Abstract
BackgroundFibrous dysplasia is a rare bone disorder, commonly associated with pain, deformity and fractures, which may significantly impact on quality of life. In this study we evaluate quality of life in patients with fibrous dysplasia using the Short Form-36 and the Brief Pain Inventory questionnaires. Data were compared with those of the general Dutch population.ResultsOut of 138 patients from a cohort of 255 patients with fibrous dysplasia that were sent questionnaires assessing quality of life and pain, the response rate was 70.3%, with 97 patients, predominantly female (65%), completing the questionnaires. Monostotic fibrous dysplasia was predominant (n = 62, 64%). Fibrous dysplasia patients had significantly lower quality of life outcome scores than the general Dutch population for all tested domains of the Short Form-36 except for the “Mental health” and the “Role emotional” domains. More severe forms of fibrous dysplasia, had the more severe Short-Form-36 quality of life outcomes, but there was no significant difference in Brief Pain Inventory domains between different subtypes of fibrous dysplasia. Quality of life was lower in patients with higher disease burden, as reflected by high skeletal burden scores (p = 0.003) and high levels of P1NP (p = 0.002).ConclusionWe demonstrate impairments in all domains of quality of life, except for ‘Mental health’ and ‘Role emotional’ domains, across the wide spectrum of fibrous dysplasia including its milder forms. We identified high skeletal burden scores, reflecting disease severity, as the most consistent predictor of impaired quality of life. Our findings hold significant clinical implications as they draw attention to the clinically unmet need to address quality of life issues in the management of patients with all subtypes of fibrous dysplasia, including its milder forms.
Highlights
Fibrous dysplasia is a rare bone disorder, commonly associated with pain, deformity and fractures, which may significantly impact on quality of life
Quality of life (QoL) can be assessed by the use of generic questionnaires, such as the Short-Form 36 (SF-36) questionnaire, or by disease-specific or domain-specific questionnaires, including questionnaires on symptoms associated with the condition under study such as the Brief Pain Inventory (BPI) to assess pain [4, 5]
In this study we demonstrate that patients with Fibrous dysplasia (FD) report significantly impaired quality of life in all tested domains except for Mental Health and Role Emotional
Summary
Fibrous dysplasia is a rare bone disorder, commonly associated with pain, deformity and fractures, which may significantly impact on quality of life. In this study we evaluate quality of life in patients with fibrous dysplasia using the Short Form-36 and the Brief Pain Inventory questionnaires. The disorder often manifests itself in childhood, presenting with bone pain, deformities or a pathological fracture, the disease may be asymptomatic, Quality of life (QoL) is defined as “the functional effect of an illness and its consequent therapy upon a patient, as perceived by the individual patient” [3] and may significantly vary between individual patients with the same disease. QoL can be assessed by the use of generic questionnaires, such as the Short-Form 36 (SF-36) questionnaire, or by disease-specific or domain-specific questionnaires, including questionnaires on symptoms associated with the condition under study such as the Brief Pain Inventory (BPI) to assess pain [4, 5]. Social and Emotional domains were not affected by disease severity, illustrating the relative independence of ‘objective’ severity of a medical condition and its effects on the quality of life of patients [6]
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