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. doi: 10.1007/s11136-016-1273-y. Epub 2016 Apr 1.


Purpose: Huntington disease (HD) is an autosomal dominant neurodegenerative disease which results in several progressive symptoms, including bulbar dysfunction (i.e., speech and swallowing difficulties). Although difficulties in speech and swallowing in HD have a negative impact on health-related quality of life, no patient-reported outcome measure exists to capture these difficulties that are specific to HD. Thus, we developed a new patient-reported outcome measure for use in the Huntington Disease Health-Related Quality of Life (HDQLIFE) Measurement System that focused on the impact that difficulties with speech and swallowing have on HRQOL in HD.

Methods: Five hundred and seven individuals with prodromal and/or manifest HD completed 47 newly developed items examining speech and swallowing difficulties. Unidimensional item pools were identified using exploratory factor analysis and confirmatory factor analysis (EFA and CFA, respectively). Item response theory (IRT) was used to calibrate the final measures.

Results: EFA and CFA identified two separate unidimensional sets of items: Speech Difficulties (27 items) and Swallowing Difficulties (16 items). Items were calibrated separately for these two measures and resulted in item banks that can be administered as computer adaptive tests (CATs) and/or 6-item, static short forms. Reliability of both of these measures was supported through high correlations between the simulated CAT scores and the full item bank.

Conclusions: CATs and 6-item calibrated short forms were developed for HDQLIFE Speech Difficulties and HDQLIFE Swallowing Difficulties. These measures both demonstrate excellent psychometric properties and may have clinical utility in other populations where speech and swallowing difficulties are prevalent.

Keywords: HDQLIFE; Health-related quality of life; Huntington disease; Neuro-QoL; PROMIS; Patient-reported outcome (PRO); Speech difficulties; Swallowing difficulties.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Computers / statistics & numerical data*
  • Deglutition Disorders / therapy*
  • Female
  • Humans
  • Huntington Disease / psychology*
  • Male
  • Middle Aged
  • Reproducibility of Results
  • Sickness Impact Profile
  • Speech Disorders / therapy*
  • Surveys and Questionnaires
  • Young Adult