How long is it possible to live with invasive ventilation in ALS?

The prolongation of life through invasive ventilation varies greatly and depends on numerous medical and social factors. Modern ventilation technology allows ventilation for many years, possibly even decades. The limitations of invasive ventilation lie only in exceptional cases in respiratory medicine, but predominantly in the severity of neurological symptoms or psychosocial factors.

Overall, there are four aspects that can limit long-term ventilation: Firstly) ALS can spread to non-motor body functions during long-term ventilation. In some patients, the autonomic nervous system, which is responsible for controlling the heart rhythm, intestinal activity and other organ functions, can be affected. Cardiac arrhythmia, the occurrence of an intestinal obstruction or the failure of other organs are life-limiting circumstances in this situation.

Secondly) ALS can also affect other areas of the central nervous system in addition to the motor centers of the brain. Some ALS patients on long-term ventilation develop dementia, which can progress to a complete loss of cognitive function. In this constellation, the continuation of long-term ventilation does not make medical sense and usually leads to the termination of ventilation therapy and palliative treatment.

Thirdly) In the course of long-term ventilation, a loss of ocular motor function (ophthalmoplegia) and impairment of visual function can occur, which is experienced as so stressful by the majority of affected patients that they refuse to continue ventilation therapy. The involvement of the oculomotor system is the main reason for the voluntary discontinuation of ventilation therapy in long-term ventilated patients with ALS.

Fourthly) Despite ventilation, the motor symptoms of ALS progress. In some long-term ventilated patients, the symptom burden is considered unacceptable and the continuation of life-prolonging ventilation therapy is rejected. In this constellation, termination of invasive ventilation and palliative treatment is possible. Overall, the options for prolonging life and the limiting factors of long-term ventilation are very individual and require a differentiated assessment and consideration of the medical and social factors in a doctor-patient dialog