Title: Developing a psychological intervention to improve ultraviolet protection and clinical outcomes in Xeroderma Pigmentosum.
Funder: Department of Health and Social Care

Abstract: Background: Xeroderma Pigmentosum (XP) is a rare inherited disease characterised by multiple skin cancers from childhood onwards. Average life expectancy is 32 years with premature death generally due to malignant melanoma. The genetic defect impairs repair of DNA damaged by ultraviolet (UV). Absolute and lifelong protection from daylight is the only way to prevent skin cancers and increase lifespan. Poor UV protection is common in XP despite the efforts of clinical staff. Despite the central importance of UV protection and psychosocial issues in the clinical management of XP, no research has ever been done in this area. Rationale: Professor Weinman s work in other diseases has identified the relationship between illness beliefs and patients adherence to medical treatments. He has developed cognitively based interventions which improve treatment adherence. We postulate that poor UV protection in XP patients may result from such psychosocial factors, and may be amenable to intervention. Aims: examine XP patients' psychological responses to their disease and to UV protection measure their UV protection behaviour. Identify psychosocial factors associated with poor UV protection. design and trial a behaviour change intervention and assess its effectiveness. Research Plan The project will be based in the NCG XP Service in London, the centre for all UK patients. However, all assessments and interviews will be in patients homes. TRIAL DESIGN (see diagrammatic summary diagramworkstreams.pdf supplementary document): A) Phase 1: Identification of psychosocial and biological factors associated with poor UV protection. (i) An in-depth study on 45 patients in the UK: detailed qualitative psychosocial interviews measurement of biological disease-specific variables affecting photoprotection objective measurement of photoprotection behaviour using wearable UV monitors and electronic patient activity diaries. N-of-1 study to investigate intra-individual variation in photoprotection behaviours over a longer time period, to find barriers and facilitators, and situational and emotional predictors of photoprotection behaviours. (ii) Cross-sectional International Questionnaire Study (UK, Germany, France, Tunisia and Japan). A cross-sectional questionnaire study to investigate photoprotection behaviour and psychosocial and biological factors in 400 Patients. This will complement the data from the in depth UK study with the statistical power of a larger group. It will also include a Health Economics evaluation of the Costs of XP . B) Phase 2: Development and controlled randomised trial of an Intervention to improve photoprotection: In phase 1, we will identify psychosocial, situational and social factors associated with poor UV protection. From these findings, we will develop a multicomponent behaviour change Intervention toolbox . This will contain the tools to design a personalised Intervention for each patient, aimed at modifying the specific psychological, social and behavioural determinants of adherence identified for that individual. We will use the Intervention in a Randomised Controlled Trial of 20 non-adherent patients and evaluate its effect on illness beliefs and on UV protection behaviour. The trial has a RCT with waiting list control design in view of the small size of the patient group. Evaluation includes Health Economics. Patient Recruitment: In the UK, we will recruit from our National XP clinic. Colleagues in the four countries will recruit their patients for the questionnaire study. Project duration: Four years and seven months. Outputs and dissemination of results: we will produce a Manual of the Intervention toolbox, in print and on an open access website. A DVD, and videos on the website, will demonstrate the intervention. We will publish findings in journals and feed back directly to patients. PPI: Our 6 member PPI panel has met the Project Team. Their suggestions have led to changes in Trial Design. Relevant Expertise: Our XP NCG Service cares for the largest cohort of XP patients in the world and is run by Dr Sarkany, Dr Fassihi and Prof Lehmann. Professor Weinman s work in Health Psychology has elucidated the psychological basis of non-adherence and has led to the development of effective interventions to improve treatment adherence in other diseases. Prof Morgan: Qualitative expert. Prof Wulf: real-time UV exposure measurement. Drs Sniehotta and Araujo-Soares: N-of-1 methodology and Intervention development. Dr Norton: Statistics; Dr Mander: trial design and analysis; Prof Mccrone: Health Economics; Dr Craythorne: skin cancer. Outcomes and Impacts: Improved photoprotection should reduce XP patients' skin cancer surgery long term and increase lifespan. Lessons may be transferable to non-XP related skin cancer and other rare inherited diseases. Results may inform photoprotection educational strategies in the general public. Project management A Steering Group will benchmark progress against targets every 3 months. Justification of costs The main change since the October 2013 application is the randomised controlled element of the Trial, using the 'RCT with waiting list control' design. The delayed intervention element of this has increased programme duration by a year. Other costs: The work takes place in patients homes entailing travel costs for the Researchers. The work generates huge amounts of UV wristwatch data requiring labour intensive analysis.

Lay abstract: Xeroderma Pigmentosum and Ultraviolet light protection: Xeroderma pigmentosum (XP) is a very rare inherited disease. Most patients die by the age of 35, usually from skin cancer. The skin in an XP patient is defenceless against cancer-causing ultraviolet light, so small amounts of normal daylight cause skin cancers to start appearing from around the age of 8 years. There is no cure. The only way to prevent the skin cancers is drastic, absolute daylight avoidance and protection. Patients not only have to avoid going outside during the day, but need ultraviolet-protective films applied to home, car and school windows. When they do go out, they need to wear gloves and a full face visor. This degree of UV protection is very hard to achieve but is crucial for patients to survive. Some patients manage, but others do not protect well from UV despite the efforts of their doctors and nurses. Research in other diseases has discovered why patients sometimes do not follow treatment advice. Although practical issues can be important, patients' choices about whether to follow treatment advice are mainly affected by psychological and social factors (including patients' beliefs and motivations). Aim: We aim to improve ultraviolet protection by discovering the psychosocial, situational and social factors preventing some patients from protecting their skin. We will then use an individualised psychological behaviour change intervention to help patients deal with these factors and UV protect better. This strategy has been successfully used in patients with other diseases. The study is a partnership of the National (NCG) Xeroderma Pigmentosum Service in London, with a Health Psychology team who specialise in the underlying psychological causes which prevent patients adhering to their medical treatments. Prospective outcome: By the end of the project we expect to have: Discovered the psychosocial and other factors which make it difficult for some XP patients to practice good UV protection. Worked intensively with patients on those factors using a psychological intervention and then tested to see whether UV protection improves after the intervention. We plan to produce an intervention instruction manual for Doctors and Nurses to enable them to improve UV protection in their XP patients. Benefits of the research: To patients: reduced numbers of skin cancers and fewer premature deaths. To the NHS: cost benefits: creating a cheap psychological intervention which reduces the costs of repeated surgery for skin cancers. To the Public: The NHS encourages safe sun behaviour in the public and in skin cancer survivors. This research may help discover better ways of reaching those who continue to sunbathe or use sunbeds. We also hope that there will be lessons applicable to other rare and severe inherited diseases where the treatment is as demanding.

Research activity codes: 7.1 INDIVIDUAL CARE NEEDS
Health categories: CONGENITAL DISORDERS