nonadherent children.ResultsSeven themes were identified related to participants' beliefs and experiences with LVR: emotiona...
Introduction: Children with a history of bronchopulmonary dysplasia (BPD) may have lower physical... more Introduction: Children with a history of bronchopulmonary dysplasia (BPD) may have lower physical activity levels, but evidence to date is based on self-report. This study compared physical activity levels between children born extremely preterm with and without history of BPD, and examined their associations with pulmonary magnetic resonance imaging (MRI) and pulmonary function test (PFT) indices. Methods: This multi-centre cross-sectional study included children aged 7-9 years born extremely preterm, with and without BPD. Children wore a pedometer for one week, then completed the Physical Activity Questionnaire (PAQ), pulmonary MRI, and PFT. Spearman correlations and multivariable linear regression modelling were performed. Results: Of 45 children, 28 had a history of moderate-severe BPD. There were no differences in any physical activity outcomes by BPD status. Higher average daily step count and higher average daily moderate-vigorous physical activity (MVPA) were each correlated...
ObjectivesTo describe the current clinical practice patterns of Canadian pediatric respirologists... more ObjectivesTo describe the current clinical practice patterns of Canadian pediatric respirologists at pediatric tertiary care institutions regarding chronic tracheostomy tube care and management of home invasive ventilation.MethodsA pediatric respirologist/pediatrician with expertise in tracheostomy tube care and home ventilation was identified at each Canadian pediatric tertiary care center to complete a 59‐item survey of multiple choice and short answer questions. Domains assessed included tracheostomy tube care, caregiver competency and home monitoring, speaking valves, medical management of tracheostomy complications, decannulation, and long‐term follow‐up.ResultsThe response rate was 100% (17/17) with all Canadian tertiary care pediatric centers represented and heterogeneity of practice was observed in all domains assessed. For example, though most centers employ Bivona™ (17/17) and Shiley™ (15/17) tracheostomy tubes, variability was observed around tube change, re‐use, and clea...
Obstructive sleep apnea syndrome is a major cause of morbidity in the Down syndrome population an... more Obstructive sleep apnea syndrome is a major cause of morbidity in the Down syndrome population and is commonly treated with adenoidectomy and/or tonsillectomy (AT). However, these children are at increased risk for perioperative respiratory adverse events (PRAEs). The objective of this study was to examine risk factors for major PRAEs requiring intervention in children with Down syndrome undergoing AT and to describe their postoperative monitoring environment. This retrospective study included all children with Down syndrome aged 0–18 years who underwent a preoperative polysomnogram followed by AT at a tertiary pediatric institution. Descriptive statistics were used to summarize baseline demographic and clinical characteristics. A multivariable model for prediction of PRAEs was constructed. A priori, it was decided that minimum oxygen saturation, apnea–hypopnea index, and average oxygen saturation asleep would be included, along with medical comorbidities associated with PRAEs at p < 0.2 in univariable analyses. Fifty-eight children were included in this study; twelve had a PRAE. Cardiac disease was associated with PRAEs on univariable analysis (p = 0.03). In multivariable analysis, average oxygen saturation asleep was associated with PRAEs (OR 1.50; 95% confidence interval 1.00, 2.41; p = 0.05). For all of the remaining variables, p > 0.15. Fifty-six children were admitted for monitoring overnight; four were admitted to the intensive care unit and fifty-two were admitted to the ward. Conclusions: A multivariable model found evidence that lower average oxygen saturation while asleep was associated with PRAEs requiring intervention in children with Down syndrome. This study highlights the difficulty in predicting complications in this population. What is known: • Obstructive sleep apnea syndrome is a major cause of morbidity in the Down syndrome population and is commonly treated with adenoidectomy and/or tonsillectomy. • However, children with Down syndrome are at increased risk for perioperative respiratory adverse events (PRAEs) following adenoidectomy and/or tonsillectomy. What is new: • We found that a lower average oxygen saturation asleep is associated with increased odds of PRAEs, adjusting for age, total apnea–hypopnea index, cardiac comorbidity, and minimum oxygen saturation. • This study highlights the difficulty in predicting complications in this population. What is known: • Obstructive sleep apnea syndrome is a major cause of morbidity in the Down syndrome population and is commonly treated with adenoidectomy and/or tonsillectomy. • However, children with Down syndrome are at increased risk for perioperative respiratory adverse events (PRAEs) following adenoidectomy and/or tonsillectomy. What is new: • We found that a lower average oxygen saturation asleep is associated with increased odds of PRAEs, adjusting for age, total apnea–hypopnea index, cardiac comorbidity, and minimum oxygen saturation. • This study highlights the difficulty in predicting complications in this population.
STUDY OBJECTIVES Despite the importance of treating sleep-disordered breathing (SDB), positive ai... more STUDY OBJECTIVES Despite the importance of treating sleep-disordered breathing (SDB), positive airway pressure (PAP) adherence rates in children are low. Identifying readily-available predictors of non-adherence would enable the development of targeted interventions and supports, but literature is limited. Our objective was to identify baseline clinical predictors of six-month PAP therapy non-adherence in children with SDB through a retrospective cohort study. METHODS This study evaluated children (8-17 years) prescribed PAP therapy for SDB between 2011-2017 at a single pediatric tertiary hospital. The primary outcome was non-adherence at six months, measured using both machine downloads and self-report. Candidate baseline predictors included demographics, comorbidities, and SDB characteristics. Relative risks (RR) and 95% confidence intervals (95%CI) were estimated using a modified Poisson regression. Missing data were imputed prior to analysis. RESULTS The study included 104 children. The independent predictors most strongly associated with greater non-adherence were older age (RR=1.08 for a one year increase, 95%CI=1.00-1.16) and higher oxygen saturation nadir (RR=1.03 for a 1% increase, 95%CI=1.00-1.05), while those most strongly associated with lower non-adherence were higher arousal index (RR=0.97 for a one event/hour increase, 95%CI=0.95-1.00), developmental delay (RR=0.58, 95%CI=0.30-1.13), and asthma (RR=0.72, 95%CI=0.44-1.17). CONCLUSIONS Overall, children who are older, have less severe SDB, or less disrupted sleep at baseline are more likely to be non-adherent to PAP therapy, and may benefit from additional supports to acclimatize to therapy. As clinical predictors were only weakly associated with non-adherence, non-clinical characteristics may play a larger role in predicting adherence.
STUDY OBJECTIVES Positional obstructive sleep apnea (POSA) is a phenotype of obstructive sleep ap... more STUDY OBJECTIVES Positional obstructive sleep apnea (POSA) is a phenotype of obstructive sleep apnea (OSA) where sleep-related obstructive events occur predominantly in supine position. Limited knowledge exists regarding the presence of POSA in children with obesity. The study objective was to determine the prevalence of POSA while identifying factors associated with POSA in children with obesity. METHODS This was a cross-sectional study of children with obesity, aged 8 to 18 years, with a diagnostic polysomnogram (PSG) between 2012 to 2019, who were referred for the evaluation of sleep-related breathing. POSA was defined as an overall obstructive apnea-hypopnea index (OAHI) ≥ 5 events per hour, and a supine OAHI to non-supine OAHI ratio of ≥2. Patient demographics, anthropometrics, and PSG data were recorded. RESULTS Of the 112 children with obesity with a diagnostic PSG, 43 (38%) had OSA. Amongst those with OSA, 25/43 (58%) had POSA (mean age 14.6±2.3 years, mean BMI 37.7±7.6 kg/m², 68% male) and 18/43 (42%) had no-POSA (mean age 13.9 ± 2.8 years, mean BMI 37.9 ± 7.2 kg/m², 78% male). Among those with POSA, 13/25 (52%) had mild OSA, 7/25 (28%) had moderate OSA and 5/25 (20%) had severe OSA. No significant differences were found in age, gender and anthropometric measures between POSA and no-POSA groups. Time spent in supine and non-supine sleep did not differ significantly between groups. CONCLUSIONS In children with obesity and OSA, POSA occurs frequently. Identifying POSA allows for potential targeted positional therapy for children with obesity.
(VA) and changes in rates of adolescent motor vehicle crashes and school night sleep duration. Cr... more (VA) and changes in rates of adolescent motor vehicle crashes and school night sleep duration. Crash rates in FC were also compared to those in the rest of the state during the same time period. Methods: VA Department of Motor Vehicles crash data (including overall crash rates, distraction, speed, and alcohol-related accidents) in 16-18 year old drivers between September and June of each year in Fairfax County versus the rest of the state were compared in the combined two year periods preceding (2013-14 and 2014-15; T1) and following (2015-16 and 2016-17; T2) school start time change in the fall of 2015. Average sleep duration was obtained from the Fairfax County Youth Survey (FCYS) a county-wide, anonymous, self-report survey administered annually to all 8th, 10th, and 12th-grade public school students. The results were also compared (pre-and post-school start time change). Results: The crash rate per 1000 in 16-18 year old licensed drivers in FC during T1 was significantly higher compared to T2, 31.63 vs 29.59 accidents per 1000 (95% CI, 1.0-1.14, OR 1.07, P¼0.03). In contrast, adolescent crash rates in the rest of Virginia were not statistically significantly different at T1 vs T2. With regards to subtypes of crashes, distractionrelated crashes were significantly higher per 1000 in FC at T1 compared to T2 at 7.01 vs 6.13 (95% CI, 0.99-1.31, OR 1.14, P¼0.05), but were not significantly different in the remainder of the state.In the T2 period in FC, 47% 12th-grade students endorsed sleeping 7 hours during school nights, which has increased by 5% (p¼0.001) after the implementation of the school start time change in the 2015-16. Conclusion: The results of this study suggests that school start time delay is associated with decreased adolescent motor vehicle crash risk, with significant implications for public health and safety.
Purpose of Review: Myotonic dystrophy type 1 (DM1) is a severe, progressive genetic disease that ... more Purpose of Review: Myotonic dystrophy type 1 (DM1) is a severe, progressive genetic disease that affects approximately 1 in 2,500 individuals globally [Ashizawa et al.: Neurol Clin Pract 2018;8(6):507–20]. In patients with DM1, respiratory muscle weakness frequently evolves, leading to respiratory failure as the main cause of death in this patient population, followed by cardiac complications [de Die-Smulders et al.: Brain 1998;121(Pt 8):1557–63], [Mathieu et al.: Neurology 1999;52(8):1658–62], [Groh et al.: Muscle Nerve 2011;43(5):648–51]. This paper provides a more detailed outline on the diagnostic and management protocols, which can guide pulmonologists who may not have experience with DM1 or who are not part of a neuromuscular multidisciplinary clinic. A group of neuromuscular experts in DM1 including pulmonologists, respiratory physiotherapists and sleep specialists discussed respiratory testing and management at baseline and during follow-up visits, based on their clinical ex...
Lung volume recruitment (LVR) is a common respiratory therapy for people living with restrictive ... more Lung volume recruitment (LVR) is a common respiratory therapy for people living with restrictive neuromuscular disease. LVR is important in acute respiratory compromise, during respiratory rehabilitation and as a preventative therapy. Cohort data associate LVR use with increased survival is diseases such as Duchenne Muscular Dystrophy.<sup>1</sup> While LVR is a commonly prescribed therapy, we have previously had to rely on self-report alone for any indication of adherence with prescribed therapy. This has significantly hampered both clinical decision making and research into effectiveness. In the paper from Canada and Australia that these data support, two large clinical services have collaborated to develop an objective LVR counter, to undertake extensive bench-testing, physiological testing, and to assess counter performance compared with diary self-report. The clinical validation was undertaken within a large, single-centre randomized controlled trial. The counter performed well and we believe the innovation will impact clinical care and research. These instructions, example output, and macro-enabled Excel spreadsheet are contined herein. 1. McKim DA, Katz SL, Barrowman N, et al. Lung Volume Recruitment Slows Pulmonary Function Decline in Duchenne Muscular Dystrophy. <em>Archives of Physical Medicine and Rehabilitation</em> 2012;93(7):1117-22.
Long-term non-invasive ventilation (NIV) is a common modality of breathing support used for a ran... more Long-term non-invasive ventilation (NIV) is a common modality of breathing support used for a range of sleep and respiratory disorders. The aim of this scoping review was to provide a summary of the literature relevant to long-term NIV use in children. We used systematic methodology to identify 11,581 studies with final inclusion of 289. We identified 76 terms referring to NIV; the most common term was NIV (22%). Study design characteristics were most often single center (84%), observational (63%), and retrospective (54%). NIV use was reported for 73 medical conditions with obstructive sleep apnea and spinal muscular atrophy as the most common conditions. Descriptive data, including NIV incidence (61%) and patient characteristics (51%), were most commonly reported. Outcomes from sleep studies were reported in 27% of studies followed by outcomes on reduction in respiratory morbidity in 19%. Adverse events and adherence were reported in 20% and 26% of articles respectively. Authors reported positive conclusions for 73% of articles. Long-term use of NIV has been documented in a large variety of pediatric patient groups with studies of lower methodological quality. While there are considerable data for the most common conditions, there are fewer data to support NIV use for many additional conditions.
Respiratory management of Duchenne muscular dystrophy (DMD) is not well studied and may vary acro... more Respiratory management of Duchenne muscular dystrophy (DMD) is not well studied and may vary across centers and practitioners. Our objective was to describe and compare the respiratory management practices of Canadian Pediatric Respirologists and Neuromuscular specialists for children with DMD. Methods: A web-based survey was sent to all 56 practicing Canadian Pediatric Respirologists and to all 24 members of the Canadian Pediatric Neuromuscular Group (CPNG) who follow children with neuromuscular diseases. The survey included 28 questions about timing and indications for respiratory consultation, sleep disordered breathing (SDB) assessments, and treatments. Results: Thirty eight (68%) pediatric respirologists and 17 (71%) CPNG members responded. Respirologists provide initial consultation after a patient's first admission to hospital with respiratory complications (14/38, 37%) and when symptoms of SDB are present (14/38, 37%). Half of the CPNG members request initial Respirology consultation at the time of DMD diagnosis. Both groups request routine pulmonary function tests. Ninety-six percent of respirologists use maximal inspiratory (MIP) and expiratory pressures (MEP) to assess respiratory muscle strength, whereas 82% of CPNG members additionally use peak cough flow. Assessment for SDB is requested by both groups when pulmonary function is abnormal or patients are symptomatic. Respirologists favor polysomnography, whereas CPNG members use overnight pulse oximetry. Nocturnal non-invasive ventilation and lung volume recruitment (LVR) are used in a minority of patients. Conclusions: Respirologists and CPNG members provide similar respiratory management of DMD patients, but differ in timing of consultation and choice of tests for pulmonary function and SDB. Canadian practices differ from the American Thoracic Society and Centre for Disease Control guidelines.
Increased neck circumference is a risk factor for obstructive sleep apnea in adults. With rising ... more Increased neck circumference is a risk factor for obstructive sleep apnea in adults. With rising obesity prevalence in children, it may be an important identifier of obstructive sleep apnea in children. The reliability of measuring neck circumference in children has not been systematically evaluated. To determine the inter- and intra-rater reliability of neck circumference measurements in children aged 2-16 years. Children aged 2-16 years with limb fractures were recruited. Neck circumference was measured by three investigators each using two separate unmarked paper tapes in the 2-5 year age group (N = 43), and three separate tapes in the 6-10 and 11-16 year age groups (N = 18 and 40). Neck circumference measurements showed excellent inter-rater reliability for children 6-10 and 11-16 years (ICC = 0.952 and 0.989). Substantial variation was observed for the 2-5 year age group (ICC = 0.701). Good intra-rater reliability was demonstrated for the three groups (ICC range: 0.776, 0.950). Repeatability coefficients were 2.5-3.4 cm in the youngest age group and were 1.2-1.4 cm in the 6-16 year age group. In children 6-16 years old, neck circumference shows very good inter and good intra-rater reliability. Multiple measurements are not required for precision and reliability.
Obese youth with sleep-disordered breathing are treated with positive airway pressure to improve ... more Obese youth with sleep-disordered breathing are treated with positive airway pressure to improve sleep and cardiovascular status. While improvements in sleep parameters have been confirmed, a study by Katz et al. showed no major improvement in ambulatory blood pressure. The aim of this ancillary study was to analyze short-term blood pressure variability, following positive airway pressure treatment, as a more sensitive marker of cardiovascular health. We analyzed 24-h blood pressure variability data in 17 children, taken at baseline and after 12 months of treatment. These data were derived from an already published prospective, multicenter cohort study conducted in 27 youth (8–16 years) with obesity who were prescribed 1-year of positive airway pressure for moderate–severe sleep-disordered breathing. Significant decreases were found in 24 h systolic blood pressure (p = 0.040) and nighttime diastolic blood pressure (p = 0.041) average real variability, and diastolic blood pressure (p = 0.035) weighted standard deviation. Significant decreases were noted in nighttime diastolic blood pressure time rate variability (p = 0.007). Positive airway pressure treatment resulted in a significant decrease in blood pressure variability, suggesting a clinically significant improvement of sympathetic nerve activity in youth with obesity and sleep-disordered breathing. Cardiovascular variability, as measured by blood pressure variability, is improved in children following positive airway pressure treatment. Our novel findings of improved blood pressure time rate variability are the first described in the pediatric literature. Future studies aimed at analyzing target organ damage in this patient population will allow for a better understanding as to whether alterations in blood pressure variability translate to decreasing target organ damage in children, as seen in adults. Cardiovascular variability, as measured by blood pressure variability, is improved in children following positive airway pressure treatment. Our novel findings of improved blood pressure time rate variability are the first described in the pediatric literature. Future studies aimed at analyzing target organ damage in this patient population will allow for a better understanding as to whether alterations in blood pressure variability translate to decreasing target organ damage in children, as seen in adults.
Objective: To determine the scope of sleep concerns, clinical features and polysomnography result... more Objective: To determine the scope of sleep concerns, clinical features and polysomnography results and to identify factors that predict obstructive sleep apnea (OSA) in a cohort of children with obesity. Methods: The study was a multi-center retrospective chart review. Data were collected from 3 pediatric sleep laboratories over a 2-year period for all children 8-16 years of age with a BMI ≥95 th centile undergoing polysomnography. Data sources included clinical charts and polysomnography results. Clinical and polysomnography factors were examined as predictors of OSA. Results: A total of 210 children met inclusion criteria and 205 had sufficient data for analysis. The mean age was 12.5 ± 2.7 years; 65% were male. Multiple sleep concerns and comorbidities were reported in most children (90% and 91% respectively). Polysomnography identified OSA in 44% of children; 28% of children demonstrated moderate/severe OSA. Mouth breathing/nasal congestion (OR 0.33, 95%CI 0.18-0.61), syndrome/multiple anomalies (OR 2.4, 95%CI 1.22-4.93) and family history of OSA (OR 2.7, 95% CI 1.2-5.8) or sleep problems (OR 12.4, 95% CI 1.5, 99.6) were the only factors predictive of OSA. Polysomnographic oxygen desaturation index <6 events/h showed an odds ratio of 4.96 (95% CI 2.27-10.86) for the absence of OSA. Conclusions: Children with obesity undergoing polysomnography are medically complex with multiple sleep concerns including a high burden of daytime symptoms; just under half demonstrate polysomnographic features of OSA. Earlier identification of OSA, recognition of non-OSA sleep concerns, and treatment strategies to improve sleep may contribute to overall health outcomes for children with obesity.
BACKGROUND Positive airway pressure (PAP) is used to treat children with concurrent obesity and s... more BACKGROUND Positive airway pressure (PAP) is used to treat children with concurrent obesity and sleep-disordered breathing (SDB), but achieving adherence remains challenging. We aimed to identify factors associated with PAP adherence in a prospective cohort of children with obesity prescribed PAP for newly diagnosed SDB. METHODS A questionnaire to assess factors related to PAP adherence was administered to participants and their parent ≥ 12 months after enrolment. Adherence (PAP use ≥ 4 hours/night on > 50% of nights), was measured with PAP machine downloads, diaries and physician assessments. Questionnaire responses were compared between adherent/non-adherent participants and between children/parents. Age, total and obstructive apnea-hypopnea index (OAHI), lowest oxygen saturation, and highest CO₂ were compared between adherent/non-adherent children with univariate differences of medians, with 95% confidence intervals. RESULTS Fourteen children (median age 14.3 years, 93% male; all with obstructive sleep apnea) were included. Eleven (79%) were adherent to PAP. SDB symptom improvement was reported in 9/14 (64%); 8/14 (57%) had positive experiences with PAP. Most children assumed an active role in PAP initiation and felt supported by the clinical team. Responses between adherent/non-adherent groups and between children/parents were similar. Oxygen saturation nadir (median difference between non-adherent and adherent groups 8.9% (95% CI 1.7, 16.1)), but not age, AHI, OAHI or maximum CO₂, was associated with PAP adherence. CONCLUSION Children with obesity-related SDB with lower nocturnal oxygen saturation nadir were more likely to adhere to PAP therapy. Ensuring adequate understanding of PAP therapy and medical team support are key factors in PAP success.
BackgroundDespite recommendations for regular lung volume recruitment (LVR) use in clinical pract... more BackgroundDespite recommendations for regular lung volume recruitment (LVR) use in clinical practice guidelines for children with neuromuscular disease, adherence to LVR is poor. We aimed to describe the experience of LVR by boys with Duchenne muscular dystrophy (DMD), their families, and healthcare providers (HCPs), as well as to identify the barriers and facilitators to LVR use.MethodsThis multicenter, qualitative study evaluated boys with DMD (n = 11) who used twice‐daily LVR as part of a randomized controlled trial, as well as their parents (n = 11), and HCPs involved in the clinical use of LVR (n = 9). Semistructured interviews were conducted to identify participants' understanding of LVR therapy and their beliefs, barriers and facilitators to its use. Thematic analysis was conducted using an inductive approach. A subanalysis compared adherent and nonadherent children.ResultsSeven themes were identified related to participants' beliefs and experiences with LVR: emotiona...
Introduction: Children with a history of bronchopulmonary dysplasia (BPD) may have lower physical... more Introduction: Children with a history of bronchopulmonary dysplasia (BPD) may have lower physical activity levels, but evidence to date is based on self-report. This study compared physical activity levels between children born extremely preterm with and without history of BPD, and examined their associations with pulmonary magnetic resonance imaging (MRI) and pulmonary function test (PFT) indices. Methods: This multi-centre cross-sectional study included children aged 7-9 years born extremely preterm, with and without BPD. Children wore a pedometer for one week, then completed the Physical Activity Questionnaire (PAQ), pulmonary MRI, and PFT. Spearman correlations and multivariable linear regression modelling were performed. Results: Of 45 children, 28 had a history of moderate-severe BPD. There were no differences in any physical activity outcomes by BPD status. Higher average daily step count and higher average daily moderate-vigorous physical activity (MVPA) were each correlated...
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