Pain experiences of adults with osteogenesis imperfecta: An integrative review

Background: Pain is a common symptom of osteogenesis imperfecta (OI) among children and adolescents. However, little is currently known of the pain experiences of adults with OI. Aims: The aims of this study were to critically appraise the studies assessing OI pain, to synthesize the pain experiences of adults with OI, and to compare the adult OI pain experiences to childhood. Methods: An integrative review was conducted. Five electronic bibliographic databases were searched. Published quantitative, qualitative, and/or mixed-method studies assessing pain in adults with OI were screened, reviewed, and appraised. Descriptive statistics were used to calculate quality scores, summarize sample characteristics, and synthesize findings. Extracted pain data were analyzed using constant comparison and consolidated into meaningful themes. Results: From the 832 titles identified, 14 studies including seven case reports met the inclusion criteria. Study appraisal scores ranged from low to moderate using the Quality Assessment Tool and the Case Report Checklist. The majority of studies assessed pain as a secondary outcome (71.4%) using well-established tools (64.2%). Adults with OI experience pain of mild to moderate intensity, which may interfere with completion of daily activities. Two themes emerged from analysis of the data: mild chronic pain persists despite surgical, pharmacological, or nonpharmacological interventions and past fractures and structural deformities may trigger onset of chronic pain in adulthood. Conclusion: Limited attention has been given to exploring the pain experience of adults diagnosed with OI. Pain is a long-term symptom of OI requiring further in-depth investigation to better understand and manage pain in adults with OI.