Avaliação da qualidade de vida de crianças e adolescentes com doença falciforme

Abstract

The objective of this work is to translate and transculturally adapt the specific questionnaire PedsQLTM 3.0 Sickle Cell Disease (SCD) Module for the Brazilian Portuguese, in children and adolescents with sickle cell disease (SCD), in two public institutions in Rio de Janeiro; evaluate the psychometric properties of two instruments of quality of life (QOL), generic and specific, in this pediatric population with SCD and their caregivers and to evaluate the association between clinical and sociodemographic factors and QOL scores found in these questionnaires. After MAPI authorization, providing the questionnaires, and the Research Ethics Comitee of Hemorio and HUPE / UERJ authorization, the PedsQL SCD was translated and adjustment, and the final version was applied to the patients and their caregivers, from 2014 to 2016, during outpatient visits, along with the PedsQLTM 4.0 generic core scale, Brazil Criteria for socio-economic assessment and a medical record. The PedsQL DF consists of 43 items, divided into 9 dimensions: pain, impact of pain, pain management, worry I, worry II, emotions, treatment, communication I and communication II. Validity was assessed by Pearson correlation (between the items and scales, between the generic and specific questionnaires and between the responses of patients and caregivers) and factor analysis, and reliability by calculating Cronbach's alpha. Also,using T Tests, the QoL scores were compared for known groups, as to the severity of the disease, sex, age, genotype, social class, total and fetal hemoglobin dosage and use of hydroxyurea, and these data were re-evaluated by linear regression univariate, complete and stepway multivariate analysis.. SPSS 19.0 and STATA 11 were used.The study included 203 patients between 8 and 18 years (mean 14,3 years) and their caregivers; 56% male; 80.5% with SS genotype. Mothers accounted for 80.3% of caregivers and parents 10%. Regarding socioeconomic status, 30% of patients were classified as class A and B and 70% as C, D and E. The average of the 43 items ranged from 49.3 to 88.2, and the scores of the dimensions ranged from 54.1 to 75.4. In general, there was a greater correlation between items and their own scales, except for the scales Emotions and Communication II, which showed correlation. Good internal consistency was demonstrated, with Cronbach's alpha coefficients of the scales ranging from 0.62 to 0.93. The scores of patients with severe desease were significantly lower than the scores of the group of patients with mild SCD (p <0.0001) in all the dimensions of the two QOL questionnaires, both by the reporting of patients and caregivers. Correlations were appropriate and in accordance with the few existing studies on the subject.It was proven the validity and reliability of PedsQL DF and PedsQLTM 4.0 to measure QOL in this pediatric population with SCD. The severity of the SCD has a negative impact on QoL of children and adolescents.