Respiratory problems

ALS and respiration

Respiration is to a certain extend an involuntary action, even though rhythm and depth can be adjusted consciously. When the muscles that assist in respiration are affected, the patient experiences a reduced endurance and tires more easily. But it is important to know that these changes occur only slowly. It's not common for ALS-patients to develop pulmonary damage.

There are a number of methods that can help in dealing with a reduced breathing capacity.

2 Simple methods

- A deep respiration exercise that can be done two times a day. The patient inhales slowly, holds his breath for two seconds and then exhales. This simple exercise will not will not inverse the progress of muscular deterioration but will help maintaining lung capacity and suppleness of the muscles. Some doctors believe that patients doing deep respiration exercises show a decreased tendency to develop pulmonary infections and partial lung collapse. A physiotherapist can assist in using the exercise equipment.

- Stop smoking.

Testing of the pulmonary function

ALS affects respiration gradually. For a doctor, the testing of the pulmonary function is a way to determine to what extend respiration is affected. A series of painless tests will measure the effectiveness of the respiration. Measuring the inhalation and expiratory pressure measures the strength of the muscles assisting in respiration, the one-second value test shows how much oxygen passes through the respiratory tract in one breath, the maximum breathing capacity measures how much oxygen moves quickly in and out of the lungs in a certain amount of time. Airflow tests determine how easily the airflow passes through the respiratory tract.


Symptoms of respiratory impairment


When ALS affects certain motorial nerves, these are rendered incapable of sending impulses from the brain to the muscles they control. Because of this, a smaller amount of muscular cells has to do the work of what in normal conditions would be done by the entire number, resulting in a more rapidly showing of muscular fatigue.

When the respiratory muscles are affected by the disease, their ability to draw oxygen into the lungs decreases. With increased activity the lungs will experience difficulty in providing the body with oxygen. Other metabolic changes occur and the patient feels fatigued.

ALS changes the life of a patient drastically, often resulting in a constant state of stress, that possibly presents itself in the form of fatigue. Periods of rest are crucial in the daily planning of an ALS patient. Efforts have to be dosed within the limits and boundaries of the patient's body.


Most problems are not specifically connected to poor quality sleep or respiratory malfunction but have everything to do with the diagnosis of ALS. Significant signals can be, general fatigue, decreased motivation and concentration, shortness of breath, a general feeling of discomfort, loss of weight (which can also be the result of difficulties in swallowing and nutritional problems), decreased appetite and a decreased resistance when exercising.

And of course there is a huge variety of symptoms related to decreased breathing, both during the day and night time. Some of the less known symptoms are: morning headaches are a possible sign of night time breathing difficulties, being out of breath more frequently, and orthopnoea, which means that people have the feeling they can not lie down anymore.
This usually means that the diaphragm, one of the most important respiratory muscles, fails to function properly. People can be aware of the fact that they wake up during the night feeling like they are suffocating or are out of breath, or their partner can notice respiratory problems during the night. Another possible problem can be the sudden stopping of breathing, which is called apnoea. Even snoring can be an indicate a problem.

Morning fatigue

Some ALS patients wake up still feeling tired, sometimes with a headache and a sense of having had an insufficient night's rest. This can be caused by a weakening of the diaphragm. When in a horizontal position, the organs in the abdomen press against the diaphragm, which in it's turn needs more power to move downwards while breathing, as described above. This way, during the night breathing becomes less effective. To counter this problem the patient has to try to elevate his head and shoulders while sleeping. This can be achieved by placing an extra pillow under the patient's head and shoulders, by using a wedge shaped pillow or by elevating the head end of the bed itself by 10 to 15cm.

Shortness of breath when exercising

This could be the first symptom of a weakening of the respiratory muscles. Shortness of breath can be noticed after ascending a flight of stairs, carrying heavy loads, etc. It can also occur without any significant exercise and decrease with reduced activity. The following positions can help to relax:

- The patient sits at a table with his back straightened while leaning forward and resting the head on a pillow on the table.

- The patient sits on a chair or the side of a bed and bends forward with a straightened back and leans with his elbows and forearms on his upper legs.

- The patient leans against a wall positioning his feet approximately 30cm away from the wall. His lower back leans against the wall while the upper back moves away from the wall.

Insufficient cough

People suffering from ALS have normal coughing reflexes, although their muscles can be weakened and unable to induce a strong cough. However, a cough can be strengthened with a technique commonly known as a splint of the abdomen. The cough may however be reinforced with a technique called splinting of the abdomen. While coughing, the arms have to be pressed firmly against the body in order to achieve an inward and downward motion of the abdomen. It is important to synchronise this pressing and bending over with the moment of exhalation while coughing.

A similar motion can be produced while sitting on a hard surface. The patient crosses his arms over his abdomen and the moment he coughs, he bends forward and presses in an inward and downward motion. If the arms are too weak for this, assistance from another person is required.

Excessive mucus and secretion

With some patients, excessive mucus and secretions can accumulate to the extend that it becomes impossible for the patient to cough them up.

This occurs mostly in the morning when secretions have had enough time during the night to accumulate. A physiotherapist can help the patient learning methods to secrete mucus more easily and move it towards the mouth so it can be disposed of. 

A method of dealing with mucus and accumulated saliva is suction through a motor, collecting bottle or reservoir, tubes or catheter.


Treatment for respiratory problems

Respiratory insufficiency can be caused by disorders in the gas exchange, which is determined by the quality of the lung tissue and/or by defects in the ventilation, or the pump.

When disorders in the gas exchange occur, ALS patients can be preventively treated by means of chest physiotherapy ( breathing exercises) as well as posture advice ( no collapsed down position, proper positioning of the arms, half sitting up or side-lying in bed)

When salivation dietary advice can be giving in addition to chest physiotherapy and postural advice (less food that’s less stimulating to saliva production)

Medically speaking, it’s important to treat pulmonary embolism as well as infections. ( that is if the patient wants this)

The degradation of the pumping function may have three reasons:

- The drive

- Mechanical component

- Metabolism

In case of ‘the drive;, it’s best to reduce the use of respiratory depressant medication, cause in the end phase ALS patients may develop a strong sense of stuffiness with fear with an increase of the oxygen demand as a result. Administering low doses of morphine may provide relief, but this is a measure of the terminal policy

In case of the ‘mechanical component’ chest physiotherapy plays an important, supporting role: breathing exercises ( like sighing with the moving of the joints of the ribs, sternum and vertebrae) and teaching a more effective cough technique or assisted coughing. The half upright bed position is very supportive for the diaphragm operation.

At the ‘metabolic’ cause the diet advices and a feeding tube can keep the nutritional stage adequate for a long time. In the terminal phase one can offer oxygen, but this enhances the risk of increasing the hypercapnia.

Both in the Netherlands and in Flanders, there is the possibility of rescue-breathing; home ventilation. The decision to agree to rescue breathing must be discussed and made way before the need arises. This choice has to be known by all attending physicians. This prevents a panic situation from occurring in moments of infection or choking, in which the patient unwillingly will be placed at a breathing device.

It must be said, that with ALS the greatest reluctance regarding the home ventilation is exercised. But the discrepancy that may arise between patients, as well as motivations for extending live and character of the patient play an important part in the dialogue between patient and physician. The environmental situation, as is there sufficient support in the homecare, may not be decisive in the decision, but can very well mean that the patient can’t stay home. How the disease progresses and the ways of new acquired applications must be considered in the advice from the physician and in the decision of the patient.


Breathing diseases

ALS-patients better avoid people with a cold or the flu, because they are much more vulnerable to have these respiratory infections evolved into pneumonia. Due to swallowing problems food or drinks may end up going into the respiratory tract, this may very well irritate the lungs and cause pneumonia.

In case of fever, coughing up bloody chest mucus or in case of yellowish mucus on has to notify the physician right away, because these symptoms may also lead to pneumonia.

First Breathing aids​

ALS may eventually lead to insufficiency of the respiratory muscles. The modern medical technology can offer portable support devices, but the decision of using them wil have big consequences for the way of life of the patient as well as his or her family.

Ventilators are a kind of controlling devices that take over the breathing. The decision of using a ventilator, should only be granted after consulting the family, the physician and others health professionals. It is important to note that recent technology has produces lightweight portable ventilators, that allow considerable mobility including travel. If at all possible, the decision on a respirator should be taken in a thoughtful way and way before a respiratory emergency occurs.

Tracheotomy or invasive ventilation

There is a distinguish between non-invasive and invasive ventilation. In the first case, the patient has a facial mask that’s connected to the breathing machine by means of air tubes In the second case a tube will be inserted into the upper respiratory tract. Sometimes it’s needed to make a incision, from the outside, into the airways to connect the air tubes.This is called tracheotomy. It’s a way of invasive ventilation. This option only comes up when there are no other possibilities.

Most ALS patients are not in this situation, but there are circumstances where this is an issue. This for sure is a very tough decision to make. Through tracheotomy the airways can be better maintained, which can sometimes be the difference between life and death. But the patient -and his close surroundings – will get more and more depending on care. It’s also possible that because of the surgery the suffering of the patient will be extended Therefore it’s very difficult for both the patient and his family, to possibly decide to stop the treatment.

The risk of wrong decision making is the greatest when there has been no prior dialogue with the physician, the family and caregivers about ventilators, tracheotomy or potential stopping of the treatment. The patients often mistakenly think that physicians will know what‘s the best for them.


What can be done for the ALS patient in case of lung failure?

Most ALS-patients get problems with the respiratory muscles mostly with the diaphragm. There is medication for breathlessness as well as physical therapy and various ways of ventilation ( see previous sections). People with swallow- or bulbar problems may also develop pneumonia or other serious conditions. That is why it is not possible to give a general answer to this question. The best thing is talking about it with the treating physician. He is the only one to know best the disease progression of the patient, and therefore be best placed to provide a more specific and custom answer