Tradução, adaptação cultural e validação do questionário CVID_QoL e avaliação da qualidade de vida de adultos com Imunodeficiência Comum Variável
Data
2023-07-14
Autores
Batista, Letícia de Oliveira 
Orientadores
Wandalsen, Gustavo Falbo
Tipo
Dissertação de mestrado
Título da Revista
ISSN da Revista
Título de Volume
Resumo
Introdução: Os erros inatos da imunidade (EII) representam um grupo crescente e heterogêneo de doenças que causam alterações no sistema imunológico e cursam com maior suscetibilidade a infecções recorrentes, manifestações inflamatórias, alergias, distúrbios autoimunes e neoplasias. A avaliação da qualidade de vida relacionada à saúde (QVRS) desses pacientes auxilia na busca de um equilíbrio entre prolongar a sobrevida e preservar seu bem-estar, auxilia na avaliação da progressão da doença e é capaz de identificar problemas que podem ser atenuados através de intervenção clínica, sendo, dessa forma, essencial para otimizar a qualidade dos cuidados clínicos. Entretanto, não há registros na literatura de instrumentos específicos que avaliem a qualidade de vida dos pacientes com EII na Língua Portuguesa.
Objetivos: Traduzir, adaptar culturalmente e validar para a Língua Portuguesa (cultura brasileira) o questionário CVID_QoL e avaliar a QVRS e as preferências de tratamento de pacientes com Imunodeficiência Comum Variável (IDCV) em uso de imunoglobulina humana no Brasil.
Métodos: Este foi um estudo observacional, transversal e unicêntrico que incluiu pacientes adultos diagnosticados com IDCV em uso de imunoglobulina humana. O questionário CVID_QoL foi traduzido e adaptado culturalmente para o Português. Posteriormente, foi realizada a validação do questionário e a avaliação da QVRS desses pacientes por meio do questionário traduzido e do SF-36. Também foram coletados dados demográficos e clínicos e avaliado o desejo de alterar a via de administração da imunoglobulina humana (de subcutânea [IgSC] para intravenosa [IgIV] e vice-versa).
Resultados: O estudo incluiu 87 pacientes: 54% do sexo feminino e 46% do sexo masculino, com idade média de 37,5 anos (±15,9). A taxa de respostas ausentes foi de 0% para todos os itens do questionário. O questionário demonstrou ser de fácil e rápida aplicação, sendo autoadministrado e levando cerca de 10 a 15 minutos para seu preenchimento. Em relação ao escore global do CVID_QoL, observamos correlações moderadas com o SF-36 (-0,47 para o componente físico e -0,52 para o componente mental). Foi observado elevado índice de concordância na avaliação teste-reteste (CVID_QoL Global, ICC=0,90, 95% CI 0,83-0,94) e elevada consistência interna (α de Cronbach 0,93). Em relação aos determinantes da QVRS, observamos maior impacto relacionados ao medo de ficar sem o tratamento de imunoglobulina, preocupações com o futuro, preocupações com piora da saúde e cansaço, e menor impacto relacionados aos problemas no relacionamento com outros pacientes e familiares. Somente três pacientes (3,4%) estavam em uso de IgSC e 84 pacientes (96,6%) usavam IgIV. Do total de participantes, 39 (44,8%) apresentaram desejo de mudar a via de administração da imunoglobulina, 19 (21,8%) não quiseram e 29 (33,3%) não tinham certeza. Foi observado pior qualidade de vida entre os pacientes que tinham desejo de mudar a via de administração quando comparados aos pacientes que não tinham esse desejo (p=0,011; diferença entre médias=13,56 [IC 95% 2,44 a 24,67]).
Conclusão: A versão brasileira do CVID_QoL mostrou-se de rápida aplicação e fácil compreensão. O questionário apresentou correlação moderada quando comparado ao SF-36, comprovando sua validade e, além disso, demonstrou boa reprodutibilidade e consistência interna. Através do questionário foram identificados itens que impactam na QVRS dos pacientes com IDCV e que não seriam identificados em questionários genérico, como a preocupação em ficar sem tratamento com imunoglobulina.
Introduction: Inborn errors of immunity (IEI) represents a growing and heterogeneous diseases group that cause changes in the immune system and make it more susceptible to recurrent infections, inflammatory manifestations, allergies, autoimmune disorders and neoplasias. The assessment of the health-related quality of life (HRQoL) of these patients helps in the search for a balance between prolonging survival and preserving their well-being, helps in assessing the progression of the disease and is able to identify problems that can be mitigated through clinical intervention, thus being essential to optimize the quality of clinical care. However, there are no records in the literature of disease-specific instruments that assess the quality of life of patients with IEI in Portuguese. Objectives: Translate, culturally adapt and validate the CVID_QoL questionnaire for the Portuguese language (Brazilian culture) and to evaluate the HRQoL and treatment preferences of patients with Common Variable Immunodeficiency (CVID) using human immunoglobulin in Brazil. Methods: This was an observational, cross-sectional, single-center study that included adult patients diagnosed with CVID using human immunoglobulin. The CVID_QoL questionnaire was translated and culturally adapted into Portuguese. Subsequently, the questionnaire was validated, and the HRQoL of these patients was assessed using the translated questionnaire and the SF-36. Demographic and clinical data were also collected, and the desire to change the route of human immunoglobulin administration (from subcutaneous [IgSC] to intravenous [IVIg] and vice-versa) was evaluated. Results: The study included 87 patients: 54% female and 46% male, with a mean age of 37.5 years (±15.9). The absent response rate was 0% for all items in the questionnaire. The questionnaire proved to be easy and quick to apply, being self-administered and taking about 10 to 15 minutes to complete. Regarding the global CVID_QoL score, we observed moderate correlations with SF-36 (-0.47 for the physical component and -0.52 for the mental component). A high level of concordance was observed in the test-retest assessment (Global CVID_QoL, ICC=0.90, 95% CI 0.83-0.94) and high internal consistency (Cronbach's α 0.93). Regarding the determinants of HRQoL, we observed a greater impact related to the fear of running out of immunoglobulin treatment, concerns about the future, concerns about worsening health condition and tiredness, and a smaller impact related to problems in relationships with other patients and family members. Only three patients (3.4%) were using IgSC and 84 patients (96.6%) were using IVIg. Of the total number of participants, 39 (44.8%) wanted to change the immunoglobulin administration route, 19 (21.8%) did not want to and 29 (33.3%) were not sure. A worse quality of life was observed among patients who wanted to change the route of administration when compared to patients who did not (p=0.011; difference between means=13.56 [CI 95% 2.44 to 24.67]). Conclusion: The Brazilian version of the CVID_QoL proved to be quick to apply and easy to understand. The questionnaire presented moderate correlation when compared to the SF-36, proving its validity and, in addition, demonstrated good reproducibility and internal consistency. Through the questionnaire, items were identified that impact the HRQoL of patients with CVID and that would not be identified in generic questionnaires, such as the concern about running out of immunoglobulin treatment.
Introduction: Inborn errors of immunity (IEI) represents a growing and heterogeneous diseases group that cause changes in the immune system and make it more susceptible to recurrent infections, inflammatory manifestations, allergies, autoimmune disorders and neoplasias. The assessment of the health-related quality of life (HRQoL) of these patients helps in the search for a balance between prolonging survival and preserving their well-being, helps in assessing the progression of the disease and is able to identify problems that can be mitigated through clinical intervention, thus being essential to optimize the quality of clinical care. However, there are no records in the literature of disease-specific instruments that assess the quality of life of patients with IEI in Portuguese. Objectives: Translate, culturally adapt and validate the CVID_QoL questionnaire for the Portuguese language (Brazilian culture) and to evaluate the HRQoL and treatment preferences of patients with Common Variable Immunodeficiency (CVID) using human immunoglobulin in Brazil. Methods: This was an observational, cross-sectional, single-center study that included adult patients diagnosed with CVID using human immunoglobulin. The CVID_QoL questionnaire was translated and culturally adapted into Portuguese. Subsequently, the questionnaire was validated, and the HRQoL of these patients was assessed using the translated questionnaire and the SF-36. Demographic and clinical data were also collected, and the desire to change the route of human immunoglobulin administration (from subcutaneous [IgSC] to intravenous [IVIg] and vice-versa) was evaluated. Results: The study included 87 patients: 54% female and 46% male, with a mean age of 37.5 years (±15.9). The absent response rate was 0% for all items in the questionnaire. The questionnaire proved to be easy and quick to apply, being self-administered and taking about 10 to 15 minutes to complete. Regarding the global CVID_QoL score, we observed moderate correlations with SF-36 (-0.47 for the physical component and -0.52 for the mental component). A high level of concordance was observed in the test-retest assessment (Global CVID_QoL, ICC=0.90, 95% CI 0.83-0.94) and high internal consistency (Cronbach's α 0.93). Regarding the determinants of HRQoL, we observed a greater impact related to the fear of running out of immunoglobulin treatment, concerns about the future, concerns about worsening health condition and tiredness, and a smaller impact related to problems in relationships with other patients and family members. Only three patients (3.4%) were using IgSC and 84 patients (96.6%) were using IVIg. Of the total number of participants, 39 (44.8%) wanted to change the immunoglobulin administration route, 19 (21.8%) did not want to and 29 (33.3%) were not sure. A worse quality of life was observed among patients who wanted to change the route of administration when compared to patients who did not (p=0.011; difference between means=13.56 [CI 95% 2.44 to 24.67]). Conclusion: The Brazilian version of the CVID_QoL proved to be quick to apply and easy to understand. The questionnaire presented moderate correlation when compared to the SF-36, proving its validity and, in addition, demonstrated good reproducibility and internal consistency. Through the questionnaire, items were identified that impact the HRQoL of patients with CVID and that would not be identified in generic questionnaires, such as the concern about running out of immunoglobulin treatment.