ABSTRACT
BACKGROUND: Many studies have recorded significant impairment of health-related quality of life in systemic sclerosis patients using validated scales. However, these instruments are not specifically designed for facial signs. OBJECTIVES: To develop and validate a specific questionnaire to assess the burden on patients with facial signs of systemic sclerosis and which we have named "Burden of Face Affected" (BoFA). METHODS: BoFA was developed using standard methodology in 3 phases: exploration, development and validation. In all, 197 patients completed questionnaires. We analysed the degree of internal consistency (Cronbach's α) and external validity between BoFA and the 12-Item Short Form Healthy Survey (SF-12), the Mouth Handicap In Systemic Sclerosis Scale (MHISS), Rosenberg's self-esteem scale, and the Perceived Stress Scale (PSS). To assess reproducibility, a test-retest analysis was conducted. The original French version was translated into English and underwent cultural validation. RESULTS: The questionnaire comprises 20 items grouped into 4 dimensions. BoFA showed good internal consistency (Cronbach's α: 0.93). External validity was demonstrated in terms of good correlation between BoFA and other questionnaires, in particular MHISS (r=0.54). The test-retest analysis demonstrated good reproducibility (0.92). The BoFA score varied significantly according to the severity of facial scleroderma as assessed by the patients themselves. DISCUSSION: Facial involvement in systemic sclerosis may be considered by physicians to be a minor consequence of the disease and is often overlooked. Nevertheless, it is crucial for patients' quality of life. A number of studies have assessed the impact of facial signs on health-related quality of life using instruments such as DLQI (Dermatology Life Quality Index), SWAP (Satisfaction With Appearance Scale), Brief SWAP and SSPRO (Scleroderma Skin Patient-Reported Outcome). However, these are not specific for facial signs and focus on other sites. BoFA has good reliability and construct validity, and it assesses disability specifically involving the face in patients with systemic sclerosis. CONCLUSION: To our knowledge, BoFA is the first specific tool for assessing burden in patients with facial scleroderma. It is an easy-to-use tool for evaluating the burden of facial signs and may also be used to assess the degree of burden before and after treatment.
Subject(s)
Quality of Life , Scleroderma, Systemic , Humans , Mouth , Reproducibility of Results , Scleroderma, Systemic/diagnosis , Surveys and QuestionnairesABSTRACT
Between 9000 and 14000 people have systemic sclerosis (ScS) in France. The work is often affected. Our study aims to assess the frequency of professional difficulties (DP) of scleroderma patients, identify these DP, the symptoms involved and the solutions used. A heteroquestionnaire was offered to scleroderma patients the Lille University Hospital. Data were cross-checked with the clinical database of the National Observatory of scleroderma patients. We used 104 questionnaires of which 84% are women. A total of 62.5%, (95% CI [52.5 to 71.8]) patients had experienced DP related to SSc, 55% reported symptoms worsened at work, 41% lower efficiency and a 19% job loss. Symptoms responsible for their DP were mainly asthenia, Raynaud syndrome, arthralgias and finger ulcerations. Only 40% of patients in difficulty were initiating steps with the county home for disabled people and 45% in informing their doctor at work (MT). The MT information absence was related to a misunderstanding of possible aid in 45% of cases, 57% of patients had not found a solution, 41% of them have lost their jobs and 31% of patients in the cohort experienced a drop in income. The impact of SSc the work is important. The aid job retention devices are under-used due to lack of knowledge of steps to take and the role of MT. Taking into account the symptoms responsible for PD must be improved to participate in this retention.
Subject(s)
Occupational Stress/epidemiology , Occupations/statistics & numerical data , Scleroderma, Systemic/complications , Scleroderma, Systemic/epidemiology , Adaptation, Physiological , Adult , Aged , Asthenia/epidemiology , Cross-Sectional Studies , Disabled Persons/statistics & numerical data , Female , France/epidemiology , Humans , Job Satisfaction , Male , Middle Aged , Occupational Stress/psychology , Occupations/standards , Quality of Life , Surveys and QuestionnairesABSTRACT
According to ethological procedures and concepts, the author tries to describe the shape and the functional value of stereotyped movements in disabled children. These pathological behaviors are not a fixed response, they take place within an intricate behavioral sequence which associates many items; they are one aspect of a pathological answer to social stimulation. They prove the disabled child's intense motivation to communicate, but show the impossibility for him to do so. The inability to generate an adapted behavioral sequence shows that the deficit concerns an essential interaction process.
