Participation in daily life activities and its relationship to strength and functional measures in boys with Duchenne muscular dystrophy

Abstract

Purpose: While most studies of Duchenne muscular dystrophy (DMD) have focused on physical impairment, there is a need to explore how impairment impacts real-life experiences in order to provide intervention strategies focused on participation. Objectives were: (1) to investigate the domains of participation in a sample of boys with DMD; (2) to compare a younger (<10 years) and older (≥10 years) group of boys with DMD with regard to participation; (3) to investigate strength and timed functional tests in a sample of boys with DMD; (4) to compare a younger (<10 years) and older (≥10 years) group of boys with DMD with regard to strength and timed functional tests; and (5) to explore associations between participation and strength and timed functional tests for our DMD cohorts.

Methods: This cross-sectional study included 60 boys with DMD (mean 9.3 years ± 0.3). Boys completed strength testing, timed functional tests, the Children's Assessment of Participation and Enjoyment and the ACTIVLIM. Independent samples t-tests were used to test for differences in all measures between our younger and older cohorts; Spearman's (rank) correlation was used to assess relationships between participation and strength and time functional tests.

Results: Significant differences were found between our younger and older boys with DMD in the areas of recreational (p < 0.01), social (p < 0.001), and skill-based activities (p < 0.05), as well as with whom and where the activities were performed (p < 0.05 and 0.001, respectively). Older boys with DMD report lower levels of participation in these areas, as well as less engagement in activities with individuals other than family members and less participation outside of the home. Lower levels of strength and slower rates of functional performance correlate with participation in fewer physical activities for our younger cohort and fewer physical and social activities for our older cohort.

Conclusions: Strength and function relate to the variability and type of activities in which boys with DMD participate. A key finding is the significant decline in social activities and community-based engagement as the boys with DMD age. The ultimate goal of an intervention is for our children to be as actively engaged in life as they desire. This requires addressing participation when measuring outcomes in order to more fully understand limitations and provide appropriate strategies for continued participation for boys and their families.

Implications for rehabilitation: Duchenne muscular dystrophy is a devastating progressive neuromuscular disorder that leads to significant strength and functional limitations, which affect physical and social participation for these boys. The ability to move beyond clinically-based outcomes and assess and monitor a child's daily activities through participation measures may provide information for therapeutic interventions. Rehabilitation specialists have a role as advocates for social and community engagement for children with physical limitations. Providing families with information on community-based opportunities, and the strategies and environmental modifications available may increase social participation for our youth growing up with a neuromuscular disorder.

Keywords: Duchenne muscular dystrophy; functional outcomes; participation; social engagement; strength outcomes.

Conflict of interest statement

Declaration of Interests

The project described was supported by Award Number K01HD064778 from the Eunice Kennedy Shriver National Institute of Child Health & Human Development. The content is solely the responsibility of the authors and does not necessarily represent the official views of the Eunice Kennedy Shriver National Institute of Child Health & Human Development or the National institutes of Health. Additional funding for recruitment and participant support was provided through Parent Project Muscular Dystrophy, the Muscular Dystrophy Association, and the National Institute of Arthritis and Musculoskeletal and Skin Diseases, and National Institute of Neurological Disorders and Stroke (PI, K. Vandenborne – R01AR056973). All authors report no further declarations of interest.

Figures

Figure 1

Children’s Assessment of Participation and Enjoyment (CAPE) report of scores in participation for younger (n=35) and older (n=25) boys with DMD. Score 0=lowest participation to 7=highest participation. Means ± standard error of the mean presented. *p≤0.05; **p≤0.01; ***p≤0.001.

Figure 2

Timed functional tests measured in seconds: Time to walk or run 10-meters (10m walk/run), time to rise from the floor (SupineUp), and time to walk up 4 stairs (4 Stairs). Includes censored data. Means ± standard error of the mean presented. * p

Figure 3

Correlation between participation in physical activities (CAPE) and time to walk up 4 stairs for younger boys with DMD (r=−0.40, p ≤0.05). Correlation between participation in social activities (CAPE) and time to walk up 4 stairs for older boys with DMD (r= −0.46; p≤0.05). Does not include censored data. Dashed line (trend) provided for visual purposes only.