Bendixen Roxanna M, Lott Donovan J, Senesac Claudia, Mathur Sunita, Vandenborne Krista
Department of Occupational Therapy, University of Pittsburgh , Pittsburgh, PA , USA .
Disabil Rehabil. 2014;36(22):1918-23. doi: 10.3109/09638288.2014.883444. Epub 2014 Feb 6.
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.
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.
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.
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.
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.
虽然大多数关于杜氏肌营养不良症（DMD）的研究都集中在身体损伤方面，但仍有必要探讨损伤如何影响现实生活体验，以便提供侧重于参与度的干预策略。目标如下：（1）调查DMD男孩样本中的参与领域；（2）比较年龄较小（<10岁）和年龄较大（≥10岁）的DMD男孩在参与度方面的差异；（3）调查DMD男孩样本中的力量和定时功能测试；（4）比较年龄较小（<10岁）和年龄较大（≥10岁）的DMD男孩在力量和定时功能测试方面的差异；（5）探索我们的DMD队列中参与度与力量及定时功能测试之间的关联。
这项横断面研究纳入了60名DMD男孩（平均年龄9.3岁±0.3）。男孩们完成了力量测试、定时功能测试、儿童参与和享受评估以及ACTIVLIM。采用独立样本t检验来测试我们年龄较小和较大队列在所有测量指标上的差异；使用Spearman（秩）相关性来评估参与度与力量及时间功能测试之间的关系。
在娱乐（p<0.01）、社交（p<0.001）和基于技能的活动（p<0.05）领域，以及活动的参与者和地点方面（分别为p<0.05和0.001），年龄较小和较大的DMD男孩之间存在显著差异。年龄较大的DMD男孩在这些领域的参与度较低，与家庭成员以外的人参与活动较少，在家外的参与也较少。力量水平较低和功能表现速度较慢与年龄较小队列中较少参与体育活动以及年龄较大队列中较少参与体育和社交活动相关。
力量和功能与DMD男孩参与的活动的可变性和类型有关。一个关键发现是，随着DMD男孩年龄增长，社交活动和基于社区的参与度显著下降。干预的最终目标是让我们的孩子能够按照自己的意愿积极参与生活。这需要在测量结果时关注参与度，以便更全面地了解限制因素，并为男孩及其家庭提供持续参与的适当策略。
杜氏肌营养不良症是一种毁灭性的进行性神经肌肉疾病，会导致严重的力量和功能限制，影响这些男孩的身体和社会参与。超越基于临床的结果，通过参与度测量来评估和监测儿童的日常活动的能力可能为治疗干预提供信息。康复专家在倡导身体有局限的儿童参与社会和社区方面发挥着作用。向家庭提供有关基于社区的机会以及可用的策略和环境改造的信息，可能会增加患有神经肌肉疾病的青少年的社会参与度。
