Loss of the muscles involved in breathing is the gravest problem in advanced
ALS. The respiratory diaphragm, a muscle that lies under the lungs, normally
moves up and down when we breathe. As we breathe in, the diaphragm moves
down, allowing the lungs to fill with air; as we breathe out, it moves
up, letting the lungs expel air. There are also other muscles, located
between the ribs, and in the neck and abdomen, that aid in respiration.
All these muscles are weakened or paralyzed in ALS.
As the respiratory muscles are losing strength, the person with advanced
ALS is also having difficulty coughing and clearing his throat because
of weakened abdominal and throat muscles. Normally, the lungs are constantly
performing housecleaning chores, moving excess mucus and
inhaled particles up toward the mouth, to a spot where they can be coughed
up. In the person with ALS, this material doesnt get coughed up,
but falls back down into the lungs, where it can cause respiratory irritation
Weakened swallowing muscles make aspirating (inhaling) food and liquids
into the lungs more likely, and these inhaled particles add to the irritation
of the respiratory tree. They can also lead to pneumonia or acute choking.
Raising the head of the bed, including during sleep, is a simple but helpful
step. This makes both breathing and swallowing easier. You can help the
person youre caring for rid himself of respiratory irritants by
learning how to do an assisted cough. A doctor, nurse or respiratory therapist
can show you how to do this maneuver, which involves pressing on the patients
abdomen as he coughs. After the assisted cough, youll need to use
mechanical suction to remove secretions from the mouth. This is also something
you can learn from a health professional. Youll need the suction devices.
As the person with advanced ALS breathes less effectively, the exchange
of oxygen and carbon dioxide thats supposed to occur in the lungs
becomes less effective. A person in respiratory distress because of weak
muscles can only speak in short phrases. He doesnt seem to have
enough air to speak in full sentences. He cant sing or shout. Hes
unable to cough effectively or sniff hard. His rate of breathing increases,
and the breathing appears labored. Sometimes you can see muscles working
in the neck or abdomen, trying to compensate for lost function in the
When respiratory distress causes a person to retain excess carbon dioxide,
he may have early morning headaches (after sleep), and may be excessively
sleepy during the day but unable to sleep well at night. The person may
look exhausted and may lose weight rapidly. At this stage, the doctor
will probably begin to discuss respiratory (ventilator) support and ask
the client to begin thinking about what kind of support he wants to have.