Development of a disease specific health related quality of life measure for adults and adolescents with cystic fibrosis

Abstract

Background-Health related quality of life (HRQoL) measurement is important in determining the impact of disease on daily functioning and subsequently informing interventions. In cystic fibrosis (CF) generic HRQoL measures have been employed but these may not be suYciently specific. The aim of the current work was to develop and validate a disease specific HRQoL measure for adults and adolescents with cystic fibrosis. Methods-Areas of concern to adults and adolescents with CF were identified by unstructured interviews, selfadministered questionnaires, consultation with multidisciplinary specialist staV, a review of the relevant literature, and examination of other HRQoL measures. Advances in the care and treatment of cystic fibrosis (CF) have resulted in most patients surviving into adulthood.1 Despite these improvements, CF remains a progressive and ultimately fatal multisystem disease that has a heavy treatment regimen.2 Given this, a new goal for intervention in CF should be to measure and improve health related quality of life (HRQoL) in relation to medical and psychosocial interventions. The measurement of HRQoL complements clinical measures of disease status such as respiratory function tests. Once developed a questionnaire would be useful as (a) an outcome measure in clinical trials, (b) for the assessment of disease progression, and (c) for the monitoring of individual patients.To date, HRQoL in adults with CF has been measured using either generic scales 3-5 or disease specific respiratory measures.6-9 These measures were not developed for the CF population and are limited since they do not reflect areas of functioning that are particularly salient to the adult with CF. Because of this the data are likely to lack sensitivity and be problematic in their interpretation. This work aims to develop, test, and validate a disease specific measure of HRQoL for adults and adolescents with CF. The measure should include areas of functioning that are meaningful to adults with CF, be brief enough to be applied in a clinical setting (that is, completion time of about 10 minutes), be simple to administer and score (for use in clinical settings and postal surveys), and be sensitive enough to detect both changes in health within the individual and diVerences between levels of disease severity.The development, testing, and validation of the Cystic Fibrosis Quality of Life (CFQoL) questionnaire took place over four phases: (1) initial item generation and testing of a preliminary questionnaire; (2) testing and validation of a second version of the questionnaire including concurrent and discriminative validity; (3) test-retest reliability of a third and final version of the questionnaire; and (4) sensitivity testing of the final version of the questionnaire. The step by step development and validation of the CFQoL is reflected in the structure of this paper with the various methods and results sections reported in sequential order for each stage of development and testing. Thorax 2000;55:946-954 946

Percepção de qualidade de vida de pessoas com fibrose cística: um estudo sobre a adequação dos instrumentos de medida

“…Cinco instrumentos de medição de QV foram analisados: um instrumento genérico (WHOQOL-100) 34 , um relacionado à saúde (Short Form-36) 35 e três instrumentos relacionados à FC: 1) Cystic Fibrosis Questionnaire -Revised 27 , versão norte-americana do Cystic Fibrosis Questionnaire (CFQ) 22 , originalmente publicado em francês; 2) Cystic Fibrosis Quality of Life (CFQoL) 9 ; e 3) Questions on Life Satisfaction -CF Module (FLZ M -CF) 19 . O FLZ M -CF pressupõe o uso conjunto de um módulo genérico (FLZ M General) e de um módulo relacionado à saúde (FLZ M Health).…”

“…Nesse sentido, avaliações de qualidade de vida (QV) têm se mostrado um importante subsídio para o desenvolvimento de intervenções médicas e psicológicas mais sensíveis e eficazes, levando em consideração a perspectiva do próprio paciente 7 . Avaliações de QV são importantes para o planejamento e acompanhamento do tratamento, para a identificação dos fatores preditores de QV e para o desenvolvimento de estratégias de promoção de bem-estar e de prevenção de saúde mental [8][9][10][11][12][13] .…”

“…Com isso, há na atualidade um grande cuidado na realização de estudos populacionais 8,9,19 -os quais envolvem o levantamento de experiências, de informações, de sentimentos e de crenças da população alvo sobre QV -antes de se desenvolver um instrumento de medida. Uma vez desenvolvido, o instrumento pode ser traduzido e validado para novos idiomas e culturas.…”

Validação lingüística dos questionários de qualidade de vida em fibrose cística

“…The four final CFQ versions were then produced in Portuguese and named CFQ [6][7][8][9][10][11] , CFQ 12-13 CFQ 14+ and CFQ (the full texts of the questionnaires are available online at www.jped.com.br/ing).…”

“…3 Many disease-specific instruments and questionnaires have been developed over the last twenty years to assess QoL in CF, for both children and adults. [4][5][6][7][8][9] Some of these questionnaires have been validated in several different languages over the last ten years. [10][11][12] A suite of questionnaires originally developed and validated in France in 1996 are of most interest since they cover individuals from childhood to adulthood.…”