We are delighted to announce that the "Hand surgery and hand therapy clinical practice guideline for epidermolysis bullosa" has been published! Depending on the type and severity, EB can affect any area of skin. This includes the hands, which can be subjected to repeated applications of pressure or friction throughout the day. Those who usually require hand surgery and/or therapy have recessive dystrophic EB (RDEB).
This guideline has been developed to help to aid hand surgeons, occupational therapists (OTs), physiotherapists, hand therapists, and those living with EB to understand operative, non-operative, and post-operative management strategies.
The guideline is based on the priorities and experiences of children and adults living with EB and their care givers. At the 2017 DEBRA UK AGM, individuals were asked what they wanted from hand surgery and therapy; and to share experiences of what had and had not worked with skin grafting, dressing changes, removal of wires and long-term splinting. They were also asked to identify and add any new or key issues.
The guideline seeks to answer the following questions:
does hand surgery improve hand function?
does patient selection/preparation influence outcomes of hand surgery?
do specific surgical techniques/resources influence safety/outcomes of hand surgery?
does non-operative treatment alter the natural hisotry of deformity?
does postoperative hand therapy help maintain surgical results?
do assistive devices and activity modifications improve hand function/quality of life/independence?
The journey to this guideline started in 2015 with Rachel Box (hand therapist EB adult specialist) from Guy's and St Thomas' NHS Foundation Trust, London, UK when she attended training in the Scottish Intercollegiate Guidelines Network (SIGN) methodology. In 2016, a DEBRA International scoping project identified hand surgery and hand therapy as a priority clinical area to develop a clinical practice guideline. In 2017, Rachel and Roger Cornwall (hand surgical lead) from Cincinnati Children's Hospital Medical Center, Cincinnati, USA were awarded a grant to develop this guideline from DEBRA UK.
First panel meeting of members of the Hand surgery and hand therapy CPG for EB
They lead an international panel of 18 members from seven countries. Members included eight hand surgeons and six OTs, hand therapy EB experts, and four individuals living with EB and their caregiver representatives. They conducted their first panel meeting in Salzburg, Austria in 2017 at the EB Research and EB Clinet conference, and reconvened at a recommendation meeting in Zermatt in 2018 at the DEBRA International Congress. The final manuscript was reviewed by nine international reviewers.
On 7th November 2022, the manuscript was accepted by Orphanet Journal of Rare Diseases to be published online open access. We would like to congratulate and thank Rachel, Catina, and the panel on completing this excellent CPG. We would also like to thank everyone who contributed to this work. The COVID-19 lockdown added pressure in the final stages of the panel's work; it was a mammoth task but they achieved their final goal. The guideline will be a great resource that will help many people living with EB around the world.
In memoriam
The panel extends its gratitude to Matija Zmazek, born with recessive dystrophic EB (1983 – 2018), Croatia, for his devotion and participation in the development of this guideline. He passed away during the guideline development process as a result of EB-related complications. He was involved in five panels and he has left an amazing legacy to the international EB community.
The guideline is available to download for FREE here.