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. 2021 Sep 30;16(1):406.
doi: 10.1186/s13023-021-01997-w.

Physiotherapy for epidermolysis bullosa: clinical practice guidelines

Amy Weisman  1 , Jennifer M Chan  2 , Chantal LaPointe  3 , Kaye Sjoholm  4 , Kristy Steinau  5 , Kaycie Artus  6 , Suci Widhiati  7 , Rebecca Bodan  8 , Michelle Wood  9 , Julio C Salas-Alanis  10   11 , Anna Carolina Rocha  12 , Beata Faitli  9 , Phuong Khuu  2
Affiliations

Physiotherapy for epidermolysis bullosa: clinical practice guidelines

Amy Weisman et al. Orphanet J Rare Dis. .

Abstract

Epidermolysis bullosa (EB) is characterized by skin fragility with blister formation occurring spontaneously or following minor trauma such as gentle pressure or friction. Current physiotherapy practice is based on anecdotal care, clinical expertise and creative problem solving with caregivers and individuals with EB. Evidence based intervention is needed to establish a foundation of knowledge and to guide international practitioners to create and improve standards of care to effectively work with individuals living with EB. This clinical practice guideline (CPG) was created for the purpose of providing evidence based interventions and best clinical practices for the physiotherapy management of individuals with EB. A survey was conducted within the EB community and six outcomes were identified as a priority to address in physiotherapy management, including (1) attaining developmental motor milestones, (2) identifying safe and functional mobility in the natural environment, (3) encouraging ambulation endurance, (4) supporting safe ability to bear weight, (5) improving access to physiotherapy services, and (6) optimizing interaction with the community. A systematic literature review was conducted and articles were critically analyzed by an international panel consisting of thirteen members: healthcare professionals (including physiotherapist, doctors, and occupational therapist), caregivers, and individuals with EB. Recommendations were formulated from evidence and panel consensus. An external panel of twelve were invited to improve the quality and gather feedback on draft manuscript and recommendations. This CPG describes the development of recommendations for physiotherapy management including several best practice interventions. This guideline lays the foundational work for physiotherapist throughout the world to provide high quality services while improving and maintaining functional mobility and independence within the EB community. The CPG outlines limitations in the evidence available and possible future research needed to improve physiotherapy practice.

Keywords: Clinical practice guideline; Epidermolysis bullosa; Mobility; Motor development; Physical therapist; Physiotherapy; Weight bearing.

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Conflict of interest statement

The working panel members and external reviewers declared no conflicts of interest with respect to the publication of this guideline. No one involved in the guideline has any connection to manufacturers.

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References

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    1. Chan JM, Weisman A, King A, Maksomski S, Shotwell C, Bailie C, et al. Occupational therapy for epidermolysis bullosa: clinical practice guidelines. Orphanet J Rare Dis. 2019;14:129. doi: 10.1186/s13023-019-1059-8. - DOI - PMC - PubMed
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