Studies on direct and indirect costs are lacking in Canada

Huntington’s disease (HD) is a fatal, autosomal dominant disorder that causes progressive neuronal dysfunction and death in areas of the brain underlying voluntary movement. HD has an estimated disease prevalence of 10.4-13.7 individuals per 100,000 in Canada. Current evidence suggests that the prevalence of HD may be increasing in Canada, as well as globally. Estimates extrapolated from a previous study suggest that there are up to 4,700 individuals affected with HD and 14,000 at 50% risk for HD in Canada. The typical onset of HD occurs in the prime of adult life. Subtle motor, cognitive and behavioural changes may manifest up to 15 years before a formal clinical diagnosis of motor dysfunction. However, once overt motor dysfunction is present the average survival is 18 years. In the absence of an effective disease-modifying therapy, the management of HD focuses on treating symptoms. Management requires an extensive, multidisciplinary approach through medical and non-medical means to support optimal function and quality of life. Consequently, the burden of HD on patients, families, and healthcare utilization is significant, and increases as patients lose functionality and independence. Direct healthcare costs for HD have been estimated in the US to be between $3,257-4,947 USD in early stages, rising steeply to $22,582-37,495 USD in late stages (annualized cost per patient). However, these costs do not include informal care or other societal costs. A study from the UK that included indirect costs found that later stages reached an average cost of £89,760 per person per year, with informal care as the cost driver. While there are significant differences between the US and UK health care systems, collectively, these results highlight a significant burden on healthcare utilization, as well as patients and caregivers. Similar studies on direct and indirect costs of HD are lacking in Canada. In addition, the incidence and prevalence of Huntington’s Disease in Canada is poorly understood. A Real World Evidence study examining the incidence, prevalence, direct and indirect costs, and impact of disease on patients, families and caregivers is needed. Real World Data on the impact of disease progression, treatment patterns, healthcare utilization, and societal burden will be vital to future economic analyses. References

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