A PSYCHOLOGICAL APPROACH TO MEASURING QUA LITY-OF-LIFE IN DUCHENNE DEBOULOGNE MUSCULAR DYSTROPHIC PATIENTS UNDER HOME VENTILATORY ASSISTANCE

28 male Duchenne de Boulogne muscular dystrophic patients (DMD13), age ranging from 15 to 27 years with a mean of 21, were examined in a med ico-psychological investigation of quality of psychic life. Psychologi cal examination included a semi-structured interview, the PM38 (one fo rm of Raven's Progressive Matrices) to assess intelligence, the Rorsch ach test to assess various aspects of personality, and the FACE III qu estionnaire designed to assess family functioning (Olson et al.,1980). The results show that the mean I. Q. for the group is 100 with a norm al distribution. There is no difference with the normative data for Fr ench non-patient adult population. The semi-structured interview indic ates regular feelings of boredom, crying bouts or plain sadness, anxie ty, complaints but satisfactory social relations and projects for the future. The Rorschach was administered, scored and interpreted using t he Comprehensive System (Exner, 1986, 1991) and shows that the subject s are mostly depressed, frequently overwhelmed by emotions, understimu lated, and feel socially isolated and have a negative self-image with low self-esteem. The FACES III indicates that the mothers' attitudes a re located in the extreme range (<<disengaged>>) whereas the fathers' attitudes are located exactly as in normative samples (<<structured>>) .