title: Perceived quality of life among caregivers of children with a childhood-onset dystrophinopathy: a double ABCX model of caregiver stressors and perceived resources.
abstract: BACKGROUND
  Duchenne 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.
  
  
  METHODS
  We 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.
  
  
  RESULTS
  Higher 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.
  
  
  CONCLUSIONS
  Findings 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.
authors.affiliation: Department of Epidemiology, The University of Iowa, Iowa City, USA.|Present address: General Dynamics Information Technology, Coralville, IA, USA.
authors.forename: Natalia
authors.initials: N
authors.lastname: Frishman
authors.affiliation: Department of Epidemiology, The University of Iowa, Iowa City, USA.
authors.forename: Kristin Caspers
authors.initials: KC
authors.lastname: Conway
authors.affiliation: Department of Pediatrics, The University of Arizona, Tucson, USA.
authors.forename: Jennifer
authors.initials: J
authors.lastname: Andrews
authors.affiliation: Department of Biostatistics, The University of Iowa, Iowa City, USA.
authors.forename: Jacob
authors.initials: J
authors.lastname: Oleson
authors.affiliation: Departments of Pediatrics and Neurology, The University of Iowa, Iowa City, USA.
authors.forename: Katherine
authors.initials: K
authors.lastname: Mathews
authors.affiliation: Departments of Neurology and Pediatrics, University of Rochester Medical Center, Rochester, USA.
authors.forename: Emma
authors.initials: E
authors.lastname: Ciafaloni
authors.affiliation: Department of Physical Medicine and Rehabilitation, University of Colorado and Children's Hospital Colorado, Aurora, USA.
authors.forename: Joyce
authors.initials: J
authors.lastname: Oleszek
authors.affiliation: Department of Epidemiology, Colorado School of Public Health, Aurora, USA.
authors.forename: Molly
authors.initials: M
authors.lastname: Lamb
authors.affiliation: Department of Physical Medicine and Rehabilitation, University of Colorado and Children's Hospital Colorado, Aurora, USA.
authors.forename: Dennis
authors.initials: D
authors.lastname: Matthews
authors.affiliation: National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention, Atlanta, USA.
authors.forename: Pangaja
authors.initials: P
authors.lastname: Paramsothy
authors.affiliation: Department of Epidemiology, The University of Iowa, Iowa City, USA.
authors.forename: Lowell
authors.initials: L
authors.lastname: McKirgan
authors.affiliation: Departments of Epidemiology and Biostatistics and Interdisciplinary Program in Toxicology, The University of Iowa, College of Public Health, S416 CPHB, 145 N Riverside Dr, Iowa City, IA, 52242, USA. paul-romitti@uiowa.edu.
authors.forename: Paul
authors.initials: P
authors.lastname: Romitti
mesh_terms: D000223:Adaptation, Psychological; D000293:Adolescent; D000328:Adult; D017028:Caregivers / Q000523:psychology*; D002648:Child; D002675:Child, Preschool; D015897:Comorbidity; D005260:Female; D006295:Health Resources; D006801:Humans; D007223:Infant; D007389:Internal-External Control; D008016:Life Change Events; D008297:Male; D008875:Middle Aged; D008960:Models, Psychological; D020388:Muscular Dystrophy, Duchenne / Q000523:psychology* / Q000628:therapy*; D011788:Quality of Life / Q000523:psychology*; D012189:Retrospective Studies; D012944:Social Support; D013315:Stress, Psychological / Q000150:complications*; D011795:Surveys and Questionnaires; D055815:Young Adult
keywords: Becker muscular dystrophy; Caregivers; Duchenne muscular dystrophy; Dystrophinopathy; Muscular dystrophies; Quality of life