Aim: The aim of this study was to determine the role of parents after extubation of their children affected by Spinal Muscular Atrophy Type 1 (SMA1) in the Pediatric Intensive Care Unit. Currently, children affected by SMA1 are often treated with non-invasive mechanical ventilation and mechanical support of cough. During the first two or three years of life they frequently present severe respiratory failure requiring intubation. Extubation may be at severe risk of failure even because of inadequate care.
Methods: Parents of SMA1 children were offered an early education on the most critical aspects and a training in non-invasive respiratory support after diagnosis. They were asked and allowed to stay as longer as possible with their child after extubation. Quality and quantity of care given by parents during the first 24 hours after extubation were recorded.
Results: All parents participated to the success of the weaning procedure: they gave continuous care and all children could be extubated.
Conclusion: The presence of parents after extubation of SMA1 patients is important for the success of the procedure; otherwise, the presence of a skilled nurse is needed, with a nurse-patient ratio of 1:1.