The Psychological Impact of Cystic Fibrosis and Primary Ciliary Dyskinesia in Adolescence: A Systematic Review

Adolescence is marked by significant emotional changes, which may be intensified by the presence of a rare respiratory disease. The purpose of this study was to explore the psychological outcomes and challenges associated with these conditions during adolescence. Following the PRISMA guidelines, a systematic review was conducted in Web of Science, ProQuest and Scopus databases, identifying 677 articles. Following screening, 18 studies met the inclusion criteria and three additional eligible studies were identified through manual snowball searching, resulting in 21 studies included in the final synthesis. Most studies focused on symptoms of anxiety and depression as key psychological variables. Some also assessed health-related quality of life, psychological comorbidities, treatment adherence, disease knowledge and perceived self-efficacy. Most studies focused on adolescents with cystic fibrosis, whereas evidence concerning primary ciliary dyskinesia was considerably more limited (20 studies included participants with cystic fibrosis and 3 with primary ciliary dyskinesia; 1 study included both conditions). Findings regarding anxiety, depressive symptoms, and health-related quality of life were heterogeneous across studies. Despite the adequate methodological quality of these studies in general, the findings highlight inconsistent conclusions in the literature, with no clear consensus on the extent or nature of the psychological impact. There is a clear need for further high-quality research to better understand the psychological difficulties and consequences associated with rare respiratory diseases during adolescence. Regular psychological assessment may be considered part of multidisciplinary care to identify psychological difficulties and support the well-being of adolescents with CF and PCD. The main limitations were the heterogeneity of study populations and methodologies, the predominance of cross-sectional designs, and the restriction of the search to three databases.