https://pubmed.ncbi.nlm.nih.gov/34044196/ Wilsons

J Acad Consult Liaison Psychiatry. 2021 May 24;S2667-2960(21)00078-1.
doi: 10.1016/j.jaclp.2021.04.004. Online ahead of print.

Effect of Mental Health, Neurological and Liver Disease on Quality of Life in Patients with Wilson Disease

Michelle A Camarata 1, Aftab Ala 2, Ayse K Coskun 3, Yanhong Deng 4, Regino Gonzalez-Peralta 5, Kaitlin R Maciejewski 4, Amar Patel 6, Susan Rubman 7, Uyen To 3, Ricarda Tomlin 3, Michael L Schilsky 3, Paula C Zimbrean 7
Affiliations expand
PMID: 34044196

DOI: 10.1016/j.jaclp.2021.04.004

Abstract

Purpose: Wilson Disease (WD) is a chronic disorder of copper metabolism which may affect patient's quality of life (QOL). Our aim was to assess the relationship between mental QOL (M-QOL) and physical QOL (P-QOL) and severity of liver, neurological disease and mental health in WD patients.

Methods: At enrollment into our multi-site international WD registry, adults (n=62) were administered exams assessing QOL (SF-12), cognition and mood. Patients also underwent hepatology and neurological assessments.

Results: Patients had lower M-QOL compared to P-QOL scores, p=0.0006. Patients with major depressive disorder (MDD) (n=22) had worse M-QOL scores, p=0.0017 but not P-QOL. We found no association with impaired cognition (n=37) and QOL. The P-QOL scores have a moderate negative association with neurological disease severity based on Unified Wilson Disease Rating Scale (UWDRS) score (total (r= -0.38, p<0.003), part 2 (r= -0.50, p<0.0001) and part 3 (r = -0.37, p=0.004)). M-QOL was not associated with UWDRS scores. Worse P-QOL, but not M-QOL, was found in higher cirrhosis severity indicated by Child-Pugh (r= -0.80, p=0.002) and MELD scores (r= -0.64, p=0.03).

Conclusions: M-QOL was associated with depression but not cognitive impairment, neurological or liver disease severity, suggesting that mental health issues may affect overall QOL independent of the degree of liver or neurologic disease. P-QOL was affected by the severity of neurological and liver disease but not mental health but also contributes to overall QOL in WD. An appreciation of the range of problems that affect QOL in adults with WD will help healthcare providers address issues that could improve overall well-being. The SF-12 may provide a useful instrument for QOL surveillance in WD.