Participatory co-design of patient-reported outcome indicators and N-of-1 evaluation of a dressing glove for Epidermolysis bullosa

Abstract

Objective: In autosomal recessive dystrophic Epidermolysis bullosa, repeat blistering results in finger webbing and severe contractures of the hands. The aim of this study was to codesign patient-reported outcome indicators for hand therapy with patients, carers and clinicians, and use these to proof-of-concept test a novel dressing glove for recessive dystrophic Epidermolysis bullosa, with cost analysis. Method: Qualitative interviews and focus groups with patients and carers generated content for the indicators. Validity and reliability were established through expert review, piloting and consensus between patients, carers and clinicians. The indicators were self-reported by patients before and while wearing the dressing glove in an N-of-1 study. Time for dressing changes and use of conventional products were also self-reported. Results: A total of 11 indicators were initially generated from the thematic analysis. Expert review, piloting and consensus involved six patients, five carers and eight clinicians (total n=19). Participants agreed 14 indicators, covering hand skin condition (n=4), webbing between the digits (n=4), experiences of wearing and changing dressings (n=2), hand function (n=2), wrist function (n=1) and hand pain (n=1). In Phase 3, 12 patients scored indicators before wearing the gloves and four patients completed scoring while wearing the gloves. Statistically significant improvements between pre-glove and with-glove periods were found for most participants' experience scores. Skin appearance also improved for most participants. Conclusions: The indicators generated useful data, differentiation between scores and participants demonstrating proof-of-concept for patients with recessive dystrophic Epidermolysis bullosa who could wear the dressing gloves. The indicators are being used in routine practice, supporting clinical follow up, commercialisation and regulatory governance of the dressing glove.

Hand surgery and hand therapy clinical practice guideline for epidermolysis bullosa

“…They are designed to wear for function as they do not restrict ROM. They must be changed every other day to avoid skin maceration and increased blistering [ 59 , 71 ].

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“…Skinnies ™ reinforced web spacer gloves are bespoke, specifically designed for EB and co-designed with participants and carers in a research project [ 59 , 71 ]. They are soft, stretchy, have an anti-microbial finish and provide gentle pressure on web spaces and help protect the hand skin.…”

“…The Skinnies™ disposable dressing glove can be worn under the Skinnies™ Reinforced web-spacer glove to provide a double layer. The gloves can be full fingered or half-fingered [ 59 , 71 ] (Fig. 5 ).…”

“…They are designed to wear for function as they do not restrict ROM. They must be changed every other day to avoid skin maceration and increased blistering [59,71]. Web space contracture (pseudosyndactyly) is gradual and insidious and usually the first type of contracture type to develop [31].…”

The development of a set of key points to aid clinicians and researchers in designing and conducting n-of-1 trials

“…This is to ensure that any effects observed in this assessment can be attributed to the treatment condition of that period. Washout techniques (see key point 19) can be employed to ensure that su cient time has elapsed for carryover effects to expire.…”