Objective: To collect real-life data from a large cohort of patients with inherited ataxia in Ireland, with special attention to the individual ataxia-related healthcare resources and costs, disability due to the disease and quality of life (QoL) measures. We aimed to compare responses of patients from a heterogeneous population across groups of all ages, different underlying genetic causes and disease durations. 

Background: In Ireland a very small number of neurologists manage the majority of people with inherited ataxia. With little information in the literature and with the support of the national ataxia patient organisation people with different types of ataxia were asked to complete a comprehensive survey to evaluate disability, resource use and QoL

Methods: Over 250 anonymous surveys were distributed in clinics, by post and at ataxia meetings nationwide. We report observational descriptive data and compare groups using non-parametric statistics.

Results: We received results from 130 patients (45% males). Seventeen percent were <25 years of age, 31% were >60 years old. Nine percent were working full or part time, 47% were unable to work or retired early due to ataxia. Thirty-nine percent were wheelchair-bound. Fifty-nine percent had symptom onset <20 years, 23% had late onset >40 years. As expected, the majority (42%) had Friedreich’s ataxia, 28% did not have genetic diagnosis. Forty-five percent relied on professionally paid care. Group comparisons and QoL data will be reported.

Conclusions: To date this is the first study in Ireland and  the largest single-country ‘real-life’ patient survey in Europe looking at patients with various types of inherited ataxia with comprehensive data on disability, healthcare resource use and QoL. 

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MDS Abstracts - https://www.mdsabstracts.org/abstract/living-with-ataxia-in-ireland-2016-a-nationwide-survey-of-130-irish-patients-with-inherited-ataxia/