Psychosocial recommendations for the care of children and adults with epidermolysis bullosa and their family: evidence based guidelines.

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.

Different experiences and perspectives between head and neck cancer patients and their care-givers on their daily impact of a gastrostomy tube.

BACKGROUND: Gastrostomy feeding in head and neck cancer patients is recognised standard practice in some cancer centres with beneficial effects on outcomes for appropriately selected patients. However, the impact on patients and care-givers needs consideration. The present study aimed to understand the daily impact of gastrostomy feeding on head and neck cancer patients and their care-givers to identify improvements to services. METHODS: Twenty-one adult patients were randomly selected from the Head and Neck centre at University College London Hospital. Six head and neck cancer patients and three care-givers participated in focus groups. The sessions were recorded, fully transcribed and qualitatively thematically analysed, and the resulting data were tabulated. RESULTS: Patients and care-givers expressed opposite experiences within knowledge and understanding of why the tube was necessary; their personal perceptions and objectives of nutritional support. Themes expressing similar experiences included: developing positive coping strategies; preventing nutritional decline; tube dependency; dentures; finance; active care; and psychological support. Furthermore, both groups expressed the benefits of retaining a support network for rehabilitation with the hospital-based specialist team. Also patients and carers recognised that the gastrostomy tube helped patient survival and, with timely dietetic management, helped them wean off the tube reliance with more confidence. CONCLUSIONS: It is essential that patients and care-givers attend pretreatment clinics to discuss nutritional support via the artificial route; their quality of life can be enhanced if guided through a specialist support pathway based at the clinical site where they initiated their care, with links to key agencies.