Caregiver support and positive airway pressure therapy adherence among adolescents with obstructive sleep apnea.

INTRODUCTION: Positive airway therapy (PAP) adherence rates are suboptimal among adolescents with obstructive sleep apnea (OSA) and strategies to increase PAP adherence is a clinical priority. This study evaluates if caregiver support is associated with PAP adherence rates among adolescents with OSA. METHODS: We conducted a retrospective study and evaluated PAP adherence rates among adolescents with OSA from 2012 to 2017. Adherence was measured as continuous variables: average PAP usage (minutes per night) and average PAP usage >4 hours/night (% of all nights). We evaluated if adolescents with OSA who were receiving practical caregiver support with PAP had higher adherence than adolescents with OSA without caregiver support. RESULTS: One hundred and seven adolescents with OSA (mean age=14.1±2.5 years, 64.5% male, mean BMI percentile=89.0±21.8) seen between January 2012 and August 2017 at our institution were included. In this study, 60.7% (n=65) of adolescents with OSA were receiving practical caregiver support with PAP therapy. Adolescents with OSA receiving practical caregiver support with PAP used therapy for a significantly greater duration each night compared to adolescents who were not receiving practical caregiver support (298.5±206.7 versus 211.9±187.2 minutes; P=0.02). Greater time since the initial PAP prescription was independently associated with PAP adherence. CONCLUSION: Focusing on PAP adherence early may help adolescents with OSA incorporate therapy into their nightly routine, which may improve adherence and lead to improved health outcomes in adolescents with OSA. Practical caregiver support may be an essential component of ensuring optimal PAP adherence among adolescents with OSA.

Family functioning among adolescents with narcolepsy.

BACKGROUND: Narcolepsy is a sleep disorder with no cure with onset typically during adolescence. Caring for an adolescent with a lifelong medical condition can negatively impact family structure, cohesion, relationships, and overall functioning. The primary objective of this study was to evaluate family functioning in a cohort of adolescents with narcolepsy using the PedsQL Family Impact Module. The secondary objective was to compare family functioning in adolescents with narcolepsy to adolescents with chronic pain based on published data. METHODS: This was a cross-sectional study of adolescents (aged 10 to 18 years) with narcolepsy. The narcolepsy group was recruited from The Hospital for Sick Children in Toronto, Canada. Family functioning was assessed by the PedsQL family impact module total scores, which was completed by the patient's caregiver. The PedsQL family impact module yields a total scale which encompasses parent health-related quality of life, daily activities, family relationships, communication, and worry subscales. Lower scores suggest poorer family functioning. Secondary data analyses were used to compare participants' family functioning to a cohort of adolescents with chronic pain. RESULTS: Thirty adolescents with narcolepsy participated (mean age=13.8 ± 2.2 years, 76.7% male). Family functioning was impaired in this cohort of adolescents with narcolepsy and similar to adolescents with chronic pain (64.0 ± 19.8 versus 64.7 ± 19.5; P=0.849). CONCLUSION: Family functioning is impaired in adolescents with narcolepsy. Clinical teams should assess family functioning at routine clinic visits by asking about concerns and challenges related to caring for an adolescent with narcolepsy and providing resources and support as needed.

A retrospective analysis of clinical characteristics of childhood narcolepsy.

AIM: Narcolepsy, encompassing excessive daytime sleepiness (EDS), cataplexy, sleep paralysis and hypnogogic hallucinations, was previously considered rare in childhood. Recently, cases of childhood narcolepsy have increased significantly and the reasons for this may include the increasing awareness of narcolepsy as well as the H1N1 vaccination. The aim of this study was to describe the clinical characteristics of childhood narcolepsy, specifically focusing on cataplexy subtypes that may facilitate early recognition of narcolepsy. METHODS: We retrospectively reviewed and analyzed the medical records of 33 children diagnosed with narcolepsy at the Hospital for Sick Children, in Toronto, Ontario. All patients were seen prior to 18 years of age and symptoms were self-reported by parents and/or children themselves. RESULTS: At presentation, 32 of 33 children reported EDS and 28 of 33 reported cataplexy. Among the 28 patients with cataplexy, 18 of 28 reported cataplexy referred to as 'cataplectic facies' (e.g., facial hypotonia and/or tongue protrusion) while 10 of 28 patients reported characteristic cataplexy, defined as bilateral loss of muscle tone. Children with cataplectic facies reported higher BMI z-scores compared to those with characteristic cataplexy, 1.8 and 0.8, respectively. Children with cataplectic facies also tended to be younger than those with characteristic cataplexy, 9.2 and 11.8 years of age, respectively. Cataplectic facies appear to be related to narcolepsy close to disease onset. CONCLUSIONS: Children, especially young, obese children, presenting with a history of EDS with associated facial hypotonia or tongue protrusion raises the index of suspicion of narcolepsy and should prompt a referral to a specialized sleep facility to establish the diagnosis.

Longitudinal evaluation of sleep-disordered breathing in children with Prader-Willi Syndrome during 2 years of growth hormone therapy.

OBJECTIVE: To review longitudinal polysomnography data to assess sleep-related disordered breathing (SRDB) before and up to 2 years after initiation of growth hormone (GH) therapy in children with Prader-Willi syndrome (PWS). STUDY DESIGN: This was a retrospective review of systematic polysomnography evaluations performed in children with PWS before and at 6 weeks, 6 months, 1 year, and 2 years after initiation of GH therapy. RESULTS: A total of 15 children with PWS were reviewed. At baseline, the median age was 3.7 years (range, 0.8-15.4 years), and the median body mass index percentile was 82.4 (range, 0-100). GH was discontinued in 2 of these 15 children owing to the occurrence of severe obstructive sleep apnea after 6 weeks of GH therapy. The remaining 13 children who were followed for up to 2 years on GH therapy demonstrated no statistically significant trends over time for any adverse sleep-related outcomes, specifically obstructive or central sleep apnea. CONCLUSION: In young children with PWS with known SRDB at baseline, the first few weeks after initiation of GH therapy may represent a vulnerable time for the development of significant SRDB. However, most children with PWS did not show significant changes in SRDB after 2 years of GH therapy. We conclude that long-term GH therapy appears to be safe after an initial period of increased risk in the context of SRDB in children with PWS.

Depressive symptoms, sleep patterns, and physical activity in adolescents with narcolepsy.

STUDY OBJECTIVES: To evaluate the association between depressive symptoms, sleep patterns (duration and quality), excessive daytime sleepiness (EDS), and physical activity (PA) in adolescents with narcolepsy. METHODS: This cross-sectional study included adolescents (ages 10-18 years) with narcolepsy attending a tertiary care facility (The Hospital for Sick Children, Toronto, Canada). Adolescents with narcolepsy completed questionnaires evaluating depressive symptoms (Children's Depression Inventory-2nd edition [CDI-2]), sleep quality (Pittsburgh Sleep Quality Index), EDS (Epworth Sleepiness Scale), and PA (Godin Leisure-Time Exercise Questionnaire). Wrist-based actigraphy was worn by adolescents for 1 week to measure total sleep time (over 24 hr) and sleep efficiency percentage. RESULTS: Thirty adolescents with narcolepsy (mean age = 13.8 ± 2.2 years, 76.7% male) participated. In this cohort of adolescents with narcolepsy, 23.3% had CDI-2 total scores in the elevated range. Greater CDI-2 total scores were associated with poor sleep quality (ρ = 0.571; p = 0.02), EDS (ρ = 0.360; p = 0.05), and lower self-reported PA levels (ρ = -0.512; p < 0.01). CONCLUSIONS: Adolescents with narcolepsy report experiencing depressive symptoms, which are associated with poor sleep quality, EDS, and low PA levels. Strategies to improve nocturnal sleep quality and symptoms of EDS as well as promoting increased PA levels in adolescents with narcolepsy may provide an opportunity to improve depressive symptoms in this population. Multidisciplinary care with mental health and sleep specialists for adolescents with narcolepsy is needed.

Utility of brain MRI in children with sleep-disordered breathing.

OBJECTIVES/HYPOTHESIS: To investigate the utility of a brain magnetic resonance imaging (MRI) in children with sleep-disordered breathing (SDB), classified as isolated obstructive sleep apnea (OSA) in the absence of adenotonsillar hypertrophy, persistent OSA following adenotonsillectomy, isolated central sleep apnea (CSA) of unclear etiology, OSA with coexisting CSA of unclear etiology, or unexplained nocturnal hypoventilation (NH). STUDY DESIGN: Retrospective chart review of polysomnography (PSG) and brain MRI data. METHODS: Children with PSG evidence of SDB, as described above, and who subsequently had their first brain MRI, were included. PSG, MRI data, and subsequent interventions were recorded. RESULTS: A total of 59 of 6,087 (1%) children met inclusion criteria. Of those, 28 of 59 (47%) were nonsyndromic children and 31 of 59 (53%) were syndromic children with an underlying medical disorder. Abnormal brain MRI findings were observed in 19 of 59 (32%) children, where eight of 19 (42%) were nonsyndromic and 11 of 19 (58%) were syndromic. Abnormal brain MRI findings were most common in syndromic children with combined OSA and CSA without adenotonsillar hypertrophy. Isolated OSA was also a common PSG finding associated with an abnormal brain MRI. Of the nonsyndromic children with an abnormal brain MRI, the most common abnormal brain MRI finding was Chiari malformation (CM), observed in 88% of the group. A brainstem tumor was identified in one nonsyndromic child. Interventions following brain MRI included neurosurgery, chemotherapy, and noninvasive positive pressure ventilation (NiPPV). CONCLUSION: A brain MRI is an important diagnostic tool in syndromic and nonsyndromic children, especially in children with either isolated OSA or combined OSA and CSA without a clear etiology. LEVEL OF EVIDENCE: 4. Laryngoscope, 2016 127:513-519, 2017.

Midline posterior glossectomy and lingual tonsillectomy in obese and nonobese children with down syndrome: Biomarkers for success.

OBJECTIVES/HYPOTHESIS: To examine outcomes following midline posterior glossectomy (MPG) plus lingual tonsillectomy (LT) for the treatment of significant obstructive sleep apnea (OSA) in children with Down syndrome (DS). METHODS: Patients with DS who had persistent OSA following tonsillectomy and adenoidectomy (TA) and were relatively intolerant of positive airway pressure (PAP) therapy were evaluated by physical examination and sleep/CINE magnetic resonance imaging to determine the etiology of upper airway obstruction. Patients with relative macroglossia underwent MPG plus LT if required. Successful surgical outcome was defined as the resolution of OSA or the ability to tolerate PAP. RESULTS: Thirteen children (8 male, 5 female), mean (standard deviation) age 14.2 (4.0) years underwent MPG plus LT. Fifty-four percent of patients were obese (Body mass index [BMI] > 95th centile) and 8% were overweight (BMI 85th-95th centile) preoperatively. All patients underwent pre- and postoperative polysomnography. Postoperatively, the obstructive apnea-hypopnea index fell significantly from 47.0/hour to 5.6/hour (P <.05) in normal weight individuals who did not become obese, but not in obese patients or those who became obese postoperatively. Successful surgical outcome was seen in all (N = 6) children who were normal weight or overweight preoperatively compared with none who were obese preoperatively (N = 7). CONCLUSION: Midline posterior glossectomy and LT are beneficial in normal weight and overweight children with DS who have persistent OSA following TA and are intolerant of PAP therapy. Obesity pre- or postoperatively portends a worse prognosis following MPG, suggesting that aggressive weight loss initiatives should be considered as an adjunct to surgery in this population. LEVEL OF EVIDENCE: 4. Laryngoscope, 127:757-763, 2017.