Translation and validation of the Life Satisfaction Index for Adolescents scale with neuromuscular disorders: LSI-A Brazil

Abstract

Objective: To validate the Life Satisfaction Index for Adolescents (LSI-A) scale, parent version and patient version, for Duchenne muscular dystrophy (DMD), spinal muscular atrophy (SMA) and limb-girdle muscular dystrophy (LGMD). Methods: The parent version of the instrument was divided into Groups A, B, C and D; and the patient version, divided into B, C and D. For the statistical calculation, the following tests were used: Cronbach's α, ICC, Pearson and the ROC Curve. Results: The parent and patient versions of the instrument are presented, with the following results in the overall score, respectively: Cronbach's α, 0.87 and 0.89; reliability, r 0.98 and 0.97; reproducibility, ICC 0.69 and 0.80; sensitivity, 0.78 and 0.72; specificity, 0.5 and 0.69; and accuracy, 64% and 70.4%. Conclusion: According to the validity and reproducibility values, the LSI-A Brazil parent and patient versions, are clinically useful to assess quality of life in DMD, SMA or LGMD and may also be useful for other neuromuscular disorders.Keywords: validation studies; quality of life; neuromuscular disorders. RESUMO Objetivo: Validar a escala Life Satisfaction Index for Adolescents (LSI-A) versão pais e versão paciente para doenças neuromusculares.Método: O instrumento versão pais foi dividido nos Grupos A,B, C e D; e paciente, em B, C e D. Para cálculo estatístico utilizou-se os testes α de Cronbach, CIC, Pearson e Curva ROC. Resultados: Valor de Cronbach versão pais e paciente no escore geral, 0.87 e 0.89; confiabilidade,0.98 e 0.97;reprodutibilidade,entre 0.59 e 0.69 e, entre 0.58 e 0.80; sensibilidade, 0.78 e 0.72; especificidade, 0.5 e 0.69; e acurácia, 64% e 70.4% respectivamente. Conclusão: Conforme a validade e reprodutibilidade, o LSI-A Brasil versão pais e paciente é útil clinicamente para avaliar a Qualidade de Vida da Distrofia Muscular de Duchenne, Amiotrofia Espinhal Progressiva ou Distrofia Muscular tipo Cinturas e pode ser usado para outras doenças neuromusculares.Palavras-chave: estudos de validação; qualidade de vida; doenças neuromusculares.The importance of quantifying the quality of life of subjects with neuromuscular disorders (NMD) by use of appropriate instruments is a relevant health issue because of the lack of specific questionnaires. This study dealt with the translation and validation of a questionnaire for NMD patients. In this study, three NMDs were considered: two dystrophies and one atrophy: Duchenne muscular dystrophy (DMD), limb-girdle muscular dystrophy (LGMD) and spinal muscular atrophy (SMA), respectively. The dystrophies are characterized by irreversible degeneration of muscular fibers, which leads to muscular weakness and motor disability, originating from mutations in different genes 1 . The atrophy is characterized by degeneration of the spinal cord motor neurons, i.e., secondary muscular atrophyThere is scientific consensus that the health-related quality of life (HRQOL) involves subjective and multidimensional values, either negative or positive, according to individual perception and ex...

Measuring quality of life in Duchenne muscular dystrophy: a systematic review of the content and structural validity of commonly used instruments

“…Hu et al (2013) [67] assessed the relevance, comprehensiveness, and comprehensibility of the PedsQL 3.0 NMM in Chinese children with DMD. Simon et al (2017) [56] assessed comprehensibility of the LSIA in Brazilian children with DMD, and comprehensiveness in professionals. However, both of these studies received ratings of doubtful due to at least some unclear details/suspected problems within the qualitative methods used.…”