Mental health and coping strategies in families of children and young adults with muscular dystrophies



Living with a progressive disease as muscular dystrophy (MD) can be challenging for the patient and the entire family from both emotional and practical point of view. We aimed to extend our previously published data about mental health in patients with MDs, also investigating coping profiles of both themselves and their parents. Furthermore, we wanted to verify whether psychological adaptation of patients can be predicted by coping strategies, taking also into account physical impairment, cognitive level and socioeconomic status.


112 patients with MDs, aged 2–32 were included. Their emotional and behavioural features were assessed through parent- and self-report Achenbach System for Empirically Based Assessment questionnaires and Strength and Difficulties Questionnaires. Development and Well-Being Assessment or Autism Diagnostic Observation Schedule were administered to confirm suspected diagnoses. Coping profile of both parents and patients was assessed through the self-administered New Italian Version of the Coping Orientation to the Problems Experienced questionnaire and its relationship with emotional/behavioural outcome was examined in linear regression analyses.


High prevalence of intellectual disability and autism spectrum disorders was confirmed in Duchenne MD. Despite the high rate of internalizing symptomatology, we did not report higher rate of psychopathological disorders compared to general population. Parents tend to rely more on positive reinterpretation and less on disengagement coping. Avoidance coping, whether used by parents or patients, and ID, predicted increased emotional/behavioural problems.


Psychosocial interventions should address problems of anxiety and depression that people with MDs frequently experience, even through fostering parents’ and childrens’ engagement coping over disengagement coping.

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  1. 1.

    Carter GT (1997) Rehabilitation management in neuromuscular disease. J Neuro Rehabil 11:69–80.

    Article  Google Scholar 

  2. 2.

    Pieterse AJ, Cup EH, Knuijt S et al (2008) Development of a tool to guide referral of patients with neuromuscular disorders to allied health services. Part two. Disabil Rehabil 30:863–870.

    Article  PubMed  Google Scholar 

  3. 3.

    Straub V, Bertoli M (2016) Where do we stand in trial readiness for autosomal recessive limb girdle muscular dystrophies? Neuromuscul Disord 26:111–125.

    Article  PubMed  Google Scholar 

  4. 4.

    Birnkrant DJ, Bushby K, Bann CM et al (2018) Diagnosis and management of Duchenne muscular dystrophy, part 2: respiratory, cardiac, bone health, and orthopaedic management. Lancet Neurol 17:347–361.

    Article  PubMed  PubMed Central  Google Scholar 

  5. 5.

    Birnkrant DJ, Bushby K, Bann CM et al (2018) Diagnosis and management of Duchenne muscular dystrophy, part 3: primary care, emergency management, psychosocial care, and transitions of care across the lifespan. Lancet Neurol 17:445–455.

    Article  PubMed  PubMed Central  Google Scholar 

  6. 6.

    Birnkrant DJ, Bushby K, Bann CM et al (2018) Diagnosis and management of Duchenne muscular dystrophy, part 1: diagnosis, and neuromuscular, rehabilitation, endocrine, and gastrointestinal and nutritional management. Lancet Neurol 17:251–267.

    Article  PubMed  PubMed Central  Google Scholar 

  7. 7.

    Fayssoil A, Abasse S, Silverston K (2017) Cardiac involvement classification and therapeutic management in patients with duchenne muscular dystrophy. J Neuromuscul Dis 4:17–23.

    Article  PubMed  PubMed Central  Google Scholar 

  8. 8.

    Magri F, Nigro V, Angelini C et al (2017) The Italian limb girdle muscular dystrophy registry: relative frequency, clinical features, and differential diagnosis. Muscle Nerve 55:55–68.

    CAS  Article  PubMed  Google Scholar 

  9. 9.

    Taghizadeh E, Rezaee M, Barreto GE, Sahebkar A (2019) Prevalence, pathological mechanisms, and genetic basis of limb-girdle muscular dystrophies: a review. J Cell Physiol 234:7874–7884.

    CAS  Article  PubMed  Google Scholar 

  10. 10.

    Mercuri E, Muntoni F (2013) Muscular dystrophies. Lancet 381:845–860.

    CAS  Article  Google Scholar 

  11. 11.

    Pousada T, Groba B, Nieto-Riveiro L et al (2018) Determining the burden of the family caregivers of people with neuromuscular diseases who use a wheelchair. Medicine (Baltimore) 97:e11039.

    Article  Google Scholar 

  12. 12.

    Snow WM, Anderson JE, Jakobson LS (2013) Neuropsychological and neurobehavioral functioning in Duchenne muscular dystrophy: a review. Neurosci Biobehav Rev 37:743–752.

    Article  PubMed  Google Scholar 

  13. 13.

    Darke J, Bushby K, Le Couteur A, McConachie H (2006) Survey of behaviour problems in children with neuromuscular diseases. Eur J Paediatr Neurol 10:129–134.

    Article  PubMed  Google Scholar 

  14. 14.

    Latimer R, Street N, Conway KC (2017) Secondary conditions among males with duchenne or becker muscular dystrophy. J Child Neurol 32:663–670.

    Article  PubMed  PubMed Central  Google Scholar 

  15. 15.

    Colombo P, Nobile M, Tesei A et al (2017) Assessing mental health in boys with Duchenne muscular dystrophy: emotional, behavioural and neurodevelopmental profile in an Italian clinical sample. Eur J Paediatr Neurol 21:639–647.

    Article  PubMed  Google Scholar 

  16. 16.

    Ricotti V, Mandy WP, Scoto M et al (2016) Neurodevelopmental, emotional, and behavioural problems in Duchenne muscular dystrophy in relation to underlying dystrophin gene mutations. Dev Med Child Neurol 58:77–84.

    Article  PubMed  Google Scholar 

  17. 17.

    Banihani R, Smile S, Yoon G et al (2015) Cognitive and neurobehavioral profile in boys with duchenne muscular dystrophy. J Child Neurol 30:1472–1482.

    Article  PubMed  Google Scholar 

  18. 18.

    Young HK, Barton BA, Waisbren S et al (2008) Cognitive and psychological profile of males with Becker muscular dystrophy. J Child Neurol 23:155–162.

    Article  PubMed  Google Scholar 

  19. 19.

    Pane M, Lombardo ME, Alfieri P et al (2012) Attention deficit hyperactivity disorder and cognitive function in Duchenne muscular dystrophy: phenotype–genotype correlation. J Pediatr 161:705–9.e1.

    Article  PubMed  Google Scholar 

  20. 20.

    Caspers Conway K, Mathews KD, Paramsothy P et al (2015) Neurobehavioral concerns among males with dystrophinopathy using population-based surveillance data from the muscular dystrophy surveillance, tracking, and research network. J Dev Behav Pediatr 36:455–463.

    Article  PubMed  Google Scholar 

  21. 21.

    Miladi N, Bourguignon JP, Hentati F (1999) Cognitive and psychological profile of a Tunisian population of limb girdle muscular dystrophy. Neuromuscul Disord 9:352–354.

    CAS  Article  PubMed  Google Scholar 

  22. 22.

    Magliano L, D’Angelo MG, Vita G et al (2014) Psychological and practical difficulties among parents and healthy siblings of children with Duchenne vs. Becker muscular dystrophy: an Italian comparative study. Acta Myol 33:136–143

    PubMed  PubMed Central  Google Scholar 

  23. 23.

    Aho AC, Hultsjö S, Hjelm K (2015) Young adults experiences of living with recessive limb-girdle muscular dystrophy from a salutogenic orientation: an interview study. Disabil Rehabil 37:2083–2091.

    Article  PubMed  Google Scholar 

  24. 24.

    Sabharwal R (2014) The link between stress disorders and autonomic dysfunction in muscular dystrophy. Front Physiol.

    Article  PubMed  PubMed Central  Google Scholar 

  25. 25.

    Gagliardi BA (1991) The impact of Duchenne muscular dystrophy on families. Orthop Nurs 10:41–49.

    CAS  Article  PubMed  Google Scholar 

  26. 26.

    Kenneson A, Bobo JK (2010) The effect of caregiving on women in families with Duchenne/Becker muscular dystrophy. Health Soc Care Commun 18:520–528.

    Article  Google Scholar 

  27. 27.

    Pangalila RF, van den Bos GA, Stam HJ et al (2012) Subjective caregiver burden of parents of adults with Duchenne muscular dystrophy. Disabil Rehabil 34:988–996.

    Article  PubMed  Google Scholar 

  28. 28.

    Holroyd J, Guthrie D (1986) Family stress with chronic childhood illness: cystic fibrosis, neuromuscular disease, and renal disease. J Clin Psychol 42:552–561.;2-8

    CAS  Article  PubMed  Google Scholar 

  29. 29.

    Abi Daoud MS, Dooley JM, Gordon KE (2004) Depression in parents of children with Duchenne muscular dystrophy. Pediatr Neurol 31:16–19.

    Article  PubMed  Google Scholar 

  30. 30.

    Magliano L, Patalano M, Sagliocchi A et al (2014) “I have got something positive out of this situation”: psychological benefits of caregiving in relatives of young people with muscular dystrophy. J Neurol 261:188–195.

    Article  PubMed  Google Scholar 

  31. 31.

    Pangalila RF, Van den Bos GA, Bartels B et al (2015) Quality of life of adult men with Duchenne muscular dystrophy in the Netherlands: implications for care. J Rehabil Med 47:161–166.

    Article  PubMed  Google Scholar 

  32. 32.

    Landfeldt E, Lindgren P, Bell CF et al (2016) Health-related quality of life in patients with Duchenne muscular dystrophy: a multinational, cross-sectional study. Dev Med Child Neurol 58:508–515.

    Article  PubMed  Google Scholar 

  33. 33.

    Bendixen RM, Senesac C, Lott DJ, Vandenborne K (2012) Participation and quality of life in children with Duchenne muscular dystrophy using the International Classification of Functioning, Disability, and Health. Health Qual Life Outcomes.

    Article  PubMed  PubMed Central  Google Scholar 

  34. 34.

    Thompson RJ (1985) Coping with the stress of chronic childhood illness. In: O’Quinn A (ed) Management of chronic disorders of childhood. G. K. Hall, Boston, pp 11–41

    Google Scholar 

  35. 35.

    Thompson RJ, Gustafson KE (1996) Adaptation to chronic childhood illness. American Psychological Association, Washington DC

    Google Scholar 

  36. 36.

    Lazarus RS, Folkman S (1984) Stress appraisal and coping. Springer, New York

    Google Scholar 

  37. 37.

    Carver CS, Connor-Smith J (2010) Personality and coping. Annu Rev Psychol 61:679–704.

    Article  PubMed  Google Scholar 

  38. 38.

    Eisenbarth C (2012) Coping profiles and psychological distress: a cluster analysis. N Am J Psychol 14:485–496

    Google Scholar 

  39. 39.

    Nielsen MB, Knardahl S (2014) Coping strategies: a prospective study of patterns, stability, and relationships with psychological distress. Scand J Psychol 55:142–150.

    Article  PubMed  Google Scholar 

  40. 40.

    Compas BE, Jaser SS, Dunn MJ, Rodriguez EM (2012) Coping with chronic illness in childhood and adolescence. Annu Rev Clin Psychol 8:455–480.

    Article  PubMed  Google Scholar 

  41. 41.

    Spira AP, Zvolensky MJ, Eifert GH, Feldner MT (2004) Avoidance-oriented coping as a predictor of panic-related distress: a test using biological challenge. J Anxiety Disord 18:309–323.

    Article  PubMed  Google Scholar 

  42. 42.

    Anshel MH, Kim KW, Kim BH, Chang KJ, Eom HJ (2001) A model for coping with stressful events in sport: theory, application, and future directions. Int J Sport Psychol 32:43–75

    Google Scholar 

  43. 43.

    Thompson RJ Jr, Zeman JL, Fanurik D, Sirotkin-Roses M (1992) The role of parent stress and coping and family functioning in parent and child adjustment to Duchenne Muscular Dystrophy. J Clin Psychol 48:11–19.;2-4

    Article  PubMed  Google Scholar 

  44. 44.

    Steinberg L, Cauffman E, Woolard J, Graham S, Banich M (2009) Are adolescents less mature than adults?: minors’ access to abortion, the juvenile death penalty, and alleged APA “flip-flop”. Am Psychol 64:583–594.

    Article  PubMed  Google Scholar 

  45. 45.

    Bushby K, Finkel R, Birnkrant DJ et al (2010) Diagnosis and management of Duchenne muscular dystrophy, Part 2: implementation of multidisciplinary care. Lancet Neurol 9:177–189.

    CAS  Article  PubMed  Google Scholar 

  46. 46.

    Vissing J (2016) Limb girdle muscular dystrophies: classification, clinical spectrum and emerging therapies. Curr Opin Neurol 29:635–641.

    CAS  Article  PubMed  Google Scholar 

  47. 47.

    Padberg GW, van Engelen BG (2009) Facioscapulohumeral muscular dystrophy. Curr Opin Neurol 22:539–542.

    Article  PubMed  Google Scholar 

  48. 48.

    Hollingshead AB (1975) Four factor index of socioeconomic status. Yale J Sociol 8:21–52

    Google Scholar 

  49. 49.

    Achenbach TM, Rescorla L (2001) Manual for the ASEBA school-age forms & profiles: child behavior checklist for ages 6–18, teacher’s report form, youth self-report. An Integrated System of Multi-Informant Assessment. Research Center for Children, Youth, & Families, University of Vermont, Burlington

  50. 50.

    Achenbach TM, Rescorla L (2003) Manual for the ASEBA adult forms and profiles. Research Center for Children, Youth, & Families, University of Vermont, Burlington

  51. 51.

    Goodman R (1999) The extended version of the Strengths and Difficulties Questionnaire as a guide to child psychiatric caseness and consequent burden. J Child Psychol Psychiatry 40:791–799.

    CAS  Article  PubMed  Google Scholar 

  52. 52.

    Stone LL, Otten R, Engels RC et al (2010) Psychometric properties of the parent and teacher versions of the strengths and difficulties questionnaire for 4- to 12-year-olds: a review. Clin Child Fam Psychol Rev 13:254–274.

    Article  PubMed  PubMed Central  Google Scholar 

  53. 53.

    Goodman R (1997) The strengths and difficulties questionnaire: a research note. J Child Psychol Psychiatry 38:581–586.

    CAS  Article  Google Scholar 

  54. 54.

    Goodman R, Ford T, Richards H et al (2000) The development and well-being assessment: description and initial validation of an integrated assessement of child and adolescent psychopathology. J Child Psychol Psychiatry 41:645–655.

    CAS  Article  PubMed  Google Scholar 

  55. 55.

    Lord C, Rutter M, DiLavore PC, Risi S (1999) ADOS. Autism diagnostic observation schedule. Manual. WPS, Los Angeles

    Google Scholar 

  56. 56.

    Ford T, Goodman R, Meltzer H (2003) The British child and adolescent mental health survey 1999: the prevalence of DSM-IV disorders. J Am Acad Child Adolesc Psychiatry 42:1203–1211.

    Article  PubMed  Google Scholar 

  57. 57.

    Goodman A, Heiervang E, Collishaw S, Goodman R (2011) The “DAWBA bands” as an ordered-categorical measure of child mental health: description and validation in British and Norwegian samples. Soc Psychiatry Psychiatr Epidemiol 6:521–532.

    Article  Google Scholar 

  58. 58.

    Carver CS, Scheier MF, Weintraub KJ (1989) Assessing coping strategies: a theoretically based approach. J Pers Soc Psychol 56:267–283.

    CAS  Article  PubMed  Google Scholar 

  59. 59.

    Sica C, Magni C, Ghisi M et al (2008) Coping orientation to problems experienced-Nuova Versione Italiana (COPE-NVI): Uno strumento per la misura degli stili di coping. Psicoterapia Cognitiva E Comportamentale 14:27–53

    Google Scholar 

  60. 60.

    Frigerio A, Rucci P, Goodman R et al (2009) Prevalence and correlates of mental disorders among adolescents in Italy: the PrISMA study. Eur Child Adolesc Psychiatry 18:217–226.

    Article  PubMed  Google Scholar 

  61. 61.

    Meltzer H, Gatward R, Goodman R, Ford T (2003) Mental health of children and adolescents in Great Britain. Int Rev Psychiatry 15:185–187.

    CAS  Article  PubMed  Google Scholar 

  62. 62.

    Rothenberger A, Becker A, Erhart M et al (2008) Psychometric properties of the parent strengths and difficulties questionnaire in the general population of German children and adolescents: results of the BELLA study. Eur Child Adolesc Psychiatry 17:99–105.

    Article  PubMed  Google Scholar 

  63. 63.

    Kovess-Masfety V, Husky MM, Keyes K et al (2016) Comparing the prevalence of mental health problems in children 6–11 across Europe. Soc Psychiatry Psychiatr Epidemiol 51:1093–1103.

    Article  PubMed  PubMed Central  Google Scholar 

  64. 64.

    Narzisi A, Posada M, Barbieri F et al (2018) Prevalence of Autism Spectrum Disorder in a large Italian catchment area: a school-based population study within the ASDEU project. Epidemiol Psychiatr Sci 6:1–10.

    Article  Google Scholar 

  65. 65.

    McKenzie K, Milton M, Smith G, Ouellette-Kuntz H (2016) Systematic review of the prevalence and incidence of intellectual disabilities: current trends and issues. Curr Dev Disord Rep 3:104–115.

    Article  Google Scholar 

  66. 66.

    Polanczyk G, De Lima MS, Horta BL et al (2007) The worldwide prevalence of ADHD: a systematic review and metaregression analysis. Am J Psychiatry 164:942–948.

    Article  Google Scholar 

  67. 67.

    Fayyad J, De Graaf R, Kessler R et al (2007) Cross-national prevalence and correlates of adult attention-deficit hyperactivity disorder. Br J Psychiatry 190:402–409.

    CAS  Article  PubMed  Google Scholar 

  68. 68.

    Simon V, Czobor P, Bálint S et al (2009) Prevalence and correlates of adult attention-deficit hyperactivity disorder: meta-analysis. Br J Psychiatr 194:204–211.

    Article  Google Scholar 

  69. 69.

    Steel Z, Marnane C, Iranpour C et al (2014) The global prevalence of common mental disorders: a systematic review and meta-analysis 1980–2013. Int J Epidemiol 43:476–493.

    Article  PubMed  PubMed Central  Google Scholar 

  70. 70.

    Wallander JL, Dekker MC, Koot HM (2003) Psychopathology in children and adolescents with intellectual disability: measurement, prevalence, course, and risk. Int Rev Res Ment Retard 26:93–134.

    Article  Google Scholar 

  71. 71.

    Condly SJ (2006) Resilience in children: a review of literature with implications for education. Urban Educ 41:211–236.

    Article  Google Scholar 

  72. 72.

    Nereo NE, Fee RJ, Hinton VJ (2003) Parental stress in mothers of boys with Duchenne muscular dystrophy. J Pediatr Psychol 28:473–484.

    Article  PubMed  PubMed Central  Google Scholar 

  73. 73.

    Fee RJ, Hinton VJ (2011) Resilience in children diagnosed with a chronic neuromuscular disorder. J Dev Behav Pediatr 32:644–650.

    Article  PubMed  PubMed Central  Google Scholar 

  74. 74.

    Carter AS, Briggs-Gowan MJ, Davis NO (2004) Assessment of young children’s social-emotional development and psychopathology: recent advances and recommendations for practice. J Child Psychol Psychiatry 45:109–134.

    Article  PubMed  Google Scholar 

  75. 75.

    Sheldrick RC, Benneyan JC, Kiss IG et al (2015) Thresholds and accuracy in screening tools for early detection of psychopathology. J Child Psychol Psychiatry 56:936–948.

    Article  PubMed  PubMed Central  Google Scholar 

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The authors thank all patients and families for their collaboration and the medical, nursing and technical personnel of the IRCCS E Medea involved in the care and evaluation of the patients (E. Mornati, C. Gerosa, S. Molteni).


The work was supported by Grants of the Italian Ministry of Health Ricerca Corrente 2018 and 5 per mille funds for biomedical research (Grant no. 2013-2016).

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Correspondence to Maria Nobile.

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The study was approved by the Ethical Committee of the Scientific Institute IRCCS Eugenio Medea according to the Declaration of Helsinki.

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All patients provided written informed consent for participation in the study.

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Tesei, A., Nobile, M., Colombo, P. et al. Mental health and coping strategies in families of children and young adults with muscular dystrophies. J Neurol 267, 2054–2069 (2020).

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  • Muscular dystrophy
  • Emotional problems
  • Behavioural problems
  • Psychopathology
  • Coping strategies
  • Mental health