“Everyone has a story to tell”: A review of life stories in learning disability research and practice

In this paper, the authors review life stories in learning disability research and practice since the 1960s. Although there is consistent evidence of their value in giving people a voice and an identity beyond the service label, they are not widely used in the provision of health and social care. This is despite long‐standing policy commitments to person‐centred practice. The paper explores possible barriers to the use of life story work and what further research is needed if they are to be more widely and effectively used in practice

Factors Associated With End-of-Life Planning in Huntington Disease.

OBJECTIVE: Knowledge of one\u27s gene status for adult onset conditions provides opportunity to make advance end-of-life (EOL) plans. The purposes of these analyses were to (1) determine the prevalence of EOL plans, including advance directives (ADs) among persons across 3 stages of Huntington disease (HD) and (2) examine factors associated with having ADs in this sample. METHODS: Data are from 503 participants in the HD Quality of Life study. Participants completed an online health-related quality-of-life survey that included questions regarding EOL planning and self-reported HD symptoms. Frequencies were calculated for EOL planning by the HD stage. Bivariate analysis and logistic regression were used to identify variables associated with having ADs. RESULTS: A total of 38.2% of participants stated they had ADs and fewer than half had other EOL plans. Being older, increased HD stage, more years of education, lower anxiety, more swallowing symptoms, and higher meaning and purpose were associated with having ADs. CONCLUSION: The prevalence of ADs in our sample is comparable to the general US population, but surprisingly low, considering the severity and long disease course of HD. PRACTICE IMPLICATIONS: Health-care providers should develop specific interventions early in the disease process to increase ADs in this population

Suicidal Ideation Assessment in Individuals with Premanifest and Manifest Huntington Disease.

BACKGROUND: Huntington disease (HD) is associated with increased risk of suicide. OBJECTIVE: This study compares suicide ideation in HD to the general population, assesses factors associated with increased prevalence of suicidal thoughts, and compares clinician-rated to self-reported assessments of suicidal ideation. METHODS: We examined 496 participants with premanifest or manifest HD. Clinician-rated suicidal ideation was measured using the Problem Behaviors Assessment - short form. Self-reported ideation was measured using two items from the HDQLIFE Concern with Death and Dying item bank. Independent sample t-tests were conducted to compare the prevalence of suicidal thoughts between our HD sample and the U.S. POPULATION: Logistic regression analyses were used to determine characteristics associated with higher odds of clinically significant suicidal ideation. Kappa agreement coefficients were calculated to evaluate concurrence between clinician-rated and self-reported assessments. RESULTS: Our sample had a significantly higher occurrence of suicidal ideation (19.76%) and suicidal plans (2.1%) than the general population (p \u3c 0.0001). Odds of clinically significant suicidal ideation were 6.8 times higher in females (p = 0.04) on the clinician measure, and Hispanic/Latinos had 10.9 times higher odds than non-Hispanics (p = 0.025) on the self-report measure. Clinician-rated assessment had fair agreement (k = 0.2-0.4) with self-reported assessments, except in early stage HD where there was no overlap in the identification of participants with clinically significant suicidal ideation. DISCUSSION: Assessment for suicidal ideation and clinically significant suicidal thoughts in HD with a multimodal approach that includes clinician-rated and self-report measures is critical at all stages of the disease

HDQLIFE and neuro‐QoL physical function measures: Responsiveness in persons with huntington’s disease

BackgroundHuntington’s disease (HD) is a neurological disorder that causes severe motor symptoms that adversely impact health‐related quality of life. Patient‐reported physical function outcome measures in HD have shown cross‐sectional evidence of validity, but responsiveness has not yet been assessed.ObjectivesThis study evaluates the responsiveness of the Huntington Disease Health‐Related Quality of Life (HDQLIFE) and the Quality of Life in Neurological Disorders (Neuro‐QoL) physical function measures in persons with HD.MethodsA total of 347 participants completed baseline and at least 1 follow‐up (12‐month and 24‐month) measure (HDQLIFE Chorea, HDQLIFE Swallowing Difficulties, HDQLIFE Speech Difficulties, Neuro‐QoL Upper Extremity Function, and/or Neuro‐QoL Lower Extremity Function). Of the participants that completed the baseline assessment, 338 (90.9%) completed the 12‐month assessment, and 293 (78.8%) completed the 24‐month assessment. Standardized response means and general linear models evaluated whether the physical function measures were responsive to self‐reported and clinician‐rated change over time.ResultsSmall to moderate effect sizes for the standardized response means supported 12‐month and 24‐month responsiveness of the HDQLIFE and Neuro‐QoL measures for those with either self‐reported or clinician‐rated declines in function. General linear models supported 12‐month and 24‐month responsiveness for all HRQOL measures relative to self‐reported declines in health, but generally only 24‐month responsiveness was supported relative to clinician‐rated declines in function.ConclusionsLongitudinal analyses indicate that the HDQLIFE and the Neuro‐QoL physical function measures are sensitive to change over time in individuals with HD. Thus, these scales exhibit evidence of responsiveness and may be useful outcome measures in future clinical trials. © 2019 International Parkinson and Movement Disorder SocietyPeer Reviewedhttps://deepblue.lib.umich.edu/bitstream/2027.42/154235/1/mds27908_am.pdfhttps://deepblue.lib.umich.edu/bitstream/2027.42/154235/2/mds27908.pd

Reliability and Validity of the HD-PRO-TriadTM, a Health-Related Quality of Life Measure Designed to Assess the Symptom Triad of Huntington\u27s Disease.

BACKGROUND: Huntington\u27s disease (HD), is a neurodegenerative disorder that is associated with cognitive, behavioral, and motor impairments that diminish health related quality of life (HRQOL). The HD-PRO-TRIADTM is a quality of life measure that assesses health concerns specific to individuals with HD. Preliminary psychometric characterization was limited to a convenience sample of HD participants who completed measures at home so clinician-ratings were unavailable. OBJECTIVES: The current study evaluates the reliability and validity of the HD-PRO-TRIADTM in a well-characterized sample of individuals with HD. METHODS: Four-hundred and eighty-two individuals with HD (n = 192 prodromal, n = 193 early, and n = 97 late) completed the HD-PRO-TRIADTM questionnaire. Clinician-rated assessments from the Unified Huntington Disease Rating Scales, the short Problem Behaviors Assessment, and three generic measures of HRQOL (WHODAS 2.0, RAND-12, and EQ-5D) were also examined. RESULTS: Internal reliability for all domains and the total HD-PRO-TRIADTM was excellent (all Cronbach\u27s α \u3e0.93). Convergent and discriminant validity were supported by significant associations between the HD-PRO-TRIADTM domains, and other patient reported outcome measures as well as clinician-rated measures. Known groups validity was supported as the HD-PRO-TRIADTM differentiated between stages of the disease. Floor and ceiling effects were generally within acceptable limits. There were small effect sizes for 12-month change over time and moderate effect sizes for 24-month change over time. CONCLUSIONS: Findings support excellent internal reliability, convergent and discriminant validity, known groups validity, and responsiveness to change over time. The current study supports the clinical efficacy of the HD-PRO-TRIADTM. Future research is needed to assess the test-retest reliability of this measure

Relationships Among Apathy, Health-Related Quality of Life, and Function in Huntington\u27s Disease.

Up to 90% of individuals with Huntington\u27s disease (HD)-a progressive, inherited neurodegenerative disorder-experience apathy. Apathy is particularly debilitating because it is marked by a reduction in goal-directed behaviors, including self-care, social interactions, and mobility. The objective of this study was to examine relationships between variables of apathy, functional status, physical function, cognitive function, behavioral status/emotional function, and health-related quality of life. Clinician-rated measures of physical, cognitive, and behavioral function, including one clinician-rated item on apathy, and self-reported measures of physical function, health-related quality of life, and emotional, cognitive, and social function were collected in a single session from 487 persons with the HD mutation (prodromal, N=193; early-stage manifest, N=186; late-stage manifest, N=108). Multiple linear regression models were used to examine which outcomes best predicted clinician-rated apathy after controlling for disease stage. Greater apathy related to less independence, increased motor impairment, and more clinician-rated behavioral problems (i.e., anger, irritability, depression). Similarly, poorer self-reported health-related quality of life; greater chorea; greater upper- and lower-extremity dysfunction; greater speech and swallowing dysfunction; worse anxiety, depression, and behavioral dyscontrol; worse cognitive function; and less satisfaction with social roles related to greater apathy. In conclusion, apathy related to physical, cognitive, and behavioral dysfunction across disease stages. Future work should explore whether clinical interventions targeting different functional domains may have the potential to reduce apathy in this patient population

A New Measure for End of Life Planning, Preparation, and Preferences in Huntington Disease: HDQLIFE End of Life Planning

BACKGROUND: Huntington disease is a fatal inherited neurodegenerative disease. Because the end result of Huntington disease is death due to Huntington disease-related causes, there is a need for better understanding and caring for individuals at their end of life. AIM: The purpose of this study was to develop a new measure to evaluate end of life planning. DESIGN: We conducted qualitative focus groups, solicited expert input, and completed a literature review to develop a 16-item measure to evaluate important aspects of end of life planning for Huntington disease. Item response theory and differential item functioning analyses were utilized to examine the psychometric properties of items; exploratory factor analysis was used to establish meaningful subscales. PARTICIPANTS: Participants included 508 individuals with pre-manifest or manifest Huntington disease. RESULTS: Item response theory supported the retention of all 16 items on the huntington disease quality of life ( HDQLIFE ) end of life planning measure. Exploratory factor analysis supported a four-factor structure: legal planning, financial planning, preferences for hospice care, and preferences for conditions (locations, surroundings, etc.) at the time of death. Although a handful of items exhibited some evidence of differential item functioning, these items were retained due to their relevant clinical content. The final 16-item scale includes an overall total score and four subscale scores that reflect the different end of life planning constructs. CONCLUSIONS: The 16-item HDQLIFE end of life planning measure demonstrates adequate psychometric properties; it may be a useful tool for clinicians to clarify patients\u27 preferences about end of life care

Agreement Between Clinician-Rated Versus Patient-Reported Outcomes in Huntington Disease

BACKGROUND: Clinician-rated measures of functioning are often used as primary endpoints in clinical trials and other behavioral research in Huntington disease. As study costs for clinician-rated assessments are not always feasible, there is a question of whether patient self-report of commonly used clinician-rated measures may serve as acceptable alternatives in low risk behavioral trials. AIM: The purpose of this paper was to determine the level of agreement between self-report and clinician-ratings of commonly used functional assessment measures in Huntington disease. DESIGN: 486 participants with premanifest or manifest Huntington disease were examined. Total Functional Capacity, Functional Assessment, and Independence Scale assessments from the Unified Huntington Disease Rating scale were completed by clinicians; a self-report version was also completed by individuals with Huntington disease. Cronbach\u27s α was used to examine internal consistency, one-way analysis of variance was used to examine group differences, and paired t tests, kappa agreement coefficients, and intra-class correlations were calculated to determine agreement between raters. RESULTS: Internal consistency for self-reported ratings of functional capacity and ability were good. There were significant differences between those with premanifest, early-, and late-stage disease; those with later-stage disease reported less ability and independence than the other clinical groups. Although self-report ratings were not a perfect match with associated clinician-rated measures, differences were small. Cutoffs for achieving specified levels of agreement are provided. CONCLUSIONS: Depending on the acceptable margin of error in a study, self-reported administration of these functional assessments may be appropriate when clinician-related assessments are not feasible

Evaluating Cognition in Individuals with Huntington Disease: NeuroQoL Cognitive Functioning Measures

PURPOSE: Cognitive functioning impacts health-related quality of life (HRQOL) for individuals with Huntington disease (HD). The Neuro-QoL includes two patient-reported outcome (PRO) measures of cognition-Executive Function (EF) and General Concerns (GC). These measures have not previously been validated for use in HD. The purpose of this analysis is to evaluate the reliability and validity of the Neuro-QoL Cognitive Function measures for use in HD. METHODS: Five hundred ten individuals with prodromal or manifest HD completed the Neuro-QoL Cognition measures, two other PRO measures of HRQOL (WHODAS 2.0 and EQ5D), and a depression measure (PROMIS Depression). Measures of functioning The Total Functional Capacity and behavior (Problem Behaviors Assessment) were completed by clinician interview. Objective measures of cognition were obtained using clinician-administered Symbol Digit Modalities Test and the Stroop Test (Word, Color, and Interference). Self-rated, clinician-rated, and objective composite scores were developed. We examined the Neuro-QoL measures for reliability, convergent validity, discriminant validity, and known-groups validity. RESULTS: Excellent reliabilities (Cronbach\u27s alphas ≥ 0.94) were found. Convergent validity was supported, with strong relationships between self-reported measures of cognition. Discriminant validity was supported by less robust correlations between self-reported cognition and other constructs. Prodromal participants reported fewer cognitive problems than manifest groups, and early-stage HD participants reported fewer problems than late-stage HD participants. CONCLUSIONS: The Neuro-QoL Cognition measures provide reliable and valid assessments of self-reported cognitive functioning for individuals with HD. Findings support the utility of these measures for assessing self-reported cognition