Responsiveness of the domain climbing up and going down stairs of the Functional Evaluation scale for Duchenne Muscular Dystrophy: a one-year follow-up

Abstract

Objective:To determine the responsiveness of the domain climbing up and going down stairs of the Functional Evaluation Scale for Duchenne Muscular Dystrophy (FES-DMD-D3) in a one-year follow-up study.Method:The study included 26 patients with DMD. Effect Size (ES) and Standardized Response Mean (SRM) described the scale’s responsiveness.Results:For climbing up stairs, ES showed that responsiveness was low in the three-month assessments (0.26; 0.35; 0.13; 0.17), low to moderate in the six-month assessments (0.58; 0.48; 0.33), moderate in the nine-month assessments (0.70; 0.68), and high in the 12-month assessment (0.88). SRM showed that responsiveness was low in the three-month assessments (0.29; 0.38; 0.18; and 0.19), low to moderate in the six-month assessments (0.59; 0.51; 0.36), moderate in the nine-month assessments (0.74 and 0.70), and high in the 12-month assessment (0.89). For going down stairs, ES showed that responsiveness was low in the three- and six-month assessments (0.16; 0.25; 0.09; 0.08 and 0.48; 0.35; 0.18, respectively), low to moderate in the nine-month assessments (0.59; 0.44), and moderate in the 12-month assessment (0.71). SRM showed that responsiveness was low in the three- and six-month assessments (0.25; 0.35; 0.12; 0.09 and 0.47; 0.38; 0.21, respectively), low to moderate in the nine-month assessment (0.62; 0.49), and moderate in the 12-month assessment (0.74).Conclusion:Climbing up stairs should be assessed at intervals of nine months or longer, when responsiveness is moderate to high. Going down stairs should be assessed annually because moderate responsiveness was observed in this period.

Reliability, validity and description of timed performance of the Jebsen–Taylor Test in patients with muscular dystrophies

“…8,9 This evaluation strategy has been used in DMD for functional performance of the lower limbs, for example, the six-minute walk test, 10 in the North Star Ambulatory Assessment 11 and in the Functional Evaluation Scale for Duchenne Muscular Dystrophy. 12 The Jebsen---Taylor Test evaluates upper limbs timed performance in seven subtests that represent everyday activities, such as writing, turning cards, picking up objects or beans with a spoon, stacking checkers, and picking up light or heavy cans. The subtests assess the distal and proximal upper limb performance.…”

Evolução do tempo de execução e dos movimentos compensatórios nas atividades de sentar e levantar da cadeira em crianças com distrofia muscular de Duchenne

“…A análise de responsividade dos seus quatro domínios mostrou que a escala é capaz de identificar alterações clínicas a partir de 6 meses, aumentando gradativamente nos intervalos de nove e doze meses (Hukuda, 2014;Escorcio, 2016;Albuquerque et al, 2016).…”

“…Para facilitar a avaliação detalhada sem imputar sobrecarga física para a criança, a FES-DMD preconiza o uso de filmagem. Visando viabilizar a aplicação clínica da escala, um software específico denominado FES-DMD-DATA foi criado para auxiliar na coleta de dados a partir dos filmes e permitir a geração de relatório (Albuquerque et al, 2016).…”

“…Computador, software FES-DMD-DATA, e registros de filmes do banco de imagens (Albuquerque et al, 2016).…”

“…As avaliações do sentar e do levantar na cadeira, de acordo com a FES-DMD-D1, são compostas por três fases por atividade, e estas, são divididas em suas respectivas subfases (Quadro 1 e 2). (Albuquerque et al, 2016).…”