6533b823fe1ef96bd127ec67

RESEARCH PRODUCT

Psychometric Field Study of Hereditary Angioedema Quality of Life Questionnaire for Adults:HAE-QoL

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description

Background: Hereditary angioedema due to C1 inhibitor deficiency (C1-INH-HAE) may affect health-related quality of life (HRQoL). A specific HRQoL questionnaire for adult patients with C1-INH-HAE, the HAE-QoL, has recently been developed in Spain. Objective: The objective of this study was to perform a cross-cultural validation and psychometric study of the HAE-QoL in an international setting. Methods: Cross-cultural adaptation of the Spanish HAE-QoL draft version and an international rating phase with experts were performed. The resultant version of the HAE-QoL, a clinical questionnaire, and Short Form 36-item Health Survey Version 2.0 (SF-36v2) were pilot tested internationally. Item reduction was based on both descriptive and exploratory factor analysis. Psychometric properties were assessed. Results: Cross-cultural adaptation of the HAE-QoL was performed in 18 countries. The draft version of the HAE-QoL was pilot tested in 332 patients, and accurate data were obtained from 290 patients from 11 countries. The reduction process resulted in a new version with 25 items and 7 dimensions (treatment difficulties, physical functioning and health, disease-related stigma, emotional role and social functioning, concern about offspring, perceived control over illness, and mental health). Strong psychometric properties were observed (Cronbach´s α 0.92; test-retest reliability 0.87). Convergent validity showed mild to moderate correlations with SF-36v2 physical and mental component summaries (0.45 and 0.64, respectively) and with SF-36v2 dimensions (P < .004). HAE-QoL scores discriminated significantly among severity groups (median: asymptomatic 133.5 vs severe 84.0; P < .001); between patients with and without long-term prophylaxis (median: 101 vs 90; P = .001); and between patients with and without psychiatric and/or psychological care (median: 74 vs 103; P ≤ .001). Conclusions: The HAE-QoL, currently available in 18 languages, showed good reliability and validity evidence. Fil: Prior, Nieves. Hospital Universitario Severo Ochoa; España Fil: Remor, Eduardo. Universidad Autónoma de Madrid; España Fil: Pérez Fernández, Elia. Fundacion Hospital Alcorcon; España Fil: Caminoa, Magdalena. Instituto de Investigacion Hospital Universitario la Paz ; España Fil: Gómez-Traseira, Carmen. Instituto de Investigacion Hospital Universitario la Paz ; España Fil: Gayá, Francisco. Instituto de Investigacion Hospital Universitario la Paz ; España Fil: Aabom, Anne. Odense University Hospital; Dinamarca Fil: Aberer, Werner. Medical University; Austria Fil: Betschel, Stephen. Saint Michael's Hospital; Canadá Fil: Boccon Gibod, Isabelle. Joseph Fourier University; Francia. Grenoble University Hospital; Francia Fil: Bouillet, Laurence. Joseph Fourier University; Francia. Grenoble University Hospital; Francia Fil: Bygum, Anette. Odense University Hospital; Dinamarca Fil: Csuka, Dorottya. Semmelweis University; Hungría Fil: Farkas, Henriette. Semmelweis University; Hungría Fil: Gomide, Maria. Universidade de Sao Paulo; Brasil Fil: Grumach, Anete. Universidade de Sao Paulo; Brasil Fil: Leibovich, Iris. Chaim Sheba Medical Center; Israel Fil: Malbrán, Alejandro. Dirección Nacional de Instituto de Investigación. Administración Nacional de Laboratorio e Instituto de Salud "Dr. C. G. Malbrán"; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina Fil: Moldovan, Dumitru. Mures County Hospital; Rumania Fil: Mihaly, Eniko. Mures County Hospital; Rumania Fil: Obtulowicz, Krystyna. Jagiellonian University; Polonia Fil: Perpén, Cecilia. Dirección Nacional de Instituto de Investigación. Administración Nacional de Laboratorio e Instituto de Salud "Dr. C. G. Malbrán"; Argentina Fil: Peveling Oberhag, Adriane. University Medical Center Mainz; Alemania Fil: Porebski, Grzegorz. Jagiellonian University; Polonia Fil: Chavannes, Celine Rayonne. Saint Michael's Hospital; Canadá Fil: Reshef, Avner. Chaim Sheba Medical Center; Israel Fil: Staubach, Petra. University Medical Center Mainz; Alemania Fil: Wiednig, Michaela. Medizinische University; Austria Fil: Caballero, Teresa. Instituto de Investigacion Hospital Universitario la Paz ; España