Cystic fibrosis (CF) is a common life-shortening genetic disease and is associated with poor psychosocial and quality of life outcomes. The objective of this study was to describe the experiences and perspectives of children and adolescents with CF to direct care toward areas that patients regard as important.
MEDLINE, Embase, PsycINFO, and Cumulative Index to Nursing and Allied Health Literature were searched from inception to April 2013. We used thematic synthesis to analyze the findings.
Forty-three articles involving 729 participants aged from 4 to 21 years across 10 countries were included. We identified 6 themes: gaining resilience (accelerated maturity and taking responsibility, acceptance of prognosis, regaining control, redefining normality, social support), lifestyle restriction (limited independence, social isolation, falling behind, physical incapacity), resentment of chronic treatment (disempowerment in health management, unrelenting and exhausting therapy, inescapable illness), temporal limitations (taking risks, setting achievable goals, valuing time), emotional vulnerability (being a burden, heightened self-consciousness, financial strain, losing ground, overwhelmed by transition), and transplant expectations and uncertainty (confirmation of disease severity, consequential timeliness, hope and optimism).
Adolescents and children with CF report a sense of vulnerability, loss of independence and opportunities, isolation, and disempowerment. This reinforces the importance of the current model of multidisciplinary patient-centered care that promotes shared decision-making, control and self-efficacy in treatment management, educational and vocational opportunities, and physical and social functioning, which can lead to optimal treatment, health, and quality of life outcomes.