Amyotrophic lateral sclerosis (ALS) is a rare neurological disease that affects motor neurons, which are nerve cells that control voluntary muscle movement.
ALS varies significantly from person to person. In some people, symptoms related to breathing can either start early on or they appear much later as the disease progresses. In general, patients develop a breathing problem when their muscles that control breathing (respiratory muscles) become weaker, leading to a variety of symptoms.
1. How does ALS cause lung issues?
The respiratory muscles contract and help us breathe when the brain sends the muscles a signal. In fact, the brain is in constant communication with the muscles of the lungs, making sure it is properly conducting inhalation and exhalation. The movement of air from the lungs is known as ventilation. While we breathe using several muscles, but the majority of work is done by a muscle known as the diaphragm.
Generally, we breathe automatically without thinking about it. The brainstem, spinal cord, motor neurons, and breathing muscles work together to make sure our body is getting oxygen and expelling carbon dioxide. During active breathing, when we consciously initiate a breath, the brain communicates with the lung muscles to move and contract.
As mentioned earlier, patients with ALS lose the functioning of neurons that control voluntary actions. Hence, the nerve cells that signal from the brain to lung muscles deteriorate. As time goes on, patients’ breathing weakens and muscles receive fewer and fewer signals.
Eventually, the muscles involved in breathing have to work much harder to supply oxygen and remove carbon dioxide. Once the respiratory muscles become very weak, patients will not be able to keep up with the demands of the body and this will lead to shortness of breath.
2. Consequence and symptoms of breathing difficulties
- Gasping for air or labored breathing without exercising or physical activity
- Shortness of breath, particularly during any, even light physical activity
- Frequent yawning during the day
- Frequently waking during the night as the weakness in respiratory muscles causes breathing at night to become shallow
- Having a headache or fuzzy headed feeling (morning confusion) when waking up
- Difficulty lying down: as the diaphragm has to push the abdomen away from the chest when breathing lying down, weakness of muscles can make it very uncomfortable to lie down.
3. Techniques to help breather easier
There are several techniques and devices that can help patients breathe easier. Some of them include:
- Incorporate breathing working from yoga, pilates, Qi gong, tai chi, or other forms of movement.
- Deep and focused breathing helps direct the breath to different areas of the lungs, which helps maximize the use of lung capacity.
- “Breath stacking” makes use of a manual ventilation bag adapted with ribbed tubing and a mouthpiece. By fully and slowly breathing in and stacking one breath on top of another, the patient will be able to stretch their lung muscles and open up under-inflated air sacs in the lungs.
- Breath stacking can also be conducted using the Cough Assist machine (Philips Respironics), which helps set the pressure at a level that is comfortable and effective.
- Positive Airway Pressure Breathing (BiPAP) has been shown to improve quality of life and increase life expectancy for people with ALS. BiPAP is an assisted breathing technique that uses a device. When the patient takes a breath, the machine delivers a larger breath to make sure the patient receives adequate lung volumes.
- Mechanical ventilation refers to using assisted breathing or total ventilatory support in which air is delivered to the lungs through a breathing tube. Mechanical ventilation ensures that the lungs expand, oxygen levels are maintained, and carbon dioxide is taken out.
- Positioning is important when trying to breathe adequately. Elevating the head of the bed as well as an addition of extra pillows under the head, neck and chest can help the patient breath better.
- Conserving energy is vital for ALS patients. Patients should schedule the time to rest between activities and plan strenuous activities like bathing, dressing and eating far apart from each other so the body can relax. By conserving energy, the patient won’t be out of breath as much and will have more energy and capacity to conduct the activities that are needed.
- Portable suction units can help eliminate secretions from the mouth
- Patients should begin their breathing techniques early in the disease process as moving to assisted breathing will become an easier transition.
4. Other factors that affect breathing
- Medication: Often prescription drugs for pain, sleeping, anxiety or muscle stiffness can have a negative effect on the breathing centers in the brain, causing even more difficulty breathing. Therefore, it is important to inform the doctor about all the drugs that you are taking (including over-the-counter drugs).
- Respiratory Infections: When a person suffers from a respiratory infection, his passageways of the nose, throat, and lungs that move air can become blocked or narrowed. Furthermore, nasal congestion can block the upper airway. The bronchi (tubes that move air in and out of lungs) can become swollen so that air movement is restricted, making it even more difficult to breathe.
5. Tips for ALS patients starting to experience breathing problem:
- Stay away from people with symptoms of a cold or flu
- Get the pneumonia vaccination and the annual flu vaccine
- Discuss with your doctor the benefits of using medications to control frequent ineffective coughing, breathing techniques and appropriate use of respiratory support and airway clearance equipment.