Ventilator Management for Neuromuscular Disease

Nicholas S. Hill, MD


Semin Respir Crit Care Med. 2002;23(3) 

In This Article

Indications for Invasive Mechanical Ventilation for Neuromuscular Disease

Consensus opinion strongly supports the view that noninvasive ventilation is preferable to invasive mechanical ventilation for the long-term ventilatory support of patients with neuromuscular disease. The reasons are many, including ease of administration, less strain on caregivers, lower cost, greater portability, fewer infections, the virtual elimination of airway complications, and reduced need for hospitalization. However, not all patients are suitable candidates for noninvasive ventilation, and there remains a role for tracheostomy ventilation in the management of neuromuscular disease patients with respiratory failure, albeit much smaller than in the past. Common reasons for using invasive mechanical ventilation include many of the contraindications for noninvasive ventilation as listed in Table 5 .

Clearly, patients who have tried noninvasive ventilation and have been intolerant or have failed to experience improvement in gas exchange or symptoms after reasonable attempts with NPPV or body ventilators must resort to invasive ventilation if they desire prolongation of survival. Also, patients with anatomic abnormalities that preclude fitting of noninvasive ventilators or who are unable to cooperate must be managed invasively. Another common reason for lack of suitability for noninvasive ventilation is severe bulbar involvement. Certain neuromuscular diseases, amyotrophic lateral sclerosis (ALS) in particular, impair swallowing and impair the patient's capability for airway protection. As pointed out in the article by Dr. Benditt, noninvasive ventilation is a temporizing measure for progressive neuromuscular diseases like ALS. When combined with severe expiratory muscle weakness, severe bulbar dysfunction creates a dangerous situation that is incompatible with survival. Placement of a tracheostomy with a diversion laryngectomy to prevent continued aspiration around the tracheostomy tube is the only option that will prolong survival. However, noninvasive ventilation may still prolong survival in patients with bulbar dysfunction,[37] as long as some expiratory muscle strength persists.

Another reason for resorting to invasive ventilation is the need for full-time ventilatory support. In his article, Dr. Bach argues ardently that most such patients can be managed noninvasively, and his center has followed many such patients successfully using noninvasive ventilation.[12] However, at most centers, such patients are still managed invasively. Often, this is because caregivers lack the skill and knowledge to teach patients and their families to use full-time noninvasive ventilation. Also, although noninvasive ventilation receives higher average patient and caregiver ratings than invasive ventilation,[38] some patients still prefer tracheostomy ventilation because of greater security. Sometimes, caregivers are unavailable to manage the patient at home, and chronic ventilator facilities may be unwilling to accept a patient receiving full-time noninvasive ventilation. Also, some investigators have recommended consideration of tracheostomy when patients require more than 16 hours of ventilatory support daily.[39]

If a patient is deemed a poor candidate for noninvasive ventilation, the decision to proceed to invasive mechanical ventilation should be carefully discussed among the patient, family, and caregivers. Ideally, the patient and family should be informed about the options for ventilator care well in advance of the need to decide. They should be given a realistic description of what to expect with invasive mechanical ventilation, the demands on caregivers, the need for frequent suctioning, and possible complications. The possible need for institutionalization should be contemplated if caregivers are unlikely to be able to support the patient at home. The realities of limited financial support by insurers should also be considered. This allows patients to decide in advance whether they desire invasive ventilation, and prepares them for alternatives like hospice. In this way, patient desires can be met and agonizing situations can be averted that arise when patients and their families are not adequately prepared for disease progression.