Muscular dystrophy, incurability, eugenics

Abstract

The medical entity "muscular dystrophy" has been the object of a recent opinion campaign aimed at promoting a law in favour of euthanasia. This disease has become, in the eyes of the public, a media model of a particularly severe and incurable disease. This very widespread statement does not correspond to reality as far as concerns the life of these patients, to the condition that they have benefited from a very useful and fully provided empirical treatment. As already seen, the hope for life has already doubled, without clear limits. The idea of inducing an interruption when at death's door, as long as a systematic prevention prior to birth, does not conform with the motivated opinion of the majority of patients consulted. On the contrary, the dogma of incurability may lead to dramatic individual consequences which should be stressed, from a medical viewpoint, on account of the unacceptable risks of social injustice or eugenics that this would imply.

Figure 2

Overall view of the treated patients, in the fourth decade of life, all living at home, collected during medical control, in the hospital. Apart from a common diagnosis of Duchenne muscular dystrophy and a similar muscle weakness, each of them was able to flourish according to the particular personality, and to benefit from the prolongation of life obtained, in spite of various adverse circumstances.

Figure 1.

Evaluation of the course of the disease. Only the longitudinal follow-up of the instructive clinical elements of DMD guarantees the necessary overall analysis. The reference is the natural progression of muscular weakness (3), characterizing the evolution of each case compared with the reference data (dotted line), unfortunately rarely improved. On the contrary, the functional (1), orthopaedic (2), and respiratory (4) complications are susceptible to being efficiently limited. The data refer to a patient with an exemplary result of early nasal ventilation (JCJ). Since the muscular deficiency is strictly related to the evolution of DMD, the progression of the respiratory insufficiency can be significantly reduced. Therefore, the poor prognosis of the disease is converted.

Figure 3.

At the age of 29 years, P.S. realized an exceptional project of an independent life. At this moment, he accepted the principle of a more protected ventilation via a tracheal ostium, encouraged by the prospects of improvement in the perfection of a device specifically adapted to his needs. Unfortunately, this grounded indication was dismissed later by others.

Figure 4.

At the age of 35 years, JCJ has reached a stage of quasi-stabilization of Duchenne muscular dystrophy. He supports now the concept of a real improvement since adolescence, when his life expectancy was judged as very limited. He was one of the first children treated by early nasal ventilation. Very motivated, he carefully followed this treatment for almost 17 years, before accepting, in due time, a tracheotomy, which became indispensable (left: typical aspects of a severe DMD at 12.5 years; right: physical condition at 34.5 years).