Evolution of body image across treatment eras: a systematic review in young people and adults living with cystic fibrosis

Clinical Snapshot

Key Findings

• P Value: Not reported in abstract

• Effect Size: Treatment era effects: pre-modulator 64±27 to 70±27, initial rollout 58±19 to 81, widespread modulator era 63±32 to 67

• Primary Outcome: Body image scores varied significantly across studies: CFQ-R scores 58±19 to 78±23, CFQoL scores 21±26 to 76

• Nnt Or Sensitivity: Ivacaftor showed positive effect on body image; elexacaftor/tezacaftor/ivacaftor showed no effect

• Confidence Interval: Not reported in abstract

Clinical Application

High feasibility as CFQ-R and CFQoL are established assessment tools; body image screening could be integrated into routine CF care Highly relevant as CFTR modulators (ivacaftor, elexacaftor/tezacaftor/ivacaftor) are PBS-subsidised; aligns with RACGP guidelines for comprehensive chronic disease management Australian adults and adolescents ≥16 years with cystic fibrosis receiving care through specialist CF centres

Abstract

OBJECTIVES: Advancements in treatments for cystic fibrosis (CF) have improved prognosis, but variant-specific therapies have also driven weight gain. Traditional challenges to maintain weight are now less prevalent, with new body image concerns potentially emerging. This review aims to synthesise quantitative and qualitative evidence on body image in adults and young people (aged ≥16 years) with CF. METHODS: Systematic search of MEDLINE, Embase, the Cumulative Index to Nursing and Allied Health, the Allied and Complementary Medicine Database and the Cochrane Library (January 2011 to August 2025) using CF-related keywords including body image and physical appearance. RESULTS: Searches retrieved 29 eligible studies (26 quantitative, two qualitative, one mixed methods). The Cystic Fibrosis Questionnaire - Revised (CFQ-R) and Cystic Fibrosis Quality of Life Questionnaire (CFQoL) questionnaires assessed body image quantitatively (0-100). 15 studies reported CFQ-R body image scores (58±19 to 78±23) and five reported CFQoL body image scores (21±26 to 76). Pre-modulator era (≤2012) scores ranged from 64±27 to 70±27; initial rollout phase (2012-2019) scores ranged from 58±19 to 81 (post-ivacaftor); and in the widespread modulator era (2020 to present) were 63±32 to 67 (31). Gender effects were inconsistent. Higher body mass index, weight, and body fat were linked to better body image in some studies; psychological wellbeing and lower anxiety/depression consistently correlated with body image. Ivacaftor and life coaching improved body image, while elexacaftor/tezacaftor/ivacaftor, cognitive behavioural therapy, and self-management programmes showed no effect. Functional health was strongly linked to body image.Qualitative findings highlighted influences of gender, weight change, functional and mental health, social support, early eating experiences, and limited clinician-initiated body image discussions. CONCLUSION: This is the first systematic review of body image in CF since new drug regimens emerged. Emerging body image disturbance patterns highlight the need for clinical strategies to screen for and address body image issues in CF care.

References

1. 1.Greaney, C., Cichy, N., O'Sullivan, G., Sills, D., Cahalan, R., & Tierney, A. (2026). Evolution of body image across treatment eras: a systematic review in young people and adults living with cystic fibrosis. European Respiratory Review, 35(160), Article 0238-2025. https://doi.org/10.1183/16000617.0238-2025