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als: canadians shouldn't die while waiting for treatment
8 minute read
for those living with als, time is a precious commodity. getty
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last year in late february, while shopping at a local costco, doug massey ran out of breath rather quickly and suddenly. the retired toronto police officer returned to his car, while his wife stayed behind to finish shopping alone. massey was already living with type 2 diabetes and years earlier, he had a quadruple bypass. he was concerned and went to see his family physician who ordered multiple scans and tests, none of which showed that there was anything medically wrong.
then came the rapid weight loss — at first, he shed 25 pounds, which massey attributed to a healthy and regular diet, but when he lost another 50 pounds, he was admitted in hospital for further evaluation. there, a neurologist noticed a twitching in massey’s leg, and by then, he also had some numbness in one of his toes – he attributed that to diabetes and thought it might be neuropathy, something his brother was already living with. the neurologist ran tests and the next day, massey was diagnosed with als.
“i thought this isn’t good, this is a death sentence,” he says. “but i didn’t feel sorry for myself, i accepted my fate almost immediately. my main concern was end-of-life – i didn’t want to suffer.”
an als drug with a $700 per month price tag
he was referred to an als clinic where he spoke with a specialist about his treatment options — a common als drug was suggested, but massey says that it came with a $700 a month price tag. he didn’t qualify for funding, and he and his wife quickly came to a decision that because the drug could only give him two months of life, massey felt it wasn’t worth it — he would rather the money go to his children.
the standard line of treatment wasn’t the right option for massey, but a few months later, he was given amx0035, an experimental als drug, on compassionate grounds. since last summer, massey has been taking it twice a day — it comes in powder form and is diluted in eight ounces of water. he says that it tastes like “liquid metal,” and he’s not entirely sure whether the drug is doing anything for him — and he isn’t undergoing any tests that may help answer that question.
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amyotrophic lateral sclerosis (als) affects the brain and spinal cord and leads to progressive paralysis. patients may not typically experience pain with als, but some of the first signs include muscle cramps, twitching and weakness in the hands or legs, slurred speech, trouble swallowing, as well as cognitive and behavioural changes, such as inappropriate crying, laughing, or yawning. while it often strikes people between the ages of 40 and 60, rare cases of the disease have been documented in a much younger population. als is also known as the lou gehrig’s disease, named after henry louis gehrig who was a professional baseball player with the new york yankees when the disease ended his career at age 36.
an estimated 3,000 canadians currently live with als, and those diagnosed face respiratory issues and challenges with movement, mobility, and speech. as the disease evolves, daily activities such as swallowing, eating, talking, and showering becomes more difficult and over time, people have more complex care needs. assistive devices and equipment can help those diagnosed with als maintain their independence, but currently, there is no cure for the disease.
dr. christen shoesmith, the chair of the canadian als research network and motor and neuron disease clinical director at london health sciences centre, treats als patients in their 20s to 90s, from all walks of life and ethnicities. she says that up to ten per cent of patients have hereditary als whereas for 90 per cent of people, the cause is still unknown. there is some research that suggests that extreme exercise may be a factor in developing als, but rumblings that a history of concussions that result from contact sports, such as soccer, as well as the pesticides that might be present on a soccer field, may lead to als, are still hypotheses, according to shoesmith. that said, there is an established connection between als and military service — both the canadian and u.s. government officially recognize als as a service-oriented disease and in both countries, war veterans are eligible for disability benefits and their spouses or partners for survivor financial support.
sponsored by:
als society of canada (als canada)
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shoesmith says that canadian patients are treated at one of the 15 specialty als clinics across the country, which include a multidisciplinary team of experts, including a physiotherapist, a dietitian, an occupational therapist, a speech pathologist, a respiratory therapist and sometimes, a palliative care physician — there are no treatments centers available in territories, but those patients are referred to a clinic at a nearby province. the standard line of treatment is riluzole, a drug that’s been around for three decades and another medication that’s available to only some patients, based on individual criteria. according to shoesmith, the goal of als treatment is to help slow down the disease.
the good news is that the standard line of treatment is just the starting point — there are clinical trials that involve experimental treatment options that patients can volunteer to participate in. most of the trials can last up to two years, after which, if it shows positive results, an application is submitted to health canada, but the regulatory body may take anywhere between six months to a year to evaluate a new drug or treatment. shoesmith says that the whole process — from recruiting clinical trial participants to testing the experimental drug or treatment to filing the application with health canada — is a lengthy process that can take at least three and a half years.
dr. shoesmith has a patient who has had als for 28 years
she suggests that clinical trials offer patients a great deal of hope and an opportunity for a better outcome. in her practice, there are patients who have been living with als for 10 years or more, and one patient has been living with it for the past 28 years. the rate at which the disease progresses is unique to each patient and life expectancy varies — for example, physicist stephen hawking lived with als for 50 years. shoesmith says that the factors that allow some patients to live longer aren’t fully understood at this stage, however, she suggests that research and discovery can help more people experience better outcomes.
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“in canada, we’ve never had this many clinical trials,” says shoesmith. “there are at least five different experimental treatments being studied right now, and hopefully one or more will show effect.”
for those living with als, time is a precious commodity. tammy moore, chief executive officer of als canada says that because how drugs are approved and made available in canada is so complex, the status quo of the drug access pathway is failing to respond to the urgent reality of als.
“at als canada, we see firsthand the tremendous impact of an als diagnosis, physically, psychologically and financially,” says moore. “the realities of the disease are harsh — they reinforce the need for better government support and access within the healthcare system.”
at least five experimental als treatments are being studied now
moore says that the organization’s the time is now position paper provides concrete solutions for delivering approved therapies to canadian patients more quickly. it calls for a publicly funded program that would allow all critical treatment options to be approved, reviewed, and funded through a single condensed timeframe across the country. the paper also urges all levels of government to prioritize the needs of als patients and build a plan that ensures equitable, timely and affordable access to proven als therapies. moore says that canadians shouldn’t have to die waiting for treatment.
als canada, which receives no core government funding to support its mission work, continues to rely on fundraising efforts and generous donations to power its movement. when the ice bucket challenge went viral on social media in 2014, it raised awareness about als, and generated $16 million. als canada invested $10 million in research, and directed the rest of the funds — $6 million — towards critical patient support programs and services. according to moore, the commitment of researchers, clinicians, policy makers, caregivers, its staff, and volunteers, as well as families and canadians diagnosed with the disease, is what drives als progress in this country.
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a year into his diagnosis, a lot has changed for massey. he has regular checkups at an als clinic every two months, but he feels tired most of the time and needs lots of rest. he has been working with an occupational therapist once or twice a week, doing gentle stretching exercises.
two months after being diagnosed, he was able to go out and ride in a car, but a few weeks later, massey needed a cane. he now uses a walker and says he can only stand on his own for about a minute or two. the physical aspect of the disease is one thing, but what’s most challenging for massey is when he experiences breathing difficulties, especially when it’s humid outside. he uses a breathing machine, which he finds helpful, but because he is unable to comfortably twist his body without impacting lung capacity, he is no longer able to play with his grandchildren.
massey’s family has raised funds for als research, but he insists that more public awareness is needed to make a meaningful impact in the lives of people who are affected. his family physician had never encountered a patient who was diagnosed with als before, and he says that the real problem with this disease is that it’s still so unknown by many people, including within the medical profession.
“i hope that people would learn to give this disease more thought because there’s a big fight for research dollars and als is rather low on the totem pole,” he says. “i hope that there’s more public awareness about it, so that it can attract more funding and open up new ways of treatment.”
for more information about als and to connect with others, click here to contact als canada.
maja begovic is a toronto-based writer.
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