Title: Xeroderma Pigmentosum: Codesign and testing of a specialised visor for ultraviolet radiation protection
Funder: Department of Health and Social Care

Abstract: There are four interdependent Work Packages (WP) with discrete deliverables (Appendices 4 Gantt Chart/5 Flowchart). WP1. Co-Design &ndash; iterative visor development with patients/parents/clinicians; patient-recorded outcome measures Health Research Authority (HRA) approvals for co-design events and non-UVR exposed patient/surrogate tests. PPI events, supporting WP2. Co-design events/contacts for iterative visor development and the patient-recorded outcomes (TELER methodology). Milestone: HRA approvals for co-design/testing activities (30 month project) (Month 1-6). Milestone: Qualitative data on visor designs; patient-recorded outcome measures, developed with patients, (Month 7-15). Deliverables: 1.1 Co-design report on patient needs and iterative development of the photoprotection visor. 1.2 Patient-recorded outcomes for the visor, developed and validated with the patients/parents using TELER methodology (Appendix 6). Patient-specific cost data will be collected including resource use, treatments (e.g. skin and eye cancer). 1.3 Cost effectiveness model WP2. Visor development and testing A clear UVR-absorbent visor, to be worn with commercially available collar/clothing, bridging gaps between the visor and patients clothing. WP2a Iterative prototyping of the Visor Concept Cardiff engineers will design and engineer the passive acoustic/ventilation system and test the functions in laboratory conditions. Milestone: Visor acoustic/ventilation system; laboratory tests (Month 1-12) WP2b Rapid and iterative 3D prototyping pre-manufacture visors with surrogate and non-UVR exposed patient tests. Milestone: Pre-manufacture visor prototypes: Rapid 3D printing of multiple iterations of the visor design (clear, non-UVR absorbing materials) (Month 1-12). Milestone: Surrogate and patient tests of pre-manufacture visor prototypes: 5 surrogates (UVR-exposed); 10 patients (UVR-protected) (Month 7-12). WP 2a, 2b Deliverables (Month 1-12) 2.1 Visor acoustic/ventilation system; laboratory tests 2.2 Rapid 3D printed pre-manufacture refined visors (non-UVR absorbing). 2.3 Surrogate (UVR-exposed, n=5) and patient (UVR-protected, n=10) tests of pre-manufacture prototype visor. WP2c Investigate and test commercially available photopolymerisable resins for 3D printed visor Milestone: Visor material, patch tested for UVR absorbance, optical clarity, absence of skin sensitivity Milestone: 3D printed visor components in optimised material WP2c Deliverables (Month 7-15) 2.4 Optimised visor material, patch tests (UVR absorbance, optical clarity, sensitivity) 2.5 3D printed demonstrator prototype components in optimised UVR absorbing, data validating UVR absorbance, optical clarity, safety WP2d Assembly of visor components into a demonstrator visor using magnets Visor components will be assembled into a demonstrator visor using magnets (Appendix 3) Milestone: Assembled demonstrator visors WP2d Deliverable (Month 7-18) 2.6 Assembled (magnets) demonstrator visor prototype in optimised UVR absorbing clear material for laboratory tests WP2e Laboratory, surrogate (UVR-exposed), patient (non UVR&ndash;exposed) demonstrator visor tests Visors will be subjected to laboratory safety tests, measuring photoprotection ability, compliance with UV400 requirements (100% absorption across the UVR range). Milestone: Laboratory safety tests of UVR dosimetry and transmission (Month 13-15). Milestone: Surrogate (UVR-exposed), patient (UVR-protected) demonstrator visor tests and validation of patient-recorded outcome measures (Month 7-15). WP2e Deliverables 2.7 Laboratory test data of UVR dosimetry and transmission. 2.8 Surrogate (UVR-exposed n=5), patient (UVR-protected n=10) tests of demonstrator visor; validated patient-recorded outcome measures. WP3 Visor proof-of-concept test; visor modifications/re-tests (as required); cost effectiveness analysis Milestone: HRA approvals (Month 10-18) Milestone: N-of-1 quasi-experimental UVR-exposed proof-of-concept test (n=10) of visor performance; cost effectiveness analysis (Month 19-30). Milestone: Visor modifications/re-tests as indicated by the proof-of-concept tests (Month 22-27). Deliverables 3.1 Patient-recorded outcomes, qualitative feedback, costs 3.2 Proof-of-concept tests, patient-recorded outcomes, cost-effectiveness analysis 3.3 Device modifications/Retests, replicating WP2 process WP4 IP, Freedom to Operate searches, manufacturing, commercialisation plans, dissemination. Milestone: FTO searches; IP protection (Month 4-6; Month-18-24) Milestone: Manufacturing, commercialisation plans; identification of commercial partner (Month 4-30). WP4 Deliverables 4.1 FTO searches 4.2 IP protection 4.3 Manufacturing; commercialisation 4.4 Dissemination

Lay abstract: Xeroderma Pigmentosum (XP) is a rare, inherited, incurable condition affecting approximately one in 250,000 of the general population. People with XP cannot tolerate ultraviolet radiation (UVR) from natural light, not just sunshine. We cannot see or feel UVR, but it can cause normal cells to become cancerous. With XP, miniscule exposure can cause skin and eye cancers from around the age of eight. Life expectancy is around 32 years; skin cancer is the main cause of death. Preventing skin and eye cancers requires absolute protection from UVR. Protective films are applied to car windows, homes, schools, and offices. Outdoor activity requires full cover-up with clothing and headwear, even on a cloudy day. Clothing is purchased. Headwear is homemade. Patients and families say the headwear steams-up, interferes with seeing, talking, and they receive unkind comments on their appearance. Many avoid all of this by not covering up, with disastrous consequences including eye damage/visual impairment, eye and skin cancers. We are working with XP charities and meeting patients with XP and their families, to develop a protection visor. Aim: To co-design and test a UVR protection visor with children and adults with XP. Expected Outputs: A visor that does not steam-up, disturb breathing, vision, or communication and is acceptable to people with XP. A fully tested visor. Patient-recorded outcomes of the acceptability and functioning of the visor, and costs. Manufacturing/commercialisation plans. Benefits: To patients: A wearable visor; reduced risk of eye/skin cancers and premature deaths. To the NHS: A visor that meets the UVR protection required to avoid skin/eye cancers in XP; adoption of the visor, reduced numbers of eye/skin cancers, premature deaths and costs. To the Public: A specialist visor that can benefit people without XP, who are vulnerable to eye/skin cancers. To Industry: Business opportunities nationally and internationally.

Research activity codes: 3.2 INTERVENTIONS TO ALTER PHYSICAL AND BIOLOGICAL ENVIRONMENTAL RISKS
Health categories: SKIN;CANCER AND NEOPLASMS