Respiratory impairment not only affects the patient but also the patient’s family and social network, particularly when the patient is at home. The underlying disease and the individual clinical picture determine decisions on long-term respiratory care, whether to proceed with comfort care or support quality of life. Patients may depend on intermittent or continuous mechanically ventilation but may also simply carry a tracheal cannula for endotracheal suctioning. In any of these cases patients need out-of-hospital intensive care by professional or family caregivers. Caregivers require wide-ranging structures of support in order to fulfill their tasks and meet the expectation placed on them. This chapter discusses models of support and explores the multifaceted challenges caregivers of home mechanical ventilated patients must face; it highlights the basic skills, challenges and support systems that are essential for the care experience of patients ventilated at home.

Long-term or prolonged mechanical ventilation is a challenge facing many neurological intensive care patients. Weaning these patients from the respirator in Germany and Switzerland increasingly takes place in weaning centers specialized in early neurological-neurosurgical rehabilitation or paraplegiology, combining intensive care and rehabilitation. Prolonged or difficult weaning refers to at least three failed spontaneous breathing trials (SBTs) or mechanical ventilation of more than seven days after the first unsuccessful SBT. Neurological intensive care patients have a high burden of morbidity with disorders of consciousness and poor functional status. Often they depend on kidney replacement therapies and are colonized with multidrug resistant bacteria, In the chapter, we discuss predictors of weaning success, therapeutic interventions in mechanically ventilated neurological patient (such as physiotherapy, occupational therapy, basal stimulation, speech/swallowing therapy, psychological support and respiratory therapy), weaning strategies and home ventilation. Practical recommendations for management are provided.

This chapter discusses respiratory therapy, an essential part in the care of patients with respiratory impairment, for example, on the ICU, and the role of respiratory therapists. Respiratory therapy covers diseases of the airway, lungs and the respiratory muscle pump, including respiratory diagnostics, non-invasive and invasive ventilation, management of secretions, nebulization, oxygen therapy and other drug and non-drug therapies. While respiratory therapists originated in pneumology about 70 years ago, their field of activity extended to a variety of medical disciplines as neurology, pediatrics, critical care medicine and sleep medicine. Respiratory therapists coordinate and monitor patient care and equipment, perform advanced respiratory care, evaluate the treatment and educate teams, patients and family caregivers. They consult in all topics of ventilatory support, including technical aspects of mechanical ventilation, ventilation strategies, airway management and pharmacotherapy, especially nebulized therapy, and they are involved in research.

Major advances over the past decades have transformed the management landscape of neuromuscular disorders. Increased availability of genetic testing, innovative therapies that target specific disease pathways and mechanisms, and a multidisciplinary approach to care including both transitional and palliative care contribute to timely and more appropriate management of conditions that are associated with a severe disease burden and often also a reduction of life expectancy.

There is an increasing number of consensus recommendations/guidelines that are a useful adjunct for establishing a timely and accurate diagnosis, and enable prognostication of disease-related complications, are a guide for multidisciplinary care and treatment, and expedite initiation of disease-modifying interventions. A number of these guidelines have been referred to in various cases, such as myasthenia gravis (MG), myotonic dystrophy type 1 and 2, chronic inflammatory demyelinating neuropathies (CIDP), and Duchenne muscular dystrophy (DMD), to name a few.