Fibrous dysplasia patients with and without craniofacial involvement report reduced quality of life inclusive of stigma, depression, and anxiety.

OBJECTIVES: Fibrous dysplasia is a rare bone disorder that causes deformity, fractures, and pain that typically manifests in childhood and persists as a chronic illness. This study evaluates adult patients with fibrous dysplasia and McCune Albright syndrome to determine whether their quality of life differs from the general population and varies in relation to disease severity and lesion location. METHODS: This study uses data from the online self-report Fibrous Dysplasia Foundation Patient Registry and operationalizes quality of life using PRO measures: SF-36, Hospital Anxiety and Depression scale, Neuro-Quality of Life Stigma scale, and the Brief Pain Inventory. RESULTS: One hundred and ninety seven adults, 90% white, 84% women, constitute the sample. Mean scores for all SF-36 domains and the Neuro Q stigma scale were significantly below population benchmarks. A large minority registered moderate to severe levels of anxiety and depression. Group differences were not significant across most of the SF-36 domains but were associated with experienced stigma. DISCUSSION: This study demonstrates a social psychological impact of fibrous dysplasia on adults, in those with and without craniofacial involvement and with mild and severe forms of the disease. Clinical treatment should encompass assessment of quality of life issues and ensure access to psychosocial treatment resources for all fibrous dysplasia/McCune-Albright syndrome patients.

Stigma and psychological distress among pediatric participants in the FD/MAS Alliance Patient Registry.

BACKGROUND: Stigma, both enacted and internalized, is part of the illness experience of many chronic conditions / diseases and has been found to increase psychological distress, lower self-esteem, and impact social engagement lowering quality of life (QOL). Stigma among pediatric patients is of particular concern due to its potential impact on identity formation. Using patient data from the online FD/MAS Alliance Patient Registry (FDMASAPR), this study seeks to 1) determine levels of enacted and self-stigma in a pediatric population of fibrous dysplasia (FD) / McCune Albright syndrome (MAS) patients and 2) to explore the relationship between stigma and anxiety and depression. METHODS: This is a cross sectional analysis of deidentified self-report data from 18 pediatric patients. Key analytic variables include the Neuro-QOL stigma short form, the Hospital Anxiety and Depression Scale (HADS), diagnostic category and craniofacial involvement, and select demographics. Sample means and score distributions are examined. Bivariate relationships between stigma, anxiety and depression and patient's personal and medical characteristics are established through analysis of variance and correlation. RESULTS: Composite stigma levels for FD/MAS pediatric patients were comparable to those of children with multiple sclerosis, epilepsy, and muscular dystrophy. Self-stigma was more frequently reported than enacted/felt stigma, but few patients indicated complete freedom from either type of stigma. Diagnosis was significantly related to self-stigma. Significant bivariate relationships were found between depression and enacted/felt and self-stigma and between anxiety and self-stigma. CONCLUSIONS: This study establishes the illness experience of pediatric patients with FD / MAS is impacted by stigma and suggests they should be regularly screened for stigma and psychological distress. It supports the integration of clinical psychologists/ therapists in regular patient care, referral of families to advocacy organizations, and indicates that rare disease patient registries can be a useful tool in efforts to improve the QOL of patients.

Assessing quality of life in pediatric fibrous dysplasia and McCune Albright syndrome: PEDS-QL and HADS data from the Fibrous Dysplasia Foundation Patient Registry.

PURPOSE: The International FD/MAS Consortium recently encouraged using the Pediatric Quality of Life Inventory (PEDS-QL) and the Hospital Anxiety and Depression scales (HADS) in clinical care. This study examines scores on these measures among pediatric fibrous dysplasia and McCune Albright (FD/MAS) patients to initiate consideration of their use in clinical treatment. METHODS: This is a retrospective analysis of pediatric data from 39 minors, ages 2-17, entered in the Fibrous Dysplasia Foundation Patient Registry from July 2016 to December 2018. Sample means and score distributions are compared to general population and chronic disease benchmarks. Associations with medical and demographic variables are also explored. RESULTS: Mean PEDS-QL scores for children 2-7 were inconclusive in determining at risk status for impaired quality of life (QOL). Individual score distributions suggested up to half experienced extensive physical or social impairment. Means and individual score distributions for the physical and psychosocial components of the PEDS-QL for children 8-17 suggested many were at risk of impaired QOL. Over half of 13-17 year-olds met the clinical benchmark for anxiety. Older males scored better than females on the PEDS-QL and HADS. Pain frequency was associated with physical function for older children. CONCLUSIONS: Older children with FD/MAS may be more compromised in terms of psychosocial QOL than previously reported. Clinicians should be attentive to the influence of gender on QOL in older children. Online patient registries associated with rare diseases have the potential to serve as efficient and cost-effective mechanisms to jumpstart examination of new measures in consideration for clinical use.

Using a social marketing approach to advertise Sexual Assault Nurse Examination (SANE) services to college students.

The authors report on an advertising campaign to communicate the availability and desirability of using Sexual Assault Nurse Examination (SANE) services. They used social marketing precepts to develop posters to educate college students about using SANE as a health service and as an arm of prosecution. After 2 advertising campaigns, they conducted an anonymous survey of 1,051 college students. The findings indicated that posters placed in residence halls and public bathrooms reached students, produced a statistically significant increase in students' understanding of SANE services, and were significantly associated with their hypothetical encouragement of others to use SANE. Gender mediated some results. Posters placed in private viewing spaces were found to be a viable way to communicate information about SANE. Funding to combat violence against women on campus should be aimed at increasing students' access to SANE and should include the costs of advertising the program.