It is equally necessary to underline that current information, particularly in the media, concerning now a positive extension of life in DMD but for a limited number of years (from 5 to 10), supports, in parallel, a mistaken opinion as to the utility of an early indication or tracheal ventilation (27, 33, 38–41). Qualitative aspects Very detailed questionnaires

are now available to assess the opinion of patients deprived of functional independence and limited in their daily life. These tests have been used by URRC to ascertain the true view of MD patients Inhibitors,research,lifescience,medical followed in the past (from the most severe – Duchenne Dystrophy – to the more benign – Becker Dystrophy –, the latter being notoriously spared for a long time from a progressive respiratory risk). In general, the patients give, without hesitation, Inhibitors,research,lifescience,medical evidence of the essential benefit derived not only from the medical care provided but also from the prolonged surveillance (42). The present survey is more abridged and focuses on the essential Inhibitors,research,lifescience,medical aspects of life (Table ​(Table33). Table 3 Opinions of treated patients LY2835219 molecular weight affected by X-linked muscular dystrophy. From basic knowledge concerning opinions, which is very rarely recorded, it emerges that the majority of the patients concerned seem to be very

reluctant to the idea of lethal measures affecting their life. Interruption of life prior to its natural term is rejected (53% refusal, 20% uncertain). Also prenatal prevention, in the hypothesis Inhibitors,research,lifescience,medical that they could have the possibility to express their opinion, is likewise rejected (70% refusal, 7% uncertain). Talking of this, it was also asked whether the answer was influenced by religious or ideological

considerations, but the incidence was very slight (17% yes, 40% does not know, 43% not at all). Also the matter of suffering in the terminal stages of life has been discussed, as it was invoked to usually justify euthanasia. This aspect is not among Inhibitors,research,lifescience,medical the major worries of patients (only 23% of those interviewed categorically refuse to be in great pain). The majority consider, however, that Medicine must be able to effectively relieve suffering, without favouring radical measures. In essence, the frequently repeated remark is: “our life is worthwhile living”. Regarding the ability to accept Adenosine their handicap, paradoxically, the patients stating a resolute refusal of damaging the integrity of life are those affected by the most severe form of muscular dystrophy (DMD). On the contrary, the patients less severely affected, surprisingly, more easily accept the idea of interrupting life. Therefore, neither the severity of the handicap nor the specific diagnosis of these severe diseases could represent the only elements of assessment, frequently external to the patient, when his time is drawing near.