Quality of life in Wilson′s disease

Abstract

BACKGROUND: Assessment of Quality of life (QoL) is fast assuming significance as the measure of health in many disorders. AIM: To correlate clinical severity and QoL in patients with Wilson's disease (WD). MATERIALS AND METHODS: We evaluated patients of WD on regular follow up for at least two years and aged over 18 years using Neurological Symptom Score (NSS) for clinical severity and WHO-BREF for QoL at a university teaching hospital. Patients with inability to respond to the questionnaire due to behavioral problems, low IQ or other disease related factors were excluded. These 30 patients (M:F:: 23:7) had a mean age of 27.97 +/- 11.16 years at evaluation and the mean duration of treatment of 9.2 +/- 6.4 years. RESULTS: All four domains of WHO-QoL-BREF viz., Physical, Psychological, Social and Environmental correlated well with each other (p < 0.01). The NSS correlated inversely with the physical domain (p < 0.02), while the duration of treatment had a positive correlation with the physical domain (p < 0.01). None of the other features of QoL showed any significant correlation with age, NSS or duration of treatment. CONCLUSION: QoL is complementary to formal neurological assessment and should be routinely incorporated in the evaluation of outcome of patients with WD and other chronic neurological disorders.