Quality of life of Brazilian families who have children with Williams syndrome.

This cross-sectional and descriptive study examined the family quality of life (FQoL) among 72 Brazilian families who have children with Williams syndrome, a rare genetic disorder in which most individuals have an intellectual disability, usually mild. Data were collected using sociodemographic and clinical data forms and the Beach Center FQoL Scale. The overall FQoL score was 3.90 ± 0.45, below the limit of four points considered satisfactory. Families felt more satisfied with the family interaction (4.11 ± 0.57), parenting (4.07 ± 0.42), and disability-related support (3.94 ± 0.62) domains, and less satisfied with the family's emotional (3.49 ± 0.73) and physical/material well-being (3.73 ± 0.74) domains. Paternal education, children's cardiopathy and autonomy in activities of daily living explained 24.5% of the variance in the overall FQoL. Measures are necessary to improve the emotional and physical/material well-being of families to reduce the family burden. Monitoring the child's cardiac condition and promoting independence in activities of daily living are also the main procedures.

Qualidade de vida de famílias de filhos com deficiência intelectual moderada / Quality of life in families with children with moderate intellectual disability

RESUMO Objetivo Investigar a qualidade de vida familiar (QVF) entre famílias brasileiras que têm filhos com deficiência intelectual (DI) moderada. Métodos Estudo transversal, realizado com 50 famílias com filhos com DI moderada de São Carlos, São Paulo, Brasil. Os dados foram coletados por meio de formulários, com informações sociodemográficas, os índices de funcionalidade de Barthel e de Lawton & Brody e a Escala de Qualidade de Vida Familiar do Beach Center. Resultados Os domínios mais fortemente correlacionados com a QVF total foram "interação familiar" (r = 0,870; p < 0,001) e "cuidado dos pais com os filhos" (r = 0,845; p < 0,001). Não houve diferenças na distribuição da QVF em relação às variáveis sociodemográficas investigadas. Observou-se correlação moderada (r = 0,326) e significativa (p = 0,021) entre o índice de funcionalidade de Lawton & Brody e a QVF. O modelo de regressão linear ajustado explicou 10,6% da variabilidade encontrada na QVF (p = 0,021) e mostrou que o aumento de uma unidade no valor do índice de Lawton & Brody representou aumento de 0,092 na QVF. Conclusão A QVF das famílias investigadas encontra-se aquém de outras amostras internacionais. Ações clínicas que fortaleçam o diálogo e a coesão familiar e a construção de um plano terapêutico individualizado podem ser meios efetivos de ajuda a essas famílias.

ABSTRACT Objective To investigate the family quality of life (FQoL) among Brazilian families who have children with moderate intellectual disability (ID). Methods A cross-sectional study was carried out with 50 families who have children with moderate DI from São Carlos, São Paulo, Brazil. Data were collected by questionnaires including sociodemographic information, Barthel and Lawton & Brody functional indexes, and the Beach Center Family Quality of Life Scale. Results The domains "family interaction" (r = 0.870; p < 0.001) and "parenting" (r = 0.845; p < 0.001) were more strongly correlated with the total FQoL. There were no differences in the distribution of FQoL according to the sociodemographic variables investigated. A moderate (r = 0.326) and significant (p = 0.021) correlation was observed between the Lawton and Brody functional index and the FQoL. The adjusted linear regression model explained 10.6% of the variability found in the FQoL (p = 0.021) and showed that the increase of one point in the Lawton and Brody index represented an increase of 0.092 in the FQoL. Conclusion The FQoL of the families investigated is below other international samples. Clinical actions that strengthen dialogue and family cohesion, and the construction of an individualized therapeutic plan can be effective ways to help these families.