The management of Pseudomonas aeruginosa respiratory infection in children with cerebral palsy: A narrative review.

Children with cerebral palsy have increased respiratory morbidity and mortality. Infection with Pseudomonas aeruginosa (PA) is associated with poorer outcomes, yet there are no formal guidelines to inform treatment of respiratory infection in children with cerebral palsy. This review explores the existing literature regarding management of PA-infection in children with cerebral palsy, with the aim of synthesising clinical recommendations and identifying gaps in current understanding. Medline (Ovid), PubMed and Embase were searched using keywords. Full-text articles involving the paediatric population and antimicrobial therapy were included. There was no limit on date of publication. Four retrospective case series were identified. Respiratory microbiology, in samples collected from a range of sites along the respiratory tract, was reported in three studies. Patients who received PA-specific antibiotics clinically improved. Two studies suggest that the use of suppressive inhaled anti-pseudomonal therapy may improve respiratory morbidity in the chronic setting. There is minimal evidence to guide management of PA respiratory infection in children with cerebral palsy. Children with cerebral palsy are at risk of developing bronchiectasis, so in the absence of high-quality evidence, management should be informed by extrapolating from the non-cystic fibrosis bronchiectasis guidelines. Further research examining surveillance and management of PA-infection in this population is required given that early intervention may prevent irreversible lung damage.

An evaluation of psychosocial sleep interventions for children with chronic health conditions: A systematic review.

Chronic health conditions (CHC; e.g., cystic fibrosis, type 1 diabetes) in children are associated with disease-specific physical symptoms that contribute to a high prevalence of sleep problems. Sleep problems exacerbate other health-related sequelae and can impede therapeutic response to health treatments, increasing the overall complexity of symptom management. Psychosocial sleep interventions (PSI) improve sleep in children with typical development and neurodevelopmental conditions. Yet, the effectiveness of PSI for children with CHC has scarcely been investigated. This systematic review appraises the literature examining the effectiveness and acceptability of PSI for children with CHC. A search identified 20 studies that met inclusion criteria. Data related to participant characteristics, sleep targets, research design and methods, measures, sleep outcomes and collateral effects were extracted. Study rigor was then evaluated. Most studies evaluated youth-directed Cognitive Behavioral Therapy for Insomnia or parent-implemented behavioral sleep interventions. Twelve studies demonstrated positive sleep treatment effects and four demonstrated mixed effects. Collateral improvements were reported in child mental health and parental health and well-being, though physical health benefits for children were not consistently reported. One, five and 14 studies were rated as having strong, adequate, and weak methodological rigor respectively. Recommendations for clinical practice and future research are made.

An approach to common sleep presentations in infants and toddlers.

BACKGROUND: Healthy sleep is vital for optimal child development, yet over 30% of Australian parents report having children with disrupted sleep affecting all family members. These sleep difficulties might co-exist with sleep breathing disorders, contributing to morbidity and reduced quality of life. OBJECTIVE: This article aims to provide general practitioners (GPs) with an evidence-based, biopsychosocial approach to managing common sleep problems in infants and preschool-aged children. DISCUSSION: Strategies and techniques are outlined to aid GPs in promoting healthy sleep during infancy, educating parents on typical sleep patterns and supporting families in managing problematic sleep patterns in toddlers. Emphasis is placed on a tailored approach to developing a healthy sleep environment to meet the child's needs and parental values. Valuable resources and indications for specialist consultation are included.

Mental health, sleep, and respiratory health after initiating elexacaftor/tezacaftor/ivacaftor treatment in children with cystic fibrosis.

INTRODUCTION: Cystic fibrosis transmembrane conductance regulator (CFTR) modulator elexacaftor/tezacaftor/ivacaftor (ETI) significantly improves lung function but its effect on mental health and sleep remains poorly understood. We report on mental health, sleep, and respiratory health outcomes of adolescents with CF commenced on ETI therapy, and monitored the prevalence of neuropsychiatric issues through the coronavirus disease 2019 pandemic. METHODS: We conducted a prospective longitudinal study of 31 adolescents (aged 10-18 years) from July 2021 to October 2022. Data collected include demographics, Patient Health Questionnaire-9 (PHQ-9), General Anxiety Disorder-7 (GAD-7), Pediatric Daytime Sleepiness Scale (PDSS), Sleep Disturbance Scale for Children (SDSC) scores, and FEV1 percent predicted. Twenty of 31 adolescents had data before and after ETI initiation. Mean differences (MD) in mental health, sleep, and respiratory health pre- and post-ETI therapy commencement were estimated using paired t-tests. The prevalence and trajectories of anxiety, depression, and sleep disturbance were described between the ETI epochs, and over the pandemic period. RESULTS: FEV1 improved following ETI therapy commencement (MD, 95% confidence interval [CI]: 7.1% (4.7%-9.6%) whereas PHQ-9, GAD-7, PDSS, and SDSC scores did not change significantly. Ten percent of participants developed new-onset anxiety/depression concerns and 10% developed new sleep concerns following ETI initiation. CONCLUSION: This is the first prospective longitudinal study of mental health and sleep changes after ETI commencement in adolescents with CF. Although respiratory outcomes improved, ETI did not improve anxiety, depression or sleep.

Optimising sleep in adolescents: The challenges.

BACKGROUND: Adolescence is a stage of significant transition as children develop into young adults. Optimal sleep is crucial during this period to ensure physical, emotional and mental wellbeing. However, it is well recognised that insufficient quality and quantity of sleep is common among adolescents worldwide. OBJECTIVE: This article aims to provide general practitioners with an overview of the key issues encountered in adolescent patients relating to sleep and summarises approaches to assessment and evidence-based management of sleep problems in this population. DISCUSSION: This review highlights the physiological changes that affect sleep during adolescence and how other factors, including unhealthy sleep behaviours, influence these. It discusses the importance of healthy sleep and the consequences of sleep disturbance in adolescents. Management strategies are outlined, focusing on the key common issues that affect sleep in the teenage years, and guidance on when to consider co-management with specialist care is provided.

Interstitial lung disease and pancreatic exocrine insufficiency in CADDS: Phenotypic expansion and literature review.

Contiguous ABCD1/ DXS1357E deletion syndrome (CADDS) is a rare deletion syndrome involving two contiguous genes on Xq28, ABCD1 and BCAP31 (formerly known as DXS1357E). Only nine individuals with this diagnosis have been reported in the medical literature to date. Intragenic loss-of-function variants in BCAP31 cause the deafness, dystonia, and cerebral hypomyelination syndrome (DDCH). Isolated pathogenic intragenic variants in ABCD1 are associated with the most common peroxisomal disorder, X-linked adrenoleukodystrophy (X-ALD), a single transporter deficiency, which in its more severe cerebral form is characterised by childhood-onset neurodegeneration and high levels of very-long-chain fatty acids (VLCFA). While increased VLCFA levels also feature in CADDS, the few patients described to date all presented as neonates with a severe phenotype. Here we report a tenth individual with CADDS, a male infant with dysmorphic facial features who was diagnosed through ultra-rapid whole genome sequencing (WGS) in the setting of persistent cholestatic liver disease, sensorineural hearing loss, hypotonia and growth failure and developmental delay. Biochemical studies showed elevated VLCFA and mildly reduced plasmalogens. He died at 7 months having developed pancreatic exocrine deficiency and interstitial lung disease, two features we propose to be possible extensions to the CADDS phenotype. We also review the genetic, phenotypic, and biochemical features in previously reported individuals with CADDS.

Assessing sleep in children with Down syndrome: Comparison of parental sleep diaries, actigraphy and polysomnography.

BACKGROUND: This study compared measurements of sleep and wake assessed with actigraphy, sleep diary and polysomnography in children with Down syndrome (DS) and also compared measures of actigraphic sleep recording in children with DS and typically developing (TD) children. METHODS: Children with DS aged 3-19 years (N = 44) referred for assessment of sleep disordered breathing (SDB) underwent overnight polysomnography, together with 1 week of actigraphy with sleep diary. Actigraphy data from the children with DS were compared with data collected from TD children, matched for age and sex. RESULTS: 22 children (50%) with DS completed >3 consecutive nights of actigraphy with a matched sleep diary. There were no differences between bedtimes, wake times or time in bed on weeknights, weekends or over 7 nights between actigraphy and sleep diary. Total sleep time was over estimated by the sleep diary by almost 2 h and the number of night awakenings under-reported. Compared to matched TD children (N = 22), there was no difference in total sleep time, however children with DS fell asleep more quickly (p < 0.001), had more awakenings (p = 0.001) and more time awake after sleep onset (p = 0.007). Children with DS exhibited less variability in both bedtimes and wake times, and fewer had >1 h sleep schedule variability. CONCLUSIONS: Parental sleep diaries over-estimate total sleep time but accurately report bed and wake times compared to actigraphy in children with DS. Children with DS have more regular sleep patterns than TD children of the same age, which is important for optimising daytime functioning. The reasons behind this warrant further investigation.

Demonstration of Accuracy and Feasibility of Remotely Delivered Oximetry: A Blinded, Controlled, Real-World Study of Regional/Rural Children with Obstructive Sleep Apnoea.

Objectives: Evaluate diagnostic accuracy and feasibility of a mail-out home oximetry kit. Design: Patients were referred for both the tertiary/quaternary-centre hospital-delivered oximetry (HDO) and for the mail-out remotely-delivered oximetry (RDO). Quantitative and qualitative data were collected. The COVID-19 pandemic began during this study; therefore, necessary methodological adjustments were implemented. Setting: Patients were first evaluated in Swan Hill, Victoria. RDO kits were sent to home addresses. For the HDO, patients travelled to the Melbourne city area, received the kit, stayed overnight, and returned the kit the following morning. Participants: All consecutive paediatric patients (aged 2−18), diagnosed by a specialist in Swan Hill with obstructive sleep apnoea (OSA) on history/examination, and booked for tonsillectomy +/− adenoidectomy, were recruited. Main outcome measures: Diagnostic accuracy (i.e., comparison of RDO to HDO results) and test delivery time (i.e., days from consent signature to oximetry delivery) were recorded. Patient travel distances for HDO collection were calculated using home/delivery address postcodes and Google® Maps data. Qualitative data were collected with two digital follow-up surveys. Results: All 32 patients that had both the HDO and RDO had identical oximetry results. The HDO mean delivery time was 87.7 days, while the RDO mean delivery time was 23.6 days (p value: <0.001). Qualitatively, 3/28 preferred the HDO, while 25/28 preferred the RDO (n = 28). Conclusions: The remote option is as accurate as the hospital option, strongly preferred by patients, more rapidly completed, and also an ideal investigation delivery method during certain emergencies, such as the COVID-19 pandemic.

Persistent pleuritic chest pain in a patient with cystic fibrosis.

Pleural effusion is rare in cystic fibrosis. Infection leading to pleural effusion is likely to be polymicrobial, including contributory fungal infection; microbiology of pleural fluid is commonly discordant with sputum. https://bit.ly/3MJXrhk.

Perioperative Care of Children with Severe Neurological Impairment and Neuromuscular Scoliosis-A Practical Pathway to Optimize Peri-Operative Health and Guide Decision Making.

Neuromuscular scoliosis is a common feature in children with severe neurological impairment (SNI), including those with severe cerebral palsy. Surgical correction of scoliosis is the mainstay of treatment. This group of patients also have associated medical complexity. The complication rates post-surgery are high, although, for many, they are worth the risk. There are currently no published practice guidelines or care pathways for children with SNI who are undergoing scoliosis corrective surgery. In response to the high uptake of this surgery, coupled with the expected complication rates, our hospital established a perioperative clinic. The purpose of this paper is to describe our perioperative approach. This clinic has developed into a service beyond perioperative care and, with the collaborative meeting, enables shared decision-making to identify the right candidate for surgery. The process involves surgical expertise, understanding the family and child at the centre, and optimisation of medical care pre- and post-surgery. In this paper, we describe the process in a step-by-step manner. We provide clinical vignettes, as well as the proformas that we use, and we highlight the benefits of the team-based process.

Delivering multidisciplinary neuromuscular care for children via telehealth.

INTRODUCTION/AIMS: In response to coronavirus disease 2019 (COVID-19) pandemic restrictions int 2020, our face-to-face (F2F) multidisciplinary neuromuscular clinic (NMC) transitioned to widespread use of telehealth (TH). This study aimed to (1) understand parent/guardian, child, and clinician perceptions of TH; (2) examine TH-related changes in clinical activity; and (3) use these findings to inform a future model of care for the NMC. METHODS: A clinical audit was undertaken to examine clinical activity throughout 2018-2020. Online surveys were distributed to clinicians and parents of children attending the NMC via TH in 2020. A working group of clinicians created a checklist to guide a future hybrid model of TH and F2F care. RESULTS: Total clinical activity in 2020 was maintained from previous years; 62.8% of all appointments occurred via TH, and 82.3% of patients attended NMC by TH at least once. Ninety-nine parents (30.6% response rate), 52 children, and 17 clinicians (77% response rate) responded to the survey. All groups reported better interaction when F2F compared to TH. Eighty percent of parents identified advantages of TH and reported lower levels of stress. A lack of "hands-on" physical assessment was identified by parents and clinicians as a TH limitation. Most families (68.1% of parents; 58.8% of children) and all clinicians indicated a preference for a mix of TH and F2F NMC appointments in the future. DISCUSSION: This study has informed a checklist to guide future TH use in a new hybrid model of care. Further investigation is required to assess health impacts of TH use in pediatric neuromuscular care.

Is cardiorespiratory disease associated with increased susceptibility of SARS-CoV-2 in children?

BACKGROUND: There are limited data in pediatric populations evaluating whether chronic cardiorespiratory conditions are associated with increased risk of coronavirus disease 2019 (COVID-19). We aimed to compare the rates of chronic cardiac and respiratory disease in children testing positive severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2[+]) compared with those testing negative (SARS-CoV-2[-]) at our institution. METHOD: Prospective cohort with nested case-control study of all children tested by polymerase chain reaction (PCR) for SARS-CoV-2 by nasopharyngeal/oropharyngeal sampling between March and October 2020. Children were identified prospectively via laboratory notification with age and sex-matching of SARS-CoV-2[+] to SARS-CoV-2[-] (1:2). Clinical data were extracted from the electronic medical record. RESULTS: In total, 179 SARS-CoV-2[+] children (44% females, median age 3.5 years, range: 0.1-19.0 years) were matched to 391 SARS-CoV-2[-] children (42% female, median age 3.7 years, range: 0.1-18.3 years). The commonest comorbidities showed similar frequencies in the SARS-CoV-2[+] and [-] groups: asthma (n = 9, 5% vs. n = 17, 4.4%, p = 0.71), congenital heart disease (n = 6, 3.4% vs. n = 7, 1.8%, p = 0.25) and obstructive sleep apnoea (n = 4, 2.2% vs. n = 10, 2.3%, p = 0.82). In the SARS-CoV-2[+] group, the prevalence of symptomatic disease was similar among children with and without cardiorespiratory comorbidities (n = 12, 75% vs. n = 103, 57%, p = 0.35). A high proportion of children hospitalized with SARS-CoV-2 infection had cardiac comorbidities (23.8%). CONCLUSIONS: In this single site data set, rates of pre-existing cardiorespiratory disease were similar in SARS-CoV-2[+] and SARS-CoV-2[-] children. Rates of symptomatic infection were similar between children with and without cardiorespiratory comorbidity. High rates of comorbid cardiac disease were observed among hospitalized children with COVID-19 warranting further research to inform vaccine prioritization.

Ventilatory support at home for children: A joint position paper from the Thoracic Society of Australia and New Zealand/Australasian Sleep Association.

The goal of this position paper on ventilatory support at home for children is to provide expert consensus from Australia and New Zealand on optimal care for children requiring ventilatory support at home, both non-invasive and invasive. It was compiled by members of the Thoracic Society of Australia and New Zealand (TSANZ) and the Australasian Sleep Association (ASA). This document provides recommendations to support the development of improved services for Australian and New Zealand children who require long-term ventilatory support. Issues relevant to providers of equipment and areas of research need are highlighted.

Quality of life and mood in children with cystic fibrosis: Associations with sleep quality.

BACKGROUND: We aimed to investigate the relationship between sleep quality, mood and health-related quality of life (HRQOL) in children with CF and controls. METHODS: Children (7-12years) and adolescents (13-18years) with CF and controls completed sleep evaluation: overnight oximetry and 14days of actigraphy. Age-appropriate questionnaires assessed mood (Children's Depression Inventory; CDI or Beck's Depression Inventory), HRQOL (CF Questionnaire-Revised; CFQ-R or PedsQL), and sleepiness (Pediatric Daytime Sleepiness Scale). RESULTS: 87 CF and 55 controls recruited. Children with CF had poorer sleep quality, more sleepiness and lower mood than controls, with a negative correlation between mood score and sleep efficiency. Sleepiness score was predictive of mood score and multiple CFQ-R domains. Adolescents with CF also demonstrated poorer sleep and more sleepiness than controls, but no difference in mood. Reduced sleep quality predicted lower CFQ-R scores. No correlation between sleep, mood or HRQOL in controls. CONCLUSIONS: In children and adolescents with CF, impaired sleep quality is associated with lower mood and HRQOL in an age-specific manner. Future research will aid understanding of effective strategies for prevention and treatment of mood disorders and sleep disturbance in children with CF.

What keeps children with cystic fibrosis awake at night?

BACKGROUND: Sleep disturbance is common in children with cystic fibrosis (CF) however there are limited studies investigating the causes for poor sleep quality. In a cross sectional observational study we aimed to evaluate the clinical correlates of sleep disturbance in this population. METHODS: Children with CF (7-18years) free from pulmonary exacerbation completed medical review, overnight oximetry, the OSA-18 and 14days of actigraphy recordings with a sleep diary. RESULTS: In addition to FEV1 <80% and low baseline SpO2, CF-related diabetes, PEG feeding and co-morbid behaviour disorder were associated with lower objective sleep quantity. Paternal smoking and a family member with a mood disorder were also associated with sleep disturbance. The use of electronic devices before bedtime was associated with lower sleep quantity and quality. FEV1, nocturnal cough, age and a behaviour disorder predicted sleep duration. FEV1, nocturnal cough, SpO2 nadir and asthma predicted sleep efficiency. Conversely, sleep efficiency independently predicted FEV1. CONCLUSIONS: Reduced sleep quality in children with CF is related to lung health and co-morbidities. However, family characteristics and poor sleep hygiene in the child were also associated with sleep disturbance. Optimal management of CF would seem to be the primary intervention to alleviate children's sleep disturbance, however our data raises additional targets for attempts to improve sleep.