Epidermolysis Bullosa (EB) is a group of rare genetic disorders resulting in skin fragility and other symptoms. Commissioned by DEBRA International and funded by DEBRA Norway, this evidence-bases guideline provides recommendations to optimise psychosocial wellbeing in EB. An international multidisciplinary panel of social and health care professionals (HCP) and people living with EB was formed. A systematic international literature review was conducted by the panel following the Scottish Intercollegiate Guidelines Network (SIGN) methodology. The resulting papers underwent systematic selection and critique processes. Included papers were allocated to 6 different outcome groups to allow data synthesis and exploration: quality of life, coping, family, wellbeing, access to HCP and pain. Based on the evidence in those papers, recommendations were made for individuals living with EB, family and caregivers and HCP working in the field. Few studies have investigated interventions and which factors lead to better outcomes, but general recommendations can be made. EB is a complex disease impacting enormously on every aspect of psychosocial life. People and families living with EB need access to multidisciplinary support, including psychological guidance, in order to improve quality of life and psychosocial wellbeing. Interventions should stimulate social participation to prevent isolation. People with EB and their families should be able to access a supportive network. HCP should be well supported and educated about the complexity of EB. They should work collaboratively with those around the individual with EB (e.g. schools, employers etc.) to provide psychosocial opportunity and care. Attention should be paid to the psychosocial impact of EB as well as physical needs. Directions for research are indicated. Electronic supplementary material The online version of this article (10.1186/s13023-019-1086-5) contains supplementary material, which is available to authorized users.

Objective Our objectives were to describe trajectories of depressive symptoms and pain at hospital discharge and six weeks later and to examine the relationship of persistent depressive symptoms to pain. Methods Before and six weeks after hospital discharge, 251 cardiac surgery patients (aged 67.3 ± 9.5 years; 73% male) completed the Beck Depression Inventory (BDI) and the Brief Pain Inventory (BPI). Patients were categorized into two groups based on the presence or absence of persistent depressive symptoms (BDI>10 at both times). Between-group differences in pain interference (BPI-INT) and pain severity (BPI-SEV) were evaluated using repeated measures analysis of variance. Linear regressions were performed to determine if changes in depressive symptoms were related to BPI-INT and BPI-SEV, controlling for demographic and clinical data. Results Persistent (16.3%) or worsening depressive symptoms (15.3%) from hospital discharge to six weeks were observed; many experienced at least some persistent pain (BPI-INT - 67.8%; BPI-SEV - 47.8%). From discharge to six weeks, patients with persistent depressive symptoms sustained higher levels of BPI-INT (p<.001) and BPI-SEV (P<.003). In multivariate analysis, only changes in depressive symptoms, not clinical and demographic variables, were related to BPI-INT (p<.001) and BPI-SEV (p=.001). Conclusion Persistent depressive symptoms are independently associated with continued pain up to six weeks after hospital discharge. Successful treatment of ongoing pain should include screening for depressive symptoms and initiation of appropriate treatment.

Background Depression after cardiac surgery (CS) is associated with increased pain and decreased sleep quality. While cognitive behavioral therapy (CBT) aimed at depression is effective in relieving depressive symptoms after cardiac surgery, little is known about its ability to ameliorate other common postoperative problems that affect recovery and quality of life. Aims The purpose of this study was to evaluate the effects of CBT for depression on pain severity, pain interference, sleep, and perceived control in patients recovering from CS. Methods Depressed patients recovering from CS were randomized to receive either eight weeks of CBT or usual care. At baseline and post-intervention, patients completed questionnaires for depressive symptoms, pain, sleep, and perceived control. Group comparisons were conducted using t-tests or chi square analysis. Repeated measures analysis was used to assess the effect of the intervention in changes over time. Results The sample (n=53) included 16.9% women and had a mean age of 67.8±9.2 years. CBT for depression increased perceived control (p<0.001) and decreased pain interference (p=0.02) and pain severity (p=0.03). Group effects remained significant (p<0.05) for perceived control and pain interference and a trend was observed for pain severity (p<0.10) after controlling for variables that differed at baseline. There were no group differences in sleep disturbance over time. Conclusions A depression-focused CBT intervention yields benefits in other common postoperative problems, specifically improved perceived control and decreased pain in depressed cardiac surgery patients.