Living with RDEB

  • Research type

    Research Study

  • Full title

    Living with Recessive Dystrophic Epidermolysis Bullosa (RDEB)

  • IRAS ID

    165709

  • Contact name

    Janelle Yorke

  • Contact email

    Janelle.Yorke@manchester.ac.uk

  • Clinicaltrials.gov Identifier

    15261, Sponsor Reference

  • Duration of Study in the UK

    0 years, 4 months, 1 days

  • Research summary

    Epidermolysis Bullosa (EB) is a rare genetic skin condition caused by a mutation of the COL7A1 gene that codes for Collagen VII found in skin and mucosa. Little research has been done on the experiences of EB patients to date.

    There is currently no cure for EB. Treatments are limited to being palliative, aiming to minimize damage and complications. Although clinical trials are ongoing to assess treatment efficacy), the lack of evidence about the needs and experiences of adults living with RDEB has on occasions, posed problems when analysing quantitative trial data. Clinical endpoints can lack sensitivity to measure changes reported by patients qualitatively, or do not measure what trial participants say are important aspects to them. Quality of life questionnaires used are unvalidated or non-disease specific as, until very recently, none existed.

    This qualitative study aims to explore the experiences of living day to day life with RDEB, from the perspective of those living with the condition, rather than as imposed meanings by the researcher. To our knowledge this will be the first attempt to explore the experiences of adults with RDEB.

    The evidence of how adult individuals live with this rare life-threatening condition can inform clinicians and the general public about living with a complex inherited condition such as RDEB. In addition the evidence can be used to advise hospital clinicians, clinical trial clinicians and scientists of what is important to patients about their condition which can, in turn, define patient-centred therapeutic measures and endpoints, clinical trial therapeutic measures and endpoints and inform research priorities.

  • REC name

    West Midlands - Coventry & Warwickshire Research Ethics Committee

  • REC reference

    15/WM/0234

  • Date of REC Opinion

    8 Jul 2015

  • REC opinion

    Further Information Favourable Opinion