Occupational therapy for epidermolysis bullosa: clinical practice guidelines
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(2019) 14:129
REVIEW
Open Access
Occupational therapy for epidermolysis bullosa: clinical practice guidelines Jennifer M. Chan1*† , Amy Weisman1†, Alex King2, Susan Maksomski3, Carrissa Shotwell4, Claire Bailie5, Helen Weaver6, Rebecca Bodan7, Estrella Guerrero8, Matija Zmazek9 and Phuong Khuu1
Abstract The purpose of this article is to summarize the Dystrophic Epidermolysis Bullosa Research Association (DEBRA) International evidence-based Clinical Practice Guidelines (CPGs) for the provision of occupational therapy (OT) for children and adults living with inherited epidermolysis bullosa (EB). This is a rare genetic disorder characterized by skin fragility leading to blister formation occurring spontaneously or following minor trauma. Current OT practice for persons with EB is based on anecdotal care, clinical expertise and trial and error with collaboration between caregiver and patient. Intervention based on research is needed to establish a foundation of knowledge to guide international practitioners to create and improve standards of care and to be able to work effectively with those living with the rare diagnosis of this condition. This CPG was created by an international panel with expertise working with persons with EB. The panel was made up of 11 members including OT’s, a physiotherapist, a medical doctor, social worker, person with EB and a carer of a person with EB. It describes the development of recommendations for 5 outcomes determined by survey of persons with EB, caregivers, and experienced healthcare professionals. The outcomes include independence in activities of daily living (ADL), independence in instrumental ADL, maximization of hand function (non-surgical), fine motor development and retention, and oral feeding skills. The recommendations are supplemented with additional files that include photos and specific examples to further guide occupational therapists or, in situations where an OT is not available, other members of the healthcare team. As the disorder of EB is rare, evidence-based CPGs are needed to provide a base of knowledge and practice for OTs throughout the world with the goal of providing quality care to patients, while improving their functional independence and quality of life. In addition, this information is valuable as a basis for further research. Keywords: Epidermolysis bullosa, Occupational therapy, Clinical practice guideline, Activities of daily living
Background Epidermolysis bullosa (EB) is a rare genetic disorder characterized by skin fragility with blister formation occurring spontaneously or following minor trauma such as gentle pressure or friction. It can be broadly divided into four major subtypes: EB simplex (EBS), junctional (JEB), dystrophic (DEB), and Kindler syndrome (KS). EB can affect multiple body systems, particularly the skin. Subtypes are determined by several factors including the level of skin cleavage, phenotype, mode of inheritance, and molecular origin. * Correspondence: [email protected] † Jennifer M. Chan and Amy Weisman contribut
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