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HDQLIFE
Availability
Please email the author for information about obtaining the instrument: Dr. Noelle Carlozzi, carlozzi@med.umich.edu
 
More information is available at https://www.hdqlife.com/.
Classification
NeuroRehab Supplemental - Highly Recommended
Recommendations for Use: Indicated for studies requiring a Huntington's Disease-Specific Health-Related Quality of Life measure.
 
Supplemental - Highly Recommended: Huntington's Disease
Short Description of Instrument
Purpose: The HDQLIFE is a brief Patient Reported Outcome (PRO) measurement system designed specifically for use in adults with Huntington disease. HDQLIFE includes generic health-related quality of life measures of social, functional, psychological, and mental well-being from the Neuro-QOL and PROMIS measurement systems as well as health-related quality of life measures developed specifically for individuals with HD  including chorea, speech and swallowing difficulties, and end of life concerns.   
 
Overview: The HDQLIFE contains 5 calibrated item banks that are specific to HD, and 11 additional item banks from PROMIS/Neuro-QoL. All item banks have Likert-style items. HD-specific item banks cover the following domains: Chorea, Speech Difficulties, Swallowing Difficulties, Concern with Death and Dying, and Meaning and Purpose.  Generic Item banks from PROMIS/Neuro-QoL cover the following domains: Anger, Anxiety, Depression, Positive Affect and Well-Being, Emotional and Behavioral Dyscontrol, Upper Extremity Functioning, Lower Extremity Functioning, Applied Cognition-General Concerns, Applied Cognition -Executive Functioning, Stigma,  Ability to Participate with Social Roles and Activities, and Satisfaction with Social Roles and Activities.  
 
Administration: The HDQLIFE can be administered as short-forms or as Computer Adaptive Tests (CATs). Administration time for each measure is generally less than 1 minute. Administration time for the entire HDQLIFE measurement system is 10-20 minutes.
Scoring

All HDQLIFE measures are scored on a t metric with a mean of 50 and a standard deviation of 10. Higher scores indicate more of the construct being measured. Thus, higher scores for Chorea, Speech Difficulties, Swallowing Difficulties, Concern with Death and Dying, Anger, Anxiety, Depression, Emotional and Behavioral Dyscontrol, and Stigma indicate worse functioning, while higher scores on Meaning and Purpose, Positive Affect and Well-Being, Upper Extremity Functioning, Lower Extremity Functioning, Applied Cognition-General Concerns, Applied Cognition –Executive Functioning, Ability to Participate with Social Roles and Activities, and Satisfaction with Social Roles and Activities indicate better functioning. Thus a score of 60 is one standard deviation above the average referenced population. This could be a desirable or undesirable outcome, depending upon the concept being measured. Note that the referent group for the new measures (Chorea, Speech Difficulties, Swallowing Difficulties, Concern with Death and Dying, and Meaning and Purpose) are individuals with HD, while the referent group for the PROMIS/Neuro-QoL measures is the general population.
Scoring and Psychometric Properties
Scoring: All HDQLIFE measures are scored on a t metric with a mean of 50 and a standard deviation of 10. Higher scores indicate more of the construct being measured. Thus, higher scores for Chorea, Speech Difficulties, Swallowing Difficulties, Concern with Death and Dying, Anger, Anxiety, Depression, Emotional and Behavioral Dyscontrol, and Stigma indicate worse functioning, while higher scores on Meaning and Purpose, Positive Affect and Well-Being, Upper Extremity Functioning, Lower Extremity Functioning, Applied Cognition-General Concerns, Applied Cognition -Executive Functioning, Ability to Participate with Social Roles and Activities, and Satisfaction with Social Roles and Activities indicate better functioning. Thus a score of 60 is one standard deviation above the average referenced population. This could be a desirable or undesirable outcome, depending upon the concept being measured. Note that the referent group for the new measures (Chorea, Speech Difficulties, Swallowing Difficulties, Concern with Death and Dying, and Meaning and Purpose) are individuals with HD, while the referent group for the PROMIS/Neuro-QoL measures is the general population.
 
Psychometric Properties: Literature reviews and qualitative focus groups were conducted to determine the most relevant aspects of health-related quality of life (HRQOL) for individuals with HD.  Focus groups included six groups with individuals with diagnosed, symptomatic HD (n=24 participants), five groups with individuals either at risk for HD (i.e., have not been tested and were not diagnosed with HD yet but have a parent with HD) or with prodromal HD (i.e., have a positive gene test, but have not been diagnosed with manifest HD) (n=16 participants), three groups with non-clinical HD caregivers (e.g., family members; n = 17 participants), and two groups with HD clinicians (e.g., physicians, nurses; n = 25 participants) (Carlozzi & Tulsky, 2013).  Qualitative analysis indicated that the following HRQOL domains were relevant in HD: anxiety, stigma, anger, depression, behavioral change, positive psychological functioning, ability to participate in social roles and activities, satisfaction with social roles and activities, mobility/ambulation, adls/upper extremities, and fatigue (Carlozzi & Tulsky, 2013).  Measures to assess these domains are available as part of the PROMIS/Neuro-QOL system. In addition, several HRQOL domains were identified that were not represented within the PROMIS/Neuro-QoL system: end of life concerns, chorea, and speech and swallowing difficulties (Carlozzi & Tulsky, 2013).  Thus, the HDQLIFE study was designed to provide validation data for the existing PROMIS/Neuro-QoL measures that were identified as being relevant to individuals with HD, and to develop new measures that were not represented within this existing framework.
 
Three new item pools (end of life concerns, chorea, and speech and swallowing difficulties) were developed using cognitive debriefing with individuals with HD (each new item was reviewed by at least 5 individuals with prodromal or symptomatic HD), and expert, literacy, and translatability reviews (Carlozzi, Schilling, Lai, Paulsen et al., 2016). These item pools were field tested in 536 individuals with prodromal, early-, or late-stage HD.  Exploratory and confirmatory factor analyses, item response theory, and differential item functioning analyses were utilized to develop new item banks for Chorea (Carlozzi, Downing, Schilling et al., 2016), Speech Difficulties (Carlozzi, Schilling, Lai, Perlmutter et al., 2016), Swallowing Difficulties (Carlozzi, Schilling, Lai, Perlmutter et al., 2016), and Concern with Death and Dying (Carlozzi, Downing, McCormack, et al., 2016), with corresponding six-item short forms. A four-item short form was developed for Meaning and Purpose (Carlozzi, Downing, McCormack et al., 2016).  
 
Validity Data:  Convergent and discriminant validity was supported for the PROMIS/Neuro-QoL measures (Carlozzi, Schilling, Lai, Paulsen et al., 2016).  Specifically, the PROMIS/Neuro-QOL measures had moderate to strong relationships with generic self-report measures of HRQOL (r's ranged from .34 to .74). There were moderate relationships between PROMIS/Neuro-QoL measures and clinician-rated measures (r's ranged from .35 to .70 with the majority between .42 and .49) and correlations tended to be highest between each PROMIS/Neuro-QoL measure and the corresponding self-report measures of these same constructs  (Carlozzi, Schilling, Lai, Paulsen et al., 2016). Correlations were lowest among measures of different constructs (i.e., emotional measure vs. physical functioning measures) (Carlozzi, Schilling, Lai, Paulsen et al., 2016).
 
For almost all PROMIS/Neuro-QoL/HDQLIFE measures, prodromal HD performed better than early-HD and late-HD, and early-HD performed better than late-HD supporting known groups validity (Carlozzi, Schilling, Lai, Paulsen et al., 2016; Carlozzi, Downing, Schilling et al., 2016).  Construct validity of the new HDQLIFE measures was also supported by modest associations between these measures and CAG repeat number (r = .21, p<.01 for HDQLIFE Chorea; r = .20, p<.01 for HDQLIFE Speech Difficulties; r = .23, p<.01 for HDQLIFE Swallowing Difficulties; r = .11, p<.05 for HDQLIFE Concern with Death and Dying); (Carlozzi, Schilling, Lai, Paulsen et al., 2016).  Furthermore, convergent validity was supported for HDQLIFE Chorea by a significant positive correlation between HDQLIFE Chorea and the Unified Huntington Disease Rating Scales (UHDRS) Total Motor Score (r = 0.64, p<0.0001) (Carlozzi, Downing, Schilling et al., 2016).  
 
Demographic Considerations:  Analyses are also available examining demographic variables for the two HDQLIFE End of Life Measures (Concern with Death and Dying and Meaning and Purpose) (Carlozzi, Downing, McCormack, et al., 2016).  For HDQLIFE Concern with Death and Dying, there was a small, but significant negative relationship with age (r = -0.12, p = 0.009), but no relationship with education (r = 0.01, p = 0.76). Women (M = 50.92; SD = 9.39) report more Concern with Death and Dying than men (M = 48.80; SD = 8.24; t(493) = -2.59, p = 0.01) (Carlozzi, Downing, McCormack, et al., 2016). There was no relationship between HDQLIFE Meaning and Purpose and age (r = 0.05, p = 0.24), education (r = -0.07, p = 0.10), nor were there differences between men (M = 49.46; SD = 9.28) and women (50.42; SD 8.97; t(493) = -1.16, p = 0.25) (Carlozzi, Downing, McCormack, et al., 2016).
 
For each new HDQLIFE measure, the correlation between the CAT scores and the full item bank was 0.99, indicating that CAT based scores (which include far fewer items than the full bank) can produce results that are very similar to those obtained with administration of the entire item set (Carlozzi, Downing, Schilling et al., 2016; Carlozzi, Schilling, Lai, Perlmutter et al., 2016; Carlozzi, Downing, McCormack, et al., 2016).
 
Administration time for each of the 5 new HDQLIFE measures was consistently less than 30 seconds for prodromal participants, generally 45-60 seconds for early-HD participants and between 60 and 75 seconds for late-stage participants (Carlozzi, Schilling, Lai, Paulsen et al., 2016).
Rationale/Justification
PROMIS and Neuro-QOL are designed to provide a common metric for evaluating patient-reported outcomes across NINDS-sponsored clinical trials. These systems have been tested in large samples of individuals from both general and diverse, neurologically impaired populations.
References
Carlozzi NE, Ready RE, Frank S, Cella D, Hahn EA, Goodnight SM, Schilling SG, Boileau NR, Dayalu P. Patient-reported outcomes in Huntington's disease: Quality of life in neurological disorders (Neuro-QoL) and Huntington's disease health-related quality of life (HDQLIFE) physical function measures. Mov Disord. 2017 Jul;32(7):1096-1102.
 
Lai JS, Goodnight S, Downing NR, Ready RE, Paulsen JS, Kratz AL, Stout JC, McCormack MK, Cella D, Ross C, Russell J, Carlozzi NE. Evaluating cognition in individuals with Huntington disease: Neuro-QoL cognitive functioning measures. Qual Life Res. 2018 Mar;27(3):811-822.
 
Mestre TA, Carlozzi NE, Ho AK, Burgunder JM, Walker F, Davis AM, Busse M, Quinn L, Rodrigues FB, Sampaio C, Goetz CG, Cubo E, Martinez-Martin P, Stebbins GT; Members of the MDS Committee on Rating Scales Development. Quality of Life in Huntington's Disease: Critique and Recommendations for Measures Assessing Patient Health-Related Quality of Life and Caregiver Quality of Life. Mov Disord. 2018 May;33(5):742-749.
 
Hahn EA, Downing NR, Stout JC, Paulsen JS, Ready B, Goodnight S, Lai JS, Miner JA, Carlozzi NE. Understanding the need for assistance with survey completion in people with Huntington disease. Qual Life Res. 2018 Mar;27(3):801-810.
 
Carlozzi NE, Schilling S, Kratz AL, Paulsen JS, Frank S, Stout JC. Understanding patient-reported outcome measures in Huntington disease: at what point is cognitive impairment related to poor measurement reliability? Qual Life Res. 2018;27(10):2541-2555.
 
Carlozzi NE, Hahn EA, Goodnight SM, Kratz AL, Paulsen JS, Stout JC, Frank S, Miner JA, Cella D, Gershon RC, Schilling SG, Ready RE. Patient-reported outcome measures in Huntington disease: Quality of life in neurological disorders (Neuro-QoL) social functioning measures. Psychol Assess. 2018 Apr;30(4):450-458.
 
Carlozzi NE, Hahn EA, Frank SA, Perlmutter JS, Downing ND, McCormack MK, Barton S, Nance MA, Schilling SG; HDQLIFE Site Investigators and Coordinators. A new measure for end of life planning, preparation, and preferences in Huntington disease: HDQLIFE end of life planning. J Neurol. 2018 Jan;265(1):98-107.
 
Carlozzi NE, Hanks R, Lange RT, Brickell TA, Ianni PA, Miner JA, French LM, Kallen MA, Sander AM. Understanding Health-related Quality of Life in Caregivers of Civilians and Service Members/Veterans With Traumatic Brain Injury: Establishing the Reliability and Validity of PROMIS Mental Health Measures. Arch Phys Med Rehabil. 2019 Apr;100(4S):S94-S101.
 
Carlozzi NE, Goodnight S, Kratz AL, Stout JC, McCormack MK, Paulsen JS, Boileau NR, Cella D, Ready RE. Validation of Neuro-QoL and PROMIS Mental Health Patient Reported Outcome Measures in Persons with Huntington Disease. J Huntingtons Dis. 2019;8(4):467-482.
 
Carlozzi NE, Boileau NR, Paulsen JS, Perlmutter JS, Lai JS, Hahn EA, McCormack MK, Nance MA, Cella D, Barton SK, Downing NR. End-of-life measures in Huntington disease: HDQLIFE Meaning and Purpose, Concern with Death and Dying, and Endâ ofâ Life Planning. J Neurol. 2019 Oct;266(10):2406-2422.
 
Carlozzi NE, Boileau NR, Hahn EA, Barton SK, Cella D, McCormack MK, Ready RE. Responsiveness to Change Over Time: An Examination of the Neuro-QoL Social Function Measures in Persons with Huntington's Disease. J Huntingtons Dis. 2020;9(1):83-97.
 
Carlozzi NE, Boileau NR, Chou KL, Ready RE, Cella D, McCormack MK, Miner JA, Dayalu P. HDQLIFE and neuro-QoL physical function measures: Responsiveness in persons with huntington's disease. Mov Disord. 2020 Feb;35(2):326-336.
 
Carlozzi NE, Boileau NR, Paulsen JS, Downing NR, Ready R, Perlmutter JS, Cella D, Chou KL, McCormack MK, Barton S, Lai JS. Psychometric properties and responsiveness of Neuro-QoL Cognitive Function in persons with Huntington disease (HD). Qual Life Res. 2020 May;29(5):1393-1403.
 
Carlozzi NE, Boileau NR, Roberts A, Dayalu P, Hanifan DL, Miner JA, Claassen D, Provost EM. Understanding speech and swallowing difficulties in individuals with Huntington disease: Validation of the HDQLIFE Speech Difficulties and Swallowing Difficulties Item Banks. Qual Life Res. 2020;15(4):1-15.
 
Carlozzi NE, Boileau NR, Roche  MW, Ready RE, Perlmutter JS, Chou KL, Barton SK, McCormack MK, Stout JC, Cella D, Miner JA, Paulsen JS. Responsiveness to change over time and test-retest reliability of the PROMIS and Neuro-QoL mental health measures in persons with Huntington disease (HD). Qual Life Res. 2020 Dec;29(12):3419-3439.
 
Carlozzi NE, Downing NR, McCormack MK, Schilling SG, Perlmutter JS, Hahn EA, Lai JS, Frank S, Quaid KA, Paulsen JS, Cella D, Goodnight SM, Miner JA, Nance MA. New measures to capture end of life concerns in Huntington disease: Meaning and Purpose and Concern with Death and Dying from HDQLIFE (a patient-reported outcomes measurement system). Qual Life Res. 2016 Oct;25(10):2403-2415.
 
Carlozzi NE, Downing NR, Schilling SG, Lai JS, Goodnight SM, Miner JA, A Frank S. The development of a new computer adaptive test to evaluate chorea in Huntington disease: HDQLIFE Chorea. Qual Life Res. 2016 Oct;25(10):2429-2439.   
 
Carlozzi NE, Schilling SG, Lai J-S, Paulsen JS, Hahn EA, Perlmutter JS, Ross CA, Downing NR, Kratz AL, McCormack, MK, Nance MA, Quaid KA, Stout JC, Gershon RC, Ready RE, Miner JA, Barton SK, Perlman SL, Rao SM, Frank S, Shoulson I, Marin H, Geschwind MD, Dayalu P, Goodnight SM, Cella D. HDQLIFE: Development and assessment of health-related quality of life in Huntington disease (HD). Qual Life Res. 2016;25(10):2441-2455.
 
Carlozzi NE, Schilling SG, Lai JS, Perlmutter JS, Nance MA, Waljee JF, Miner JA, Barton SK, Goodnight SM, Dayalu P. HDQLIFE: the development of two new computer adaptive tests for use in Huntington disease, Speech Difficulties, and Swallowing Difficulties. Qual Life Res. 2016 Oct;25(10):2417-2427.  
 
Carlozzi NE, Tulsky DS. Identification of health-related quality of life (HRQOL) issues relevant to individuals with Huntington disease. J Health Psychol. 2013;18(2):212-225.
 
Document last updated January 2022