Mental wellbeing in non-ambulant youth with neuromuscular disorders: What makes the difference?

The physical and social challenges associated with neuromuscular disorders may impact mental wellbeing in non-ambulant youth during the more vulnerable period of adolescence. This cross-sectional survey investigated non-ambulant youths’ mental wellbeing and relationships with physical health, participation and social factors. The conceptual model was the International Classification of Functioning, Disability and Health (ICF). Thirty-seven youth aged 13 – 22 years old (mean age 17.4 years; n = 30 male; n = 24 Duchenne Muscular Dystrophy) and their parents provided biopsychosocial data through a comprehensive self-report questionnaire. The primary outcome measure was the Warwick Edinburgh Mental Wellbeing Scale (WEMWBS). Relationships between mental wellbeing and variables within and across each ICF domain were explored using linear regression models. Mean WEMWBS scores (55.3/70 [SD 8.1]) were higher than for typically developing youth and comparable to youth with other chronic conditions. Over half of youth reported severe co-morbidities across all body systems. Multivariable modelling indicated that mental wellbeing was independently associated with academic achievement and perceived family support but not with physical health variables. Beyond management of physical co-morbidities, enabling youths’ educational attainment and attending to social support likely optimises youth’s wellbeing

Quality of life and psychosocial wellbeing in youth with neuromuscular disorders who are wheelchair Users: A systematic review

Objective: To investigate quality of life (QoL) and psychosocial wellbeing in youth with Neuromuscular Disorders (NMD) who are wheelchair users. Data Sources: Medline, Embase, CINAHL and PsycINFO (January 2004 to April 2016) and reference lists of retrieved full-text papers. Study Selection: Peer-reviewed studies were included when data describing self-reported QoL and psychosocial wellbeing could be separately understood for those using wheelchairs and aged 12-22 years old. 2058 records were independently screened and potentially eligible papers were obtained and examined by all reviewers. Twelve observational and three qualitative studies met the inclusion criteria. Data Extraction: Population representativeness, measurement tools and outcomes, where possible with comparison groups. Two reviewers independently appraised studies for risk of bias to internal validity and generalisability. Data Synthesis: Heterogeneity of measurement and reporting precluded meta-analysis. Data were cross-sectional only. Compared to same age typically developing peers, physical QoL was scored consistently and significantly lower in youth with NMD, whilst psychosocial QoL was not. Psychosocial QoL was highest in youth non-ambulant since early childhood and in those recruited via single tertiary specialist clinics. Mental health and social participation could not be compared to same age populations. Conclusions: Despite low physical QoL, psychosocial QoL in youth with NMD appeared comparable to same age peers. The psychosocial wellbeing of younger adolescents on degenerative disease trajectories appeared most compromised, though the longitudinal impacts of growing up with NMD on mental health and social participation are unknown. Interpretation was hampered by poor description of participant age, gender and physical ability, lack of population based recruitment strategies and inconsistent use of age appropriate measures. Understanding of self-reported QoL and psychosocial wellbeing in youth with NMD transitioning to adulthood is limited

Can regular use of the Cough Assist machine maintain lung function in children and adolescents with neuromuscular disease?

Question: Does regular use of the CoughAssist maintains lung function in children/adolescents with neuromuscular disease? Design: Prospective pilot observational case-matched cohort trial, combined with retrospective medical record review. Participants: Eight children (seven male) with neuromuscular disease. Intervention: Four children regularly using the CoughAssist were matched by age, height and mobility status with four currently not using the CoughAssist acting as a control group. Participants performed regular spirometry over a four month period using a Vitalograph alpha 6000 spirometer. Outcome measures: vital capacity, peak expiratory flow rate, and peak cough flow measures. Results: Mean age was 13.8 years (range 10-17 years). All required electric wheel chair for mobility. Time series analysis revealed no differences with vital capacity (p = 0.71), peak expiratory flow (p = 0.20) or peak cough flow (p = 0.54) associated with CoughAssist use. Vital capacity, peak expiratory flow rates and peak cough flow readings were all maintained in those regularly using the CoughAssist, while the control group had varied readings. Conclusion: This pilot study has identified that regular use of the CoughAssist may assist maintenance of lung function in the short term in those with neuromuscular disease. These findings warrant substantiation in a larger sample with consideration of using a random sampling methodology

Parent carer quality of life and night-time attendance in non-ambulant youth with neuromuscular disorders

Purpose: To describe and explore carer quality of life (QoL) and night-time attendance to their child in parents of non-ambulant youth with Neuromuscular Disorders. Methods: A cross-sectional population-based, comprehensive survey including the Adult Carer QoL (ACQoL) questionnaire, measures of social context and youths’ physical status. Associations between carerQoL or frequency of parents’ night-time attendance with independent variables were explored using linear and logistic regression models, respectively. Results: Parents’ perceived lower carer-QoL (mean 76.5/120, SD 18.5) when they attended to their child twice a night or more (n = 17/35) and with shorter time since their child was prescribed noninvasive ventilation (NIV). Parental night-time attendance was not associated with youth’s actual use of NIV, but was more likely when youth required assistance to turn in bed, reported frequent sleep discomfort and had more severe joint contractures. Conclusions: To optimize parent carer-QoL, interventions must address parents’ frequency of night-time attendance and youths’ sleep comfort