As with other muscle weaknesses in people with ALS, the point at which breathing muscles begin to weaken is unpredictable. With some, these are the first muscles to weaken; for others they are the last. For most people with ALS, death comes peacefully as a result of weakened breathing muscles.

Normal “easy” breathing involves two main muscle groups. When you breathe in, the diaphragm moves down; at the same time the intercostal muscles between your ribs contract to pull your rib cage up and out. These two actions cause a partial vacuum. Fresh air rushes down the windpipe through the bronchi, the largest air passages in your lungs, and then into the small air sacs which pass fresh oxygen to the blood. This process is called inspiration.

When you breathe out, both diaphragm and intercostal muscles relax, decreasing the size of your chest cavity. Used air that contains waste carbon dioxide is pushed out of your lungs.

If you are breathing heavily, two additional muscle groups come into play. When you take a deep breath in, muscles in the neck that attach to the collarbone and upper ribs assist in breathing. When you force a breath out, your abdominal muscles help to push up the diaphragm.

Fatigue
Fatigue is a common symptom of ALS. It is caused by a number of factors.

As ALS attacks your motor neurons, they become unable to send commands from your brain to the muscle cells that they control. A smaller number of muscle cells must then try to perform jobs usually done by the full number. The result is that your muscles tire before they normally would.

As your respiratory muscles are affected by ALS, they are able to draw less air into your lungs. When activity increases, it becomes more difficult for the lungs to supply enough oxygen to the body. Other metabolic changes take place and you feel fatigued.

ALS produces many changes in your life. Change often results in general stress that can manifest itself as fatigue.

Don't let yourself become fatigued. Rest when you begin to feel tired. Try to keep your exertions within the limits that your body is now imposing. Pay attention to signals like fatigue. It may be necessary for you to change your priorities and forego less important activities. Plan your day to include regular rest periods.

Morning Fatigue
Some people with ALS experience morning fatigue. You may wake up feeling tired, sometimes with a headache, and the feeling that you slept poorly. This may be caused by a weakness in the diaphragm.

When you stand upright, the diaphragm moves down when you breathe in. When you lie down, the organs in your abdomen press against the diaphragm, and more strength is required for the diaphragm to move down during breathing. Therefore, you might breathe less effectively at night if you are lying flat.

To help cope with this problem, you should try to raise your head and shoulders during sleep. You can accomplish this by using two or more pillows under your head and shoulders, by choosing a wedge-shaped pillow, or by raising the head of your bed on blocks 4" to 6" high.

Shortness of Breath with Activity
Shortness of breath may be the first symptom of the weakening of breathing muscles. You may notice shortness of breath after rushing upstairs, carrying a load, etc. Shortness of breath can also occur with no particular exertion; you may not be able to walk or talk for long without becoming winded. You should tell your doctor about these symptoms. When you experience shortness of breath, stop what you are doing. Shortness of breath will disappear when activity is reduced. Relax in one of the following positions if you are able and breathe slowly and deeply.

Sit at a table, lean forward with a straight back and rest your head and shoulders on a pillow on the table.

Sit on a chair or the edge of your bed, lean forward, keep your back straight and rest your elbows and forearms on your thighs.

Lean against a wall with your feet about 12 inches from the wall. Rest your lower back against the wall and lean your upper back away from the wall.

Weak Cough
When you cough, your stomach muscles and intercostal muscles between the ribs contract quickly. This forces air from the lungs and up the airway. Any mucus or food in one of the air passages is forced out as well. People with ALS have normal cough reflexes, but the muscles may be weakened and unable to produce a strong cough. You can strengthen your cough using a technique known as “splinting the abdomen.”

Splinting the Abdomen
First, take a deep breath, then clasp your arms firmly across your lower abdomen and bend over a high-backed chair. Just as you are coughing out, squeeze your arms in tightly so that you push your abdomen in and down. It is important to time the bending and squeezing to coincide with the expelling phase of your cough.

A variation on the self-assisted cough can be performed while sitting on a hard surface. Clasp your arms over your abdomen and as you are about to cough out, bend forward quickly and squeeze the abdomen in and down.

If your arms are too weak to perform this maneuver, ask someone to help by pressing your forearms firmly into your abdomen, bending you forward as you cough. Ask your healthcare professional to assist you and your caregiver to perfect this technique.

Excess Mucus and Secretions
In some people, excess mucus and secretions can build up to the point where they cannot be easily cleared by coughing. This is particularly the case first thing in the morning when secretions have built up during the night. If you experience this problem, ask your physiotherapist to instruct you in methods to loosen secretions and bring them to the mouth to be spit out.

One method of dealing with mucus and saliva build-up is suctioning. Suction equipment acts very much like a dentist's suction tube; it consists of a motor, collection bottle, tubing and catheters. Talk to your nurse or physiotherapist about suction equipment; he or she will be able to tell you and your caregiver where to get the equipment and how to use it properly.