Advance Care Plan and Factors Related to Disease Progression in Patients With Spinocerebellar Ataxia Type 1: A Cross-Sectional Study in Thailand.

Abstract

Spinocerebellar ataxia type 1 (SCA1) is an autosomal dominant progressive neurodegenerative disease. Few studies have been conducted regarding advance care planning in this population. This study explores advance care planning preferences of patients with SCA1 and their association with disease progression and quality of life. The study examined 12 Thai patients with SCA1 from 2 families living in Thailand. The advance care plan followed a Gold Standards Framework. The 12 patients were interviewed and recorded in video. The research team evaluated neurocognitive functions as measured by the following tests; Scale for the Assessment and Rating of Ataxia (SARA), Berg Balance Score, Mini-Mental Status Examination, and Digit Span and Category Fluency. The quality of life was measured by a Short-Form Health Survey-36 (SF-36). Seven of 12 patients with SCA1 rated communication ability as most important for their quality of life. Patients identified becoming a burden on their family members and ventilator dependence as the most undesirable situations. Half of the patients preferred a hospital as their last place of care. Comparing patients prefer hospital to home has significantly high median SARA (23 vs 11.5; P = .03) and low SF-36 (41.4 vs 72.4; P = .02). Those patients preferring a hospital for end-of-life care exhibited more physical disability and lower quality of life than those who preferred home care. Making assisted living health-care services in the home more readily available and affordable may alleviate concerns of patients facing more severe physical challenges.