Part 2 of 3: Most people diagnosed with ALS die within two to five years. As there is no known treatment to significantly slow the disease’s progression, doctors focus on improving the quality of patients’ lives.
AT THE END OF ALS
Physicians and caregivers who specialize in caring for ALS patients work in a unique medical paradigm. Their patients will not get well; most will die within two to five years of diagnosis. And because the first symptoms of ALS are sometimes confused with those of stroke, months may pass before patients are accurately diagnosed. There is no treatment that significantly slows the disease progression, so the aim is to improve quality of life, through a series of medical interventions, in what are inevitably the last remaining years.
Once ALS is diagnosed, physicians and other caregivers (speech and occupational therapists, social workers and many others) support people with ALS and their families through a series of unimaginable losses: coming to terms with the fact that their lives will be significantly shorter than imagined, adapting to progressively more severe disability and paralysis – and ultimately, making heartrendingly difficult decisions about medical intervention to prolong life.
Wendy Johnston, the medical director of the University of Alberta ALS clinic, says, “For some of these patients, decisions about end-of-life care must be made almost immediately. When a diagnosis is made, we can’t know how the disease will progress, but with the type of ALS that is particularly aggressive, we soon begin to see indications. Most ALS patients, however, will have time to adapt to their disability over a period of years before facing these questions.”
Almost all ALS patients die of respiratory failure, which, for the majority of patients, is the final stage of a relentless, progressive deterioration of muscle waste and paralysis.
Along the way, new non-invasive ventilation support, nutritional support and other kinds of care provided by a health team have been proven to prolong life and increase quality of life for both patients and their caregivers.
As the disease progresses, the demands of caring for ALS patients at home can leave loved ones emotionally, physically and financially bankrupted – but the alternative, entering a hospital or institution, can also be devastating. Often, end-of-life questions weigh on younger patients most heavily.
(The disease typically presents in late mid-life, but can strike any time in adulthood.) “With mechanical respiratory invention, the body is no longer required to breathe for itself, so even as other paralysis occurs, we can significantly prolong life for these patients,” says Dr. Johnston. This more invasive respiratory intervention cannot be provided at home. “People will often say, ‘I want to live. But I don’t want to live in an institution,’” says Dr. Johnston. “I am treating a young man right now, in his thirties. He was a construction worker. His partner cared for him at home as long as she could, but there is no other family, and she couldn’t manage alone any longer. He is now in an institution where the other patients are elderly and most are cognitively impaired. There is no one there to talk to him, and he is having a very difficult time. Younger ALS patients really struggle. They feel the world has no place for them.”
That doesn’t have to be the case, says Dr. Johnston. “In Manitoba, for example, Brummitt-Feasby ALS House provides all of the services required for these patients at end-of-life. That is the model we aspire to.”
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