Respiratory Issues for Patients
with Amyotrophic Lateral Sclerosis (ALS)
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 will help you obtain equipment that makes
it easier to clear your lungs of secretions to help you
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 bilevel 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 Bilevel 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.
Your health care team may have given you this information as part of your care. If so, please use it and call if you
have any questions. If this information was not given to you as part of your care, please check with your doctor. This
is not medical advice. This is not to be used for diagnosis or treatment of any medical condition. Because each
person’s health needs are different, you should talk with your doctor or others on your health care team when using
this information. If you have an emergency, please call 911. Copyright ©4/2014. University of Wisconsin Hospitals
and Clinics Authority. All rights reserved. Produced by the Department of Nursing. HF6450.