Defined by attitude, not ALS
October 16, 2019
For a loud and clear patient voice, we need look no further than Adam Welburn-Ross. A shining example of the expanded patient role, Adam has explored every corner of specialty medicine.
Innovative drug treatment backed by a patient support program? Check. Clinical trial? Check. Health outcomes reported to a registry? Check. Patient advocacy? Quadruple check. As passionate about life as he is about research and patient care, he wants no part of the victim role.
So how does this super-patient maintain such energy and grace? What can he teach us about the specialty drug patient of the future? We spoke to Adam to find out.
If you expect Adam Welburn-Ross to act like a sick person, you’re in for a disappointment. Adam, 44, doesn’t roll that way. He’s too busy living his life. And writing letters to Members of Parliament. And joking with his doctors. And laughing—a lot.
Not that Adam has his head in the sand. A father of two young-adult children, he knows perfectly well that the disease he lives with, amyotrophic lateral sclerosis (ALS), gradually robs people of the ability to walk, talk, eat, and eventually breathe.17 He knows that ALS leads to changes in thinking and personality in up to half of affected people.18 He knows that the average ALS patient lives just two to five years after diagnosis.2 He knows all this, and yet he has no time for despair. “I focus on living in the moment,” he says, “and at the moment things are great.”
Adam’s optimism has strong roots in his life. During his 10 years as a hockey coach, his unrelentingly positive attitude earned him the nickname “coach sunshine.” Now that the mountain of ALS looms ahead of him, “I have to practise what I’ve been telling the players all along—to stay determined and never quit. It’s been a really important factor in my attitude since the diagnosis.”
The long road
Diagnosed for the first time in 1869, ALS is a neurologic disorder that blocks communication between the brain and the muscles, leading to progressive paralysis. Adam received his own diagnosis in mid-2018, after a year and a half of undergoing “inconclusive” investigations and waiting to see specialists. And waiting. And waiting.
That’s when his self-advocacy instincts kicked in. He asked a relative who worked in health care for help navigating the system, which led to a referral to a clinic that conducted “every test imaginable,” which led to a tentative diagnosis of ALS. He eventually found his way to Dr. John Turnbull, who heads the ALS clinic at the Hamilton Health Sciences Centre and confirmed the diagnosis. “If I were passive, there’s a good chance I still wouldn’t know what disease I have,” Adam says. “And that’s not right. We need to shorten the time to diagnosis and treatment.”
Adam clicked with Dr. Turnbull from the start. “He has a patient focus, rather than a disease focus,” he says, explaining that “he doesn’t just ask me how my leg is doing, he asks me how I’m doing as a person. He also encourages me to bring caregivers to my appointments, and checks in with them as well.”
Under Dr. Turnbull’s supervision, Adam began treatment with Rilutek, an oral medication that slows the progression of ALS symptoms,19 and joined the medication’s patient support program. His treatment journey took a twist in the fall of 2018, when Health Canada approved a new ALS medication called Radicava. With his interest in scientific innovation and appetite for new experiences, one might think Adam would be the first in line for the new drug. However, the drug’s on-off IV dosing gave him pause—a reservation shared by Dr. Turnbull, who wrote a peer-reviewed journal article questioning the overall value of the medication20—and he stepped away from the opportunity.
A champion of patient rights, Adam views fighting for treatment and stepping back from treatment as two sides of the same coin. “We have the right to an opinion about our own treatment,” he notes, reflecting the rising tide of patient agency in specialty medicine. “This includes the right to say no.”
The super-patient
Intent on leaving a legacy to future ALS patients, Adam embodies everything that today’s specialty medicine patient can be and do. On the advocacy front, he collaborates tightly with ALS Canada to prod governments into action—specifically, to make ALS drugs more accessible and affordable, to improve home and community care for ALS patients, and to allocate more funds to ALS research. Some of the items on his wish list are quite simple— “like a manual for ALS patients and caregivers on how to navigate various health care systems and a contact list for related resources.”
He has also enrolled in a clinical trial of levosimendan, an oral medication shown in previous studies to help the muscles contract more easily.21 The trial will evaluate the effect of the medication breathing function21 —an exciting hypothesis, but not the reason Adam signed up. “To tell you the truth, I didn’t care which trial I got into,” he says with a laugh. “I just wanted to help move science and ALS treatment forward.” It helps that the medication hasn’t given him any side effects. “If it made me sick, I probably wouldn’t be quite as enthusiastic.” A further bonus: if the results pan out, he will receive the drug at no cost for the rest of his life.
All told, Adam highly recommends the clinical trial experience. “As soon as this one is over, I plan to join another one,” he says. In the meantime, he has been tracking his symptoms and day-to-day activities and sending the information to a US-based real-world data collection group called the ALS Therapy Development Institute. “It’s an honour to share my health data in the name of progress,” is how he sees it.
In May 2019, when Adam still had full mobility, he participated in the Walk to End ALS, the largest volunteer-led fundraiser for Canadian ALS societies.22 “We’re after the brass ring—an actual cure—rather than improvement of symptoms,” he says. A hundred-and-fifty years after the first diagnosis, ALS remains a terminal disease—and Adam is not OK with that. In his view, “research aimed at a cure aligns more closely with Canadian values,” and he would like to see “the government agenda reflect the fabric of our country.”
A new normal
More than a year after his diagnosis, Adam can no longer walk unaided. After experiencing one too many instances of foot drop, which prevents the foot from recoiling and throws off gait, he began using a walker, and he now gets around in a wheelchair. Accepting that he has entered a new, less independent phase of his life, Adam relies on his partner, Danielle, to help him with day-to-day activities. He can still brush his teeth, but “Danielle makes it easier by putting my toothbrush and glass of water within reach,” he says. If he needs a drink of water or juice, she opens the bottle for him. If he has to leave the house, she loads his wheelchair into the car.
Adam takes none of it for granted. “I sometimes think it’s even harder for caregivers than for patients,” he says. “In a better world, the system would reward caregivers for their Herculean efforts.” For the time being, he has been able to access up to 21 hours of care per week. The rest falls to Danielle, leaving her with a double-shift of full-time work and after-hours caregiving. “She’s not only risen to the challenge, but she’s kept her spirits high,” Adam says, conceding that “my crazy positivity probably rubbed off on her.” His attitude has also inspired his mother, Gail, to follow his lead. “I told her that I’m alive right now, so let’s enjoy our time together, and that’s what we’ve been doing. When you get right down to it, the only thing we ever have is today.”
References
17 Symptoms. ALS Canada. https://www.als.ca/about-als/what-is-als/symptoms/
18 Progression. ALS Canada. https://www.als.ca/about-als/what-is-als/progression/
19 Flavell L. Rilutek (riluzole). ALS News Today. https://alsnewstoday.com/als-treatment/rilutek-riluzole/
20 Turnbull J. Is edaravone harmful? (A placebo is not a control). Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration 2019; 19:1-6.
21 Clinical trials. ALS Canada. https://www.als.ca/research/clinical-trials/
22 Walk to end ALS. ALS Society of Canada 2019. https://walktoendals.ca/