Four years ago, Christina turner, like most Canadians, had never heard of glioblastoma. In March 2013, her husband Adam was diagnosed with this devastating cancer, which originates in the brain and is considered unbeatable.
The family was packing for a March break vacation, recalls Christina, “and suddenly Adam could barely walk. His whole left side wouldn’t function.”
She called 911 and accompanied him to a local hospital. “Within an hour, the CT scan was done and the ER physician told me he had a lesion on the brain,” she says. “I know now that ‘lesion’ sometimes means ‘tumour.’”
Then Adam had an MRI. “At midnight,” says Christina, “I was told, ‘He’s got glioblastoma and he’s going to die from this. You need to know. Your husband is going to die.’”
A biopsy had confirmed glioblastoma. “It was quite advanced by the time he was diagnosed,” she says. “At the time, they estimated he would have 18 to 24 months.”
Glioblastoma invades the brain quickly and lethally, striking about 1,000 Canadians every year. It’s the most common and most aggressive of malignant tumours that originate in the brain.
Most patients survive less than two years following diagnosis, and the median survival rate is generally 15 months. The average five-year survival rate is 10 per cent.
“The minute they told me, I just burst into tears,” Christina remembers. “I’d lost an aunt and uncle to different types of brain tumours. I knew what the path was going to be.”
At the same time, she couldn’t believe what was happening.
“Adam was so vibrant and healthy. He was the guy who never got sick. He was a picture of health. He ate well, he worked out.”
So far, researchers have found that hereditary factors may be implicated in a very small number of patients with glioblastoma. But for most cases, there is no explanation for the cause.
What is known is that the tumour originates in the brain’s supportive tissue, or glia – a tentacled network with its own rich blood supply, making it a prime environment for a rapidly growing and highly invasive malignant tumour.
After being diagnosed with glioblastoma, Adam was transferred to Sunnybrook and into the care of internationally recognized glioblastoma specialists, neuro-oncologist Dr. James Perry and radiation oncologist Dr. Arjun Sahgal and their teams at the Odette Cancer Centre.
Currently, glioblastoma is treated with some combination of surgery to remove accessible tumours, with radiation therapy and with chemotherapy. But this cancer is a devious, elusive and deadly shape-shifter, making treatment difficult and outcomes poor.
Adam Turner died just nine months after his diagnosis, exactly one month after his 46th birthday, leaving his wife Christina and their three children, and a close-knit circle of family and friends.
Understanding the unstable glioblastoma
Unlike some other cancers where the cells are clones of each other, when one glioblastoma cell becomes two, the offspring cells don’t look like the parent.
“Glioblastoma is highly genetically unstable, more so than most cancers,” Dr. Perry points out. “Tumour cells in glioblastoma bear no resemblance to each other. We call this tumour heterogeneity, and this poses one of the biggest obstacles to successful treatment because the cells have different DNA mutations.”
The offspring of an entire family of glioblastoma tumour cells are all different, with different DNA machinery and different switches.
“Some of these cells respond to treatment, but some don’t, and begin to take over the tumour,” Dr. Perry explains.
That’s what makes treating glioblastoma such a challenge.
As one of North America’s most extensive practices dealing with glioblastoma and other brain cancers, Sunnybrook’s Odette Cancer Centre is, notes Dr. Perry, involved in “research embedded in care.”
The complex, challenging care provided at Sunnybrook for people with glioblastoma combines cutting-edge research and technology, clinical trials and innovative therapies and advances in precision medicine.
The goal, says Dr. Perry, is “to deliver an effective therapy without collateral damage.”
Getting inside the brain
There’s the problem of where glioblastoma is located in the brain.
Researchers have mapped areas of the brain, for example, locations linked to vision and speech. “But with glioblastoma,” says Dr. Sahgal, “everything shifts around to accommodate the tumour and there is only so much you can see with a traditional MRI.”
The neurosurgeon may think she’s cutting into a safe area, but without guided imaging that provides a map of the brain, she may be damaging critical normal tissue.
Doctors need new and better ways to see the tumour in the brain.
New technology – such as Sunnybrook’s new MR-Linac, which gives visual access to the tumour during radiation treatment and provides more advanced monitoring of the tumour as well as normal tissue during those treatments – is improving understanding of the “biomarkers of response” according to Dr. Sahgal.
“In other words, detecting and understanding the unique signals through the MR images during treatment may provide for treatments that are personalized to the patient’s unique tumour biology,” says Dr. Sahgal.
“The MR-Linac has major potential to advance the entire field of radiation response for these tumours and a new field of research,” he adds.
Meanwhile, drug therapies directed at the brain must get past the blood-brain barrier – the membrane that prevents many substances in the blood from passing into the brain and nervous system.
However, Dr. Perry is excited about an innovative new ultrasound technique – transcranial focused ultrasound – that’s making holes in the brain’s fortress.
Ultrasound energy at low intensity can temporarily loosen the barrier to allow drugs through to attack the glioblastoma tumour.
“We’re in early days of a clinical trial,” says Dr. Perry, “but low intensity focused ultrasound works beautifully and it appears to be safe.”
Getting to the bottom of the genes
“There are things that are happening now that are so much safer that the quality of life is better for patients,” says Dr. James Perry, neuro-oncologist at Sunnybrook.
Researchers have been exploring genetic mutations and biomarkers – signs of biological properties or molecules – associated with glioblastoma.
These advances in genomics are also making a difference in the treatment of glioblastoma, providing more information about which tissue and tumours will be more sensitive to radiation therapy.
That has led to the new field of radiogenomics, says Dr. Sahgal, which correlates cancer imaging features and genetic information.
Also, genomic research has made it possible, Dr. Perry continues, “to break down the vast majority of glioblastoma [cases] into four major types and to focus drug development and strategies on each of those four types” – instead of on an infinite number.
“Despite the lack of obvious new medications and therapies, there are things happening now that are so much safer that the quality of life is better for patients,” he adds. “And we’re able to be more aggressive, which is why we’re getting the gains in survival.”
While research continues into the molecular makeup of glioblastoma and how it can be targeted by using the body’s own immune system (immunotherapy) and other new approaches to bring about long-term remission and possibly a cure, the current goal is to prolong both the length and quality of life for patients.
“When we’re talking about glioblastoma,” says Dr. Perry, “it’s incurable. But the survival time is getting longer. Now 10 to 20 per cent of our patients survive for five years. Until the [oral chemotherapy] drug temozolomide came along, almost no one survived that long.
“We even have a patient who’s a 20-year survivor. Why did she get lucky? What is it about her tumour that’s not like the others? They appear identical. It’s all in the molecular profiling – the pathways that control the tumour. [It’s] just as random as that.
“Those of us who have been around a long time recognize that the effects of our treatment pale in comparison to the effects of the genetic lottery.”
As the research and innovation continue at Sunnybrook’s Odette Cancer Centre, Dr. Perry is hopeful about the future for glioblastoma patients.
“I think the two-to-five-year survival will continue to go up.”
Adds Dr. Sahgal, “There are very few places in the world that can do all of this.”
How Canadians support brain cancer awareness
When Torontonian Christina Turner learned in May that The Tragically Hip’s Gord Downie was diagnosed with glioblastoma she knew the disease would soon have a much higher profile.
And she was right.
By the end of summer, when The Tragically Hip had completed its cross-country tour, people were talking about glioblastoma. Almost $300,000 had been donated to the Gord Downie Fund for Brain Cancer Research at Sunnybrook, set up to raise funds for the disease that had ended Christina’s husband’s life. That total now sits at more than $1.2 million.
Across the country, Canadians came together on August 20 – The Hip’s final concert of their summer tour — to raise money and awareness. Here are just a few of their stories.
On that warm August evening, Debra and Will Prescott set up a big screen and a sophisticated sound system on the front porch of their lakeside cottage in the Kawarthas.
They invited friends and neighbours over to watch the broadcast and raised $510 for the fund.
“We’re definitely fans of The Hip,” says Debra, “and we recently had some news of people dealing with a health concern that’s much like Gord’s. So we wanted to do what we could to support the cause.”
“Deb had breast cancer so we’re aware of the struggle when cancer impacts a family,” Will adds. “Breast cancer has a huge awareness, so we see the value in fundraising for good causes and for diseases we need to find a cure for.”
More than 4,500 kilometres away in Whistler, B.C., Chandra Eyton and Chris Hodkinson also put up a big screen in their large backyard, borrowed a sound system and asked friends and neighbours to come and enjoy the concert broadcast, a barbecue and a keg of beer donated by a local brewery. And they let everybody know it was a fundraiser.
“The Hip played here in the ’90s in an old hangout called The Boot, just before it got demolished and turned into condos,” recalls Chandra. “That’s when I got hooked. Chris went to school in Kingston and saw them perform when he was at university.”
The night of Aug. 20 was “gorgeous,” says Chandra. “It was almost too bright to see the screen but it was plenty loud. Everybody came in shorts and T-Shirts. There was a lot of dancing and crying. We raised $805 which, I think, for a little event is pretty good.”
In Winnipeg, Hip fans gathered at The Cube in Old Market Square to watch the broadcast and raised $15,200 for the fund. Organizers Mike Del Buono and Nick Van Seggelen reported that they ran out of beer four times and the beer store was sold out by 10 p.m.
Robin Turack, whose husband Fred died of brain cancer, and her daughter, Sydney, hosted around 1,000 Hip fans packed into 150 cars at the Muskoka Drive-In to watch a live feed of the concert.
“William Alexander, owner of the Drive-In, and his staff donated their services and earnings to the fund,” reports Robin, “while Hip fans and those touched by cancer donated generously [$6,000]. It was a spectacular night for a really important cause and for a man whose music touches the hearts of so many Canadians.”
Aug. 20 was the last night of “Courage for Gord” preconcert parties in cities across Canada during the summer tour. Rob Ferreira, a member of The Hip’s active fan forum, helped organize the events.
Fans were shattered when the news broke in May about Gord’s diagnosis, says Rob. “They wanted to know, ‘How do we give back and organize fundraising for brain cancer?’ We had the tour schedule. Could we organize pre-concert fundraisers? I did three in Toronto. We had silent auctions, raffles.” Even the specially designed menus commemorating the tour were sold. “We raised $45,600,” says Rob.
In September, he and other fans launched a “Courage for Gord” website community. In one day, 1,000 key chains were sold at $25 each, raising another $25,000 for the fund. “you don’t need to be a Tragically Hip fan,” explains Rob. “It’s beyond that. It’s about Gord. He’s touched people in so many different ways.” Two of those people are Toronto sisters Clover and Avalon, whose father is also being treated for brain cancer at Sunnybrook’s Odette Cancer Centre. They brought in a cheque for the Gord Downie Fund for the $275 they raised from their lemonade stand.