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 (AC-QoL) questionnaire, measures of social context and youths' physical status. Associations between carer-QoL 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.

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 Well-Being in Youth With Neuromuscular Disorders Who Are Wheelchair Users: A Systematic Review.

OBJECTIVE: To investigate quality of life (QOL) and psychosocial well-being in youth with neuromuscular disorders (NMDs) who are wheelchair users. DATA SOURCES: MEDLINE, Embase, CINAHL, and PsycINFO (January 2004-April 2016) and reference lists of retrieved full-text articles. STUDY SELECTION: Peer-reviewed studies were included when data describing self-reported QOL and psychosocial well-being could be separately understood for those using wheelchairs and 12 to 22 years of age. There were 2058 records independently screened, and potentially eligible articles were obtained and examined by all reviewers. Twelve observational and 3 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 generalizability. DATA SYNTHESIS: Heterogeneity of measurement and reporting precluded meta-analysis. Data were cross-sectional only. Compared with same-age typically developing peers, physical QOL was scored consistently and significantly lower in youth with NMDs, whereas psychosocial QOL was not. Psychosocial QOL was highest in youth nonambulant since early childhood and in those recruited via single tertiary specialist clinics. Mental health and social participation could not be compared with same-age populations. CONCLUSIONS: Despite low physical QOL, psychosocial QOL in youth with NMDs appeared comparable with same-age peers. The psychosocial well-being of younger adolescents on degenerative disease trajectories appeared most compromised; however, the longitudinal effects of growing up with a NMD on mental health and social participation are unknown. Interpretation was hampered by poor description of participant age, sex and physical ability; lack of population-based recruitment strategies; and inconsistent use of age-appropriate measures. Understanding of self-reported QOL and psychosocial well-being in youth with NMDs transitioning to adulthood is limited.

A fine balance and a shared learning journey: Exploring healthcare engagement through the experiences of youth with Neuromuscular Disorders.

BACKGROUND: Youth with Neuromuscular Disorders (NMD) who are wheelchair users can now survive well into adulthood if their multisystem comorbidities are prudently managed. Uptake of health behaviors may optimize their health outcomes. OBJECTIVE: To explore youths' perceptions of health, health behaviors and healthcare engagement. METHODS: This qualitative study purposefully recruited 11 youth with NMD from a concurrent, population-based study for variability of age, gender, type of NMD and their ratings of motivation and engagement. Interview data were analyzed and synthesized by thematic content. RESULTS: Participants perceived healthcare engagement as being given tools (knowledge and responsibility) and using them to maintain their finely balanced health. Nested in adequate social, emotional and physical support, they took responsibility for creatively integrating health behaviors they felt were informed by credible knowledge, gained primarily through personal experience. CONCLUSION: Cognizant of their compromised health, youth with NMD in this study were motivated to maintain their physical health. Limited NMD condition specific knowledge challenged youths' uptake of health behaviors. They valued a learning partnership with their healthcare professionals. By embracing the youth's experience based knowledge and through facilitating supportive relationships, healthcare professionals co-construct youth's healthcare engagement that may optimize health behaviors and outcomes.

The value of the CoughAssist® in the daily lives of children with neuromuscular disorders: Experiences of families, children and physiotherapists.

OBJECTIVES: We explored parents', children's and physiotherapists' experiences of regular CoughAssist® use, along with their perceptions of its value as an adjunct to in their daily, home respiratory management. METHODS: All children in the care of a specialist neuromuscular service who regularly used a CoughAssist® device at home participated. Qualitative case study methods involved semi-structured interviews with three children with neuromuscular disorders (NMD), their parents and physiotherapist. Data were analysed using thematic content analysis. RESULTS: Participants (n = 9) perceived the CoughAssist® held benefits for physical, social and emotional aspects of living with NMD. Poor adherence was identified as the major barrier to effective use, governed by factors including child's resistance, time constraints, treatment preference, practitioner support and fear of pressure trauma. CONCLUSIONS: Barriers to regular CoughAssist® use must be identified and individually addressed to enable uptake into respiratory care, accurately measure its effectiveness and realise its perceived benefits to children with NMD.