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

• Published in: Health and quality of life outcomes Vol. 10; no. 1; p. 82
• Main Authors: Prior, Nieves, Remor, Eduardo, Gómez-Traseira, Carmen, López-Serrano, Concepción, Cabañas, Rosario, Contreras, Javier, Campos, Ángel, Cardona, Victoria, Cimbollek, Stefan, González-Quevedo, Teresa, Guilarte, Mar, de Rojas, Dolores Hernández Fernández, Marcos, Carmen, Rubio, María, Tejedor-Alonso, Miguel Ángel, Caballero, Teresa
• Format: Journal Article
• Language: English
• Published: England BioMed Central Ltd 20.07.2012; BioMed Central; BMC
• Subjects: Adolescent; Adult; Adults; Aged; Analysis; Angioedema; Angioedemas, Hereditary - psychology; Angioneurotic edema; C1 inhibitor deficiency; Complement C1 Inactivator Proteins - deficiency; Content analysis; Disease; Evidence-Based Medicine; Female; Hereditary angioedema; Humans; Interviews as Topic; Male; Medical research; Medicine, Experimental; Middle Aged; Pilot Projects; Psychometrics - methods; Qualitative analysis; Qualitative methodology; Qualitative Research; Quality of Life; Questionnaire; Questionnaires; Reproducibility of Results; Research projects; Severity of Illness Index; Spain; Studies; Study Protocol; Surveys and Questionnaires; Spain
• ISSN: 1477-7525; 1477-7525
• DOI: 10.1186/1477-7525-10-82

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. 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. 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.