Fatigue in young people with Duchenne muscular dystrophy

Abstract

AIM To describe fatigue in Duchenne muscular dystrophy (DMD) from patients' and parents' perspectives and to explore risk factors for fatigue in children and adolescents with DMD.METHOD A multicentre, cross-sectional study design was used. Seventy-one patients (all males; median age 12y, age range 5-17y) identified via the Canadian Neuromuscular Disease Registry, and their parents completed questionnaires. Subjective fatigue was assessed using the Pediatric Quality of Life Inventory Multidimensional Fatigue Scale by child self-report and parent proxy-report. RESULTSPatients with DMD across ages and disease stages experienced greater fatigue compared to typically developing controls from published data. Sleep disturbance symptoms were associated with greater fatigue by child self-report (q=-0.42; p=0.003) and parent proxyreport (q=-0.51; p<0.001). Depressive symptoms were associated with greater fatigue by child self-report (q=-0.46; p<0.001) and parent proxy-report (q=-0.45; p<0.001). Lower functional ability was associated with greater fatigue by parent proxy-report (q=0.26; p=0.03). Physical activity level, and musculoskeletal, respiratory, and cardiac function were not associated with fatigue.

A Case Study With Symbihand: An sEMG-Controlled Electrohydraulic Hand Orthosis for Individuals With Duchenne Muscular Dystrophy

“…ADL require sufficient grip forces and additionally the ability to modulate those, without additional effort or fatigue. In DMD, the hand grip force significantly declines after the age of 12, accompanied by early fatigue onset [8], leading in increasing inability of performing ADL [9]. However, hand treatment for such individuals is not receiving a lot of attention [10], and there is no evidence of training grip force modulation or hand fatigue reduction in individuals with DMD.…”

The association between dietary factors and body weight and composition in boys with Duchenne muscular dystrophy

“…Childhood obesity is a complex issue caused by a multitude of environmental, genetic and lifestyle factors 5 . For children with DMD, additional risk factors include physical disability which limits physical activity, 6 a potentially reduced resting energy expenditure as a result of low muscle mass, 7 an increased appetite from chronic use of glucocorticosteroids (“steroids”) 8,9 and sleep disturbance and fatigue 10 . Steroids are best practice therapy for DMD and are not only associated with improved muscle strength, function, quality of life and independent mobility, but also associated with weight gain 8,9 .…”

A muscle fatigue-like contractile decline was recapitulated using skeletal myotubes from Duchenne muscular dystrophy patient-derived iPSCs

“…A decline in muscle performance and fatigue accumulation are common symptoms across ages and disease stages of DMD patients. 4 Despite their frequencies, they are still not well recapitulated and studied in vitro . In addition, the correlation between them and DMD onset remains controversial.…”