As ALS causes muscles in the chest to weaken, breathing can become difficult. In fact, respiratory problems have long been a leading cause of death for people with ALS.
Today, though, there are things you can do to improve respiration and manage breathing problems as they occur. These include both special techniques and mechanical aids that can help with breathing and coughing.
Usually, ALS breathing problems first manifest as shortness of breath when performing physical activities. This can often be managed by simply taking breaks between activities to rest and catch your breath and by planning your schedule so that any physically demanding activities are spread out over the course of the day.
Breathing exercises can also help. Fully expand the lungs by taking five to 10 deep breaths, with short rests in between, several times each day.
Breathing can be more difficult when laying flat, leading to breathlessness in bed and trouble sleeping. Elevating the head of the bed can often alleviate this problem. This is easy to do with an adjustable hospital-style bed; with a regular bed, it can be accomplished by stacking pillows under the head, neck, and back or by placing pillows or blankets under the head of the mattress.
Coughing is important because it clears the lungs and airways of fluid, phlegm, food particles, and any other foreign particles that might have been inhaled. By keeping the respiratory system clear, coughing helps you breath easier and reduces the risk of pneumonia.
ALS can make coughing difficult. Weaker chest muscles mean weaker coughs, which aren’t as good at clearing the airways.
“Breath stacking” is one technique that can be used to create a more forceful cough. In breath stacking, the person takes a series of short breaths without exhaling, “stacking” the breaths until the lungs are full. The person holds the breath briefly and then pushes all the air out at once with a cough.
“Huffing” or “squeeze coughing” is another technique ALS patients can use. Huffing involves taking two deep breaths and then forcing all of the air out of your lungs with a series of huffs, with both the mouth and vocal cords open. By repeating this several times, phlegm from deep in your lungs can be moved upward where it is easier to cough out.
Medications or respiratory therapy can also be prescribed to reduce the amount of material that builds up in the lungs, likewise reducing the need for coughing.
There are also mechanical aids that can help ALS patients cough more effectively.
For example, “mechanical hyperinflation” involves using a resuscitator bag and mask to push more air into the lungs to produce a stronger cough. It is very similar to breath stacking, except for the additional equipment required (and the need for a caregiver to help operate the mask).
Automatic mechanical cough assist devices push more air into the lungs and help pull the air back out to simulate a deep, natural cough.
Mechanical ventilators can take on much of the workload when breathing itself becomes difficult.
For many ALS patients, noninvasive positive pressure ventilation, or NIPPV, is the preferred choice when a ventilator becomes necessary. Being “noninvasive” means that NIPPV devices do not require any incisions; they simply deliver air through a nasal or face mask. NIPPV devices are portable and removable, allowing them to be used on an as-needed basis as you go about your regular daily activities.
Tracheostomy ventilation, on the other hand, is invasive. It requires a surgical opening into the windpipe at the base of the throat. Since tracheostomy ventilation bypasses the vocal cords and mouth, a speaking valve may be required to help the patient speak normally.
Tracheostomy ventilation can either be a planned part of ALS patient care or be an emergency procedure used when the patient can no longer breathe on their own. To keep more control over your care and the progression of the disease, it is best to make decisions about the use of ventilators before they are required. The ventilator can be added before it becomes an emergency, or an advance directive can let the patient dictate their own terms for what measures are taken in their care.
A doctor can help you think through such decisions and determine which actions or equipment will work best in managing ALS breathing issues.