Respiratory Issues for Patients with Amyotrophic Lateral Sclerosis (ALS) HF#6450

Breathing problems are some of the most severe symptoms of ALS.  Breathing problems occur because the muscles involved in breathing slowly weaken.  The muscles that help you breathe and cough the most are the diaphragm, the intercostals, and the abdominal muscles. 

 

The picture shows the muscles used for breathing.

 

The diaphragm is a muscle that lies under the lungs.  It moves down and contracts during breathing in (inhalation).  It relaxes and moves up during breathing out (exhalation). 

 

The intercostal muscles are muscles that are found between and on top of the ribs.  Some of these muscles contract when we breathe in, while others contract during forced breathing out.  The abdominal muscles also help with forced breathing out and coughing.  The muscles contract when we cough to help push air out of our lungs.  This helps to move mucus up and out of our lungs. 

 

As ALS weakens the respiratory muscles, the act of breathing becomes harder.  The ability to cough and swallow well is decreased.  The respiratory therapist (RT) can assist you by teaching you breathing techniques.  RTs also help you obtain equipment that will help you clear your lungs of secretions and breathe more easily.

 

As your ALS progresses, you may have problems swallowing food.  Your swallow specialist and speech pathologist can teach you techniques that help you get proper nutrition and lessen choking.  Sadly, choking can still occur.  It is a good idea to have your family members and friends trained on the proper way to do the Heimlich Maneuver in case you do choke on food. 

 

 

The Heimlich Maneuver for Choking

 

A choking victim can't speak or breathe and needs your help right away.  Follow these steps to help a choking victim:

 

1. From behind, wrap your arms around the victim's waist. 
2. Make a fist and place the thumb side of your fist against the victim's upper belly, below the ribcage and above the navel. 
3. Grasp your fist with your other hand and press into their upper belly with a quick upward thrust.  Do not squeeze the ribcage.  Confine the force of the thrust to your hands. 
4. Repeat until the object is coughed or spit out.

 

Do not slap the victim's back.  This could make matters worse.

 

 

 

 

 

 

 

Doing the Heimlich Maneuver on Yourself

 

When you choke and you can't speak or breathe, you need help right away.  Follow these steps to save yourself from choking.

 

1. Make a fist and place the thumb side of your fist against your upper belly, below the ribcage and above the navel. 
2. Grasp your fist with your other hand and press into your upper belly with a quick upward thrust. 
3. Repeat until object is coughed or spit out. 
4. Or, you can lean over a fixed horizontal object (table edge, chair, railing) and press your upper belly against the edge to produce a quick upward thrust.  
5. Repeat until object is coughed or spit out.
   

 

Cough Assist

 

A cough assist is a maneuver that a family member or friend can perform on you when you cannot cough up phlegm on your own.  They perform this by putting their hands on your abdomen.  Then they push against the abdominal muscles to help you cough.  Below are the steps for doing a cough assist.

 

1. Have the caregiver place their hands on your abdomen over the belly button as shown in the picture below.
2. Take two slow deep breaths to expand your lungs.
3. Once you have completed the first two breaths, perform another.  When you exhale, have the caregiver push the heels of their hands into the abdomen to help the diaphragm move up and expel air and phlegm. 
4. Repeat this procedure until your secretions are cleared.

 

 

Cough Assist Machine

 

The Cough Assist Machine helps to clear secretions from the lungs by helping you with your breathing.  When you breathe in (inspiration), the machine gives you air (positive pressure) to help expand your lungs.  When you blow out (expiration), the machine creates a sucking force (negative pressure) that pulls the air out of your lungs.  This rapid change in pressure during the two phases of breathing helps make your cough stronger and more effective.

 

Portable Suction Machine

 

Many times, people with ALS also have weakness in their tongue muscles.  This weakness makes it impossible to move phlegm to the front of the mouth so that it can be spit out.  A portable suction machine can be used at home.  It can help to help suck up saliva or secretions that stay in the back of the throat.

 

 

 

Ventilation

 

As your ALS becomes worse, your breathing can become less effective.  The exchange of oxygen and carbon dioxide (CO2) that normally occurs in the lungs changes.  The changes do not allow the lungs to expel CO2 as they did in the past.  Symptoms of retained CO2 are waking up with morning headaches and feeling really sleepy during the day.  At this stage, your doctor will often suggest the use of some form of help with ventilation.  This ventilation may be noninvasive or invasive.  Non-invasive ventilation means that nothing has to be placed in your body to help you with breathing.

 

Non-invasive ventilation can be done with a respirator also known as a ventilator (vent) or a bi-level assistance device.  Bi-level assistance devices are most often used.  These devices use a small mask that is placed over your nose.  The mask is held in place by a head strap.  The Bi-level machine is attached to the mask through a hose.  Air is pushed through your nose and into your lungs.  One level of air is added to your lungs during the inhale and another is delivered during the exhale.  These pressures of air help you take deeper breaths and get more oxygen into your blood.  If you don’t breathe deeply enough at night, the bi-level device will give you extra breaths.  A mouthpiece can also be added to the Bi-level device during the day so that people can take a deep breath every once in a while to make their shortness of breath better.

 

Weakness of the breathing muscles also affects how people speak.  It becomes harder to speak in more than short phrases. 

 

 

 

A respirator also known as a ventilator (vent) can be used for invasive or non-invasive ventilation.  The same bi-level masks and mouthpieces can be adapted and attached to a respirator to help with breathing.    A vent provides air to the lungs at timed intervals.  It takes over breathing whereas the bi-level machine only helps with breathing and does not take over the breathing.

 

The most permanent type of ventilation is done by attaching a vent to a tracheostomy tube (trach).  This tube must be placed in surgery.  The tube is put through a hole made in the trachea. 

 

Tubing from the vent is attached to the trach tube.  It gives the patient a breath every few seconds.  The amount of breaths given to a person is set to meet their unique needs.  Not everyone with ALS will need or choose to have a trach tube.  This choice should not be made lightly.  The trach tube passes through the vocal cords stopping speech at first.  Other  tools to communicate are often used.  Once the trach tube has healed, devices can sometimes be added to the tube to allow people to talk again.  For people that choose, there are small ventilators that are portable and quiet. 

 

 

 

 

 

 

The information provided should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed physician should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Any duplication or distribution of the information contained herein is strictly prohibited.

Last Updated: 12/23/2011

Copyright © 12/22/2011 University of Wisconsin Hospitals and Clinics Authority. All rights reserved. Produced by the Department of Nursing. HF#6450

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