Objective: Children living with a genetic muscle disorder (GMD) report significant impairments in health-related quality of life (HRQOL) as a result of living with a chronic and progressive disorder. The challenges posed by living with a GMD may lead to additional and increasing daily stressors for these children and their families/whanau. Children use a range of coping strategies in an effort to respond to such stressors. Currently, however, limited literature is available regarding the use of coping strategies in children living with a GMD. The literature that is available focuses predominantly on a single GMD - Duchenne muscular dystrophy. This study sought to explore self-reported use and perceived effectiveness of coping strategies among children across a range of GMD and to identify associations with HRQOL.

Method: The study examines secondary data collected as part of a nationwide, epidemiological study of the prevalence and impact of GMDs in NZ (MD-PREV). This sub-study involved 48 children (aged 5 to 15 years); 29 (60.4%) were male participants. Children were invited to complete the Kidcope and Paediatric Quality of Life questionnaires as part of the initial impact assessment. Inclusion in this sub-study required a clinical confirmation of a GMD, this included muscular dystrophy, congenital myopathy and ion channel muscle disorders.

Results: Cognitive restructuring (n = 34) and wishful thinking (n = 34) were the most commonly used coping strategies, whereas, social support (a lot, 75.00%), resignation (a lot, 55.60%), and distraction (a lot, 50.00%) were perceived as the most effective coping strategies. Positive coping strategies (M = 2.36, SD = .64; p < .01) were perceived to be more effective compared to negative coping strategies (M = 2.07, SD = .77; p < .01). There were no significant associations between the types of coping strategies used and children’s age, sex, or ethnicity (p >.05). Total number of positive (p < .01) and negative (p < .01) coping strategies used, along with ethnicity (p < .05), significantly accounted for 49% of total variance observed in self-reported HRQOL (p < .01). Sex and age were also included in the model, however, did not contribute significantly to variance seen in HRQOL (p > .05). Lower total HRQOL was significantly associated with the increased use of both positive and negative coping strategies (p < .01)

Conclusions: Study findings suggest that children use a range of positive and negative coping strategies when living with a GMD. While children reported positive coping strategies as more effective than negative strategies, results suggest that childrens use of a greater number of different coping strategies is associated with poorer self-reported HRQOL. Children living with a GMD may benefit from psychoeducation around how to use coping strategies effectively. Increased education for children, and their families/whanau, may help them to better cope with stressors related to their health condition.