TY - JOUR
T1 - Perceived quality of life among caregivers of children with a childhood-onset dystrophinopathy
T2 - A double ABCX model of caregiver stressors and perceived resources
AU - Frishman, Natalia
AU - Conway, Kristin Caspers
AU - Andrews, Jennifer
AU - Oleson, Jacob
AU - Mathews, Katherine
AU - Ciafaloni, Emma
AU - Oleszek, Joyce
AU - Lamb, Molly
AU - Matthews, Dennis
AU - Paramsothy, Pangaja
AU - McKirgan, Lowell
AU - Romitti, Paul
N1 - Funding Information:
Dr. Kathy Mathews receives research funding from the National Institutes of Health and the Friedreich ataxia research alliance (FARA). Dr. Matthews is also a consultant for aTyr pharma and is a site principal investigator for industry sponsored trials for Sarepta Therapeutics, Horizon pharma, Shire ViroPharma, Eli Lilly, Pfizer, and Biomarin. The remaining authors report no competing interests.
Funding Information:
The findings and conclusions in this report are those of the authors and do not necessarily represent the official position of the Centers for Disease Control and Prevention. The writing of this manuscript was funded by CDC cooperative agreement 5U01DD000831. Data collection by the Muscular Dystrophy Surveillance, Tracking, and Research Network (MD STARnet) was funded by CDC cooperative agreements: 5U01DD000187, 5U01DD000189, 5U01DD000191, and 5U01DD000190.
Publisher Copyright:
© 2017 The Author(s).
PY - 2017/2/10
Y1 - 2017/2/10
N2 - 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.
AB - 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.
KW - Becker muscular dystrophy
KW - Caregivers
KW - Duchenne muscular dystrophy
KW - Dystrophinopathy
KW - Muscular dystrophies
KW - Quality of life
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U2 - 10.1186/s12955-017-0612-1
DO - 10.1186/s12955-017-0612-1
M3 - Article
C2 - 28187773
AN - SCOPUS:85012041397
VL - 15
JO - Health and Quality of Life Outcomes
JF - Health and Quality of Life Outcomes
SN - 1477-7525
IS - 1
M1 - 33
ER -