Abstract
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.
Highlights
Duchenne and Becker muscular dystrophies, collectively referred to as dystrophinopathies, are recessive X-linked disorders characterized by progressive muscle weakness and cardiac and respiratory failure
In 2004, MD Muscular Dystrophy Surveillance (STARnet) retrospectively identified and prospectively followed individuals born since January 1, 1982 who were diagnosed with a dystrophinopathy by age 21 years, and resided following diagnosis in an MD STARnet site (Arizona, Colorado, Iowa, western New York State)
Questionnaires were completed by 211 primary caregivers from August 2011 through February 2012 (Fig. 2)
Summary
Duchenne and Becker muscular dystrophies, collectively referred to as dystrophinopathies, are recessive X-linked disorders characterized by progressive muscle weakness and cardiac and respiratory failure. Duchenne (DMD) and Becker (BMD) muscular dystrophies, collectively termed dystrophinopathies, are X-linked disorders characterized by progressive muscle weakness [1]. Symptom onset for DMD occurs before the 5th birthday and historically, complete loss of ambulation occurs by the 12th birthday [6]. Symptom onset for BMD often occurs at a later age and disease progression is slower. Those affected by a dystrophinopathy experience progressive weakness resulting in loss of ability to walk or perform activities of daily living (ADLs). Compromised pulmonary and cardiac systems are the major contributors to premature mortality
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