Trinity College, School of Medicine, Trinity Centre for Health Sciences, Tallaght University Hospital, Dublin, Ireland.
Department of Neurology, Tallaght University Hospital, Dublin, Ireland.
Cerebellum. 2022 Apr;21(2):280-296. doi: 10.1007/s12311-021-01271-6. Epub 2021 Jul 6.
Inherited ataxias are a heterogenous group of neurodegenerative disorders characterised by progressive impairment of balance and coordination, typically leading to permanent and progressive disability. Diagnosis and management of these disorders incurs a range of direct and indirect financial costs. The aim of this study was to collect individual ataxia-related healthcare resources in a large cohort of individuals with different subtypes of inherited ataxia and calculate the associated cost of illness in the Republic of Ireland. One hundred twenty-nine respondents completed a cross-sectional study on healthcare resource utilisation for progressive ataxia in Ireland. Costs were calculated using a prevalence-based approach and bottom-up methodology. The COI for inherited ataxia in 2016 was €59,993 per person per year. Results were similar between participants with Friedreich's ataxia (FRDA, n = 56), non-FRDA (n = 18) and those with undetermined ataxia (n = 55). Indirect costs, based on productivity losses by participants or caregivers, accounted for 52% of the cost of illness. Inherited ataxia is associated with significant health and social care costs. Further funding for inherited ataxia to ease the financial burden on patients, caregivers and healthcare system and improve standards of care compliance is warranted.
遗传性共济失调是一组异质性的神经退行性疾病，其特征为平衡和协调功能进行性受损，通常导致永久性和进行性残疾。这些疾病的诊断和管理会产生一系列直接和间接的财务成本。本研究的目的是在一组患有不同类型遗传性共济失调的个体中收集与个体相关的医疗资源，并计算爱尔兰共和国的疾病负担成本。129 名受访者完成了一项关于爱尔兰进行性共济失调医疗资源利用的横断面研究。使用基于患病率的方法和自下而上的方法来计算成本。2016 年遗传性共济失调的疾病负担成本为每人每年 59993 欧元。弗里德里希共济失调（FRDA，n=56）、非 FRDA（n=18）和未确定共济失调（n=55）参与者的结果相似。基于参与者或照顾者的生产力损失的间接成本占疾病负担的 52%。遗传性共济失调与重大的健康和社会保健费用有关。需要进一步为遗传性共济失调提供资金，以减轻患者、照顾者和医疗保健系统的经济负担，并提高护理标准的合规性。
