Perceived quality of life among caregivers of children with a childhood-onset dystrophinopathy: a double ABCX model of caregiver stressors and perceived resources

Health and Quality of Life Outcomes

Research

Jacob Oleson¹  Molly Lamb²  Lowell McKirgan³  Kristin Caspers Conway³  Natalia Frishman⁴  Jennifer Andrews⁵  Joyce Oleszek⁶  Dennis Matthews⁶  Paul Romitti⁷  Emma Ciafaloni⁸  Katherine Mathews⁹  Pangaja Paramsothy^1,10 

[1] Department of Biostatistics, The University of Iowa, Iowa City, USA;Department of Epidemiology, Colorado School of Public Health, Aurora, USA;Department of Epidemiology, The University of Iowa, Iowa City, USA;Department of Epidemiology, The University of Iowa, Iowa City, USA;Present address: General Dynamics Information Technology, Coralville, IA, USA;Department of Pediatrics, The University of Arizona, Tucson, USA;Department of Physical Medicine and Rehabilitation, University of Colorado and Children’s Hospital Colorado, Aurora, USA;Departments of Epidemiology and Biostatistics and Interdisciplinary Program in Toxicology, The University of Iowa, College of Public Health, S416 CPHB, 145 N Riverside Dr, 52242, Iowa City, IA, USA;Departments of Neurology and Pediatrics, University of Rochester Medical Center, Rochester, USA;Departments of Pediatrics and Neurology, The University of Iowa, Iowa City, USA;National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention, Atlanta, USA;

关键词: Becker muscular dystrophy;    Caregivers;    Duchenne muscular dystrophy;    Dystrophinopathy;    Muscular dystrophies;    Quality of life;   

DOI  :  10.1186/s12955-017-0612-1

 received in 2016-08-25, accepted in 2017-02-04,  发布年份 2017

来源: Springer

【 摘 要 】

BackgroundDuchenne and Becker muscular dystrophies, collectively referred to as dystrophinopathies, are recessive X-linked disorders characterized by progressive muscle weakness and ultimately cardiac and respiratory failure. Immediate family members are often primary caregivers of individuals with a dystrophinopathy.MethodsWe explored the impact of this role by inviting primary caregivers (n = 209) of males diagnosed with childhood-onset dystrophinopathy who were identified by the Muscular Dystrophy Surveillance, Tracking, and Research Network (MD STARnet) to complete a mailed questionnaire measuring perceived social support and stress, spirituality, and family quality of life (FQoL). Bivariate and multivariate analyses examined associations between study variables using the Double ABCX model as an analytic framework.ResultsHigher stressor pile-up was associated with lower perceived social support (r = -0.29, p < .001), availability of supportive family (r = -0.30, p < .001) or non-family (r = -0.19, p < .01) relationships, and higher perceived stress (r = 0.33, p < .001); but not with spirituality (r = -0.14, p > 0.05). FQoL was positively associated with all support measures (correlations ranged from: 0.25 to 0.58, p-values 0.01–0.001) and negatively associated with perceived stress and control (r = -0.49, p < .001). The association between stressor pile-up and FQoL was completely mediated through global perceived social support, supportive family relationships, and perceived stress and control; supportive non-family relationships did not remain statistically significant after controlling for other mediators.ConclusionsFindings suggest caregiver adaptation to a dystrophinopathy diagnosis can be optimized by increased perceived control, supporting family resources, and creation of a healthy family identity. Our findings will help identify areas for family intervention and guide clinicians in identifying resources that minimize stress and maximize family adaptation.

【 授权许可】

CC BY   
© The Author(s). 2017