title: Understanding the outcomes of a home nursing programme for patients with epidermolysis bullosa: an Australian perspective.
abstract: Epidermolysis bullosa (EB) consists of a spectrum of genodermatoses characterised by skin fragility and various degrees of skin and mucous membrane blistering. Minimal trauma and friction can cause extensive blistering in patients with EB, resulting in a number of complications. However, wound management is the main challenge for these patients because of a high risk of infection, fluid loss and potential development of aggressive squamous cell carcinoma (SCC). Indeed, patients with EB have an increased risk for developing skin cancers compared to the general population. In 2012, a home nursing programme was established in Australia to provide assistance to families or patients with severe forms of EB. Nursing care was provided to patients with severe EB during dressing changes in their homes over a period of 2 years. Both families of patients and nurses were surveyed periodically using a developed questionnaire to assess the benefits of this home nursing and its impact on the patients, their families and the nurses. Key findings included a perceived improvement in quality of life, a better provision of support and improved family life management. These findings are the first to highlight the benefits of this national home nursing programme for EB patients within Australia and demonstrate the continued need and benefit of home nursing for patients with severe skin blistering disorders.
authors.affiliation: Brightsky Australia, Sydney, NSW, Australia.
authors.forename: Louise J
authors.initials: LJ
authors.lastname: Stevens
authors.affiliation: Dystrophic Epidermolysis Bullosa Research Association - Australia, Pittsworth, QLD, Australia.
authors.forename: Sue
authors.initials: S
authors.lastname: McKenna
authors.affiliation: Dystrophic Epidermolysis Bullosa Research Association - Australia, Pittsworth, QLD, Australia.
authors.forename: Jennifer
authors.initials: J
authors.lastname: Marty
authors.affiliation: Regenerative Medicine, Mawson Institute, University of South Australia, Adelaide, SA, Australia.
authors.forename: Allison J
authors.initials: AJ
authors.lastname: Cowin
authors.affiliation: Dystrophic Epidermolysis Bullosa Research Association - Australia, Pittsworth, QLD, Australia. zlatko.kopecki@unisa.edu.au.|Regenerative Medicine, Mawson Institute, University of South Australia, Adelaide, SA, Australia. zlatko.kopecki@unisa.edu.au.
authors.forename: Zlatko
authors.initials: Z
authors.lastname: Kopecki
mesh_terms: D000293:Adolescent; D000328:Adult; D001315:Australia; D017028:Caregivers; D002648:Child; D002675:Child, Preschool; D004820:Epidermolysis Bullosa / Q000628:therapy*; D005260:Female; D006701:Home Nursing; D006801:Humans; D008297:Male; D017063:Outcome Assessment, Health Care; D015397:Program Evaluation; D011788:Quality of Life; D055815:Young Adult
keywords: Epidermolysis bullosa; Home nursing; Wound management