Respiratory care for people with neuromuscular disorders
Although neuromuscular disorders do not affect the lungs themselves, they can affect the muscles involved in breathing and coughing. As respiratory muscles weaken, it can become difficult to maintain adequate minute ventilation (the amount of air that must move in and out of your lungs in order to inhale enough oxygen and exhale enough carbon dioxide). This, in turn, can result in recurrent chest infections, chronic headaches, ever-present fatigue and increased muscle weakness.
While not everyone with a neuromuscular disorder is at risk of developing breath problems— Charcot-Marie-Tooth disease, for instance, mainly affects the lower extremities—someone experiencing breathlessness (a key symptom of breathing impairment) should speak to his or her health-care team to determine the best course of action.
In some cases, a health-care team may prescribe mechanical ventilation for someone who is experiencing breathlessness. Mechanical ventilation assists or replaces your own breathing through mechanical means. Most often, that involves the use of a machine called a ventilator. There are two kinds of ventilation:
- Invasive ventilation delivers air from a ventilator through tubing that is inserted into a tracheostomy, a surgically created hole in the front of the neck and into the windpipe (trachea).
- Non-invasive ventilation delivers air through a mask and does not require a surgical procedure.
The right kind of ventilation depends on the person and his or her medical needs.
For people whose breathing requires a boost, non-invasive ventilation can be an effective treatment option. It works by using relatively small, quiet device called a continuous positive airway pressure (CPAP) machine to deliver a light flow of air through a heated humidifier and then up a hose to a mask.
While CPAP machines work well in the earlier stages of ventilatory problems, there also are non-invasive ventilators for people who require continuous support. Other Positive Airway Pressure or PAP treatment modalities are APAP (Auto Positive Airway Pressure) and BPAP (Bilevel Positive Airway Pressure). These more advanced machines offer additional features, such as internal batteries, different modes for triggering and delivering breaths, superior breath sensing and synchronizing, and multiple alarms.
The various CPAP machines have different settings that trained professionals can adjust to suit your particular situation and treatment needs. They also can accommodate a variety of different mask styles to ensure they fit your face comfortably and do not leak. Again, a trained CPAP professional can help you find the mask that is best for you.
Planning and decision-making
While non-invasive ventilation can be an effective means of treatment for many people, others may be advised to have a tracheostomy because of advanced bulbar palsy (the weakening of muscles used for speaking, chewing and swallowing), severe lung infection or other factors. Before making any decision, it is important to make an informed decision. You should consult with medical professionals who are experienced in treating patients with neuromuscular disorders. Speak with people who have experience with both invasive and non-invasive ventilation. Visit our Respiratory Care page to access more information. These sources—and discussions with your health-care team and family members—will give you a range of perspectives on the subject that will help you to explore the plan of care that is right for you.
VitalAire and Muscular Dystrophy Canada
VitalAire Canada, who provide CPAP products and treatment through their Canada-wide network of clinics and health-care professionals, are partnering with Muscular Dystrophy Canada in May and June of 2016 to donate $25 from each mask sold in Nova Scotia and New Brunswick (in May) and B.C. and Alberta (in June). To learn more about the VitalAire–Muscular Dystrophy Partnerships and how to see other resources related to respiratory care for people with neuromuscular disorders, please visit www.muscle.ca/vitalaire