Pain experiences of adults with osteogenesis imperfecta: An integrative review

Abstract

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. RÉSUMÉ Contexte: La douleur est un symptôme commun de l'ostéogenèse imparfaite (OI) chez les enfants et les adolescents. Toutefois, on sait actuellement peu de choses au sujet de la douleur ressentie par les adultes atteints d'OI. But: Effectuer une appréciation critique des études évaluant la douleur occasionnée par l'OI, faire la synthèse de l'information sur la douleur ressentie par les adultes atteints d'OI et comparer la douleur ressentie à l'âge adulte à celle ressentie pendant l'enfance. Méthodes: Un examen par intégration a été mené. Des recherches ont été effectuées dans cinq bases de données bibliographiques électroniques. Les études quantitatives, qualitatives ou mixtes publiées qui évaluaient la douleur chez les adultes atteints d'OI ont été sélectionnées, examinées et évaluées. Des statistiques descriptives ont été utilisées pour calculer leur score de qualité, résumer les caractéristiques de leur échantillon et synthétiser leurs conclusions. Les données sur la douleur qui avaient été extraites ont été analysées à l'aide de la méthode de la comparaison constante et regroupées en thèmes significatifs. Résultats: Parmi les 832 titres recensés, 14 études comprenant sept rapports de cas répondaient aux critères d'inclusion. Au moment de les évaluer, les études ont obtenu...

A Baseline Measurement of Quality of Life in 322 Adults With Osteogenesis Imperfecta

“…This is comparable with observations of other studies, ( 15,21 ) and has also been observed in review studies for pain in children with OI. ( 22 )…”

Chronic pain in adults with osteogenesis imperfecta and its relationship to appraisal, coping, and quality of life: A cross-sectional study

“…Nevertheless, a high incidence of chronic pain has been detected in this collective, [11,12] which disrupts everyday activities and is found to be resistant to surgical, pharmacological and nonpharmacological intervention. [13] The International Association for the Study of Pain (IASP) defines pain as "An unpleasant sensory and emotional experience associated with, or resembling that associated with, actual or potential tissue damage," [14] mentioning in its "key notes" the subjective nature of this experience, and how this is influenced by psychological and learned factors. Chronic pain, in more specific terms, is that which persists beyond the healing period (generally more than 3 to 6 months) and, therefore, has lost its adaptive value.…”

Suppressed prefrontal cortex oscillations associate with clinical pain in fibrodysplasia ossificans progressiva

“…Pain is a highly common driver of reduced quality of life across rare, musculoskeletal diseases [4,21,32]. Prominent and more accessible features of musculoskeletal disease such as skeletal deformities and lesions, or muscle atrophy and weakness garner extensive clinical and research attention, whereas the neurobiological cause(s) of pain have been less well-studied in this clinical domain.…”