Evicenter
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SKY-0515

Huntington's disease

Last updated
211 sources

Product information and disease descriptionPlace of product in therapyDisease descriptionBurden of Huntington's disease

Humanistic burden and health-related quality of life

21 evidence topics · 22 sources

HDBOI study: EQ-5D-5L, SF-6D, and H-QoL-I scores by disease stage

“A total of 2,094 PwHD were enrolled with completed eCRFs (100%) and PPIE-P forms (n = 482, 23%).” (opens the source at this quote in a new tab)

“The mean EQ-5D-5L (n = 336) utility score was 0.59 (SD, 0.27), with the highest mean utility scores [SD] in ES (0.72 [0.22]) followed by MS (0.62 [0.18]) and AS (0.37 [0.30]), p < 0.001.” (opens the source at this quote in a new tab)

“The mean SF-6D score (n = 482) was 0.57 (SD, 0.10), with mean values decreasing with advanced disease (ES, 0.61; MS, 0.56; AS, 0.50, p < 0.001).” (opens the source at this quote in a new tab)

“H-QoL-I mean scores (n = 482) also worsened with more advanced disease, from 0.58 for ES to 0.49 for MS and 0.37 for AS, p < 0.001.” (opens the source at this quote in a new tab)

“Overall proxy respondents reported on average worse outcomes than PwHD (self-reported) across all outcomes and disease stages suggesting a possible unawareness of deficits by PwHD.” (opens the source at this quote in a new tab)

Health state utility values: systematic literature review

“Of 383 articles screened, 16 articles reported utility values estimated in 11 distinct studies.” (opens the source at this quote in a new tab)

“The EQ-5D index range was 0.89 - 0.72 for mild/prodromal HD and 0.71 - 0.37 for severe/late-stage disease.” (opens the source at this quote in a new tab)

“This study uncovered high variability in published utility estimates, indicating substantial uncertainty in existing data.” (opens the source at this quote in a new tab)

Health state utility values: EHDN REGISTRY SF-6D analysis

“11,328 questionnaires were completed by 5560 respondents with HD in 12 European countries.” (opens the source at this quote in a new tab)

“Women generally had lower HSUVs than men, and HSUVs were consistently lower than population norms for those with HD in the UK, and dropped with increasing disease severity.” (opens the source at this quote in a new tab)

“with TFC score, Behavioural score, and male gender significant predictors of SF-6D values (p < 0.001).” (opens the source at this quote in a new tab)

Stage-specific utility values applied in a U.S. cost-effectiveness model

“Health utilities for SoC were derived from the Hawton et al. study” (opens the source at this quote in a new tab)

“that used SF-6D preference-based measures to estimate stage-specific utilities for HD.” (opens the source at this quote in a new tab)

Health stateUtility, base caseRange
Early“0.73”“0.7–0.9”
Middle“0.63”“0.5–0.7”
Late“0.57”“0.4–0.6”
Norway cross-sectional study: EQ-5D-3L values by disease phase

“Mean HRQoL values declined across disease phases for the 84 participants; early phase (n = 34): mean = 0.84 (SD = 0.13), middle phase (n = 19): mean = 0.74 (SD = 0.10) and advanced phase (n = 31): mean = 0.65 (SD = 0.5).” (opens the source at this quote in a new tab)

Spain: H-QoL-I and EQ-5D-3L

“Fifty-five patients and an equal number of caregivers participated.” (opens the source at this quote in a new tab)

“The usual activities and anxiety/depression were the most severely altered dimensions according to the EQ-5D-3L results.” (opens the source at this quote in a new tab)

“Increase of disease severity resulted in lower patient QoL.” (opens the source at this quote in a new tab)

Chorea health states: time trade-off utility values in the general population

“Mean ± SD TTO-derived utility values were 0.07 ± 0.52, 0.26 ± 0.50, 0.48 ± 0.47, and 0.64 ± 0.41 for severe, moderate/severe, moderate/mild, and mild chorea severity, respectively.” (opens the source at this quote in a new tab)

“Respondents were willing to give up 3.6, 5.2, 7.4, and 9.3 years during a 10-year life span to avoid living with mild, mild/moderate, moderate/severe, and severe chorea, respectively.” (opens the source at this quote in a new tab)

HDQLIFE: development of a disease-specific HRQoL measurement system

“Existing item banks and new item pools were field tested in 536 individuals with prodromal, early-, or late-stage HD.” (opens the source at this quote in a new tab)

“HDQLIFE encompasses both validated Neuro-QoL/PROMIS measures, as well as five new scales in order to provide a comprehensive assessment of HRQOL in HD.” (opens the source at this quote in a new tab)

HDQLIFE physical function measures: reliability and validity

“A total of 510 individuals with Huntington's disease completed 2 Quality of Life in Neurological Disorders (Lower Extremity Function and Upper Extremity Function) and 3 Huntington's Disease Health-Related Quality of Life (Chorea, Speech Difficulties, and Swallowing Difficulties) measures.” (opens the source at this quote in a new tab)

“Reliabilities for the new patient reported physical functioning measures were excellent (all Cronbach's α > .92).” (opens the source at this quote in a new tab)

Chorea and HRQoL: U.S. online survey using HDQLIFE

“A total of 115 participants (n = 35 caregivers; n = 80 individuals with HD) were included in this study.” (opens the source at this quote in a new tab)

“Among those experiencing chorea (n = 83, 74% of respondents), 66% indicated it was 'Very Important' to manage chorea; however, only 47% agreed that their current medication regimen helped manage their movements.” (opens the source at this quote in a new tab)

“significantly higher Anxiety (P = 0.0423) and stigma (P < 0.0001) scores were observed among respondents with high chorea than in those with low chorea.” (opens the source at this quote in a new tab)

Determinants of HRQoL: functional capacity and depressive mood

“Multiple regression analyses showed that functional capacity and depressive mood were significantly associated with HrQOL, in that greater impairments in HrQOL were associated with higher levels of depressive mood and lower functional capacity.” (opens the source at this quote in a new tab)

“Motor symptoms and cognitive function were not found to be as closely linked with HrQOL.” (opens the source at this quote in a new tab)

Psychiatric burden: suicidal ideation and sleep disturbance in the HDBOI study

“PwHD reported current (13%, n = 272) or previous (28%, n = 575) suicidal ideation, which was more common with advanced HD (ES, 11%; MS, 14%; AS, 15%; p < 0.05).” (opens the source at this quote in a new tab)

“Of 482 questionnaire respondents, 91% (n = 437) reported difficulty sleeping, which was more common with AS HD (p < 0.05; [p = 0.000]).” (opens the source at this quote in a new tab)

Psychiatric burden: suicidal ideation measured with HDQLIFE Concern with Death and Dying items

“We examined 496 participants with premanifest or manifest HD.” (opens the source at this quote in a new tab)

“Our sample had a significantly higher occurrence of suicidal ideation (19.76%) and suicidal plans (2.1%) than the general population (p < 0.0001).” (opens the source at this quote in a new tab)

U.S. survey: individuals with HD and care partners compared with Parkinson's disease and the general population

“IHD had greater comorbid anxiety (IHD = 51.2%, IPD = 28.8%, GP = 2.0%), and HD-CP had greater comorbid anxiety (HD-CP = 52.5%, PD-CP = 28.6%, GP-CP = 19.6%) and depression (HD-CP = 65.0%, PD-CP = 29.9%, GP-CP = 19.6%), relative to other cohorts (p < 0.05).” (opens the source at this quote in a new tab)

“Respective of their GP cohorts, IHD exhibited lower HRQoL (EQ-5D: 0.66 ± 0.21 vs. 0.81 ± 0.17) and greater depression (PHQ-9: 11.59 ± 7.20 vs. 5.85 ± 6.71), whereas HD-CP exhibited greater depression only (PHQ-9: 6.84 ± 6.38 vs. 4.15 ± 5.58) (p < 0.001).” (opens the source at this quote in a new tab)

Canada: HRQoL of individuals with HD and care partner strain

“A total of 62 adult individuals with HD (or their proxies) and 48 care partners met defined eligibility criteria.” (opens the source at this quote in a new tab)

“For individuals with HD, the greatest HRQoL burden (i.e., lowest score) was for the SF-36v1 Role -Physical scale (46.8 [42.9]).” (opens the source at this quote in a new tab)

“Among care partners, 55.3% experienced high strain, as indicated by the CSI.” (opens the source at this quote in a new tab)

Caregiver burden: prospective longitudinal study of patient and caregiver pairs

“105 caregiver/patient pairs were included in the analysis.” (opens the source at this quote in a new tab)

“Of the two clusters of patients with advanced disease, cluster A was characterized by high levels of irritability and obsessive-compulsive behaviors, with high and increasing burden (N = 30; 29%), cluster B, the more apathetic group, with low and decreasing burden (N = 22; 21%).” (opens the source at this quote in a new tab)

Caregiver burden: determinants in a U.S. clinic sample

“Total functional capacity and being sole caregiver were significantly associated with higher caregiver burden via MCSI.” (opens the source at this quote in a new tab)

Caregiver burden: South Korea prospective cohort

“Sixty-five patients with HD and 45 caregivers were enrolled in this cohort study.” (opens the source at this quote in a new tab)

“A higher caregiver burden was associated with a more severe Shoulson-Fahn stage (p = 0.038) of the patients.” (opens the source at this quote in a new tab)

Caregiver burden: systematic review

“Twelve studies were included in the review involving 569 caregivers of patients with HD.” (opens the source at this quote in a new tab)

“Neuropsychiatric symptoms and the hereditary nature of the disease have been identified as important correlates of caregiver strain.” (opens the source at this quote in a new tab)

Patient and caregiver experience: ICER stakeholder input

“Caregiver impact increases as the disease progresses and individuals become more dependent on others, with 24/7 care eventually needed, particularly during the last five years of life.” (opens the source at this quote in a new tab)

“Motor symptoms are also a prominent feature of the disease and affect individuals’ ability to walk, talk, eat, and sleep.” (opens the source at this quote in a new tab)

Multigenerational burden: qualitative study across six European countries

“Cognitive and psychiatric symptoms were particularly burdensome, driving declines in quality of life (QoL) and emotional well-being.” (opens the source at this quote in a new tab)

“Caregivers reported extensive physical, financial, and psychological strain, with many assuming care responsibilities from a young age or eventually caring for multiple relatives.” (opens the source at this quote in a new tab)