Lilian A. Y. Fernandes

Elaboração e confiabilidade da escala funcional do subir e do descer escada para Distrofia Muscular de Duchenne

CONTEXTUALIZACAO: Os instrumentos de avaliacao funcional utilizados para pacientes com distrofia muscular de Duchenne (DMD), citados na literatura, sao limitados e escassos, dificultando a tomada de decisao clinica fisioterapeutica. OBJETIVOS: Descrever o processo de criacao de uma escala de avaliacao funcional do subir e do descer escadas, especifica para criancas com diagnostico de DMD, e examinar sua confiabilidade inter e intraexaminadores. METODOS: A construcao da escala seguiu cinco etapas, a saber, elaboracao de um roteiro para observacao dirigida com base na literatura; observacao do subir e do descer em 120 registros filmados de 30 criancas com DMD (5 a 11 anos); elaboracao da escala, considerando o grau crescente de dificuldade de execucao dos movimentos; criacao do manual de preenchimento e submissao da escala e do manual a 10 examinadores, seguida de reajustes para criacao da versao final. A confiabilidade foi testada pelo pesquisador (repetibilidade) e dois examinadores independentes (reprodutibilidade). Utilizou-se o Indice de Correlacao Intra-Classe (ICC) e a Correlacao de Kappa Ponderado. RESULTADOS: A escala elaborada abrange cinco fases para o subir e quatro fases para o descer escadas. Encontrou-se excelente confiabilidade intra/interexaminadores, com valores da Correlacao de Kappa Ponderado > 0,78 em todas as fases e ICCs > 0,89, com p<0,05 entre todos os escores. CONCLUSAO: A escala proposta mostrou excelente repetibilidade e reprodutibilidade, sendo necessaria a continuidade do estudo com os objetivos de avaliar sua acuracia e validade e de construir uma ferramenta digital para otimizar a coleta de dados.

Evaluation Scale Development, Reliability for Sitting and Standing From the Chair for Duchenne Muscular Dystrophy

ABSTRACT The authors aimed to (a) develop a scale to evaluate non–wheelchair-dependent children with Duchenne muscular dystrophy (DMD) while sitting and standing from the chair, (b) test its reliability, and (c) correlate the scores of this scale with the time, the age and the Vignos. Thirty DMD boys performed sit-to-stand and stand-to-sit from the chair 4 times. Scale development was based on a previous movement characterization in healthy children and in DMD children and on suggestions by physical therapists with expertise in DMD. The final version of the scale was submitted to the analysis of reliability. The sitting evaluation consists of phases: flexion, contact of the hip with the chair, extension. The standing evaluation comprehends the phases: flexion; transference; extension. Sitting and standing phases presented an excellent reliability (intraclass correlation coefficient [ICC] ≥ 0.91) and a good reproducibility (ICC ≥ 0.89). The scores generated by sitting on the chair correlated to the time taken to perform the tasks (r = .69) and to the age of the patient (r = .44) and the score of standing from the chair also correlated to the time of performance (r = .66). The sit-to-stand functional evaluation scale DMD is a reliable assessment tool that allows the description and quantification of the functional performance of DMD children.

Development of an Evaluation Scale for Sitting and Standing From the Ground for Children With Duchenne Muscular Dystrophy

ABSTRACT The authors developed an evaluation scale for sit–stand from the ground for children with Duchenne muscular dystrophy (DMD) and tested its reliability. The construction occurred in stages: (a) the characterization of the movement in healthy children, (b) the characterization of the movement in children with DMD, (c) the elaboration of the 1st version of the scale and the manual, (d) the evaluation by experts and readjustments, and (e) the analysis of inter- and intraexaminer reliability and correlation with the Vignos Scale, age, and time for the execution of the activity. The scale comprehended 3 phases for sitting and 5 for the standing. A very good repeatability of the measures of sitting and standing (ICC = 0.89 and 0.84, respectively) and excellent reproducibility (ICC = 0.93 and 0.92, respectively) was demonstrated. The Kappa coefficient for the 8 phases in the interexaminer analysis varied from 0.77 to 1.00 (excellent reliability), and in the intraexaminer analysis varied from 0.80 to 1.00 (excellent reliability). Good correlation was found between the variables on the Vignos Scale (age: r = 0.58; stand: r = 0.56). The scale is a reliability instrument that allows evaluation of the activity of sitting and standing in children with DMD.

Relationship between the climbing up and climbing down stairs domain scores on the FES-DMD, the score on the Vignos Scale, age and timed performance of functional activities in boys with Duchenne muscular dystrophy.

BACKGROUND: Knowing the potential for and limitations of information generated using different evaluation instruments favors the development of more accurate functional diagnoses and therapeutic decision-making. OBJECTIVE: To investigate the relationship between the number of compensatory movements when climbing up and going down stairs, age, functional classification and time taken to perform a tested activity (TA) of going up and down stairs in boys with Duchenne muscular dystrophy (DMD). METHOD: A bank of movies featuring 30 boys with DMD performing functional activities was evaluated. Compensatory movements were assessed using the climbing up and going down stairs domain of the Functional Evaluation Scale for Duchenne Muscular Dystrophy (FES-DMD); age in years; functional classification using the Vignos Scale (VS), and TA using a timer. Statistical analyses were performed using the Spearman correlation test. RESULTS: There is a moderate relationship between the climbing up stairs domain of the FES-DMD and age (r=0.53, p=0.004) and strong relationships with VS (r=0.72, p=0.001) and TA for this task (r=0.83, p<0.001). There were weak relationships between the going down stairs domain of the FES-DMD-going down stairs with age (r=0.40, p=0.032), VS (r=0.65, p=0.002) and TA for this task (r=0.40, p=0.034). CONCLUSION: These findings indicate that the evaluation of compensatory movements used when climbing up stairs can provide more relevant information about the evolution of the disease, although the activity of going down stairs should be investigated, with the aim of enriching guidance and strengthening accident prevention. Data from the FES-DMD, age, VS and TA can be used in a complementary way to formulate functional diagnoses. Longitudinal studies and with broader age groups may supplement this information.

Thoracic cirtometry in children with Duchenne muscular dystrophy: expansion of the method

BACKGROUND Thoracic cirtometry is a simple and accessible technique to evaluate chest mobility during forced breathing. However, it does not allow for the assessment of compensatory movements commonly used by people with chronic diseases, such Duchenne muscular dystrophy (DMD). DMD is a condition characterized by progressive and irreversible degeneration of the musculoskeletal system. OBJECTIVES To expand the method of thoracic cirtometry to allow for the assessment of compensatory movements; to analyze the reliability of the tool; and to describe thoracic mobility of children with DMD during deep breathing. METHOD Sixty boys, 30 with DMD (10.1±0.5 years) and 30 healthy controls (9.5±0.6 years) participated in the study. The expanded thoracic cirtometry was organized in two phases: 1. the body could move freely, allowing the assessment of compensatory movements (free thoracic cirtometry) and 2. the body without compensatory movements, allowing for the direct study of the movements of the chest (guided thoracic cirtometry). This method includes videotaping and systematic observation of body movements using descriptive and numeric data. We investigated reliability of these measures in both groups. RESULTS Measures of axial and the xiphoid thoracic cirtometry (both free and guided) showed excellent reliability. All measures were significantly different between groups. In DMD boys, free thoracic cirtometry presented a greater value of chest expansion when compared with the guided measures, which probably occurred due to compensatory movements. The most commons were movements of the head, shoulder and torso. CONCLUSIONS The expanded thoracic cirtometry method showed excellent reliability and achieved the objectives of determining measures of chest mobility and compensatory movements during deep breath. We suggested its use in the respiratory evaluation of children with DMD.