The Challenge of Adherence in Wilson Disease: An Opportunity for Nurse‐Led Interventions

BACKGROUND AND AIMS: Life-long treatment is critical in Wilson disease (WD), a rare genetic disease leading to copper accumulation with hepatic and extrahepatic manifestations. Poor adherence is a well-known contributor to unfavourable clinical outcomes. Measuring adherence in these chronic patients may be challenging, as no gold standard method is available. We aimed at evaluating adherence in a well-characterised WD cohort and ultimately designing individualised nursing interventions. METHODS: Prospective, single-centre study, including adult WD patients. Adherence to medication was assessed by a three-dimensional approach: (1) self-reported ARMSe questionnaire; (2) pharmacy service dispensing records and (3) physician-based evaluation. Concordance between methods was assessed, and variables associated with low adherence were evaluated. RESULTS: Inclusion of 54 WD patients (54% female, median age 39 years, median time since diagnosis 20 years). According to ARMSe, 39% of the patients reported low adherence, which was significantly associated with younger age at evaluation, higher ALT and higher exchangeable copper levels. Although a trend towards a positive association between methods was observed, concordance was low and patients were not classified in a constant manner, as only 12% were noncompliant by the three methods. CONCLUSION: Despite the critical role of adherence for adequate control of WD, one-third of our cohort exhibited low adherence. The limited agreement among the different assessment methods reinforces the need for a combined strategy. The ARMSe questionnaire proved useful for guiding individualised educational nursing interventions by enabling the identification of patient-specific barriers to adherence.