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Health-related quality of life in Huntington's disease patients: a comparison of proxy assessment and patient self-rating using the disease-specific Huntington's disease health-related quality of life questionnaire (HDQoL).

ABSTRACT: Huntington's disease (HD) is a fatal, neurodegenerative disease for which there is no known cure. Proxy evaluation is relevant for HD as its manifestation might limit the ability of persons to report their health-related quality of life (HrQoL). This study explored patient-proxy ratings of HrQoL of persons at different stages of HD, and examined factors that may affect proxy ratings. A total of 105 patient-proxy pairs completed the Huntington's disease health-related quality of life questionnaire (HDQoL) and other established HrQoL measures (EQ-5D and SF-12v2). Proxy-patient agreement was assessed in terms of absolute level (mean ratings) and intraclass correlation. Proxies' ratings were at a similar level to patients' self-ratings on an overall Summary Score and on most of the six Specific Scales of the HDQoL. On the Specific Hopes and Worries Scale, proxies on average rated HrQoL as better than patients' self-ratings, while on both the Specific Cognitive Scale and Specific Physical and Functional Scale proxies tended to rate HrQoL more poorly than patients themselves. The patient's disease stage and mental wellbeing (SF-12 Mental Component scale) were the two factors that primarily affected proxy assessment. Proxy scores were strongly correlated with patients' self-ratings of HrQoL, on the Summary Scale and all Specific Scales. The patient-proxy correlation was lower for patients at moderate stages of HD compared to patients at early and advanced stages. The proxy report version of the HDQoL is a useful complementary tool to self-assessment, and a promising alternative when individual patients with advanced HD are unable to self-report.

SUBMITTER: Hocaoglu MB

PROVIDER: S-EPMC3432781 | biostudies-other | 2012 Sep

REPOSITORIES: biostudies-other

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Health-related quality of life in Huntington's disease patients: a comparison of proxy assessment and patient self-rating using the disease-specific Huntington's disease health-related quality of life questionnaire (HDQoL).

Hocaoglu Mevhibe B MB Gaffan E A EA Ho Aileen K AK

Journal of neurology 20120306 9

Huntington's disease (HD) is a fatal, neurodegenerative disease for which there is no known cure. Proxy evaluation is relevant for HD as its manifestation might limit the ability of persons to report their health-related quality of life (HrQoL). This study explored patient-proxy ratings of HrQoL of persons at different stages of HD, and examined factors that may affect proxy ratings. A total of 105 patient-proxy pairs completed the Huntington's disease health-related quality of life questionnair ...[more]

PMID: 22392579

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Project description:BackgroundTo assess the quality of life of people with dementia, measures are required for self-rating by the person with dementia, and for proxy rating by others. The Quality of Life in Alzheimer's Disease scale (QoL-AD) is available in two versions, QoL-AD-SR (self-rating) and QoL-AD-PR (proxy rating). The aim of our study was to analyse the inter-rater agreement between self- and proxy ratings, in terms of both the total score and the items, including an analysis specific to care setting, and to identify factors associated with this agreement.MethodsCross-sectional QoL-AD data from the 7th Framework European RightTimePlaceCare study were analysed. A total of 1330 cases were included: n = 854 receiving home care and n = 476 receiving institutional long-term nursing care. The proxy raters were informal carers (home care) and best-informed professional carers (institutional long-term nursing care). Inter-rater agreement was investigated using Bland-Altman plots for the QoL-AD total score and by weighted kappa statistics for single items. Associations were investigated by regression analysis.ResultsThe overall QoL-AD assessment of those with dementia revealed a mean value of 33.2 points, and the proxy ratings revealed a mean value of 29.8 points. The Bland-Altman plots revealed a poor agreement between self- and proxy ratings for the overall sample and for both care settings. With one exception (item 'Marriage' weighted kappa 0.26), the weighted kappa values for the single QoL-AD items were below 0.20, indicating poor agreement. Home care setting, dementia-related behavioural and psychological symptoms, and the functional status of the person with dementia, along with the caregiver burden, were associated with the level of agreement. Only the home care setting was associated with an increase larger than the predefined acceptable difference between self- and proxy ratings.ConclusionsProxy quality of life ratings from professional and informal carers appear to be lower than the self-ratings of those with dementia. QoL-AD-SR and QoL-AD-PR are therefore not interchangeable, as the inter-rater agreement differs distinctly. Thus, a proxy rating should be judged as a complementary perspective for a self-assessment of quality of life by those with dementia, rather than as a valid substitute.

Project description:BackgroundHuntington's disease (HD) is a progressive genetic neurodegenerative disease accompanied by mental and neurocognitive disabilities, which requires long-term and comprehensive treatment and care. Information on the health and economic burden of HD is scarce, but essential for conducting health economic analyses, in light of the prospect of new therapies for HD. In this study, we aim to identify values for Health-Related Quality of Life (HRQoL), describe service utilization and costs, and their associations with clinical and socio-demographic variables across all phases of HD.MethodsA cross-sectional study including 86 patients across all phases of HD. Values of HRQoL were calculated based on EQ-5D-3L index scores. Additionally, health care and societal costs were estimated based on service utilization collected using the Client Service Receipt Inventory (CSRI) and data from the patients' interviews. Total societal costs included costs of primary and secondary health care services, informal care and productivity loss of the patients. Multiple regression analyses were used to investigate associations between socio-demographic and clinical variables on HRQoL and costs.ResultsHRQoL values declined, while total costs increased across disease severity. Total six-month healthcare costs and total societal costs were € 18,538 and € 66,789 respectively. Healthcare and societal costs doubled from early to middle phase, and tripled from middle to advanced disease phase. Main six-month cost components for the three disease phases were informal care costs (€ 30,605) accounting for approximately half the total societal costs, and costs due to production loss (€ 18,907) being slightly higher than the total healthcare costs. Disease severity and gender were found to have the strongest effect on both values of HRQoL and costs.ConclusionsReported values of HRQoL and costs including costs for production loss may be used in modelling the cost-effectiveness of treatment for HD. Our results highlight the crucial role the informal caregivers play in the care provided to HD patients in all disease phases. Future research should focus on the estimation of productivity loss among informal caregivers.

Project description:BACKGROUND:The implementation of neonatal screening and the early initiation of lifelong therapy have helped to prevent severe complications and enabled much more favorable outcomes for early-treated phenylketonuria (ETPKU) patients. However, PKU patients tend to develop subtle cognitive and psychosocial abnormalities and the strict dietary therapy can present financial and social burden. Thus, PKU is expected to affect the quality of life (QoL) of these patients. There is insufficient evidence regarding the relationship between metabolic control and Health-Related QoL (HRQoL). We aimed to assess the effect of short- and long-term therapy on QoL among Hungarian adult PKU patients using the standardized PKU-specific PKU-QoL questionnaire. Methods: We conducted a single-centre, cross-sectional, observational study in Hungary. We included adult PKU patients treated with diet and amino acid supplements only. Patients reported HRQoL using the standardized adult PKU-QoL questionnaire and mean blood Phe concentrations were assessed for three different time periods: the previous 10 years, the previous year and concentration at the time of completing the questionnaire. The correlation between patients' QoL scores and their Phe levels was assessed. The classical PKU group was further divided into "good" and "suboptimal" adherence groups based on individual mean Phe levels in the examined time period. We evaluated differences in QoL among the two subgroups of classical PKU patients. QoL scores between classical and non-classical patients were also compared. Results: Data from 88 adult patients were analysed (66 had classical PKU). No median PKU-QoL score reached major or severe impact/frequent symptoms in any domain. The highest scores (meaning larger burden) were mostly related to emotional impact of PKU and disease management. When performing correlation analysis between Phe levels and QoL scores by all patients we found weak to fair positive correlation in several domains either short or long term. Patients with classical PKU reported greater financial impact of PKU than patients with less severe PKU. Classical PKU patients with good therapy adherence tended to report better HRQoL scores than patients with suboptimal adherence. Conclusion: We conclude that patients showed good HRQoL using the PKU-specific questionnaire. Our study demonstrates that suboptimal metabolic control is negatively associated with patients' HRQoL.

Project description:Several measures of oral health-related quality of life have been developed for children. The most frequently used are the Child Perceptions Questionnaire (CPQ), the Child Oral Impacts on Daily Performances (C-OIDP) and the Child Oral Health Impact Profile (COHIP). The aim of this study was to assess the methodological quality of the development and testing of these three measures.A systematic search strategy was used to identify eligible studies published up to December 2012, using both MEDLINE and Web of Science. Titles and abstracts were read independently by two investigators and full papers retrieved where the inclusion criteria were met. Data were extracted by two teams of two investigators using a piloted protocol. The data were used to describe the development of the measures and their use against existing criteria. The methodological quality and measurement properties of the measures were assessed using standards proposed by the Consensus-based Standards for the Selection of Health Measurement Instruments (COSMIN) group.The search strategy yielded 653 papers, of which 417 were duplicates. Following analysis of the abstracts, 119 papers met the inclusion criteria. The majority of papers reported cross-sectional studies (n?=?117) with three of longitudinal design. Fifteen studies which had used the original version of the measures in their original language were included in the COSMIN analysis. The most frequently used measure was the CPQ. Reliability and construct validity appear to be adequate for all three measures. Children were not fully involved in item generation which may compromise their content validity. Internal consistency was measured using classic test theory with no evidence of modern psychometric techniques being used to test unidimensionality of the measures included in the COSMIN analysis.The three measures evaluated appear to be able to discriminate between groups. CPQ has been most widely tested and several versions are available. COHIP employed a rigorous development strategy but has been tested in fewer populations. C-OIDP is shorter and has been used successfully in epidemiological studies. Further testing using modern psychometric techniques such as item response theory is recommended. Future developments should also focus on the development of measures which can evaluate longitudinal change.

Dataset Information

Health-related quality of life in Huntington's disease patients: a comparison of proxy assessment and patient self-rating using the disease-specific Huntington's disease health-related quality of life questionnaire (HDQoL).

Publications

Health-related quality of life in Huntington's disease patients: a comparison of proxy assessment and patient self-rating using the disease-specific Huntington's disease health-related quality of life questionnaire (HDQoL).

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