Caregiver burden in cystic fibrosis: a systematic literature review

Abstract

Background: Cystic fibrosis (CF) affects more than 80,000 people worldwide, having a considerable impact on the quality of life of patients and their caregivers, who assist patients with time-consuming treatment regimens. Despite this, a review of the available evidence has not been previously undertaken. This systematic literature review aimed to identify the humanistic and economic burdens of CF on caregivers. Methods: A systematic literature review was conducted, in accordance with Preferred Reporting Items for Systematic reviews and Meta-Analyses guidelines. Publications reporting outcomes for the caregivers of people with CF, including utility data, health status, and occupational impact, were reviewed. Sources searched were Embase (OvidSP), Medline (PubMed), the Cochrane Database of Systematic Reviews, and the Epistemonikos database, from 2010 to March 2020. A subsequent search with updated terms identified articles up to April 2020. Electronic searches were supplemented by hand searches to capture all relevant literature. Results: A total of 889 articles reporting humanistic burden and 310 reporting economic burden were identified. Following full-text screening by two independent reviewers, 72 articles were included in the review, of which 65 and 17 reported data on humanistic and economic burdens, respectively, with 10 reporting on both. The reviewed literature covered several outcomes and identified multiple key findings: greater disease severity is associated with the reporting of greater caregiver burden and lower utility scores of quality of life; reduced patient lung function is associated with increased caregiver depression and anxiety; and caregiving causes significant occupational impact, with pulmonary exacerbations decreasing caregiver productivity by up to a third compared with the patient being in a ‘well’ state. Conclusion: Findings from this systematic literature review highlight the substantial humanistic and economic burdens borne by the caregivers of people with CF. Future research would help to further inform on the link between disease severity and caregiver burden.

Parents as informal caregivers of children and adolescents with spinal muscular atrophy: a systematic review of quantitative and qualitative data on the psychosocial situation, caregiver burden, and family needs

“…Even though not all parents might be affected by SMA in a way that calls for additional support, the findings of the included studies indicate that receiving the SMA diagnosis can cause post-traumatic symptoms and that a substantial proportion of parents experience physical and mental health problems in the course of SMA due to the caregiving situation. These findings are in line with reviews focusing on the psychosocial situation of parents with chronically ill children and adolescents with other rare diseases (e.g., cystic fibrosis, paediatric cancer) and indicate a strong recommendation for parents to receive early and ongoing assessment of their mental health needs with access to appropriate interventions to optimise parent, child, and family well-being [ 71 – 73 ].…”

Health-Related Quality of Life in Adults with Cystic Fibrosis: Familial, Occupational, Social, and Mental Health Predictors

“…Very few studies investigated the impact of family status on CF HRQoL. Previous reports broadly focused on caregiver burden and quality of life of parents with CF children [ 42 ]. We highlighted that living with parents was negatively associated with the “weight” domain, and partner presence with the “digestive symptoms” domain.…”