Knowing What to Expect Can Help You Prepare
If you’ve been recently diagnosed with amyotrophic lateral sclerosis (ALS) or other motor neuron disease, you’ve probably got some questions and concerns regarding your future. As of yet, there’s no cure for these diseases. But that doesn’t mean you can’t get help. There are lots of resources available to help you. Many of these resources will help keep you as comfortable as possible, and others discussed below could even effect how long you are able to live.
ALS slowly robs people of the strength required to move. Since adequate nutrition is important for maintaining strength, it is easy to imagine how important nutrition might be to someone suffering from ALS. But eating is not always easy, especially in advanced stages of the disease. The muscles that help swallowing may not work as well. The ability to cough if food goes down the wrong tube may be compromised. As a result, it’s important to make sure there are no signs of choking while eating. At some point, patients with ALS will benefit from an evaluation of their ability to swallow, such as a barium swallow study. They may only be able to eat and drink food and liquid of a certain consistency, such as soft foods or thickened liquids. Eventually, a percutaneous endoscopy tube (PEG) will likely be necessary to provide adequate levels of nutrition. While a PEG probably improves survival time by increasing the level of general nutrition, no specific vitamin or other supplement has been proven effective in helping with ALS.
You don’t need to be a health professional to recognize that breathing is important, or that it takes some muscular effort to breathe. As ALS progresses, however, the simple act of breathing can become more complicated, and even require a team of medical experts. In addition to extending the lifespan of people with ALS, good respiratory care can also improve energy, vitality, daytime sleepiness, difficulty concentrating, sleep quality, depression and fatigue. For these reasons, it may be a good idea to have your breathing evaluated early and often, even if you don’t feel you’re having any difficulties.
Respiratory assistance may first begin at night with a noninvasive ventilation method such as CPAP or BiPAP. These support the airway and ensure that even when the body is most at rest, it receives enough oxygen and blows off enough carbon dioxide. As ALS progresses, noninvasive ventilation may be required during the day as well as night. Eventually, more invasive methods such as mechanical ventilation will need to be considered. Another option is diaphragmatic pacing, in which the muscle responsible for expanding the lungs is rhythmically stimulated with electricity to help it contract when motor neurons no longer send it this message. All of these options are best discussed with a team including a neurologist, respiratory therapist, and perhaps a pulmonologist as well.
Protecting the Airway
In addition to the act of expanding the lungs, breathing requires that all the airways be open, rather than plugged with mucous, mouth secretions, or food. When we are healthy we protect our airway by swallowing regularly, coughing, and taking occasional deep breaths, all without really thinking about it. If we are too weak to swallow or cough well, help is needed to protect the airway.
Many techniques are available to help keep the lungs open. Sleeping with the head slightly upright can help prevent secretions from going down the wrong tube at night. Respiratory therapists can teach friends and family how to manually assist a cough to make it more effective. More technologically advanced options include Mechanical insufflation/exsufflation (MIE), which involves a device that slowly inflates the lungs, then rapidly changes the pressure to simulate a cough. High-frequency chest wall oscillation (HFCWO) involves a vibrating vest that, when worn by a patient, helps break up mucous in the lungs so that it can be coughed up more easily. While early reports on effectiveness of HFCWO have been mixed, many patients feel it is helpful.
Another part of protecting the airway is to reduce the amount of secretions produced by the nose and mouth. They can cause drooling, and also put a patient at increased the risk of breathing those secretions into the lungs. A wide variety of medications are available to help control these secretions.
There’s no way around it. Eventually, we all die, and people with ALS die sooner than others. By the time many of the more invasive options above are required, people with ALS will likely suffer from dramatic changes in their ability to communicate. Some will suffer from an ALS-related dementia, and others will simply lose control of their mouth, tongue, and vocal cords. At this point, the medical professionals involved with caring for that patient will need to rely either on earlier statements about what the patient would want regarding their care, or rely on a surrogate decision-maker.
Under what circumstances, if any, would you want life-prolonging treatment with mechanical ventilation, feeding tubes, and more to stop? These are very personal decisions, with legal, ethical, and religious implications. It is critical to plan ahead by arranging a living will or power of attorney, so that you can approach the end of life with the dignity you deserve.
R. G. Miller, MD, FAAN, C. E. Jackson, MD, FAAN, E. J. Kasarskis, MD, PhD, FAAN, J. D. England, MD, FAAN, D. Forshew, RN, W. Johnston, MD, S. Kalra, MD, J. S. Katz, MD, H. Mitsumoto, MD, FAAN, J. Rosenfeld, MD, PhD, FAAN, C. Shoesmith, MD, BSc, M. J. Strong, MD and S. C. Woolley, PhD. Practice Parameter update: The care of the patient with amyotrophic lateral sclerosis: Drug, nutritional, and respiratory therapies (an evidence-based review). Report of the Quality Standards Subcommittee of the American Academy of Neurology, Neurology, October 13, 2009 vol. 73 no. 15 1218-1226
R. G. Miller, MD, FAAN, C. E. Jackson, MD, FAAN, E. J. Kasarskis, MD, PhD, FAAN, J. D. England, MD, FAAN, D. Forshew, RN, W. Johnston, MD, S. Kalra, MD, J. S. Katz, MD, H. Mitsumoto, MD, FAAN, J. Rosenfeld, MD, PhD, FAAN, C. Shoesmith, MD, BSc, M. J. Strong, MD and S. C. Woolley, PhD. Practice Parameter update: The care of the patient with amyotrophic lateral sclerosis: Multidisciplinary care, symptom management, and cognitive/behavioral impairment (an evidence-based review). Report of the Quality Standards Subcommittee of the American Academy of Neurology, Neurology October 13, 2009 vol. 73 no. 15 1227-1233
Orla Hardiman, Management of respiratory symptoms in ALS. Journal of Neurology, Volume 258, Number 3, 359-365,
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