title: Development of a disease-specific quality of life questionnaire for adult patients with hereditary angioedema due to C1 inhibitor deficiency (HAE-QoL): Spanish multi-centre research project.
abstract: BACKGROUND
  There is a need for a disease-specific instrument for assessing health-related quality of life in adults with hereditary angioedema due to C1 inhibitor deficiency, a rare, disabling and life-threatening disease. In this paper we report the protocol for the development and validation of a specific questionnaire, with details on the results of the process of item generation, domain selection, and the expert and patient rating phase.
  
  
  METHODS
  Semi-structured interviews were completed by 45 patients with hereditary angioedema and 8 experts from 8 regions in Spain. A qualitative content analysis of the responses was carried out. Issues raised by respondents were grouped into categories. Content analysis identified 240 different responses, which were grouped into 10 conceptual domains. Sixty- four items were generated. A total of 8 experts and 16 patients assessed the items for clarity, relevance to the disease, and correct dimension assignment. The preliminary version of the specific health-related quality of life questionnaire for hereditary angioedema (HAE-QoL v 1.1) contained 44 items grouped into 9 domains.
  
  
  CONCLUSIONS
  To the best of our knowledge, this is the first multi-centre research project that aims to develop a specific health-related quality of life questionnaire for adult patients with hereditary angioedema due to C1 inhibitor deficiency. A preliminary version of the specific HAE-QoL questionnaire was obtained. The qualitative analysis of interviews together with the expert and patient rating phase helped to ensure content validity. A pilot study will be performed to assess the psychometric properties of the questionnaire and to decide on the final version.
authors: Allergy Department, IdiPaz Hospital La Paz Health Research Institute, Madrid, Spain. npg74m@hotmail.com
authors: Nieves
authors: N
authors: Prior
authors: Eduardo
authors: E
authors: Remor
authors: Carmen
authors: C
authors: Gómez-Traseira
authors: Concepción
authors: C
authors: López-Serrano
authors: Rosario
authors: R
authors: Cabañas
authors: Javier
authors: J
authors: Contreras
authors: Ángel
authors: Á
authors: Campos
authors: Victoria
authors: V
authors: Cardona
authors: Stefan
authors: S
authors: Cimbollek
authors: Teresa
authors: T
authors: González-Quevedo
authors: Mar
authors: M
authors: Guilarte
authors: Dolores Hernández Fernández
authors: DH
authors: de Rojas
authors: Carmen
authors: C
authors: Marcos
authors: María
authors: M
authors: Rubio
authors: Miguel Ángel
authors: MÁ
authors: Tejedor-Alonso
authors: Teresa
authors: T
authors: Caballero
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D054179:Angioedemas, Hereditary / Q000523:psychology*; D003174:Complement C1 Inactivator Proteins / Q000172:deficiency*; D019317:Evidence-Based Medicine; D005260:Female; D006801:Humans; D007407:Interviews as Topic; D008297:Male; D008875:Middle Aged; D010865:Pilot Projects; D011594:Psychometrics / Q000379:methods*; D036301:Qualitative Research; D011788:Quality of Life; D015203:Reproducibility of Results; D012720:Severity of Illness Index; D013030:Spain; D011795:Surveys and Questionnaires
chemical_list: D003174:Complement C1 Inactivator Proteins