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INTRODUCTION: Xeroderma pigmentosum (XP) is a rare genetic condition caused by defective nucleotide excision repair and characterised by skin cancer, ocular and neurological involvement. Stringent ultraviolet protection is the only way to prevent skin cancer. Despite the risks, some patients' photoprotection is poor, with a potentially devastating impact on their prognosis. The aim of this research is to identify disease-specific and psychosocial predictors of photoprotection behaviour and ultraviolet radiation (UVR) dose to the face. METHODS AND ANALYSIS: Mixed methods research based on 45 UK patients will involve qualitative interviews to identify individuals' experience of XP and the influences on their photoprotection behaviours and a cross-sectional quantitative survey to assess biopsychosocial correlates of these behaviours at baseline. This will be followed by objective measurement of UVR exposure for 21 days by wrist-worn dosimeter and daily recording of photoprotection behaviours and psychological variables for up to 50 days in the summer months. This novel methodology will enable UVR dose reaching the face to be calculated and analysed as a clinically relevant endpoint. A range of qualitative and quantitative analytical approaches will be used, reflecting the mixed methods (eg, cross-sectional qualitative interviews, n-of-1 studies). Framework analysis will be used to analyse the qualitative interviews; mixed-effects longitudinal models will be used to examine the association of clinical and psychosocial factors with the average daily UVR dose; dynamic logistic regression models will be used to investigate participant-specific psychosocial factors associated with photoprotection behaviours. ETHICS AND DISSEMINATION: This research has been approved by Camden and King's Cross Research Ethics Committee 15/LO/1395. The findings will be published in peer-reviewed journals and presented at national and international scientific conferences.

Original publication

DOI

10.1136/bmjopen-2017-018364

Type

Journal article

Journal

BMJ Open

Publication Date

21/08/2017

Volume

7

Keywords

Xeroderma pigmentosum, adherence, dosimeter, mixed methods, photoprotection, rare conditions, Adolescent, Adult, Aged, Aged, 80 and over, Child, Child, Preschool, Cross-Sectional Studies, Face, Female, Health Behavior, Health Knowledge, Attitudes, Practice, Humans, Infant, Male, Middle Aged, Radiation Dosage, Radiation Protection, Research Design, Self Report, Skin Neoplasms, Sunscreening Agents, Ultraviolet Rays, United Kingdom, Xeroderma Pigmentosum, Young Adult