Permanent standard time is the optimal choice for health and safety: an American Academy of Sleep Medicine position statement.

The period of the year from spring to fall, when clocks in most parts of the United States are set one hour ahead of standard time, is called daylight saving time, and its beginning and ending dates and times are set by federal law. The human biological clock is regulated by the timing of light and darkness, which then dictates sleep and wake rhythms. In daily life, the timing of exposure to light is generally linked to the social clock. When the solar clock is misaligned with the social clock, desynchronization occurs between the internal circadian rhythm and the social clock. The yearly change between standard time and daylight saving time introduces this misalignment, which has been associated with risks to physical and mental health and safety, as well as risks to public health. In 2020, the American Academy of Sleep Medicine (AASM) published a position statement advocating for the elimination of seasonal time changes, suggesting that evidence best supports the adoption of year-round standard time. This updated statement cites new evidence and support for permanent standard time. It is the position of the AASM that the United States should eliminate seasonal time changes in favor of permanent standard time, which aligns best with human circadian biology. Evidence supports the distinct benefits of standard time for health and safety, while also underscoring the potential harms that result from seasonal time changes to and from daylight saving time. CITATION: Rishi MA, Cheng JY, Strang AR, et al. Permanent standard time is the optimal choice for health and safety: an American Academy of Sleep Medicine position statement. J Clin Sleep Med. 2024;20(1):121-125.

Education and implementation of home spirometry in an adolescent cystic fibrosis population.

BACKGROUND: Lung function in individuals with cystic fibrosis (CF) is closely monitored as an objective marker of clinical status. The COVID-19 pandemic shifted our ability to assess individuals from in-person to remote monitoring using telehealth. As part of efforts to monitor individuals remotely during this time, this study describes the process of education and implementation of home spirometry in an adolescent CF population at Nemours Children's Hospital in Wilmington, Delaware, USA. In addition, this study reviews the ability of adolescents with CF to produce reliable, consistent, and accurate results using home spirometry. METHODS: This was a quality-improvement study over a 9-month period at a single CF center. Home spirometers were supplied by the CF Foundation to 40 adolescents with CF. An educational curriculum was used for initial training on the device by a dedicated CF respiratory therapist. After training, participants reported spirometry results weekly until reliable technique was established. Once reliable technique was achieved, participants reported results monthly. Results were retrospectively reviewed to determine accuracy and consistency. The percentages of patients who were able to achieve reliable, consistent, and accurate results were recorded as well as the need for additional training or other reasons for inability to produce ongoing results. RESULTS: Home spirometers were distributed to 40 adolescents. Out of these 40 participants, 35 (88%) completed initial training; 29 (83%) sent at least one set of results, and 24 (60%) established reliable technique after an average of 5 weekly attempts. When home spirometer results were retrospectively reviewed, 83% (20/24) were deemed accurate in comparison to spirometry completed in clinic, and 83% (20/24) showed consistency between efforts sent. CONCLUSION: Home spirometry, when properly implemented with structured education and active participant engagement, has potential to provide meaningful data and feedback to CF care teams. Implementation of this process requires substantial resources and active participation from an adolescent population who are at higher risk for non-adherence. Future studies are needed to determine the impact of home spirometry on clinical outcomes and reliability over time and to address barriers to consistent and enduring engagement in the adolescent population.

Daytime Sleepiness in Children With Asthma: Examining Respiratory and Non-respiratory Factors.

Objective Daytime sleepiness is common in youth with asthma (YWA). Treatments designed to mitigate daytime sleepiness in YWA require an understanding of the primary causes of this problem. We examined respiratory- and non-respiratory-related factors associated with daytime sleepiness in YWA. Methods One hundred YWA (eight to 17 years old) were included in a cross-sectional study. Daytime sleepiness, quality of life, anxiety, bedtime cellphone use, and respiratory symptoms were self-reported. Asthma severity, lung function, and the number of prescribed medications were obtained from electronic medical records. Multivariable regression models identifying variables associated with daytime sleepiness were generated. Results Participants were 54% male and 45% Black, with a mean age of 12.1 years. The multivariable regression model showed decreased quality of life (b = -0.328, p = 0.004) and increased bedtime cellphone use (b = 0.300, p = 0.004)were significantly related to daytime sleepiness, while anxiety (b = 0.213, p = 0.05), prescribed asthma medications (b = 0.173, p = 0.05), and worse lung function (b = -0.173, p = 0.05)were marginally related to daytime sleepiness. Conclusions In addition to optimizing asthma control, strategies targeting anxiety, quality of life, and nocturnal cellphone use are important to mitigate daytime sleepiness in YWA.

Acceptability and feasibility of a brief behavioral sleep intervention for youth with CF.

Youth with CF are at increased risk for physiological and behavioral sleep difficulties due to disease-specific symptoms and more general pediatric sleep challenges. Despite evidence suggesting that behavioral sleep interventions are effective for improving common sleep difficulties, no interventions exist for youth with CF. SLEEP-CF was designed to fill this gap by providing tailored, flexible behavioral sleep support to youth with CF. Results suggest that SLEEP-CF is an acceptable and feasible behavioral sleep intervention, even in a population with normative sleep habits. There may be benefit in terms of improving sleep knowledge and sleep hygiene. Technology use during and after bedtime is prevalent. CF care team members are encouraged to assess sleep as part of routine CF care, and to provide support as indicated.

Bringing At-home Pediatric Sleep Apnea Testing Closer to Reality: A Multi-modal Transformer Approach.

Sleep apnea in children is a major health problem affecting one to five percent of children (in the US). If not treated in a timely manner, it can also lead to other physical and mental health issues. Pediatric sleep apnea has different clinical causes and characteristics than adults. Despite a large group of studies dedicated to studying adult apnea, pediatric sleep apnea has been studied in a much less limited fashion. Relatedly, at-home sleep apnea testing tools and algorithmic methods for automatic detection of sleep apnea are widely present for adults, but not children. In this study, we target this gap by presenting a machine learning-based model for detecting apnea events from commonly collected sleep signals. We show that our method outperforms state-of-the-art methods across two public datasets, as determined by the F1-score and AUROC measures. Additionally, we show that using two of the signals that are easier to collect at home (ECG and SpO2) can also achieve very competitive results, potentially addressing the concerns about collecting various sleep signals from children outside the clinic. Therefore, our study can greatly inform ongoing progress toward increasing the accessibility of pediatric sleep apnea testing and improving the timeliness of the treatment interventions.

Adolescent E-cigarette or Vaping Use-Associated Lung Injury in the Delaware Valley: A Review of Hospital-Based Presentation, Management, and Outcomes.

E-cigarette or vaping use-associated lung injury (EVALI) remains a major concern due to ongoing use of nicotine and/or cannabis-containing products and resulting acute lung injury. There are few published reports describing the clinical features, comorbidities, severity of disease, and outcomes of treatment in adolescents. This report describes the experience of a single tertiary care children's hospital in the Delaware Valley and reviews data from all patients diagnosed with EVALI in the emergency department and inpatient setting from July 2019 to June 2021 at the Nemours Children's Hospital in Wilmington, Delaware. Demographic, clinical, therapeutic, diagnostic features, and outcomes are presented. Abstinence and steroids improved outcomes in our population. Obtaining a vaping history, negative infectious testing, elevated inflammatory markers, and characteristic computed tomography findings were key to making the diagnosis of EVALI.

A Qualitative Exploration of Sleep Habits and Intervention Needs Among Youth with Cystic Fibrosis.

Evidence suggests that individuals with Cystic Fibrosis (CF) experience difficulties with sleep architecture and hygiene, although research is limited. There are currently no behavioral sleep interventions for youth with CF. The current study used qualitative methods to understand sleep needs and concerns among youth with CF, as well as to obtain feedback about potentially useful behavioral sleep intervention strategies. Semi-structured interviews were conducted with youth with CF between the ages of 11-17 and their parents. Themes were extracted from the data and will be used to inform the development of a brief behavioral sleep intervention for youth with CF. Youth and their parents described several CF-specific sleep concerns, often related to respiratory symptoms, and a number of strategies used to manage these concerns. They also described concerns that apply to the general population, including pervasive use of technology around bedtime. Many evidence-based behavioral sleep intervention strategies are acceptable to youth with CF, although modifications are appropriate to reduce time burden and address CF-specific needs. Youth with CF experience a number of disease-specific and more generalized sleep concerns which are likely amenable to behavioral intervention. Results from this study will be used to inform the development of a brief behavioral sleep intervention for youth with CF.

Polysomnographic characteristics and treatment modalities in a referred population of children with trisomy 21.

Background: Children with Trisomy 21 (T21) are at an increased risk of sleep-disordered breathing (SDB), which can impact daily functioning and cause other health complications. Accordingly, it is imperative to diagnose and treat SDB in this population. Current guidelines recommend screening polysomnogram by age 4 or sooner if clinically indicated. There are limited published studies describing characteristics of SDB in children with T21, particularly in infants and young children. Objective: The objective of this study is to characterize SDB and treatment modalities in infants and young children with T21. Methods: This is a retrospective review of a cohort of children (≤60 months of age) with T21 who completed a polysomnogram (PSG) between 2015 and 2020 at a pediatric referral center. Demographic information, relevant medical history, polysomnography parameters, and treatment details of these children were collected from EMR. Descriptive and comparative statistics were calculated for the cohort; additional subgroup analysis was completed by age 0-35 months and 36-60 months. Results: Most of the cohort met criteria for sleep apnea (84.1%), and airway surgery was the most common treatment modality (71.4%). The mean AHI was high (21.4 events/hour) with a trend towards hypoventilation (mean EtCO2 = 55.9 mmHg; mean percentage of TST with EtCO2 > 50 mmHg 20.8%). Mean arousal index was elevated (32 events/hour). There were no significant differences in SDB by age when we compared children 0-35 months and 36-60 months. Conclusions: This cohort of referred children with T21 showed high prevalence of SDB with a trend towards hypoventilation and disrupted sleep quality with no significant differences by age. These data highlight the importance of maintaining a high index of suspicion for SDB in young patients with T21 and obtaining PSG testing to characterize sleep and breathing.

Comparing decannulation failures and successes in pediatric tracheostomy: An 18-year experience.

OBJECTIVES: There is a paucity of published literature identifying patients at higher risk of decannulation failure. The purpose of this study is to evaluate patient factors that may predict successful decannulation of pediatric tracheostomy patients and analyze factors contributing to tracheostomy decannulation failures. METHODS: A retrospective chart review of tracheostomy outcomes was conducted at a pediatric referral hospital. Successful and failed decannulations were compared using the following patient variables: age at tracheostomy, sex, ethnicity, gestational age and weight, the primary indication for tracheostomy, comorbidities, age at decannulation attempt, polysomnography data, and status of airway before decannulation as assessed endoscopically by airway team. RESULTS: Four hundred thirty-nine tracheostomies were performed over the 18-year period with 173 decannulation attempts. The overall rate of successful decannulation on the first attempt was 91.9% (159 of 173), with an eventual decannulation success rate of 97.1% (168 of 173). Compared with failed decannulations, the patients with successful decannulations had a shorter duration of tracheostomy and no medical comorbidities. Gestational age and weight approached, but did not achieve, statistical significance. After 25 months with a tracheostomy, approximately 50% of patients are decannulated with very few decannulations occurring after 75 months. The overall mortality rate in this cohort was 18.6% (78 of 420) with a tracheostomy-related mortality rate of 0.95% (4 of 420). CONCLUSIONS: The decannulation protocol at this institution is successful nearly 92% of the time. Fewer medical comorbidities, shorter duration of tracheostomy placement, and older gestational age may improve the likelihood of successful decannulation. Future studies are needed to determine the optimal timing and workup to evaluate patients for decannulation.

Machine learning for automatic identification of thoracoabdominal asynchrony in children.

BACKGROUND: The current methods for assessment of thoracoabdominal asynchrony (TAA) require offline analysis on the part of physicians (respiratory inductance plethysmography (RIP)) or require experts for interpretation of the data (sleep apnea detection). METHODS: To assess synchrony between the thorax and abdomen, the movements of the two compartments during quiet breathing were measured using pneuRIP. Fifty-one recordings were obtained: 20 were used to train a machine-learning (ML) model with elastic-net regularization, and 31 were used to test the model's performance. Two feature sets were explored: (1) phase difference (ɸ) between the thoracic and abdominal signals and (2) inverse cumulative percentage (ICP), which is an alternate measure of data distribution. To compute accuracy of training, the model outcomes were compared with five experts' assessments. RESULTS: Accuracies of 61.3% and 90.3% were obtained using ɸ and ICP features, respectively. The inter-rater reliability (i.r.r.) of the assessments of experts was 0.402 and 0.684 when they used ɸ and ICP to identify TAA, respectively. CONCLUSIONS: With this pilot study, we show the efficacy of the ICP feature and ML in developing an accurate automated approach to identifying TAA that reduces time and effort for diagnosis. ICP also helped improve consensus among experts. IMPACT: Our article presents an automated approach to identifying thoracic abdominal asynchrony using machine learning and the pneuRIP device. It also shows how a modified statistical measure of cumulative frequency can be used to visualize the progression of the pulmonary functionality along time. The pulmonary testing method we developed gives patients and doctors a noninvasive and easy to administer and diagnose approach. It can be administered remotely, and alerts can be transmitted to the physician. Further, the test can also be used to monitor and assess pulmonary function continuously for prolonged periods, if needed.

Diagnostic differences in respiratory breathing patterns and work of breathing indices in children with Duchenne muscular dystrophy.

RATIONALE: Pulmonary function testing (PFT) provides diagnostic information regarding respiratory physiology. However, many forms of PFT are time-intensive and require patient cooperation. Respiratory inductance plethysmography (RIP) provides thoracoabdominal asynchrony (TAA) and work of breathing (WOB) data. pneuRIPTM is a noninvasive, wireless analyzer that provides real-time assessment of RIP via an iPad. In this study, we show that pneuRIPTM can be used in a hospital clinic setting to differentiate WOB indices and breathing patterns in children with DMD as compared to age-matched healthy subjects. METHODS: RIP using the pneuRIPTM was conducted on 9 healthy volunteers and 7 DMD participants (ages 5-18) recruited from the neuromuscular clinic, under normal resting conditions over 3-5 min during routine outpatient visits. The tests were completed in less than 10 minutes and did not add excessive time to the clinic visit. Variables recorded included labored-breathing index (LBI), phase angle (Φ) between abdomen and rib cage, respiratory rate (RR), percentage of rib cage input (RC%), and heart rate (HR). The data were displayed in histogram plots to identify distribution patterns within the normal ranges. The percentages of data within the ranges (0≤ Φ ≤30 deg.; median RC %±10%; median RR±5%; 1≤LBI≤1.1) were compared. Unpaired t-tests determined significance of the data between groups. RESULTS: 100% patient compliance demonstrates the feasibility of such testing in clinical settings. DMD patients showed a significant elevation in Φ, LBI, and HR averages (P<0.006, P<0.002, P<0.046, respectively). Healthy subjects and DMD patients had similar BPM and RC% averages. All DMD data distributions were statistically different from healthy subjects based on analysis of histograms. The DMD patients showed significantly less data within the normal ranges, with only 49.7% Φ, 48.0% RC%, 69.2% RR, and 50.7% LBI. CONCLUSION: In this study, noninvasive pneuRIPTM testing provided instantaneous PFT diagnostic results. As compared to healthy subjects, patients with DMD showed abnormal results with increased markers of TAA, WOB indices, and different breathing patterns. These results are similar to previous studies evaluating RIP in preterm infants. Further studies are needed to compare these results to other pulmonary testing methods. The pneuRIPTM testing approach provides immediate diagnostic information in outpatient settings.

Status asthmaticus requiring extracorporeal membrane oxygenation associated with rhinovirus infection.

Objective: Evolving research links human rhinovirus (HRV) with status asthmaticus (SA) as well as severe respiratory illness in patients with atopy and asthma. This case series reviews five episodes of HRV-associated SA that required extracorporeal membrane oxygenation (ECMO). Methods: Charts of four patients, five total episodes of ECMO, with SA secondary to HRV were reviewed in this IRB-approved case series. Outcomes included demographic information, past medical history, clinical parameters and spirometry. Results: Patients (three male, one female), mean age 9 years (range 7-12 years) at the time of admission, were African American, on Medicaid, carried a diagnosis of persistent asthma, and had documented non-adherence to prescribed, daily controller medications. One patient had passive smoke exposure. All patients had a mean IgE of 734 (range 12-2497) with seasonal allergic rhinitis was diagnosed in three patients. Cases occurred in spring (3/5) and fall (2/5). Venous/venous ECMO (4/5) or venous/arterial ECMO (1/5) was continued for a mean duration of 4.2 days (range 3-7 days). Spirometry after hospitalization had a mean FEV1 of 1.59 L (81% predicted, range 69%-91%), and an FEF25%-75% 1.13 L (47.5% predicted, range 41%-65%) at an average of 16.7 weeks post ECMO. Conclusions: This case series highlights the association between persistent, poorly controlled asthma and severe SA with HRV infection resulting in ECMO. Despite life-threatening illness, these patients did not demonstrate significant large-airway obstruction following infection. However, patients showed persistently abnormal small airway function, which could be a risk factor or early evidence of vulnerability to infection.

Persistent Central Apnea and Long-Term Outcome After Posterior Fossa Decompressive Surgery for Arnold Chiari Type 1 Malformation in a Pediatric Patient.

ABSTRACT: Arnold Chiari malformation (ACM) is the most common cause of central sleep apnea (CSA) in otherwise healthy children. Although there are several case reports and series reported on this topic, there are limited descriptions of the long-term course of these children after the surgical interventions. Posterior fossa decompression surgery to relieve pressure of the herniating cerebellum on the brainstem is generally thought to significantly improve CSA in most cases, however, there are very limited data on the natural course of CSA in children following decompression surgery. There may be a subset of children in whom it may take much longer for CSA to resolve, and in some it may not resolve completely. Hence, these children need to be followed closely with sleep studies to document resolution of CSA. In this case report, we describe a 10-year-old male with severe CSA who was subsequently diagnosed with type 1 ACM and underwent posterior fossa decompressive surgery. However after surgery, although there was improvement in his CSA, he still had a significant degree of residual CSA which required bilevel positive pressure therapy and took more than 7.5 years to resolve. This case report illustrates the need for close follow-up in these children and for providers to understand the natural course so they can accurately counsel families about expectations after surgical treatments.

Measures of respiratory inductance plethysmography (RIP) in children with neuromuscular disease.

BACKGROUND: Pulmonary function testing (PFT) is essential for the clinical assessment of respiratory problems. Respiratory inductance plethysmography (RIP) is a non-invasive method of PFT requiring minimal patient cooperation. RIP measures the volumetric change in the ribcage and abdomen, from which work of breathing (WOB) indices are derived. WOB indices include: phase angle (Ф), percent ribcage (RC%), respiratory rate (RR), and labored breathing index (LBI). Heart rate (HR) is collected separately. AIM: The goal of this study was to assess the utility of a newly developed RIP system, the pneuRIP, in an outpatient clinic setting in children with neuromuscular (NM) disease. METHOD: The pneuRIP system measures and displays the WOB indices in real-time on an iPad display. Forty-three subjects, 22 NM patients and 21 healthy children (ages: 5-18 years) were enrolled. RESULTS: Patients' means showed an increase of 119.8% for Ф, 15.7% for LBI, and 19.9% for HR compared with healthy subjects, when adjusted for age and gender. The study found significant differences between the mean values of the healthy subjects and patients in Ф (P = 0.000), LBI (P = 0.001), and HR (P = 0.001). No differences were noted for RC% and RR between groups. Data for Ф in NM patients were diffusely distributed as compared with healthy subjects based on analysis of histograms. CONCLUSION: Non-invasive pneuRIP testing provided instantaneous PFT results. As compared to healthy subjects, NM patients showed abnormal results with increased markers of thoracoabdominal asynchrony, WOB indices, and biphasic breathing patterns likely resulting from NM weakness.

Risk factor analysis for mortality among infants requiring tracheostomy.

INTRODUCTION: An increasing number of tracheostomies are performed in infants with complex comorbidities including bronchopulmonary dysplasia (BPD) and congenital heart disease (CHD). With this shift in indications, there is an urgent need to characterize outcomes in this population. METHODS: This 5-year retrospective chart review assessed rates of 12-month mortality in infants who were ≤12 months of age at the time of tracheostomy at a tertiary care pediatric hospital and risk factors associated with death. Patient characteristics evaluated included chronologic age and post-menstrual age at tracheostomy placement, gestational age and weight, sex, ethnicity, indication for tracheostomy, and comorbidities including BPD, CHD, subglottic stenosis (SGS), craniofacial syndromes, and chromosomal trisomy syndromes. Subgroup analysis was performed in infants with CHD. RESULTS: One hundred thirty-two tracheostomies were performed during the study period with an overall 12-month mortality of 14.4% (19/132). Mortality was increased in patients with CHD (35%) and decreased in patients with SGS (3.7%). No other patient characteristics were associated with differences in mortality. There was a trend towards improved mortality outcomes among patients born at earlier gestational ages. CONCLUSIONS: Among infants with tracheostomy in this cohort, overall mortality rates were relatively low but not insignificant. CHD was associated with increased mortality; however, children with SGS showed more favorable outcomes. Other patient characteristics were not associated with differences in mortality. These data clarify outcomes in a group of infants with tracheostomy.

Pseudomonas eradication and clinical effectivness of Ivacaftor in four Hispanic patients with S549N.

Ivacaftor was approved for rarer class-III CFTR mutations including S549N in 2014. Since these mutations are uncommon, ongoing reports of patient experiences with Ivacaftor and these mutations are important. This case series describes the clinical effectiveness (including airway infection status, lung function, and growth) of Ivacaftor therapy in four pediatric Hispanic patients with S549N and F508del over 24 months. In these patients, Ivacaftor was highly efficacious with no further Pseudomonas-positive cultures despite prior chronic colonization in three patients as well as notable improvements in lung function and growth. The remarkable improvements in lung function and growth were similar to G551D patients with more striking changes in airway infection status. Pediatr Pulmonol 2017;52:E37-E39. © 2017 Wiley Periodicals, Inc.