Quality of Life of Patients with Wilson's Disease and Their Families

Ojas Unavane; Kritika Tiwari; Aabha Nagral; Ritika Aggarwal; Nikita Garg; Nishtha Nagral; Bela Verma; Ajay Jhaveri; Maninder S Setia

doi:10.1016/j.jceh.2021.05.013

Quality of Life of Patients with Wilson's Disease and Their Families

J Clin Exp Hepatol. 2022 Mar-Apr;12(2):461-466. doi: 10.1016/j.jceh.2021.05.013. Epub 2021 Jun 9.

Authors

Ojas Unavane¹, Kritika Tiwari², Aabha Nagral³, Ritika Aggarwal⁴, Nikita Garg⁴, Nishtha Nagral⁵, Bela Verma⁶, Ajay Jhaveri⁴, Maninder S Setia⁷

Affiliations

¹ Seth G.S Medical College and KEM Hospital, Mumbai, India.
² Matushree Gomati Hospital, Mumbai, India.
³ Jaslok Hospital and Research Centre, Mumbai, Apollo Hospital, Navi Mumbai, India.
⁴ Jaslok Hospital and Research Centre, Mumbai, India.
⁵ Fortis Hospital, Mulund, Mumbai, India.
⁶ Sir J.J. Hospital and GMC, Mumbai, India.
⁷ MGMIHS, Navi Mumbai, India.

Abstract

Objectives: Wilson's disease (WD) is a chronic disease caused by altered copper metabolism requiring lifelong therapy. Its long-term and debilitating nature has the potential to affect the quality of life (Qol) of patients as well as their families. Our study aims to assess this impact of the disease on patients and their families.

Methods: We conducted a prospective, observational study over 2 years on 73 patients and 73 age-matched controls with 33 children and 40 adults in each group. The Qol of cases and controls was assessed using the PedsQL Generic Core Scales and World Health Organisation Quality of Life BREF (WHOQOL-BREF) for children and adults, respectively. Families of child and adult patients were interviewed using PedsQL Family Impact Module and Family Attitude Scale (FAS), respectively. The data were statistically analyzed.

Results: Mean age of the cases was 22.04 ± 11.8 years. Qol scores for both adults and children were worse in cases with neuropsychiatric disease than in those with hepatic disease. For children, the mean scores of overall psychological functioning were lower in cases compared with controls (P = 0.0001). Qol of parents of the patients was significantly lower than those of parents of the controls as was the family functioning (P = 0.0001 and P = 0.016). Family Attitude Scale scores for adults did not differ significantly between cases and controls.

Conclusion: The Qol of patients with neuro-WD is worse than that of hepatic disease. The disease impacts the psychological functioning of the children and the Qol of their families, which improves with the duration of the disease.

What is known: WD is a long-term, debilitating disease. Patients have to take lifelong treatment with frequent medical visits and often multiple hospitalizations.

What is new: WD affects the Qol of not only the patients but also their families. Qol of patients with neuro-WD is worse than that of patients with hepatic disease.

Keywords: FAS, Family Attitude Scale; Family Attitude Scale; PedsQL; Qol, quality of life; WD, Wilson's disease; WHOQOL-BREF, World Health Organisation Quality of Life BREF; hepatic; neuropsychiatric.