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NINDS CDE Notice of Copyright
HDQLIFE
Availability
Please email the author for information about obtaining the instrument: Dr. Noelle Carlozzi, carlozzi@med.umich.edu
Classification
Supplemental for 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.  
 
Time: 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.
Rationale/Justification
Psychometric Properties Development: 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).  Qualitiative 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 contructs (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).  
 
Other Important Notes: 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, N.E., Downing, N.R., McCormack, M.K., Schilling, S.G., Perlmutter, J.S., Hahn, E. A., Lai, J.-S., Frank, S., Quaid, K.A., Paulsen, J. S., Cella, D., Goodnight, S.M., Miner, J.A., & Nance, M.A.  (2016).  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).  Quality of Life Research, 25(10), 2403-2415.
 
Carlozzi, N.E., Downing, N.R., Schilling, S.G., Lai J.-S., Goodnight, S.M., Miner, J.A., & Frank, S. (2016).  The development of a new computer adaptive test to evaluate chorea in Huntington Disease: HDQLIFE Chorea. Quality of Life Research, 25(10), 2429-2439.
 
Carlozzi, N.E., Schilling, S.G., Lai J.-S., Paulsen, J.S., Hahn, E.A., Perlmutter, J.S., Ross, C.A., Downing, N.R., Kratz, A.L., McCormack, M.K., Nance, M.A., Quaid, K.A., Stout, J.C., Gershon, R.C., Ready, R.E., Miner, J.A., Barton, S.K., Perlman, S.L., Rao, S.M., Frank, S., Shoulson, I., Marin, H., Geschwind, M.D., Dayalu, P., Goodnight, S.M., & Cella, D. (2016).  HDQLIFE: Development and assessment of health-related quality of life in Huntington disease (HD). Quality of Life Research, 25(10), 2441-2455.
 
Carlozzi, N. E., Schilling, S.G., Lai, J.-S., Perlmutter, J.S., Nance, M.A., Waljee, J.F., Miner, J.A., Barton, S.K., Goodnight, S.M., & Dayalu, P. (2016).  HDQLIFE: The development of two new computer adaptive tests for use in Huntington disease, Speech Difficulties and Swallowing Difficulties.  Quality of Life Research, 25(10), 2417-2427.
 
Carlozzi, N. E., & Tulsky, D.S. (2013). Identification of health-related quality of life (HRQOL) issues relevant to individuals with Huntington disease. J Health Psychol, 18(2), 212–225.
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