It likely crosses the mind of every individual who receives a diagnosis of ALS, if not immediately then soon thereafter — but it’s the question no one wants to bring up.
What happens at the end?
Despite their reluctance to ask, people want to know: When the end comes is it going to hurt? Will I choke or feel like I’m suffocating? Will it be scary?
The answers to these questions, like the experience itself, can’t be identical for everyone, but overall the course of ALS does tend to follow a fairly predictable path. If you’re more comfortable not knowing about the end of your journey with ALS, perhaps this isn’t the read for you. But if you’re one of those who wants, who needs to know, then read on for some words of wisdom — and reassurance — from a number of ALS experts.
What will happen?
The vast majority of deaths in ALS are the result of respiratory failure.
Other far-less-common causes of death include malnutrition as a result of swallowing problems, pulmonary embolism (a blockage in one of the arteries of the lungs), abnormalities in the heart’s electrical pacing system called cardiac arrhythmias, and pneumonia as the result of aspiration (when food or fluid gets into the lungs).
Mark Bromberg, director of the MDA/ALS Center at the University of Utah in Salt Lake City describes respiratory failure as a process that progresses slowly over months.
Shortness of breath develops, first with activity such as dressing, then when lying flat or sitting quietly.
At this point, people may experience a lack of energy or find they have difficulty sleeping.
Finally, says David Lacomis, director of the MDA/ALS Center at the University of Pittsburgh, “the individual’s carbon dioxide levels build up, and they slip into painless coma.”
A common phrase heard from family members whose loved ones have passed is, “It was very peaceful at the end.” Rather than struggling and gasping for air, their loved ones “went to sleep and just slipped away.”
Will I choke or feel like I can’t breathe?
Choking, and the panic that can be induced by shortness of breath, are often the first and worst fears after a diagnosis of ALS.
“Patients fear choking,” says Lacomis, “but that would be an exceedingly uncommon cause of respiratory arrest.”
And Julie Rowin, director of the new MDA/ALS Center at the University of Illinois in Chicago, notes that although it’s common for people to worry about choking to death, she has never seen it actually happen.
As respiratory failure progresses, some may feel a shortness of breath that’s exacerbated by anxiety or fear, but in most cases medication is administered to provide relief.
Most commonly, explains Merit Cudkowicz, director of the MDA/ALS Center at Massachusetts General Hospital in Boston, morphine (a narcotic painkiller that also acts on the central nervous system as a mood enhancer) is used to prevent any discomfort caused by shortness of breath. Depressants called benzodiazepines, such as Ativan, reduce anxiety.
At MDA clinics, physicians work closely with palliative care teams to coordinate treatment with in-home hospice care providers, assisted living facilities or inpatient hospice settings. Such cooperation helps ensure the person with ALS has the most peaceful and painless experience possible.
Hospice care focuses on providing comfort and quality of life in the final months or days, by supporting the physical, emotional and spiritual needs of the individual with ALS and their family members. (For more information on the ins and outs of hospice, see “Not Gloom and Doom: Demystifying Hospice,” MDA/ALS Newsmagazine, January-February 2010.)
Is it different on a vent?
The decision to use assisted ventilation when breathing becomes difficult may — or may not — determine how the end transpires.
In those who choose to forgo assisted ventilation, by far the most common cause of death is respiratory failure.
Noninvasive ventilation aids in alleviating shortness of breath, Rowin says, but eventually as respiratory muscles weaken, it becomes insufficient to prevent respiratory failure.
Those who use invasive ventilation (delivered through a tracheostomy or opening in the neck) may delay respiratory failure for some time. Occasionally, complications occur with equipment, such as disconnected tubing or mechanical problems, and sometimes the individual may still have a respiratory failure, such as when the lungs become unable to expand as a result of pneumonia or lung collapse.
In many cases, invasive ventilation provides users with more time and a better quality of life. When the end does come, the experience of those on a vent and those who aren’t “should be very similar,” says Bromberg.
What can I do to prepare?
Advance planning can alleviate some of the stress involved in facing end-of-life issues.
Shafeeq Ladha, director of the MDA/ALS Center at St. Joseph’s Hospital and Medical Center in Phoenix, notes the importance of planning and discussion early in the course of the disease to prevent fear, mistakes and discomfort in times of crisis.
Ladha advises people with ALS to create a living will with advance directives and to discuss their wishes with whomever will be making medical decisions for them in the event they’re unable to do it themselves. “Too often,” Ladha says, “physicians and family members are afraid or too uncomfortable to breach this topic, and so when the time for end-of-life decisions comes, no one is prepared.”
Be sure to discuss palliative care options with your physician, and contact your local MDA office for assistance with finding hospice care and other support and resources.
“I think the most important message,” says Rowin, “is that if the patient has an experienced neurologist and hospice care in place, they do not have to be fearful of terminal physical pain and suffering, because they will be well taken care of.”