HDQLIFE and Neuro‐QoL Physical Function Measures: Responsiveness in Persons with Huntington's Disease

Carlozzi, Noelle E.; Boileau, Nicholas R.; Chou, Kelvin L.; Ready, Rebecca E.; Cella, David; McCormack, Michael K.; Miner, Jennifer A.; Dayalu, Praveen

doi:10.1002/mds.27908

Cited by 15 publications

(13 citation statements)

References 56 publications

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“…HDQLIFE Chorea 6 , 47 assesses the impact that chorea (i.e., the dance‐like movements characteristic of HD) has on physical activity and participation; data support its reliability, validity, and responsiveness in HD. 6 , 47 , 48 , 49 …”

Section: Methodsmentioning

confidence: 99%

“…HDQLIFE Speech Difficulties 6 , 50 assesses how difficulty with oral expression, language production, and articulation affects communication and well‐being; data support its reliability, validity, and responsiveness in HD. 6 , 48 , 49 , 50 …”

Section: Methodsmentioning

confidence: 99%

“…HDQLIFE Swallowing Difficulties 6 , 50 assesses how swallowing and choking problems impact well‐being and eating; data support its reliability, validity, and responsiveness in HD. 6 , 48 , 49 , 50 …”

Section: Methodsmentioning

confidence: 99%

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Meaning and purpose in Huntington’s disease: a longitudinal study of its impact on quality of life

Sokol

Troost

Kluger

et al. 2021

Ann Clin Transl Neurol

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Objective Previous work in Huntington’s disease (HD) has shown that a sense of meaning and purpose (M&P) is positively associated with positive affect and well‐being (PAW); however, it was unknown whether HD‐validated patient‐reported outcomes (PROs) influence this association and how M&P impacts PROs in the future. Our study was designed to examine if HD‐validated PROs moderate the relationship between M&P and PAW and to evaluate if baseline M&P predicts 12‐ and 24‐month changes in HD‐validated PROs. Methods This was a longitudinal, multicenter study to develop several PROs (e.g., specific for the physical, emotional, cognitive, and social domains) for people with HD (HDQLIFE). The sample consisted of 322 people with HD (n = 50 prodromal, n = 171 early‐stage manifest, and n = 101 late‐stage manifest HD). A single, multivariate linear mixed‐effects model was performed with PAW as the outcome predicted by main effects for M&P and several moderators (i.e., an HD‐validated PRO) and interactions between M&P and a given PRO. Linear‐mixed models were also used to assess if baseline M&P predicted HD‐validated PROs at 12 and 24 months. Results Higher M&P was positively associated with higher PAW regardless of the magnitude of symptom burden, as represented by HD‐validated PROs, and independent of disease stage. In our primary analysis, baseline M&P predicted increased PAW and decreased depression, anxiety, anger, emotional/behavioral disruptions, and cognitive decline at 12 and 24 months across all disease stages. Interpretation These findings parallel those seen in the oncology population and have implications for adapting and developing psychotherapeutic and palliative HD interventions.

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Section: Methodsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

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Meaning and purpose in Huntington’s disease: a longitudinal study of its impact on quality of life

Sokol

Troost

Kluger

et al. 2021

Ann Clin Transl Neurol

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“…Pearson correlations were used to assess convergent validity (when pairs of variables were normally distributed) or Spearman correlations (when at least one variable was not normal) in order to determine the relationship between the WHODAS 2.0 and the H-QoL-I [ 23 ]. Correlations less than 0.3 were considered weak, 0.3–0.6 sufficient, and 0.6 or more were regarded as good or very good evidence of convergent validity [ 24 ].…”

Section: Methodsmentioning

confidence: 99%

Psychometric Properties and Validation of the Polish Version of the 12-Item World Health Organization Disability Assessment Schedule 2.0 in Patients with Huntington’s Disease

Ćwirlej-Sozańska

Sozański

Kupczyk

et al. 2021

JCM

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Background: Huntington’s disease is a progressive neurodegenerative disorder that usually manifests in adulthood and is inherited in an autosomal dominant manner. The main aim of the study was to assess the psychometric properties of the 12-item WHO Disability Assessment Schedule (WHODAS) 2.0 in studying the level of disability in people with Huntington’s disease. Method: This is a cross-sectional study that covered 128 people with Huntington’s disease living in Poland. We examined scale score reliability, internal consistency, convergent validity, and known-group validity. The disability and quality of life of people with Huntington’s disease were also assessed. Results: The scale score reliability of the entire tool for the research group was high. The Cronbach’s α test result for the whole scale was 0.97. Cronbach’s α for individual domains ranged from 0.95 to 0.79. Time consistency for the overall result was 0.99 and for particular domains ranged from 0.91 to 0.99, which confirmed that the scale was consistent over time. All of the 12-item WHODAS 2.0 domains negatively correlated with all of the Huntington Quality of Life Instrument (H-QoL-I) domains. All correlation coefficients were statistically significant at the level of p < 0.001. The results obtained in the linear regression model showed that with each subsequent point of decrease in BMI the level of disability increases by an average of 0.83 points on the 12-item WHODAS 2.0 scale. With each subsequent year of the disease, the level of disability increases by an average of 1.39 points. Conclusions: This is the first study assessing disability by means of the WHODAS 2.0 in the HD patient population in Poland, and it is also one of the few studies evaluating the validity of the WHODAS 2.0 scale in assessing the disability of people with HD in accordance with the recommendations of DSM-5 (R). We have confirmed that the 12-item WHODAS 2.0 is an effective tool for assessing disability and changes in functioning among people with Huntington’s disease.

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“…Although research efforts offer great promise, currently there is no cure for HD [7]. The gold standard for treatment is comprehensive multidisciplinary care to manage drug responsive symptoms, maximize function and maintain the highest possible quality of life until death [7][8][9][10][11], Huntington's Disease Society of America (HDSA) Centers of Excellence (COEs) are one source of onsite, same day multidisciplinary care, and are the entry point for nearly all HD research performed in the U.S. [11][12][13][14]. However, there are only 41 of these specialty centers across the U.S. in 2017, and they are not equally distributed from a geospatial perspective [14].…”

Section: Introductionmentioning

confidence: 99%

Geographic Barriers Drive Disparities in Specialty Center Access for Older Adults with Huntington’s Disease

Nguyen

Bravo

Gonzalez‐Alegre

et al. 2022

JHD

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Background: Huntington’s Disease Society of America Centers of Excellence (HDSA COEs) are primary hubs for Huntington’s disease (HD) research opportunities and accessing new treatments. Data on the extent to which HDSA COEs are accessible to individuals with HD, particularly those older or disabled, are lacking. Objective: To describe persons with HD in the U.S. Medicare program and characterize this population by proximity to an HDSA COE. Methods: We conducted a cross-sectional study of Medicare beneficiaries ages ≥65 with HD in 2017. We analyzed data on benefit entitlement, demographics, and comorbidities. QGis software and Google Maps Interface were employed to estimate the distance from each patient to the nearest HDSA COE, and the proportion of individuals residing within 100 miles of these COEs at the state level. Results: Among 9,056 Medicare beneficiaries with HD, 54.5% were female, 83.0% were white; 48.5% were ≥65 years, but 64.9% originally qualified for Medicare due to disability. Common comorbidities were dementia (32.4%) and depression (35.9%), and these were more common in HD vs. non-HD patients. Overall, 5,144 (57.1%) lived within 100 miles of a COE. Race/ethnicity, sex, age, and poverty markers were not associated with below-average proximity to HDSA COEs. The proportion of patients living within 100 miles of a center varied from < 10% (16 states) to > 90% (7 states). Most underserved states were in the Mountain and West Central divisions. Conclusion: Older Medicare beneficiaries with HD are frequently disabled and have a distinct comorbidity profile. Geographical, rather than sociodemographic factors, define the HD population with limited access to HDSA COEs.

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HDQLIFE and Neuro‐QoL Physical Function Measures: Responsiveness in Persons with Huntington's Disease

Cited by 15 publications

References 56 publications

Meaning and purpose in Huntington’s disease: a longitudinal study of its impact on quality of life

Meaning and purpose in Huntington’s disease: a longitudinal study of its impact on quality of life

Psychometric Properties and Validation of the Polish Version of the 12-Item World Health Organization Disability Assessment Schedule 2.0 in Patients with Huntington’s Disease

Geographic Barriers Drive Disparities in Specialty Center Access for Older Adults with Huntington’s Disease

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