As HD progresses, physical dependence and cognitive impairment increase, and the high care needs of HD can be difficult to manage at home. Non-medical factors, such as access to home care services and informal care, and financial considerations may influence when people with HD transition to LTC. 

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When a bed came up for my mom, we were ready to go. She’d had a series of bad falls and was hospitalized, and she got bumped to the top of the emergency list to get a bed. Before my mom’s admission to LTC, my family had challenges accessing sufficient home care services where the hours provided were too low. We were dependent on government-funded services, which limited what we could do. — Jenna Shea, Family caregiver, Ontario

In 2021–2022, there were 496 LTC residents with HD in Canada. They showed varied health characteristics, many of which were consistent with mid- to late-stage disease.Reference2  Many health characteristics are worse among LTC residents with HD compared with the general LTC population; LTC residents with HD are about 18 years younger, on average. ºìÁì½í¹Ï±¨ characteristics were slightly better among the 83 newly admitted LTC residents with HD who still showed signs of disease progression. (See data tables for more information.)

• 53% of LTC residents with HD had severe cognitive impairment, and 59% were dependent or totally dependent on others for activities of daily living (ADLs).Footnote ii
• Residents with impaired cognition generally showed worse health characteristics than those with intact cognition.  
• Many LTC residents with HD had difficulty swallowing (75%), significant recent weight loss (25%) and incontinence (60%), and were dependent on a wheelchair for mobility (63%). 
• About 50% of LTC residents with HD exhibited mental and behavioural symptoms, including depression and aggression. 
• A subset of LTC residents with HD (10%) had intact cognition and could perform ADLs independently.

Figure 1 Prevalence of selected health characteristics among LTC residents with HD and the general LTC population, 2021–2022  

Notes
ADLs: Activities of daily living, which include personal hygiene, toilet use, locomotion and eating.
Statistics for LTC residents with HD include data from Newfoundland and Labrador, New Brunswick, Ontario, Saskatchewan, Alberta and British Columbia.

Sources
*  Continuing Care Reporting System and Integrated interRAI Reporting System, 2021–2022; Canadian Institute for ºìÁì½í¹Ï±¨ Information.
†  Canadian Institute for ºìÁì½í¹Ï±¨ Information. Profile of Residents in Residential and Hospital-Based Continuing Care, 2021–2022. Ottawa, ON: CIHI; 2022.

Over a 1-year period, 91% of LTC residents with HD were prescribed 5 or more drug classesFootnote iii  while 15% were prescribed 15 or more drug classes (versus 88% and 20% of all LTC residents, respectively). 

• Antipsychotics were the top group of drugs prescribed to LTC residents with HD (they were prescribed to 81% of LTC residents with HD). The top chemicals were olanzapine (31%), risperidone (29%), haloperidol (22%) and quetiapine (22%).
• Tetrabenazine was prescribed to 10% of LTC residents with HD.
• Drugs for depression, seizures and anxiety were also commonly prescribed, and their rate of use exceeded the prevalence of these associated health conditions among LTC residents with HD.

Figure 2 Top 7 drugs prescribed to LTC residents with HD over a 1-year period,* by rate of use

Notes
* See the methodology notes for details.
Drug groups  correspond to the third level of the World ºìÁì½í¹Ï±¨ Organization (WHO) Anatomical Therapeutic Chemical (ATC) classification. 
This figure includes data from Newfoundland and Labrador, Ontario, Saskatchewan and British Columbia.

Sources
National Prescription Drug Utilization Information System, 2021 to 2023; and Continuing Care Reporting System and Integrated interRAI Reporting System, 2021–2022, Canadian Institute for ºìÁì½í¹Ï±¨ Information.

CIHI’s drug data does not contain the reason for prescribing, making it difficult to interpret off-label use, which is common for patients with HD. For example, antipsychotics are often prescribed off-label for chorea, given the side effects of tetrabenazine and the ability of antipsychotics to manage other symptoms of HD, including psychosis, aggression and depression.Reference3 Reference4 Second-generation atypical antipsychotics (e.g., olanzapine, risperidone, quetiapine) are first-line therapies given their tolerability.Reference4 Reference5

With the availability of genetic testing and other medical advancements, misdiagnosis and delays in diagnosis of HD are becoming less common. However, they can still occur due to the rarity of the disease, the delay between onset of neuropsychiatric and motor symptoms, and the lack of information on family history due to the stigma associated with HD, among other factors.Reference6

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My maternal grandfather lived in an isolated Ontario village and was misdiagnosed for 20 years, including with schizophrenia, until the late 1960s. My mother had complete loss of personal insight — lack of self-awareness — in early adulthood, delaying her diagnosis for over 20 years. HD tricked my mother into believing she was okay; she refused doctors’ examinations despite wobbly walking, falling, depression and paranoia. The only way to get her help was to become her legal guardian. — Timothy Irwin, Person living with HD, Ontario

Additionally, non-specialist health care providers may still not recognize or have experience with HD, which can lead to misdiagnosis and suboptimal care.Reference7Reference8Reference9

• In CIHI’s LTC analysis, 6% of LTC residents with HD were repeatedly diagnosed with schizophrenia despite clinical diagnosis criteria (Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition, or DSM-5) indicating not to diagnose it when symptoms are caused by another medical condition.Reference10
• As well, in CIHI’s analysis of outpatient clinic data, a small number of patients with HD had concurrent diagnoses of Parkinson’s disease or Pick’s disease (frontotemporal dementia). These conditions are unlikely to occur with HD but reflect the symptoms of HD. 
• A high percentage (45%) of LTC residents with HD did not receive any form of therapyFootnote iv despite showing motor and/or cognitive symptoms, even though best practice guidelines indicate its benefits for this population.Reference3

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Often [LTC] homes share occupational [therapists] and physiotherapists and they may not have capacity to take on more residents. Huntington Society of Canada’s social workers also need to educate service providers so that they understand the importance of these services for residents affected by HD to maintain their abilities and strength longer. — Angèle Bénard, Former National Director of Family Services, Huntington Society of Canada, Ontario

The LTC population with HD is relatively young and complex, and prescription medications are a key component of their care. While many residents are in the later stages of the disease, factors other than disease progression, such as access to informal and formal community care services, may also influence when people with HD transition to LTC.

This analysis also examined care for patients with HD in outpatient settings, using available clinic data. Home care and physician billing data was not analyzed because of challenges identifying patients with HD and limited jurisdictional data coverage.Reference12 The availability of richer clinic data across Canada and more community-based data to link across care settings would improve our understanding of care for patients with HD (and other rare diseases) at different disease stages.

• Drugs for rare diseases
• Data Learnings for Rare Disease Analysis (PDF)

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