Medication Adherence and Relevance of the SPUR Behavioural Diagnostic Tool in the Management of Patients with Wilson's Disease: The WilSPUR Study.

BACKGROUND: Wilson's disease (WD) is a rare autosomal-recessive disorder of copper metabolism that can cause hepatic and/or neuropsychiatric manifestations, most often presents in young people. Lifelong treatment adherence is essential to prevent neurological deterioration, hepatic decompensation and death, yet non-adherence is frequent. Centres of the French national WD network evaluated the feasibility and the perceived utility of SPUR, a behavioural diagnostic tool, in the management of people living with WD. METHODS: In this non-interventional, prospective, multicentre, non-comparative study, 100 adults with WD from four French centres completed the self-administered digital SPUR questionnaire, which estimates a non-adherence risk (NAR, 0-100) and identifies the individual behavioural drivers of that risk. Healthcare professionals (HCPs) then reported whether the SPUR results changed their perception of the patient, changed their support, and were useful overall. RESULTS: The mean NAR was low (15.8/100). The predominant behavioural drivers were social (societal and immediate) and financial. Perceived health condition and perceived mental well-being were both inversely associated with the NAR. HCPs considered SPUR useful in 82% of cases, although the tool changed their perception of the patient in only 26.3% and their support in only 27.4% of cases. These findings are exploratory given the subjective clinician-reported endpoint and the selected cohort. CONCLUSION: Clinicians perceived SPUR as useful in a selected, hepatology-recruited WD cohort. Because the study had no comparator group, no objective adherence measure and no outcome follow-up, its effect on adherence behaviour and clinical outcomes remains to be established in prospective, controlled studies using objective adherence markers.