Steve Waters knew something wasn’t right inside his body. He was exhausted for no reason, and had debilitating diarrhea. His doctor said it was irritable bowel syndrome and that it would come and go throughout this life.
“I just wasn’t satisfied with that,” recalls Steve, who is 46. “I knew something else was wrong.”
After several more tests and visits to specialists, Steve recalls getting a call from his doctor while driving home one rainy night and being told he had two tumours on his liver.
“They were neuroendocrine tumours [NETs], and the hormones secreted from the tumours had already started doing damage to my heart.”
NETs are very rare abnormal growths in the neuroendocrine cells. These cancers are usually slow-growing and can affect any organ, but most commonly occur in the lungs, gastrointestinal tract or pancreas.
In Ontario, about 600 people are diagnosed with NETs each year.
“Endocrine cells are present in most of our organs and secrete different types of hormones, most commonly serotonin,” says Dr. Julie Hallet, a surgical oncologist at Sunnybrook. “That’s why NETs have two unique characteristics: slow cancer growth and hormonal secretions.”
If left undiagnosed, the hormones released by the tumours can wreak havoc on the body. For Steve, that meant his right-side heart valves were damaged and having trouble functioning.
He was referred to the Susan Leslie Clinic for Neuroendocrine Tumours at Sunnybrook’s Odette Cancer Centre. The clinic, established in June 2009, brings together a multidisciplinary team of experts to simultaneously assess and care for patients like Steve. It’s the only clinic of its kind in Canada and sees more than 1,000 new and follow-up patients every year.
After an appointment with medical oncologist and NETs clinic co-founder Dr. Simron Singh, Steve’s first major treatment was having his heart valves replaced by Dr. Gideon Cohen in the Schulich Heart Program. For Steve, it was the first of many surgeries.
But Steve counts himself as one of the lucky ones; his journey to diagnosis and treatment took just a few months. For other people suffering the effects of NETs, it takes much, much longer.
Jeanne Yee was an active 37-year-old massage therapist when she began not feeling well in 2000.
“I lost weight, had heart palpitations, was vomiting, sweating and flushing,” Jeanne, now 53, recalls. “I had consistent and excruciating abdominal pain, which was made worse with eating, so I ate very little.”
For more than a decade, she suffered. She took a leave of absence from work, lost more than 30 pounds from her already slight frame and visited many doctors. She was diagnosed with a variety of ailments, including irritable bowel syndrome, menopause, food allergies and a stomach bug believed to have been picked up on a trip to China.
Finally, an internal specialist in 2014 referred her to Sunnybrook: Jeanne had NETs.
“I had never heard of NETs and I actually thought the specialist was pulling my leg,” Jeanne admits. Soon after her referral, she had major life-saving surgery to remove three primary tumours from her small intestine.
“In medical school, we are taught, ‘When you hear hoofbeats, think horses,’ meaning think straight and consider the obvious diagnosis. But with NETs, it’s more like ‘When you hear hoofbeats, think zebras.’ ”
– Dr. Julie Hallet, surgical oncologist
According to Dr. Hallet, it’s not uncommon for NETs to go undiagnosed for years. “The signs and symptoms are very non-specific. There’s often diarrhea or some facial flushing or wheezing, but those things can happen with so many diseases,” Dr. Hallet says. “Often, patients will seek out health care for a very long time and get labelled with a bunch of other diagnoses that are not NETs, most commonly asthma or irritable bowel syndrome.”
Another reason diagnosis can take so long is that these rare tumours just aren’t on the radar.
“Even as health-care providers, we just don’t think about NETs. So you can’t consider something you don’t know about when seeking to diagnose someone,” Dr. Hallet explains. “In medical school, we are taught, ‘When you hear hoofbeats, think horses,’ meaning think straight and consider the obvious diagnosis. But with NETs, it’s more like ‘When you hear hoofbeats, think zebras.’ ”
Dr. Hallet and the NETs team at Sunnybrook are currently conducting a long-term research study to help better understand this mysterious disease. Even though the tumours are rare, the number of people living with NETs is increasing, as patients with these kinds of tumours can, with careful care management, live for a long time.
“We are focusing on finding enablers to more timely diagnoses,” Dr. Hallet says. “We are examining the data of 6,000 patients with NETs in Ontario and asking: ‘How were they eventually diagnosed? What path led to diagnosis and treatment? Does this vary across regions?’ From there, we’re hopeful we can identify what works and ways we can improve diagnosis and treatment.”
Because this type of tumour is so rare, there’s no standard pathway of care, Dr. Hallet points out. Contributing to that variance in treatment is the fact that each tumour is truly unique; the tumours grow at different rates, release different hormones and may or may not be responsive to chemotherapy. Surgery, interventional radiology and anti-hormone injections are all options.
“Treatment really needs to be personalized for each tumour, so that we can control growth and hormonal secretion,” she says. “But what is the right treatment at which point in a patient’s journey? How do we sequence the treatments? That’s really the challenge. This is a marathon, not a sprint.”
Dr. Hallet says Sunnybrook’s NETs clinic aims to raise awareness of the disease and improve access to care at Sunnybrook and beyond.
“We work with community partners, so patients may come here for initial assessment and treatment recommendations, but then they can undergo that treatment at their home hospital. Eventually, if there are progressions or concerns or questions, we can revisit and reassess.”
“I pray a little longer, meditate a little longer, breathe deeper, smile more and laugh a lot.“
– Jeanne Yee, NETS patient
Steve has attended conferences in the U.S. and made contact with other NETs patients from around the world. He has heard first-hand how challenging it can be to access specialized treatment in some smaller health-care centres.
“I know that having the multidisciplinary care team [at Sunnybrook] really increases my chance of survival. I am so grateful to have expertise in my corner.”
Living with this chronic illness is challenging, Steve says, and he can feel his health worsening. But he also has good days and was able to travel to California this year for a vacation. “Cancer is such a generalized term and it’s hard to explain to people how I am doing. I can explain it in day-to-day functioning – I am able to make my daughter’s lunches and put dinner on the table.”
Jeanne retired from her physically demanding job as a massage therapist. She receives monthly anti-hormone injections. Fatigue is an ongoing challenge, as is the knowledge that the cancer will likely progress.
“But for now, the clinic keeps a close watch on me, so I don’t worry about the future,” she says. “I only care about my quality of life. I savour the time with my family and friends. I still cycle on sunny days, I go for walks and run a little. I pray a little longer, meditate a little longer, breathe deeper, smile more and laugh a lot.”