FATORES ASSOCIADOS À QUALIDADE DE VIDA DE CRIANÇAS E ADOLESCENTES COM DISTROFIA MUSCULAR DE DUCHENNE

Abstract

Duchenne muscular dystrophy is a genetic and recessive neuromuscular disease characterized by muscle weakness, gait loss, and functional dependence up to the second decade of life. There is a need for a knowledge not only of the patient's physical affliction, but also of any psychosocial aspect, with emphasis on the quality of life. To optimize the treatment of patients with Duchenne muscular dystrophy, it is important that the assessment of quality of life takes into account the opinion not only of the parents or guardians, but also of the patients themselves. The dissertation was constructed in scientific article. The first article deals with an integrative review of the literature and aimed to analyze the quality of life of children and adolescents with Duchenne muscular dystrophy under their own perception through the instrument Pediatric Quality of Life InventoryTM. The research was conducted with the Latin American and Caribbean Literature in Health Sciences (LILACS), National Library of Medicine, National Institutes of Health (PubMed), Web of Science, Cumulative Index to Nursing & Allied Health Literature (CINAHL), and American Psychological Association (PsycINFO), with studies published from 2007 to 2017. Nine studies were selected for review. The results showed that children and adolescents with Duchenne muscular dystrophy have a poor quality of life under their own perception, highlighting the physical ability domain as the worst. Some factors such as advanced age, reduced mobility, non-use of corticosteroids, low level of family income and educational level negatively influence the quality of life of boys with Duchenne muscular dystrophy. The second article aimed to relate sociodemographic, clinical and functional capacity to the quality of life of patients with Duchenne muscular dystrophy; and to compare the quality of life of the patients under their own perception with that of the parents. It is a quantitative and cross-sectional study. The sample consisted of 30 patients with Duchenne muscular dystrophy between the ages of four and 18 years and their parents or guardians who were being treated at a Rehabilitation and Rehabilitation Center in Goiânia, Goiás, Brazil. To evaluate the functional capacity was used the Vignus Scale, the Motor Function Measure, the Egen Klassification Scale and the Modified Barthel Index; and for quality of life assessment, the Pediatric Quality of Life InventoryTM version 4.0. The mean age of the patients was 12 (± 3.06) years. The monthly income of their families indicated a salary average of R $ 2054.00 (± 2418.8) and the majority did not wander (86.7%). The monthly income showed a positive correlation only with the physical ability domain of the quality of life. The functional capacity assessment instruments had a positive correlation with the domains physical capacity, emotional aspect and total quality of life score. There was only negative correlation with the domain school activity. All domains of the instrument of quality of life presented significant difference between the perception of the children and their parents, except for the domain school activity. In the parents' version, all domains had an average score worse than that of their children. It can be observed that patients with Duchenne muscular dystrophy with greater impairment of functional capacity have a poorer quality of life, especially regarding physical capacity, since it is a disabling and progressive disease. In addition, parents also ended up underestimating their children's quality of life. The results of this research may be relevant to clinical practice and planning interventions for this population and their families. It is expected that there will be a restructuring and reorganization of the health practices, for a more humanized care, that preserves the quality of life of patients with Duchenne muscular dystrophy. Keywords: Duchenne muscular dystrophy; Quality of life; Child; Adolescent.