Burden of Wilson Disease among patients and care partners in the United States: results from a cross-sectional survey.

OBJECTIVE: This study assessed the burden of Wilson Disease (WD) among patients and care partners (WD-CPs) in the US and compared it to a US general population of adults (GPs) and care partners (GP-CPs). METHODS: This cross-sectional, self-reported survey included patients with WD and WD-CPs aged ≥18 years recruited through the Wilson Disease Association (WDA), while data for GPs and GP-CPs were obtained from the 2022 National Health and Wellness Survey. GPs and GP-CPs were propensity score matched (3:1) with WD patients and WD-CPs for demographics and health characteristics. Bivariate analysis evaluated differences in comorbidity burden and health-related outcomes of the WD cohorts compared to matched GP cohorts. RESULTS: Thirty-seven patients with WD and 53 WD-CPs completed the survey. Most patients reported some treatment burden (73.3%), experienced sleep problems (60%), and visited a healthcare provider (HCP) in the past 6 months (91.9%). Compared with matched GPs, patients with WD had a significantly higher mortality risk (p < .001) and reported greater rates of chronic liver disease, cirrhosis (both, p < .001), migraines (p = .032), non-alcoholic steatohepatitis (p = .004), sleep problems (p = .009) and HCP visits (p = .002). Most WD-CPs (75.5%) reported high burden of caring (mean ZBI-12 score, 26.5) and more negative impact on esteem than GP-CPs. CONCLUSION: This study highlights the burden of WD experienced by patients and WD-CPs, with patients experiencing high treatment burden, comorbidity burden and healthcare resource utilization, and WD-CPs experiencing high impact of caring, including impact on employment and self-esteem.

Wilson Disease (WD) is a rare genetic disorder that results from copper building up in the liver and the central nervous system. The management of WD has been consistent for the past 50 years. We surveyed patients with WD and family members of patients with WD ("care partners," hereby referred to as WD-CPs) residing in the US, to understand the burden of WD. We also used data from the 2022 National Health and Wellness Survey to compare patients with and WD-CPs to a sample of adults and care partners of adults in the general population (hereby referred to as GP and GP-CPs). The study found that the majority of the patients with WD reported some treatment burden (73.3%), experienced sleep problems (60.0%), and visited a healthcare provider (HCP) in the past 6 months (91.9%). Compared to GPs, patients with WD had a significantly higher risk of dying in the next 10 years and reported greater rates of other health conditions (chronic liver disease, cirrhosis, migraines, and non-alcoholic steatohepatitis), sleep problems, and visits to healthcare providers in the last 6 months. The majority of WD-CPs (75.5%) reported high burden of caring and more negative impact on their self-esteem than GP-CPs. Overall, this study highlights the burden of WD and suggests the need for more effective treatments that can reduce this burden.

Medication adherence following coronary artery bypass graft surgery: assessment of beliefs and attitudes.

BACKGROUND: The medication management of patients following coronary artery bypass graft (CABG) surgery may include antiplatelet agents, beta-blockers, angiotensin-converting enzyme inhibitors, and statins. However, poor adherence is common, and patient attitudes and beliefs play a role in adherence. OBJECTIVE: To evaluate the association between self-reported adherence and the beliefs patients have about cardiovascular medicines used after CABG. METHODS: Adults were surveyed 6-24 months following CABG. The validated Beliefs about Medicines Questionnaire (BMQ) assessed attitudes concerning the Specific Necessity, Specific Concerns, General Harm, and General Overuse of medicines. The validated medication adherence scale assessed self-reported adherence. Analysis included univariate comparison (BMQ scales) and multivariate logistic regression (identification of adherence predictor variables). RESULTS: Of 387 patients surveyed, 132 (34%) completed the questionnaire. Nonparticipants were more likely to be female and have undergone 1- or 2-vessel CABG procedures compared with 3- or 4-vessel procedures. Subjects were primarily English-speaking, white, and male. Adherent behavior was reported in 73 of 132 patients (55%). The average period between CABG and the survey was 16 months. Nonadherent patients were in stronger agreement on the General Overuse (p = 0.01) and General Harm (p = 0.04) scales. The adjusted odds of adherent behavior were significantly lower, with an increasing General Overuse score (OR 0.83; 95% CI 0.72 to 0.95; p = 0.007); an annual income of $50,000 to $100,000 relative to less than $20,000 (OR 0.36; 95% CI 0.14 to 0.91; p = 0.031), and a living status of "alone" compared with "with adults and no children" (OR 0.20; 95% CI 0.06 to 0.65; p = 0.007). The odds ratio of self-reported adherence was higher with increasing age (OR 1.05; 95% CI 1.01 to 1.09; p = 0.023). CONCLUSIONS: In summary, patient beliefs and attitudes regarding medications, along with other social, economic, and demographic factors, help explain differences in self-reported adherence to standard drug therapy following CABG.

Enhancing adherence to long-term medical therapy: a new approach to assessing and treating patients.

Poor adherence to medical therapy may cause as much as dollar 100 billion in unnecessary healthcare expenses each year. Nonadherence is a complicated and dynamic problem that involves patient, medication, and prescriber factors. The Case Management Adherence Guidelines (CMAG-1) are an evidence-based algorithm that introduces concepts and strategies for assessing medical knowledge and readiness to change (motivational interviewing). Once the assessment has been made, the guidelines can be used regularly to help patients become and remain adherent to their medication regimens. CMAG-1 and its tools, based on up-to-date information about adherence and ways to promote behavioral change, are designed to identify motivational and knowledge deficiencies that may block adherence. As information accumulates from centers that use it, CMAG-1 will be modified accordingly, with the goal of creating structured interaction with patients that will increase their knowledge and motivation to take medication appropriately.