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01.12.2012 | Research | Ausgabe 1/2012 Open Access

Health and Quality of Life Outcomes 1/2012

Participation and quality of life in children with Duchenne muscular dystrophy using the International Classification of Functioning, Disability, and Health

Zeitschrift:
Health and Quality of Life Outcomes > Ausgabe 1/2012
Autoren:
Roxanna M Bendixen, Claudia Senesac, Donovan J Lott, Krista Vandenborne
Wichtige Hinweise

Electronic supplementary material

The online version of this article (doi:10.​1186/​1477-7525-10-43) contains supplementary material, which is available to authorized users.

Competing interests

The authors declare that they have no competing interests

Authors’ contributions

RMB conceived and designed the study, acquired the data, analyzed and interpreted the data and drafted and revised the manuscript. CS recruited the subjects, scheduled times for the evaluations, and helped to draft and revise the manuscript. DJL participated in the design of the study and helped to draft and revise the manuscript. KV is the principal investigator of the larger study from which this pilot data was collected; KV participated in the design of the study and was involved in the drafting of the manuscript and revising for critically important intellectual content. All authors participated sufficiently in the work to take public responsibility for appropriate portions of the content. All authors read and approved the final manuscript.

Abstract

Background

Duchenne muscular dystrophy (DMD) is characterized by muscle damage and progressive loss of muscle function in male children. DMD is one of the most devastating genetically linked neuromuscular diseases for which there is currently no cure. Most clinical studies for DMD utilize a standard protocol for measurement exploring pathophysiology, muscle strength and timed tasks. However, we propose that examining broader components of health as emphasized by the International Classification of Functioning, Disability and Health-Children and Youth Version (ICF-CY) may be of great value to children and their families, and important outcomes for future clinical trials.

Methods

Fifty boys with DMD and 25 unaffected age-matched boys completed two self-report measures: the Children’s Assessment of Participation and Enjoyment and the Pediatric Quality of Life InventoryTM 4.0. We investigated differences between the two groups with regard to participation in life activities and perceived quality of life (QoL). Additionally, we compared participation in activities and QoL in both cohorts of younger and older boys.

Results

Participation in physical activities was significantly lower in boys with DMD than unaffected boys. Perceived QoL was markedly diminished in children with DMD relative to unaffected controls, except in the emotional domain. The amount of time boys engage in an activity, as well as participation in social activities, declined for our older boys with DMD but no changes were observed for our older unaffected boys. For both groups, QoL remained constant over time.

Conclusions

The ICF-CY provides a conceptual framework and specific terminology that facilitates investigation of the consequences of impairment in children and youth. Our study is one of the first to explore participation in a cohort of boys with DMD. It was not surprising that activities of choice for boys with DMD were less physical in nature than unaffected boys their age, but the consequences of less social engagement as the boys with DMD age is of great concern. Results from our study underscore the need to further evaluate activities that children elect to participate in, with special emphasis on facilitators and barriers to participation and how participation changes throughout the course of a disease.
Zusatzmaterial
Authors’ original file for figure 1
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Authors’ original file for figure 2
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Authors’ original file for figure 3
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Literatur
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