Infant spinal muscular atrophy (SMA) is the second most common neuromuscular disorder in children. There are 3 types (from I to III) depending on the age of onset of symptoms and the maximum functional abilities reached. In type I, paralysis begins before the age of six months and most children die before the age of one. The arrival of innovative curative therapies are changing the game, but do not resolve the ethical questions raised by the fate of these children.
In an article published in February 2020, French neuropediatricians report the results of a study carried out as part of a hospital clinical research program (PHRC). The aim was to compare three distinct populations of children with ADM type I: one comprising 39 infants, analyzed in a prospective framework for the period 2012-2016; another, retrospective, of 43 children over the same period; and that of a historical natural history of SMA in 222 children followed between 1989 and 2009. Even if practices and attitudes have changed in recent times, the authors emphasize that the pre-eminence of the palliative approach in these very dependent infants remains a reality. The parents’ investment in the very heavy care of these children is another reality. Supporting the end of life of these children is also a frequent and necessary practice.
