Box 1.

Case vignette 1.

Patient features
Age	An 8-week-old boy
Diagnosis	Spinal muscular atrophy-type I (SMA-I), genetically assured
Prognosis	Rapid deterioration of muscular function, death likely in first year of life due to respiratory insufficiency
Medical history	Little movements in last weeks of pregnancy, considerable deterioration of motor function at 6 weeks after birth
Current status	Beginning dysphagia and nutritional disturbance, progressive respiratory insufficiency
	Patient seems attentive and communicates non-verbally with parents
Family	One elder sister (3 years)
	Parents emotionally distressed, think about the impact on their child’s and their own life
Discussed treatment options and outcomes
Invasive and non-invasive ventilation	Significant prolongation of survival, at best into second decade of life (for invasive ventilation) Loss of motor functions, pulmonary infections, severe osteoporosis, scoliosis, impaired language production, normal cognitive development
Palliative care	Alleviation of respiratory distress by palliative interventions, opioids, and benzodiazepines
Question asked	How do you judge the medical indication for mechanical ventilation?