Coping in Children and Adolescents with a Genetic Muscle Disorder –Findings from a Population-Based Study

Article type: Research Article

Authors: Jones, Kelly M.^{a; *} | Anand, Aaron^a | Bright, Charmaine^b | O’Grady, Gina^c | Rodrigues, Miriam J.^{d; e} | Ranta, Annemare^f | Roxburgh, Richard H.^{d; g} | Theadom, Alice^a | on behalf of the MD-PREV study group¹

Affiliations: [a] National Institute for Stroke and Applied Neurosciences, School of Clinical Sciences, Faculty of Health and Environmental Sciences, Auckland University of Technology, Auckland, New Zealand | [b] School of Clinical Sciences, Faculty of Health and Environmental Sciences, Auckland University of Technology, Auckland, New Zealand | [c] Paediatric Neurology, Starship Children’s Health, Auckland, New Zealand | [d] Neurology Department, Auckland City Hospital, Auckland, New Zealand | [e] Muscular Dystrophy Association of New Zealand, Auckland, New Zealand | [f] Department of Neurology, Wellington Regional Hospital, University of Otago, New Zealand | [g] Centre for Brain Research, University of Auckland, Auckland, New Zealand

Correspondence: [*] Correspondence to: Dr Kelly M. Jones National Institute for Stroke and Applied Neurosciences, School of Clinical Sciences, Faculty of Health and Environmental Sciences, Auckland University of Technology, Private Bag 92006, Auckland 1142, New Zealand. Tel.: +64 9 921 9999; E-mail: kelly.jones@aut.ac.nz.

Note: [1] MD-PREV Study Group members listed below.

Abstract: Background:The impacts of genetic muscle disorders on quality of life in affected children are well-documented. However, few studies have investigated children’s coping strategies and relationships between coping and quality of life. Objectives:To determine coping strategy use, efficacy, and associations with quality of life in children with a genetic muscle disorder. Methods:Forty-eight children (6–15 years, 58% male) with a genetic muscle disorder were identified as part of a national prevalence study. Children completed the Kidcope in response to a specific stressor (doctors visits) and the Pediatric Quality of Life Inventory Neuromuscular Module. Results:‘Wishful thinking’ (75%, 36/48) and ‘cognitive restructuring’ (71%, 34/48) were the most frequently used coping strategies. ‘Self-criticism’ (12%, 6/48), and ‘blaming others’ and ‘resignation’ (both 19%, 9/48) were the least used strategies. Coping strategy use did not differ across age and sex groups (p’s from 0.08 to 1.00). Positive coping strategies tended to be more effective (medians ranged from 2.00 to 2.75) than negative strategies (medians ranged from 1.38 to 2.50). Using a greater number of different types of positive (F(4, 46) = 5.79, p = 0.001) and/or negative (F(4, 44) = 5.64, p 0.001) coping strategies was linked to poorer health-related quality of life. Conclusion:We conclude that children with genetic muscle disorders use a wide range of positive and/or negative coping strategies in response to stressors associated with a doctor visit and may benefit from greater support to improve health-related quality of life. Findings support the value of routine screening of children’s coping to identify those who would benefit from support.

Keywords: Neuromuscular diseases, muscle diseases, coping, quality of life, child, adolescent

DOI: 10.3233/JND-200564

Journal: Journal of Neuromuscular Diseases, vol. 8, no. 6, pp. 1069-1078, 2021