The diaphragm is an important muscle for maintaining respiration because it controls the ability to breathe. As ALS progresses, the diaphragm weakens causing respiratory inefficiency, preventing oxygen from entering the lungs. On a more positive note, there are exercises and specialized devices that can greatly improve respiration and quality of life.
Yoga, meditation and breathing exercises support the diaphragm and should be included as a daily regimen for breath control and awareness. Using extra pillows under the head, neck and back when sleeping or lying down can be helpful. It's important to rest between daily activities to reduce shortness of breath. Try to take 5 to 10 deep breaths to fully expand the lungs several times a day, holding your breath, then exhaling through pursed lips. If you enjoy singing or playing a wind instrument, all the better.
Respiratory therapists at clinics can perform a variety of tests to measure respiratory efficiency. A spirometer test during an overnight sleep study can provide effective solutions. If additional support is needed, the Non Invasive Positive Pressure Ventilation (NIPPV), also known as NIP-V in the UK, pushes air into the nose and/or mouth through a mask into the lungs. NIPPV doesn’t have to be used all the time, but it is generally helpful for sleep and can be extended to daytime use. The Continuous Positive Airway Pressure (CPAP), Bilevel Positive Airway Pressure (BiPap) and Ventilation Positive Airway Pressure (VPAP) are options for sleep apnea and can also be helpful for ALS as well. Several mask options are available and it’s important to select one that is most comfortable as well as effective. Bronchodilators, such as Albuterol and Proventil, are sometimes prescribed for people with ALS because they open the airway and can provide relief.
The Diaphragm Pacing System rhythmically stimulates, or paces, the diaphragm through surgically implanted electrodes. Electrodes send signals to the muscle, causing it to contract and assist breathing efforts. The NeuRx Diaphragm Pacing System (DPS) by Synapse Biomedical, can be used in conjunction with noninvasive ventilation or by itself. Though DPS doesn’t slow or stop general ALS progression, it delays the need for invasive ventilation and also improves both sleep and quality of life.
“Invasive” techniques require a surgical hole in the windpipe known as a tracheostomy. Air is delivered through a breathing hose into the trachea on a timed cycle. Many ventilators can be adjusted to either work with natural breathing patterns or to take over the respiration process entirely.
Some medications prescribed for insomnia, depression and anxiety may also decrease respiration and should be carefully reviewed by a medical professional to avoid invasive side effects. An accumulation of phlegm can also affect respiration and even lead to pneumonia. The Vac-Assist Suction Aspirator operates like a vacuum with a wand attached that removes excessive phlegm and mucus from the mouth and throat.
Becoming informed about all of the opportunities available to protect respiration is important for both the patient and the caregiver as well as being aware of potential negative side effects.