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Introduction Accurate assessment of hypersomnia depends upon consideration of several factors, including use of medications that affect alertness and sleep organization. Urine drug screens (UDS) are recommended when assessing hypersomnia, but there is little standardization with respect to screening methods, types of substances detected, and use in maintenance of wakefulness test (MWT) interpretation, in part because there is scant literature relating UDS results to patients’ characteristics, MWT findings and implications. Methods A retrospective analysis was performed in adolescents evaluated at Cincinnati Children's Sleep Center between 2008 and 2021 who underwent MWT with concurrent UDS to determine the adequacy of hypersomnia treatment. UDS in our laboratory were performed by Qualitative Immunoassay/Gas Chromatography-Mass Spectrometry/Liquid Chromatography-Tandem Mass Spectrometry. Results A total of 109 MWTs were accompanied by UDS in 79 patients. Patients were 17.7 [16.6, 18.6] years old (median, [IQ range]), 41.3% female, 68.8% White, 25.7% Black, 5.5% Other, with a BMI of 25.8 [22.1, 31.8] kg/m2. 85.3% had narcolepsy. In addition to prescribed medications, caffeine was positive in 54.1% of UDS, and diphenhydramine was positive in 58.7%. No patients reported use of caffeine or diphenhydramine on the day of MWT. There were no significant demographic differences between those who tested positive and negative for caffeine. The median sleep latency in those with caffeine-positive UDS was longer than those with caffeine-negative UDS, although it did not reach statistical significance (27.3 [14.7, 38.3] vs 19.1 [9.8, 36.1] minutes; P=0.15). Patients with a positive UDS for diphenhydramine all took modafinil/armodafinil. In addition, 80% of patients taking modafinil/armodafinil had diphenhydramine-positive results. Nicotine and cannabinoids were detected in 2 UDS. Conclusion Two unexpected substances (caffeine and diphenhydramine) were found on UDS during MWTs in a significant proportion of our cohort. Caffeine may influence the results of MWTs, although further investigations are warranted. The unexpected presence of diphenhydramine is a false positive for those patients on modafinil/armodafinil (supported by literature and confirmed by our laboratory director). Sleep clinicians should be aware of these findings and the implications of unexpected substances when interpreting MWTs. Support (If Any) Cincinnati Children's Hospital Research Fund

Introduction CPAP Adherence in children with OSA is often sub-optimal and there is concern for worsening during the COVID-19 pandemic. Although telehealth has been used in sleep clinics during the pandemic, there is little known about telemedicine’s effectiveness in specialized care. The purpose of this study is to compare CPAP adherence and related outcome metrics in pediatric OSA patients seen before and during the COVID-19 pandemic and between those seen in person and by telemedicine. Methods This retrospective cohort consisted of patients who were diagnosed with OSA, prescribed CPAP, and were seen in a CPAP clinic between 1/1/2018 and 9/31/2021. CPAP adherence data and outcome metrics including the Epworth Sleepiness Scale (ESS), Michigan Sleep-disordered Breathing (PSQ) and Pediatric Quality of Life questionnaires (Ped-QOL) were collected. Only patients with one pre-pandemic and at least one post-pandemic visit were included. All variables were reported as mean± SD. Statistical comparison was performed using the mixed-effects model. Results 147 patients met criteria with 353 total visits. 147 were before the pandemic (pre) and 206 were during the pandemic (post). Demographic data indicated 57% male, 82% white, and 63% 12 years and older. Post-pandemic visits consisted of 78% telemedicine. Comparison between pre and post pandemic data demonstrated no statistically significant differences in % CPAP usage>4hrs (71±31% [pre] vs 73±33% [post], p=0.95), ESS (6.4±5.5 [pre] vs 5.9±5.8 [post], p=0.27), or PSQ (0.38±0.22 [pre] vs 0.36±0.22 [post] p=0.90). However, there was a significant difference in Psychosocial Health domain of Ped-QOL (68.6±19.4 [pre] vs 74.5±17.9 [post], p=0.012). Comparison between telemedicine and in-person visits during the pandemic showed no differences in %CPAP usage>4hrs (73±31% [Tele] vs 73±37%, p=0.73), ESS, PSQ, or Ped-QOL. Conclusion In our cohort, children with OSA had no significant changes in CPAP adherence, sleepiness, or OSA symptoms during the COVID-19 pandemic. CPAP telehealth visits provided the same effectiveness as in-person visits, as evidenced by similar adherences and outcome metrics. Interestingly, patients reported higher psychosocial health during the pandemic despite no difference in CPAP adherence. Factors not related to OSA management may contribute to the improvement of quality of life in this population. Support (If Any) Cincinnati Children’s Research Fund

Introduction Children with central hypersomnia often have residual daytime sleepiness despite treatment with medical therapies. The Maintenance of Wakefulness Test (MWT) is an objective measure used to assess daytime alertness and responsiveness to treatment in patients with hypersomnia. There are limited data on MWT characteristics among pediatric populations. The purpose of this study was to: (1.) Compare MWT characteristics and subjective assessment between patients who passed and failed the MWT; (2.) Determine clinical management changes following MWT. Methods A retrospective review was conducted of all children who underwent MWT at Cincinnati Children’s Hospital Medical Center from September, 2008 to June, 2021. Pass and fail assessment designations were determined by the clinician as recorded in the medical record. Demographics, Epworth Sleepiness Scores (ESS), MWT characteristics, and pharmacological modifications were recorded. A majority of patients underwent MWT prior to driving. Descriptive statistics as well as Chi-square, Fisher’s exact, and Wilcoxon rank-sum testing were utilized. All variables were reported as medians with interquartile ranges. Results 109 MWTs were performed on 79 children with a median age of 17.7 years [16.6, 18.6]. MWTs were indicated for 4 primary diagnoses: hypersomnia (n=5), idiopathic hypersomnia (n=11), narcolepsy type 1 (n=56), and narcolepsy type 2 (n=37). 55 (50.5%) were documented as pass (P) and 54 as fail (F). No differences in age (17.5 years) [16.2,18.5] (P) vs. (17.8) [16.9,18.9] (F) (p=NS) or other demographics existed between the two groups. As expected, mean sleep latency was significantly higher among those who passed (37.3 minutes) [31.2,40.0] (P) vs. (10.8) [7.9,16.6] (F) (p Conclusion MWT provides useful objective assessment and often leads to management changes in adolescents with central hypersomnia. A subjective sleepiness assessment (ESS) does not correlate with objective MWT assessments. We speculate that an altered perception of sleepiness in children with hypersomnia may play a role in the inaccuracy of subjective assessments commonly utilized in clinical practice. Support (If Any) Cincinnati Children’s Research Foundation.

Introduction We present a patient with chronic insomnia, excessive daytime sleepiness, and sleep-related hypoventilation that improved with a wake promoting agent in conjunction with BPAP. Report of Case A 15-year-old male with Cystic Fibrosis (single F508del mutation, positive sweat test, FEV1% of 98%) and Autism who presented with frequent headaches, chronic insomnia, and daytime sleepiness (PDSS of 22). Current medications included albuterol as needed. Sleep history was significant for restless leg symptoms. Labs revealed low serum ferritin. Sleep study two years prior showed an AHI of 3.4 and PMLD of 18.4. After completion of iron therapy and initiation of gabapentin, his chronic insomnia and serum ferritin improved. However, patient continued to have aggressive behavior, headaches, and snoring. Sleep study was repeated and revealed mild OSA with hypoventilation (AHI of 6.41, >28% of total sleep time with TCO2 above 50 mm Hg). Initial serum bicarbonate was 29 mmol/L and progressively increased to 34 mmol/L. Due to evidence of persistent chronic hypoventilation, patient was trialed on BPAP. Subsequent sleep study with BPAP showed an AHI of 0, but with worsening sleep related hypoventilation (TCO2 of 56mmHg, > 70% total sleep time with TCO2 above 50 mm Hg). Genetic, endocrine, and neurological work up for hypoventilation was negative. Due to persistent daytime sleepiness (PDSS of 24), MSLT was performed and showed evidence of hypersomnia. Modafinil was then initiated. With the combination of nocturnal BPAP use and Modafinil, daytime sleepiness improved (PDSS of 15) and he had normalization of CO2 and bicarbonate levels. Conclusion We present a patient with CF, with minimal lung disease, who demonstrated a negative work up for sleep related hypoventilation, in which dysregulation of control of breathing improved with the use of a wake promoting agent in conjunction with BPAP.