XPAND Trial: Enhancing XP Photoprotection Activities - New Directions

People with Xeroderma Pigmentosum (XP) have a genetic condition which stops their skin repairing damage from Ultraviolet Radiation (UVR). This means they are much more likely to develop potentially fatal skin cancers. The only way to reduce this damage is to rigorously protect the skin, by limiting UVR exposure. This done in a number of ways including: staying indoors; wearing protective clothing, sunscreen and glasses. People with XP can find it difficult to maintain this level of protection, putting themselves at risk. This research will test whether an intervention designed to enhance photoprotection activities is successful. It will use a randomised controlled trial design to compare the amount of UVR reaching the face, between participants receiving the intervention and those receiving standard clinical care. The amount of UVR reaching the face is important, as this is where people with XP develop most cancers. It is dependent on the overall level of exposure to UVR in the environment, and photoprotection used. The intervention involves a tailored conversation with the participant about their photoprotection practices. It will target both the overall exposure to UVR and the photoprotection used when outdoors, and will be conducted in 7 sessions with an intervention facilitator. The content will be dependent on the specific photoprotection behaviour being targeted (e.g., poor sunscreen application) and the reasons for poor photoprotection for each person. This could be low motivation related to doubts about the need to protect and concerns about protecting. Other barriers to protection might be lack of routines. The facilitator will provide information tailored to these beliefs and use other standard behaviour change techniques to encourage the development of "better" photoprotection habits. The investigators predict that the intervention group will have a lower mean daily dose of UVR to the face compared to the control group in two time periods in the summer months.

Sex

All

Minimum age

16 Years

Inclusion Criteria:

1. A confirmed diagnosis (on the basis of DNA repair study results) of Xeroderma Pigmentosum
2. Age \>16 years
3. Sub-optimal adherence to photoprotection when outdoors, identified by clinical team from data held in medical notes.

For those who participated in our previous research study, sub-optimal adherence will be confirmed from data collected during that study. They will be eligible if they meet any of the following criteria:

1. Score \<20 on the Adherence to Photoprotection scale (total score is 25 - optimal protection) from a cross-sectional questionnaire completed during our previous research.
2. Using photoprotective clothing that have been assessed by the clinical team as anything other than "very good or excellent" on anytime outdoors, recorded on the daily UVR protection diary.
3. Having a "resistant" mode of adjustment to photoprotection in the qualitative analysis

Exclusion Criteria:

1. Adults diagnosed with cognitive impairment
2. Adults with inadequate English to be able to converse with the intervention facilitators
3. Adults diagnosed with current clinical depression or anxiety

• BACKGROUNDWalburn J, Sarkany R, Norton S, Foster L, Morgan M, Sainsbury K, Araujo-Soares V, Anderson R, Garrood I, Heydenreich J, Sniehotta FF, Vieira R, Wulf HC, Weinman J. An investigation of the predictors of photoprotection and UVR dose to the face in patients with XP: a protocol using observational mixed methods. BMJ Open. 2017 Aug 21;7(8):e018364. doi: 10.1136/bmjopen-2017-018364. PMID 28827277
• DERIVEDWalburn J, Norton S, Sarkany R, Sainsbury K, Araujo-Soares V, Morgan M, Canfield M, Foster L, Heydenreich J, McCrone P, Mander A, Sniehotta FF, Wulf HC, Weinman J. Evaluation of a personalised adherence intervention to improve photoprotection in adults with Xeroderma Pigmentosum (XP): protocol for the trial of XPAND. BMJ Open. 2019 Jul 17;9(7):e028577. doi: 10.1136/bmjopen-2018-028577. PMID 31320353