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

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.

Iterative codesign and testing of a novel dressing glove for epidermolysis bullosa.

OBJECTIVE: Recessive dystrophic epidermolysis bullosa (RDEB) is a rare genetic skin disorder which requires intensive hand therapy to delay fusion of the digits. Existing dressings do not conform to the complex structure of the hand and are applied in patches held with additional bandages, leading to an occlusive environment. The aim was to co-design with patients a dressing glove based on their user experiences and needs. METHOD: Qualitative interviews and focus groups with children and adults with RDEB, and their carers, were conducted. Iterative feedback of design cues, bench and surrogate testing of materials and prototype refinement were achieved through collaborative codesign with patients, carers, clinicians and manufacturers. RESULTS: Thematic analysis generated eight user needs and corresponding design cues, addressing issues of absorbency, adherence, comfort, adaptability, ease of application and removal, breathability, protection, and hand hygiene. A prototype was selected for proof of concept testing. CONCLUSION: This novel dressing glove design met the patient's requirements for a dressing, which conformed to the hand structure and sat in the web spaces to keep fingers separated. Proof of concept testing has since been undertaken with patients to determine performance, value for money and whether further developments are required.

Hand surgery and hand therapy clinical practice guideline for epidermolysis bullosa.

WHAT IS ALREADY KNOWN ABOUT THIS TOPIC?: Epidermolysis bullosa (EB) causes blistering and scarring of the hands resulting in contractures fused web spaces and altered function. Surgery is needed to release contractures and web spaces and hand therapy is essential to maintain results, approaches for both differ. WHAT DOES THIS STUDY ADD?: These guidelines aim to provide information on the surgical and conservative therapeutic hand management of children and adults diagnosed with EB. They are based on available evidence and expert consensus to assist hand surgeons and therapists in decision making, planning and treatment. They highlight the importance of a holistic multidisciplinary team (MDT) approach, where patient priorities are paramount.

Accessibility, accountability, affordability: healthcare policy in orthopedic trauma.

PURPOSE OF REVIEW: This review provides historical background on trauma care in the USA and summarizes contemporary trauma-related health policy issues. It is a primer for orthopedic surgeons who want to promote improvements in research, delivery, and cost reduction in trauma care. RECENT FINDINGS: As of 2010, funding for trauma research accounted for only 0.02% of all National Institutes of Health research funding. This is disproportionate to the societal burden of traumatic injury, which is the leading cause of death and disability among people aged 1 to 46 years in the USA. The diagnosis-related group model of hospital reimbursement penalizes level-I trauma centers, which typically treat the most severely injured patients. Treatment of traumatic injury at level-I and level-II trauma centers is associated with lower rates of major complications and death compared with treatment at non-trauma centers. Patient proximity to trauma centers has been positively correlated with survival after traumatic injury. Inadequate funding has been cited as a reason for recent closures of trauma centers. Orthopedic surgeons have a responsibility to engage in efforts to improve the quality, accessibility, and affordability of trauma care. This can be done by advocating for greater funding for trauma research; choosing the most cost-effective, patient-appropriate orthopedic implants; supporting the implementation of a national trauma system; leading high-quality research of trauma patient outcomes; and advocating for greater accessibility to level-I trauma centers for underserved populations.

Surgery of the hand in recessive dystrophic epidermolysis bullosa.

The underlying genetic abnormalities of epidermolysis bullosa (EB) cause destabilization at the dermo-epidermal junction. Patients with EB characteristically are subject to blistering following relatively minor trauma (the Nikolsky sign), and suffer from ulcers and erosions in all areas subject to persistent or repeated friction, such as the hand. Hand deformities occur in most patients with dystrophic EB (DEB), and include adduction contractures of the first web space, pseudosyndactyly, and flexion contractures of the interphalangeal, metacarpophalangeal, and wrist joints. All structures in the hand may be involved. The severity of the deformity worsens with age, and surgical correction becomes increasingly difficult. Recurrent deformity occurs within 2 to 5 years. Meticulous skin care and the use of well-fitted splints supervised within a multidisciplinary team setting are essential. To date there is no strong evidence base on which to plan surgical treatment of the hand in DEB.