Consensus statements for the transdisciplinary care of patients with epidermolysis bullosa in South Africa : Part 1

Abstract

BACKGROUND : Epidermolysis bullosa (EB) is a rare, painful and blistering genodermatosis with no cure. Treatment aims to prevent new lesions and manage complications. Previously, there were no management guidelines or consensus recommendations for patients with EB in Africa. AIM : The aim of this study is to produce a comprehensive, transdisciplinary and practical care guide that is contextually appropriate to the cultural setting and resource limitations in South Africa. SETTING : Multicentre, multiprovincial study involving healthcare practitioners from five South African provinces – KwaZulu-Natal, Gauteng (Johannesburg, Pretoria), Western Cape (Cape Town, Stellenbosch), Free State (Bloemfontein) and the Eastern Cape (Umtata). METHODS : Consensus recommendations for the care of patients with EB were developed by a transdisciplinary team of specialists in consultation with EB patients. The modified Delphi technique was used to reach a robust consensus with a threshold of 80% for each action point to ensure the validity and reliability of the recommendations. RESULTS : In all, 16 consensus statements were developed, and the main themes included the clinical clues to the diagnosis, complications as per the subtype of EB, diagnostics in a resource-limited environment, management of EB, pruritus and pain, palliative care and genetic counselling. CONCLUSION : A transdisciplinary approach is essential for the holistic care of patients and their families with EB in the context of their resource limitations and cultural diversity providing much-needed guidance for clinicians in South Africa and similar settings. CONTRIBUTION : This is the first consensus recommendation of care for patients with EB in Africa.