Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease that affects the nerves that control muscle function. The disease leads to muscle weakness, including the muscles needed for breathing. This can be very serious, with respiratory failure being the primary cause of death in ALS patients. Supporting breathing with ventilation may prolong survival and improve patients’ quality of life.
There are two types of ventilation that can be used in ALS patients: noninvasive ventilation or invasive mechanical ventilation.
Noninvasive ventilation
Noninvasive ventilation (NIV) is breathing support that does not involve surgery. It facilitates breathing with the help of a mask on the face or nose connected via tubes to a small portable ventilator.
NIV provides a survival benefit to people with ALS whose muscles for speaking, swallowing, and chewing (bulbar muscles) are only mildly affected by the disease. It also helps maintain quality of life in patients with mild to moderate bulbar weakness. In people with ALS who have a severe bulbar weakness, neither an improvement in survival nor quality of life could be shown in randomized clinical trials using NIV.
Invasive mechanical ventilation
Invasive mechanical ventilation (IMV) involves the permanent insertion of a plastic tube through an opening in the neck into the windpipe. This is done through a surgical procedure called a tracheostomy.
IMV does not require the use of respiratory muscles because the ventilator machine takes over the respiratory work. It is used when NIV can no longer adequately support breathing. IMV prolongs survival in ALS patients who are not able to breathe independently.
Most ALS patients who use IMV must be connected to the ventilator 24 hours a day. Its use, therefore, requires specially trained individuals who can provide support throughout the day. For example, secretions can block off the breathing tube or airways and need to be suctioned. This can happen at any time of day, and patients using IMV often require care in a nursing home.
Patients with IMV also often lose the ability to eat, drink, or speak. They then require a feeding tube or assistive technology for communication.
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