Determinants of impaired quality of life in patients with fibrous dysplasia

Majoor, B.C.J.; Andela, Cornelie D.; Bruggemann, Jens; Sande, Michiel A. J. van de; Kaptein, Ad A.; Hamdy, Neveen A. T.; Dijkstra, P. D. S.; Appelman‐Dijkstra, Natasha M.

doi:10.1186/s13023-017-0629-x

Cited by 25 publications

(36 citation statements)

References 17 publications

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“…Consistent with previous studies, (4,28) we observed that pain is more frequent and severe in adults compared with pediatric subjects with FD. A novel finding from this study was that BTM levels did not vary according to the presence or absence of pain.…”

Section: Discussionsupporting

confidence: 93%

Age-Related Changes and Effects of Bisphosphonates on Bone Turnover and Disease Progression in Fibrous Dysplasia of Bone

Florenzano

Pan

Brown

et al. 2019

Journal of Bone and Mineral Research

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Fibrous dysplasia (FD) is a mosaic disease in which bone is replaced with fibro‐osseous tissue. Lesions expand during childhood, reaching final burden by age 15 years. In vitro data suggest that disease activity decreases in adulthood; however, there is no clinical data to support this concept. Bone turnover markers (BTMs) have been used as markers of disease activity in FD; however, the natural history of BTM changes, the effects of antiresorptive treatment, and their association to clinical outcomes have not been described. The goals of this study are to describe 1) the natural history of FD disease activity and its association with pain; 2) the impact of bisphosphonates on the natural history of BTMs; and 3) the effect of bisphosphonates on progression of FD burden during childhood. Disease burden scores and alkaline phosphatase, osteocalcin, NTx, FGF23, and RANKL levels from 178 subjects in an FD/MAS natural history study were reviewed, including 73 subjects treated with bisphosphonates. BTMs, RANKL, and FGF23 demonstrated a sustained reduction with age. Bisphosphonate treatment did not significantly impact this age‐dependent decrease in BTMs. Pain was more prevalent and severe in adults compared with children and was not associated with BTMs. In children, the progression of disease burden was not affected by bisphosphonates. In conclusion, FD is associated with an age‐dependent decline in bone turnover and other markers of disease activity. Pain, in contrast, is more frequent and severe in adults with FD and is not related to bone turnover. Bisphosphonate treatment does not significantly impact the age‐dependent decrease in bone turnover, nor does it prevent the progression of FD disease burden in children. These findings, in association with the established adverse effects of antiresorptives, should be considered when evaluating use and response to bisphosphonates in patients being treated for FD and in any study using BTMs as surrogate endpoints. © 2019 American Society for Bone and Mineral Research.

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Section: Discussionsupporting

confidence: 93%

Age-Related Changes and Effects of Bisphosphonates on Bone Turnover and Disease Progression in Fibrous Dysplasia of Bone

Florenzano

Pan

Brown

et al. 2019

Journal of Bone and Mineral Research

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“…As part of the protocol used in our ongoing study addressing various aspects of QoL in patients with FD [ 1 , 4 ], three different questionnaires were sent to 255 consecutive patients with a confirmed diagnosis of FD, aged ≥ 16 years, who attended the outpatient clinic of the Centre for Bone Quality of the Leiden University Medical Center between 2013 and 2016. Ninety-two of 138 eligible patients completed the questionnaires, representing a response rate of 67% (Fig.…”

Section: Methodsmentioning

confidence: 99%

“…Questionnaires used were the Utrecht Coping List Questionnaire (UCL) to evaluate coping strategies, and the Short Form-36 (SF-36) and Brief Pain Inventory (BPI) questionnaires to evaluate QoL [ 15 – 18 ]. For a detailed description of the SF-36 and BPI please refer [ 1 ]. The UCL is a widely used validated questionnaire to study coping strategies, which is based on the assumption that individuals prefer a certain way of coping, regardless of the situation: the coping strategy.…”

Section: Methodsmentioning

confidence: 99%

“…In a recent study, it was shown that patients with fibrous dysplasia (FD), particularly those patients with bone pain, reported significant impairment in quality of life (QoL), and that disease severity, as reflected by higher skeletal burden scores (SBS) and elevated serum bone turnover markers, was associated with more severe impairment in QoL [ 1 ]. According to the Common-Sense Model of self-regulation (CSM), QoL is influenced by two major determinants: illness perceptions and coping strategies [ 2 , 3 ].…”

Section: Introductionmentioning

confidence: 99%

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Passive Coping Strategies Are Associated With More Impairment In Quality Of Life In Patients With Fibrous Dysplasia

et al. 2018

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Impairments in quality of life (QoL) have been reported in patients with fibrous dysplasia (FD). Here, we examine coping strategies in FD and assess whether these coping strategies are associated with QoL and disease severity. Ninety-two patients (66% females) filled out the Utrecht Coping List (UCL), Short Form-36, and the Brief Pain Inventory (BPI). Coping strategies of patients with FD were compared with reference data from a random sample of Dutch women and patients with chronic pain. Compared to healthy adults, patients expressed more emotions (p < 0.01). Compared to patients with chronic pain, patients with FD used more active coping strategies (p < 0.001), and sought more distraction (p = 0.01) and more social support (p < 0.001). Using more passive coping strategies was associated with more impairment in social function, physical role, mental health, vitality (all p < 0.001), and general health (p < 0.01). Using more avoidant coping strategies was associated with worse mental health and less vitality (both p < 0.01). More expression of emotions was associated with worse mental health (p < 0.01). Type and clinical severity of FD were not associated with coping behavior. Patients with FD have different coping strategies compared to random Dutch reference populations with or without pain. In FD, using more passive coping strategies was associated with more impairment in several aspects of QoL. There was no relationship between coping behavior and clinical characteristics, pointing to biomedical variables not determining the way patients cope with their illness. Recognition of less effective coping strategies can be helpful in the understanding and adaptation of these coping strategies, improving personalized clinical care, with the ultimate goal to improve QoL in patients with FD.

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“…Quality of Life (QoL) has been reported to be more severely affected in patients with the more severe types of FD. Determinants of severe disease including a High Skeletal Burden Score (SBS) and increased bone turnover markers (BTM), have been identified as determinants of impaired QoL [ 2 , 3 ]. Other factors than those directly related to the disease which also influence QoL are ‘illness perceptions’: the thoughts and emotions that a patient has concerning his or her disease and its treatment [ 4 ].…”

Section: Introductionmentioning

confidence: 99%

Illness Perceptions are Associated with Quality of Life in Patients with Fibrous Dysplasia

et al. 2017

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Fibrous dysplasia (FD) is a rare bone disorder in which normal bone is replaced by fibrous tissue resulting in pain, deformities, pathological fractures or asymptomatic disease. Illness perceptions are patients’ cognitions and emotions about their illness and its treatment, which may impact on Quality of Life (QoL). Here, we explore illness perceptions in patients with FD compared to other disorders, identify factors associated with illness perceptions and evaluate their relationship with QoL. Ninety-seven out of 138 eligible patients from the LUMC FD cohort completed the Illness Perception Questionnaire-Revised (IPQ-R) and the Short Form-36 (SF-36). Age, Gender, Skeletal Burden Score (SBS), FGF-23 levels, type of FD and SF-36 scores were analysed for an association with illness perceptions. We observed significant (p < 0.01) differences in patients’ illness perceptions between FD subtypes in the domains: identity, timeline acute/chronic and consequences. Patients with craniofacial FD reported to perceive more consequences (p = 0.022). High SBS was associated with perceiving more negative consequences and attributing the cause of FD to psychological factors (p < 0.01), and high FGF-23 levels with attributing more symptoms to the disease and perceiving more consequences (p < 0.01). The IPQ-R domain identity, timeline acute/chronic, timeline cyclical, consequences, emotional representations and treatment control were significantly associated with impairments in QoL. Illness perceptions in patients with FD relate to QoL, differ from those in patients with other disorders, and are associated with disease severity. Identifying and addressing maladaptive illness perceptions may improve quality of life in patients with FD.Electronic supplementary materialThe online version of this article (doi:10.1007/s00223-017-0329-5) contains supplementary material, which is available to authorized users.

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Determinants of impaired quality of life in patients with fibrous dysplasia

Cited by 25 publications

References 17 publications

Age-Related Changes and Effects of Bisphosphonates on Bone Turnover and Disease Progression in Fibrous Dysplasia of Bone

Age-Related Changes and Effects of Bisphosphonates on Bone Turnover and Disease Progression in Fibrous Dysplasia of Bone

Passive Coping Strategies Are Associated With More Impairment In Quality Of Life In Patients With Fibrous Dysplasia

Illness Perceptions are Associated with Quality of Life in Patients with Fibrous Dysplasia

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