Research Article| Volume 29, ISSUE 1, P48-58, January 2019

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

Published:September 13, 2018DOI:


      • Non-ambulant youth with NMD rated their mental wellbeing higher than youth in the general population.
      • More frequent health complaints were associated with lower mental wellbeing.
      • Mental wellbeing was not independently associated with physical health variables.
      • Mental wellbeing was independently associated with academic achievement and family support.
      • Enabling youths’ educational attainment and attending to social support may optimise youth's wellbeing.


      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.


      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'


      Subscribe to Neuromuscular Disorders
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect


        • Woodcock IR
        • Fraser L
        • Norman P
        • Pysden K
        • Manning S
        • Childs A-M
        The prevalence of neuromuscular disease in the paediatric population in Yorkshire, UK; variation by ethnicity and deprivation status.
        Dev Med Child Neurol. 2016; 58: 877-883
        • Bushby K
        • Finkel R
        • Birnkrant DJ
        • Case LE
        • Clemens PR
        • Cripe L
        • et al.
        Diagnosis and management of Duchenne muscular dystrophy, part 1: diagnosis, and pharmacological and psychosocial management.
        Lancet Neurol. 2010; 9: 77-93
        • D'Amico A
        • Mercuri E
        • Tiziano FD
        • Bertini E
        Spinal muscular atrophy.
        Orphanet J Rare Dis. 2011; 6: 71
        • Ryder S
        • Leadley RM
        • Armstrong N
        • Westwood M
        • de Kock S
        • Butt T
        • et al.
        The burden, epidemiology, costs and treatment for Duchenne muscular dystrophy: an evidence review.
        Orphanet J Rare Dis. 2017; 12: 79
        • Mah JK
        • Thannhauser JE
        • McNeil DA
        • Dewey D
        Being the lifeline: the parent experience of caring for a child with neuromuscular disease on home mechanical ventilation.
        Neuromuscul Disord. 2008; 18: 983-988
        • Coverdale GE
        • Long AF
        Emotional wellbeing and mental health: an exploration into health promotion in young people and families.
        Perspect Public Health. 2015; 135: 27-36
        • Weinberg M
        • Tomyn A
        Community survey of young Victorians’ resilience and mental wellbeing. Full report: part A and part B.
        Victorian Health Promotion Foundation, Melbourne, Australia2015
      1. World Health Organisation. What is ICF? ICF education. Available from: 2015 [accessed: 13.02.2017]

        • Patton GC
        • Sawyer SM
        • Santelli JS
        • Ross DA
        • Afifi R
        • Allen NB
        • et al.
        Our future: a Lancet commission on adolescent health and wellbeing.
        Lancet. 2016; 387: 2423-2478
        • Travlos V
        • Bulsara C
        • Patman S
        • Downs J
        A fine balance and a shared learning journey: exploring healthcare engagement through the experiences of youth with neuromuscular disorders.
        NeuroRehabil. 2016; 39: 519-534
        • Travlos V
        • Patman S
        • Wilson A
        • Simcock G
        • Downs J
        Quality of life and psychosocial wellbeing in youth with neuromuscular disorders who are wheelchair users: a systematic review.
        Arch Phys Med Rehabil. 2017; 98 (1104-1017)
        • Landfeldt E
        • Lindgren P
        • Bell CF
        • Guglieri M
        • Straub V
        • Lochmüller H
        • et al.
        Quantifying the burden of caregiving in Duchenne muscular dystrophy.
        J Neurol. 2016; : 1-10
        • Hufton M
        • Roper H
        Variations in Duchenne muscular dystrophy course in a multi-ethnic UK population: potential influence of socio-economic factors.
        Dev Med Child Neurol. 2017; 59: 837-842
        • Shakkottai A
        • Kidwell KM
        • Townsend M
        • Nasr SZ
        A five-year retrospective analysis of adherence in cystic fibrosis.
        Ped Pulmonol. 2015; 50: 1224-1229
        • Faint NR
        • Staton JM
        • Stick SM
        • Foster JM
        • Schultz A
        Investigating self-efficacy, disease knowledge and adherence to treatment in adolescents with cystic fibrosis.
        J Paediatr Child Health. 2017; 53: 488-493
        • Landfeldt E
        • Lindgren P
        • Bell CF
        • Guglieri M
        • Straub V
        • Lochmüller H
        • et al.
        Health-related quality of life in patients with Duchenne muscular dystrophy: a multinational, cross-sectional study.
        Dev Med Child Neurol. 2016; 58: 508-515
        • Pangalila R
        • van den Bos GA
        • Bartels B
        • Bergen MP
        • Kampelmacher MJ
        • Stam HJ
        • et al.
        Quality of life of adult men with Duchenne muscular dystrophy in the Netherlands: implications for care.
        J Rehabil Med. 2015; 47: 161-166
        • Pangalila RF
        • van den Bos GA
        • Bartels B
        • Bergen M
        • Stam HJ
        • Roebroeck ME
        Prevalence of fatigue, pain, and affective disorders in adults with Duchenne Muscular Dystrophy and their associations with quality of life.
        Arch Phys Med Rehabil. 2015; 96: 1242-1247
        • van Huijzen S
        • van Staa A
        Chronic ventilation and social participation: experiences of men with neuromuscular disorders.
        Scand J Occup Ther. 2013; 20: 209-216
        • Carter B
        • Qualter P
        • Dix J
        Social relationships, loneliness and adolescence: the potential for disruption by chronic illness.
        J Child Health Care. 2015; 19: 421-422
      2. Treat-NMD Neuromuscular Network. Registry of outcome measures United Kingdom Jamarau Administration Unification. Available from: 2014 [accessed: January–June 2014]

        • Clarke A
        • Putz R
        • Friede T
        • Ashdown J
        • Adi Y
        • Martin S
        • et al.
        Warwick-Edinburgh Mental Well-being Scale (WEMWBS) acceptability and validation in English and Scottish secondary school students (The WAVES project).
        Warwick Medical School and Univeristy of Edinburgh, Edinburgh2010
        • Clarke A
        • Friede T
        • Putz R
        • Ashdown J
        • Martin S
        • Blake A
        • et al.
        Warwick-Edinburgh Mental Well-being Scale (WEMWBS): validated for teenage school students in England and Scotland. A mixed methods assessment.
        BMC Public Health. 2011; 11: 487
        • Werlauff U
        Methods to assess physical functioning, and their clinical applicability, in patients with spinal muscular atrophy and congenital myopathy.
        University of Copenhagen, Copenhagen2013
        • Fagoaga J
        • Girabent-Farrés M
        • Bagur-Calafat C
        • Febrer A
        • Steffensen BF
        Functional assessment for people unable to walk due to spinal muscular atrophy and duchenne muscular dystrophy. Translation and validation of the Egen Klassifikation 2 scale for the Spanish population.
        Rev Neurol. 2015; 60: 439-446
        • Currie C
        • Inchley J
        • Molcho M
        • Lenzi M
        • Veselska Z
        • Wild F
        Health Behaviour in School-aged Children (HBSC) study protocol: background, methodology and mandatory items for 2013/14 survey.
        Child and Adolescent Health Research Unit, St. Andrews2014
        • Ravens-Sieberer U
        • Erhart M
        • Torsheim T
        • Hetland J
        • Freeman J
        • Danielson M
        • et al.
        An international scoring system for self-reported health complaints in adolescents.
        Eur J Public Health. 2008; 18: 294-299
        • Drake C
        • Nickel C
        • Burduvali E
        • Roth T
        • Jefferson C
        • Badia P
        The Pediatric Daytime Sleepiness Scale (PDSS): sleep habits and school outcomes in middle-school children.
        Sleep Med. 2003; 26: 455-458
        • McCrea GL
        • Miaskowski C
        • Stotts NA
        • Macera L
        • Hart SA
        • Varma MG
        Review article: self-report measures to evaluate constipation.
        Aliment Pharmacol Ther. 2008; 27: 638-648
        • Tuffrey C
        • Bateman BJ
        • Colver AC
        The Questionnaire of Young People's Participation (QYPP): a new measure of participation frequency for disabled young people.
        Child Care Health Dev. 2013; 39: 500-511
        • Tuffrey C
        • Bateman BJ
        • Colver A
        Questionnaire of young people's participation version 2.0.
        Solent NHS Trust and Newcastle University, Fareham, UK2013
      3. Gramsch K, Kirschner J, Rodrigues M. Care-NMD questionnaire about care for people with Duchenne muscular dystrophy. New Zealand version. 2012.

        • Elwick H
        • Joseph S
        • Becker S
        • Becker F
        Manual for the Adult Carer Quality of Life questionnaire (AC-QoL).
        The Princes Royal Trust for Carers, London2010
        • Merrick H
        • McConachie H
        • Le Couteur A
        • Mann K
        • Parr JR
        • Pearce MS
        • et al.
        Characteristics of young people with long term conditions close to transfer to adult health services.
        BMC Health Serv Res. 2015; 15: 435
      4. IBM Support. Obtaining a confidence interval for a median IBM. Reference #: 1476966. Historical Number:21267]. Available from: 2016 [accessed: 19.02.2018]

        • Gibson BE
        • Zitzelsberger H
        • McKeever P
        ‘Futureless persons’: shifting life expectancies and the vicissitudes of progressive illness.
        Sociol Health Illn. 2009; 31: 554-568
        • Vuillerot C
        • Hodgkinson I
        • Bissery A
        • Schott-Pethelaz A-M
        • Iwaz J
        • Ecochard R
        • et al.
        Self-perception of quality of life by adolescents with neuromuscular diseases.
        J Adolesc Health. 2010; 46: 70-76
        • Magliano L
        • Patalano M
        • Sagliocchi A
        • Scutifero M
        • Zaccaro A
        • D'Angelo MG
        • et al.
        Burden, professional support, and social network in families of children and young adults with muscular dystrophies.
        Muscle Nerve. 2015; 52: 13-21
        • Sonneveld HM
        • Strating MM
        • van Staa AL
        • Nieboer AP
        Gaps in transitional care: what are the perceptions of adolescents, parents and providers.
        Child Care Health Dev. 2013; 39: 69-80
        • Nierse CJ
        • Abma TA
        • Horemans AMC
        • Van Engelen BGM
        Research priorities of patients with neuromuscular disease.
        Disabil Rehabil. 2013; 35: 405-412
        • Lamb C
        • Peden A
        Understanding the experience of living with spinal muscular atrophy: a qualitative description.
        J Neurosci Nurs. 2008; 40: 250-256
        • O'Dea SM
        • Shuttleworth RP
        • Wedgwood N
        Disability, doctors and sexuality: do healthcare providers influence the sexual wellbeing of people living with a neuromuscular disorder.
        Sex Disabil. 2011; 30: 171-185
        • Young HK
        • Lowe A
        • Fitzgerald DA
        • Seton C
        • Waters KA
        • Kenny E
        • et al.
        Outcome of noninvasive ventilation in children with neuromuscular disease.
        Neurology. 2007; 68: 198-201
        • Cotton SM
        • Voudouris NJ
        • Greenwood KM
        Association between intellectual functioning and age in children and young adults with Duchenne muscular dystrophy: further results from a meta-analysis.
        Dev Med Child Neurol. 2005; 47: 257-265
        • Battini R
        • Chieffo D
        • Bulgheroni S
        • Piccini G
        • Pecini C
        • Lucibello S
        • et al.
        Cognitive profile in Duchenne muscular dystrophy boys without intellectual disability: the role of executive functions.
        Neuromuscul Disord. 2018; 28: 122-128
        • Wisk LE
        • Weitzman ER
        Expectancy and achievement gaps in educational attainment and subsequent adverse health effects among adolescents with and without chronic medical conditions.
        J Adolesc Health. 2017; 61: 461-470
        • Hamilton MJ
        • Longman C
        • O'Hara A
        • Kirkpatrick M
        • McWilliam R
        Growing up with spinal muscular atrophy with respiratory distress (SMARD1).
        Neuromuscul Disord. 2015; 25: 169-171
        • Hull J
        • Aniapravan R
        • Chan E
        • Chatwin M
        • Forton J
        • Gallagher J
        • et al.
        British Thoracic Society guideline for respiratory management of children with neuromuscular weakness.
        Thorax. 2012; 67: i1-i40
        • Read J
        • Simonds A
        • Kinali M
        • Muntoni F
        • Garralda ME
        Sleep and well-being in young men with neuromuscular disorders receiving non-invasive ventilation and their carers.
        Neuromuscul Disord. 2010; 20: 458-463
        • Kraus D
        • Wong BL
        • Horn PS
        • Kaul A
        Constipation in duchenne muscular dystrophy: prevalence, diagnosis, and treatment.
        J Pediatr. 2016; 171: 183-188
        • Moore GE
        • Lindenmayer AW
        • McConchie GA
        • Ryan MM
        • Davidson ZE
        Describing nutrition in spinal muscular atrophy: a systematic review.
        Neuromuscul Disord. 2016; 26: 395-404
        • Noonan VK
        • Lyddiatt A
        • Ware P
        • Jaglal SB
        • Riopelle RJ
        • Bingham CO
        • et al.
        Montreal Accord on Patient-Reported Outcomes (PROs) use series. Paper 3: patient-reported outcomes can facilitate shared decision-making and guide self-management.
        J Clin Epidemiol. 2017; 89: 125-135
        • Bartlett SJ
        • Witter J
        • Cella D
        • Ahmed S
        Montreal Accord on Patient-Reported Outcomes (PROs) use series. Paper 6: creating national initiatives to support development and use—the PROMIS example.
        J Clin Epidemiol. 2017; 89: 148-153
        • Langer T
        • Kirschner J
        Outcomes in Duchenne muscular dystrophy: nature, nurture, culture–or all three.
        Dev Med Child Neurol. 2017; 59: 780-781