Subjective Quality of Life in Patients with Progressive Muscular Dystrophy.

Abstract

This study describes and analyzes a group of progressive muscular dystrophy (PMD) patients in terms of demographic characteristics, functional status, and subjective quality of life. In addition, the subjective quality of life responses were compared with those of a control group who answered the same questionnaires. Thirty five patients and 101 healthy young people participated as subjects in this study. Three questionnaire scales were used to evaluate self-esteem (SE), the life satisfaction index (LSI), and the holistic health index (HHI). In this study Cronbach’s α coefficient for each scale was 0.77, 0.60, 0.73 for PMD patients, and 0.84, 0.63, 0.71 for controls. The majority of the PMD patients were at a severe disability level with regard to the functional status of their limbs. Significant differences in SE, LSI, and HHI were not discerned between PMD patients and controls. There were positive and significant correlations among the scores of the three questionnaires in the control group (coefficients ranging from 0.48-0.65, p<0.0001). However, the LSI score of PMD patients correlated negatively and significantly with both the SE score (r=-0.63, p<0.0001) and the HHI score (r=-0.48, p<0.01). The age of the PMD patients correlated negatively and significantly with the SE score (r=-0.40, p<0.05), but positively with the LSI score (r=0.42, p<0.05). There were significant differences in the SE score (p<0.05) and LSI score(p<0.005) which were dependent on the presence of the patient’s mother. Functional abilities did not have a significant impact on the subjective quality of life. These results suggest that the PMD patients had accepted the unavoidable progression of functional disability, and were leading substantive lives according to their abilities. It is postulated that other factors are associated with the subjective quality of life, and accordingly, the need for a multidimentional description of the PMD patient’s subjective quality of life is indispensable. In addition, when considerating therapeutic intervention it is necessary in order to promote the PMD patient’s independence.