Assessment of hand contractures in epidermolysis bullosa (ACE-EB): Describing the development of a novel hand assessment for children

Introduction: Patients with Severe Recessive Dystrophic Epidermolysis Bullosa (RDEB-S) develop hand contractures that progress over time and impact function and quality of life. The role of the Occupational Therapist includes hand assessment and interventions to help maintain function. We developed the Assessment of Hand Contractures in Epidermolysis Bullosa (ACE-EB) to facilitate sensitive and detailed assessment. The purpose of this article is to describe its development and use. Methods: We undertook a literature search of existing hand assessments and evaluated their use for children with RDEB-S. Most measure contracture recurrence post-operatively and none was sensitive enough to capture emerging contractures seen in children. We developed the ACE-EB by adapting the most relevant existing assessments, to include specific component contracture measurements, a Hand Deformity Grade and a hand surgery Patient Reported Outcome Measure. We explored the validity by surveying clinicians in the field of Epidermolysis Bullosa and we tested the inter-rater reliability. Results: We describe the development of the ACE-EB. The results of the validity survey showed most respondents agreed that the ACE-EB had sufficiently sensitive grading scales, included relevant aspects of hand assessment and was a useful clinical or research tool. Inter-rater reliability tests showed good strength of agreement. Discussion: The ACE-EB is a novel clinical tool designed for use with children with RDEB-S. It can be used to monitor changes, track natural history of hand contractures, guide practice and serve as a post-operative outcome measure in children and adults. Further testing of its validity and reliability is required.