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

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.

Burden of caregivers of patients with duchenne muscular dystrophy: relationship to functional capacity / Sobrecarga de cuidadores de pacientes com Distrofia Muscular de Duchenne: relação com a capacidade funcional

“…16 The support of social networks and support professionals are essential, as they help to reduce stress factors, through coping strategies. 19,20 In the current study, only 20% of caregivers performed psychological counseling, and 42.7% did not have leisure activities. Caregivers of individuals with chronic illnesses need emotional support, as they need someone to listen to them, be empathetic, provide comfort, security, confidence and dedication.…”

Needs management in families affected by childhood-onset dystrophinopathies

“…More advanced disease is often associated with increased caregiver stress. 26,27,43,44 Consistent with this observation, counseling for caregivers was identified as a need for a significantly higher proportion of caregivers when the diagnosed individual was non-ambulatory. Social support, resiliency, increased perceived control, and healthy family identity are factors that have been associated with less caregiver stress.…”

“…Social support, resiliency, increased perceived control, and healthy family identity are factors that have been associated with less caregiver stress. 23,27,45 Our study, along with others, suggests that recommendations for the management of psychosocial issues have not been effectively translated at point of care, 46,47 despite evidence that adequate resources have been shown to protect against related stresses. 27,45 Our study is not without limitations.…”

“…20 Studies on the quality of life of individuals diagnosed with a dystrophinopathy and on the burden to their families have reported lower adaptability among those individuals with increasing health demands. [22][23][24][25][26][27][28][29][30][31] Although the measurement of burden and adaptation have varied across studies, adequate practical and psychological resources have been shown to reduce the effect of these demands on the diagnosed individual and their families. 23,26,27,32,33 To assist healthcare providers, public health practitioners, and policymakers in gaining a better understanding of the needs of caregivers and families of individuals with a dystrophinopathy diagnosis, we administered a comprehensive…”

Perceived mental health in parents of children with rare congenital surgical diseases: a double ABCX model considering gender

“…In the context of rare congenital surgical diseases, a crisis event (x) is the diagnosis of a rare disease and, as given in this study, the circumstances caused by the COVID-19 pandemic. In recent research on families of children with a chronic condition, different indicators have been used to operationalize the cumulative demands forming stressor pile-up (aA), among them scales assessing stressful life events of the family [ 15 ], life stress [ 16 ], disease severity, behavioral problems of the child [ 17 ], or single items such as parental educational attainment, marital status [ 18 ], and income [ 19 ]. Adaptive resources (bB) were assessed as social or family support and family functioning or family environment [ 16 , 18 , 20 ].…”