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

Overview

Journal J Acad Consult Liaison Psychiatry

Specialty Psychiatry

Date 2021 May 27

PMID 34044196

Citations 4

Authors

Michelle A Camarata

Aftab Ala

Ayse K Coskun

Yanhong Deng

Regino P Gonzalez-Peralta

Kaitlin R Maciejewski

Amar Patel

Susan Rubman

Uyen To

Ricarda Tomlin

Michael L Schilsky

Paula C Zimbrean

Affiliations

Soon will be listed here.

Abstract

Background: Wilson disease (WD) is a chronic disorder of copper metabolism which may affect patient's quality of life (QOL).

Objective: Our aim was to assess the relationship between mental QOL (M-QOL) and physical QOL (P-QOL) and severity of the liver, neurological disease and mental health in patients with WD.

Methods: At enrollment into our multisite international WD registry, adults (n = 62) were administered examinations assessing QOL (Short-Form 12-Item Health Survey), cognition, and mood. Patients also underwent hepatology and neurological assessments.

Results: Patients had lower M-QOL than P-QOL scores, P = 0.0006. Patients with major depressive disorder (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 the Unified Wilson Disease Rating Scale 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 Unified Wilson Disease Rating Scale 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 Model for End Stage Liver Disease scores (r = -0.64, P = 0.03).

Conclusions: M-QOL was associated with depression but not cognitive impairment, neurological disease, or liver disease severity, suggesting that mental health issues may affect overall QOL independent of the degree of liver or neurological 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 health care providers address issues that could improve overall well-being. The Short-Form 12-Item Health Survey may provide a useful instrument for QOL surveillance in WD.

Citing Articles

Marino Z, Berenguer M, Pena-Quintana L, Olveira A, Miralpeix A, Sastre I J Clin Med. 2023; 12(14).

PMID: 37510937 PMC: 10381913. DOI: 10.3390/jcm12144823.

Quality of life and depression in Wilson's disease: a large prospective cross-sectional study.

Chevalier K, Rahli D, de Veyrac L, Guillaume J, Obadia M, Poujois A Orphanet J Rare Dis. 2023; 18(1):168.

PMID: 37386576 PMC: 10308610. DOI: 10.1186/s13023-023-02777-4.

Wilson disease in Northern Portugal: a long-term follow-up study.

Garrido I, Marques M, Liberal R, Cardoso H, Lopes S, Macedo G Orphanet J Rare Dis. 2022; 17(1):82.

PMID: 35197085 PMC: 8867740. DOI: 10.1186/s13023-022-02245-5.

Quality of Life in Wilson's Disease: A Systematic Literature Review.

Balijepalli C, Yan K, Gullapalli L, Barakat S, Chevrou-Severac H, Druyts E J Health Econ Outcomes Res. 2021; 8(2):105-113.

PMID: 34963883 PMC: 8655222. DOI: 10.36469/jheor.2021.29987.

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