Amyotrophic lateral sclerosis (ALS) is a rare neurological disease that is characterized by the loss or deterioration of motor neurons (nerve cells that control voluntary muscle movement).
ALS leads to weakness in several muscles of the body, including muscles that control breathing and swallowing. As the muscles that control breathing become weak, more serious respiratory problems develop, leading to major issues.
As respiratory issues are serious and can be life-threatening, it is important to inform the physician right away. First, however, it is important to recognize the early symptoms of breathing problems. These are the early symptoms:
1. Shortness of breath
- Generally, this the first respiratory symptom that an ALS patient experiences.
- Shortness of breath may be particularly noticeable when engaged in physical activities such as running, exercising, walking, or climbing stairs.
2. Difficulty breathing at night
- This symptom tends to occur as chest muscles relax too much to assist with breathing during the dreaming phase of sleep.
- Difficulty breathing at night can often lead to issues with sleep, fatigue or morning headaches.
- Difficulty breathing at night can also cause the patient to wake up frequently during the night.
3. Problems with swallowing
- Swallowing issues can also interfere with breathing. The main reason for that interferance is a weakness of swallowing muscles that can cause food and saliva to be more easily inhaled into the lungs (which sometimes leads to choking).
- The food or saliva that are introduced into the respiratory system can also carry bacteria, which leads to a higher risk of developing respiratory infection and pneumonia.
- Fever is often a sign of respiratory infection and should be treated with antibiotics.
Diagnosing breathing symptoms
When an ALS patient experiences breathing difficulties, it is vital to inform a physician. In order to determine the severity of the breathing difficulties, the physician will administer a breathing test that assesses the patient’s vital capacity (VC), which is a measure of the amount of air that a patient can blow out. Most physicians will routinely do this test to monitor any changes that may occur in breathing ability.
Another type of breathing test that may be administered at the clinic is the maximum inspiratory pressure test, which measures the strength of breathing muscles. Other tests include measuring the level of oxygen saturation in blood, called pulse oximetry, through the use of a small probe placed on the patient’s finger throughout a night either at home or a sleep study lab.
Treatment for patients with breathing complications
- Early on in the disease when the patient only experiences shortness of breath when exerting themselves, one simple technique is to take breaks in between activities to rest and catch your breath.
- Plan your schedule so that the activities that require high energy are spread out throughout the rest of the day
- Entirely expand your lungs by taking 5-10 deep breaths with short rests in between. Do this several times a day.
- Breathing is more difficult when laying down, and so elevating the head of the bed can help relieve this problem. This can be done by stacking pillows under the head.
- Coughing is an important part of maintaining healthy lungs as it gets rid of fluid, phlegm, food particles, and any other foreign particles in order to clear the lungs
- Coughing can also help patients breathe easier and lessens the risk of developing pneumonia.
- ALS can make coughing difficult due to weakness of chest muscles
- Breath stacking is a commonly used technique to produce a forceful cough. This involves taking a series of short breaths without exhaling and “stacking” the breaths until the lungs are full. Then, you will breathe briefly and push all the air out at once with a cough.
- Huffing, also known as squeeze coughing, is another technique that can be beneficial to ALS patients. This technique involves taking 2 deep breaths and then forcing out all the air that is in the lungs through a series of huffs with the mouth and vocal cords open. By conducting this technique many times, the phlegm that is located deep in the lungs goes upward, which makes it easier to cough out.
- Mechanical aids can help patients with ALS cough more effectively.
- Mechanical hyperinflation, a type of breathing equipment, uses a resuscitator bag and mask to push extra air into the lungs so that patient can have a stronger cough. This is similar to breath stacking. A caregiver may need to help operate the mask.
- Mechanical ventilators take on the workload of breathing so that patients don’t have to exert themselves to be able to breathe, particularly when the muscles become too weak to work effectively
- Noninvasive positive pressure ventilation (NIPPV) is the preferred choice of ventilation for most ALS patients. NIPPV devices, which are portable, help deliver air through a nasal or face mask and do not require any incision to be made. NIPPV uses a ventilator that is either called BiPAP or VPAP, depending on the manufacturer. Research has shown that the use of NIPPV devices improves quality of life and prolongs survival.
- Tracheostomy ventilation is an invasive type of ventilation. This form of ventilation requires a surgical opening of the windpipe at the base of the throat. As the tracheostomy ventilation bypasses the vocal cords and mouth, a speaking valve may be needed to help the patient speak normally. Tracheostomy can either be planned or be used in a time of emergency.