Spinal muscular atrophy with respiratory disease (SMARD): an ethical dilemma

Excerpt

The classic riposte of the countryman when asked by a stranger for directions was ‘Well I wouldn't start from here, if I were you’! He would probably have said much the same after reading the contribution by Giannini et al. in Intensive Care Medicine reporting two babies with spinal muscular atrophy with respiratory disease (SMARD) who were diagnosed only when they were irrevocably ventilator dependent [1]. There can be no more difficult question to address in a paediatric intensive care unit than what is in the best interests of a baby with completely normal central neurological function but virtual complete and untreatable peripheral and respiratory muscle paralysis who is totally ventilator dependent. What lessons can be learned from this report? …