Yetimakman et al also draw attention to our interpretation of stability in older patients who were already ventilated and who were more likely to remain stable. We do acknowledge that the interpretation of these data is difficult, because natural history data reporting possible changes in hours or modalities of ventilation are scanty and often depend on compliance with standards of care, availability of resources, or, in the past, different approaches to proactive respiratory care. Although we agree completely that these data should be interpreted with caution, we have had feedback from the families that many older ventilated patients were not always stable and had to change parameters over time, often in the setting of infections. Infections or other adverse events often triggered an increased need for ventilation that often persisted after the infection subsided. Furthermore, from our personal experience during continuous interviews with parents and families, most parents report that stability represents one of their targets when choosing treatment for their children. We also fully agree on the need for better surrogates for respiratory function in which proactive care and type and approach to ventilatory care should play a minor part. This may prove to be challenging in our children with type 1 SMA. Vital capacity, as suggested, is definitely a good measure, but not applicable to children younger than 6 years of age or in very weak older patients. More longitudinal data over longer periods will hopefully help to define what is the best measure to monitor ventilatory progression in the treated patients who are developing new phenotypes, compared with those classically identified in the different forms of SMA.