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 living with ALS.

But there are things you can do to improve respiration and manage breathing problems as they occur. Examples include special techniques and mechanical aids that can help with breathing and coughing.

Breathing Techniques 

ALS breathing problems usually manifest first 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 lying 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 Techniques 

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 breathe 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, you take a series of short breaths without exhaling, “stacking” the breaths until the lungs are full. You hold the breath briefly and then push all the air out at once with a cough.

“Huffing” or “squeeze coughing” is another technique people living with ALS can use. Huffing involves taking two deep breaths and then forcing all 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’s easier to cough out.

Medications or respiratory therapy can also be prescribed to reduce the amount of material that builds up in your lungs – likewise, reducing the need for coughing.

Coughing Equipment 

There are mechanical aids that can help people living with ALS 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’s 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.

Ventilators 

Mechanical ventilators can take on much of the workload when breathing itself becomes difficult.

For many people living with ALS, noninvasive positive pressure ventilation, or NIPPV, is the preferred choice when a ventilator becomes necessary. Being “noninvasive” means that NIPPV devices don’t require any incisions; they simply deliver air through a nasal or face mask.

NIPPV devices are portable and removable, so they can 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 might be required to help you speak normally.

Tracheostomy ventilation can either be a planned part of ALS patient care or an emergency procedure used when you can no longer breathe on your own.

To maintain more control over your care and the progression of the disease, it’s best to make decisions about the use of ventilators before they’re required. The ventilator can be added before it becomes an emergency, or an advance directive can let you dictate your own terms for what measures are taken in your care.

A doctor can help you think through decisions and determine which actions or equipment will work best in managing ALS breathing issues.