OBJECTIVES: Friedreich ataxia (FA) is an inherited disease resulting in progressive damage to the nervous system, leading to loss of coordination and muscle control. The aim of this study was to systematically review and synthesize evidence on resource utilization and costs in FA.

METHODS: A systematic search was carried out in Medline, Embase, and INAHTA databases from Jan 2001 to Jan 2021; according to PRISMA guidelines and guided by PICO criteria. Studies published in English that reported cost, resource utilization, and cost-effectiveness of any treatment for patients with FA and their caregivers were included. Two reviewers independently performed screening and data extraction.

RESULTS: The search retrieved 57 studies, of which 5 fulfilled the eligibility criteria. Among these, 2 reported resource utilization, and 5 reported cost data. No economic evaluation was identified. Neurologists and cardiologists were the most frequently visited physicians, seen by 61-86% and 57-86% of FA patients, respectively. About 23-46% of FA patients were hospitalized for an average of 5-9 days per year. Mean annual direct medical and non-medical cost per patient ranged from £8.893 in the UK to CA$34.683 in Canada. Indirect costs were measured in productivity loss of patients with FA and their caregivers. The mean time lost per week among FA patients was 1-2 hours for those who were employed, and 4-5 hours for those attending classes. Caregiver support was needed among 60-77% of FA patients. Indirect cost due to productivity loss of adult patients and their caregivers was estimated at £14.822 per patient per annum.

CONCLUSIONS: Only limited evidence was identified among a few countries; and no economic evaluation has been identified in FA, indicating unmet needs to create such evidence.