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RESPIRATORY

What are the symptoms of respiratory muscle weakness?
How is respiratory muscle weakness detected or diagnosed?
What are the treatment options for weakness of respiratory muscles?

 

What are the symptoms of respiratory muscle weakness?

Weakness of the respiratory muscles may lead to shortness of breath. Initially, this may only be apparent with physical exertion, but may also be noticed when walking, climbing stairs of even just laying down flat. Respiratory muscle weakness may also affect your breathing at night, which in turn disrupts your sleep, reduces the quality of your sleep and may result in morning headaches, irritability or difficulty concentrating during the day. Weakness of the diaphragm, one of the most important respiratory muscles, may also impair your ability to cough and to clear secretions or phlegm.

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How is respiratory muscle weakness detected or diagnosed?

The most commonly performed test is the vital capacity (VC) and this is performed routinely in most ALS clinics. The negative inspiratory force (NIF) and the sniff nasal inspiratory pressure (SNIP) are other tests commonly used to detect respiratory muscle weakness. Sometimes there is value in performing these tests both in the upright position and when laying down (i.e. in the supine position).

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What are the treatment options for weakness of respiratory muscles?

Non-invasive ventilation (NIV) is the most commonly used treatment for ALS patients who have developed respiratory muscle weakness. It is typically recommended when the vital capacity falls to around 50% of normal, or once symptoms have emerged. Studies have shown that NIV improves quality of life and also prolongs survival. Typically, it is initially used at night, but as the disease progresses and respiratory muscle weakness worsens, it is typically also used during the day.

With increasing respiratory muscle weakness and difficulty coughing, a cough-assist device may become useful. This machine, which is also sometimes called a mechanical insufflator-exsufflator, works by applying a positive pressure to the airway (helping you to take a deep breath of air) and then rapidly shifting to a negative pressure (producing a high expiratory flow) that simulates a natural cough.

Breath stacking is a technique that many ALS patients use to improve their cough ability. It is simple to learn, and can be very effective. Using a resuscitator bag to help push air into your lungs, allowing you to take in a series of breaths without exhaling. That expands your lungs, which by itself is useful. It also gives you a larger volume to exhale forcefully with a cough. Breath stacking can also improve swallowing safety.

If the breathing muscles become very weak and non-invasive ventilation throughout the day and night is insufficient to produce adequate oxygenation, then invasive ventilation via a tracheostomy (i.e. a hole in the breathing pipe) is an option that you should discuss with your neurologist and pulmonologist.

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