Queensland is the world melanoma capital
NEWLYWEDS Nicole and Craig Johnson are in a doctor's office anxiously awaiting the results of a scan. Not yet married a year, the couple from Gladstone in Central Queensland, has been trying for a baby but this isn't the type of scan they've been hoping to see. Nicole, still in her 30s, has recently been told she has metastatic malignant melanoma - cancer that has spread beyond the skin - and she's had a full-body scan to find out where it has infiltrated. Tumours are in her brain, on her lungs, a hip, a shoulder, an arm and a leg. She has "bits and pieces" throughout her body.
If she'd been diagnosed a few years earlier, chances are Craig would have become a widower before their second wedding anniversary. For decades, the average prognosis for such advanced melanoma was nine months. However, a new era is emerging in melanoma medicine. Nicole's cancer specialist, Brisbane's Dr Paul Mainwaring, doesn't mention death sentences at that first meeting and hasn't since. He gives her treatment options - and hope.
Craig and Nicole, now both 41, first met in their 20s. Mutual friends had tried to play Cupid to no avail. Both had been single for years when they reconnected "out of the blue" in 2012. "He Facebooked me - as you do," she says with a laugh, joyous at telling their love story. "He wanted to know if I wanted to catch up." Craig was working on a project overseas at the time. They got to know each other again chatting on the phone. This time, romance developed quickly. He returned to Gladstone and, by February 2013, they were living together. Craig proposed in December of that year and they were married in August 2014. They had dreams of starting a family.
A few months after their wedding, Nicole noticed her arm and shoulder were sore after a gym workout. She went fo a massage and, within days, felt a lump about the size of a marble between her shoulder and elbow on her right arm. It disappeared, but a few weeks later re-emerged much bigger than before. Another lump had also popped up on her abdomen. "I just put it down to getting older, lumps and bumps," she says. Not one to catastrophise, she did not suspect advanced skin cancer, although she'd had a mole cut out of her neck a couple of years earlier and it had been diagnosed as a melanoma. With her fair complexion, Nicole had always been vigilant about having regular skin checks and was not one to sunbake. Doctors thought they had caught the cancer early.
But when her general practitioner sent her for a scan to check out the growing masses inside her arm and abdomen, Nicole was given reason to start worrying. "I was told it didn't look good," she says. She had surgery in Gladstone in early 2015 to remove the growth inside her abdomen and pathology tests revealed it was melanoma. One of more than 13,000 Australians diagnosed with the skin cancer annually, Nicole was referred to Mainwaring, a respected Brisbane cancer specialist. As she sat in his office days later to receive the results of a more detailed positron emission tomography scan - a molecular map of where the cancer was invading her body - the growth on her arm had expanded to the size of a tennis ball. "It was massive," she says.
With the cancer already in her brain, the diagnosis was dire. Nicole could not hold back her tears, but Mainwaring was optimistic. In the past few years, new drugs have revolutionised the way melanoma is treated. When he first started as a cancer specialist almost 30 years ago, all Mainwaring could offer metastatic melanoma patients was toxic chemotherapy, which usually didn't work. In the past five years, two new types of drugs have arrived on the scene - medications targeting genetic drivers within the cancer and immunotherapy.
The gene-targeted drugs only have a chance of working in the 40 per cent of melanoma patients, such as Nicole, whose tumours test positive to a mutation in the BRAF gene, which has been implicated in many types of cancer, promoting its growth and spread. Of the eligible patients, about 6 per cent receive no benefit at all and by 11 months of treatment, half have stopped responding. Only 13 per cent are still taking the drugs at five years.
Although they were effective for a few months in Nicole, and the lump in her arm disappeared, Mainwaring switched her to a new immunotherapy medication, Keytruda, when her cancer started to grow again in mid-2015. The drug, and others like it, is designed to dismantle the protective wall surrounding the cancer, allowing the patient's immune system to start attacking it.
About a week after Nicole's first dose of Keytruda, given intravenously, she lost the use of her right leg, ending up in a wheelchair. More scans showed her brain tumour had doubled in size, growing from 5mm to 1cm in diameter. She was admitted to hospital in Brisbane for 12 sessions of radiotherapy, hoping it would reduce the tumour enough to get her walking again. At that stage, it was far too early to know whether the immunotherapy was working. She had an agonising wait of another two months before being told the Keytruda was shrinking her cancer. "Since then, everything's been great," she says. She regained movement in her leg, but had to give up her driver's licence for 18 months.
With no evidence of the cancer still being in her brain, Nicole was given the go-ahead to start driving again just before Christmas last year. She continues to have infusions of immunotherapy every three weeks.
Almost three years since her devastating diagnosis, she's working full-time - seven days on, seven days off - as a tug scheduler in Gladstone Harbour, and considers herself lucky, despite having cancer. Lucky to be diagnosed in an era when drugs are working for some advanced melanoma patients; lucky she has no side-effects; lucky to have well-trained doctors and to live in a country, such as Australia, with good healthcare. Mainwaring has told her she would have been dead by now without immunotherapy drugs and the persistent scientists who developed them.
"There were lots of immunotherapy trials that were negative - fail after fail after fail," he says. "I became incredibly immunotherapy-sceptical. It was really upsetting. But these new agents have blown us all away."
Although Nicole still has two "quite small" tumours on her lungs, the cancer remains stable. But it's far too soon to use the C-word - cure. Nobody knows if the Keytruda will keep working. Her future remains uncertain.
"It sucks but there's no point dwelling on what-ifs," Nicole says. "It doesn't solve anything. Life keeps going. Going off the rails is not going to make anything better."
Princess Alexandra Hospital oncologist Associate Professor Victoria Atkinson, who has not been involved in Nicole's case, says the new drugs are undoubtedly extending the average survival time for metastatic melanoma patients. Atkinson says about half of those on Keytruda, or the biologically similar drug Opdivo, will still be alive after three years. "It's a significant improvement," she says. New studies are showing even better results - about 58 per cent survival after three years - among skin cancer patients treated with Opdivo combined with an earlier form of immunotherapy, Yervoy.
For a small, but substantial group of patients, doctors are starting to whisper the C-word. "There are patients around with no evidence of disease who have stopped their treatment who we hope the cancer will not come back," Atkinson says, choosing her words carefully.
"About 15 per cent of patients on immunotherapy drugs will get rid of their disease completely. But we need a little bit more time to say that we are curing people. I suspect we may be. We really need probably two more years before we can say (that)."
Amid the excitement about the prospect of curing some patients, there's also the cruel reality that for many people, for reasons still largely unknown, the new drugs fail to work. One in three melanoma patients on Keytruda or Opdivo will get no response. It's a medical lottery. However, Atkinson says new classes of medication may be on the horizon. Trials of more drugs, and combinations of therapies, have emerged, providing ongoing hope that response rates, and ultimately overall survival, will improve further. "Treatment options for melanoma have increased very rapidly in a short period of time but there are still hurdles that need to be overcome," Atkinson says. "There's still lots and lots of work, and lots of trials, going on."
At the same time, University of Queensland Professor of Chemistry Matt Trau is optimistic about the development of groundbreaking, inexpensive diagnostic tests that would give doctors real-time evidence of whether melanoma medications are effective in individual patients rather than having to rely on the torturous wait between scans. Trau says nanotechnology can monitor circulating tumour cells in a patient's bloodstream as they evolve. "These are the rogue cells that everybody is afraid of that break away from the original cancer and travel through the blood to other sites," he explains.
In what amounts to cancer forensics, researchers are also working on tests that can detect trace DNA in blood, urine or saliva left over when cancer cells die. "It's like a crime scene," Trau says. "They leave behind DNA fingerprints."
If the tests pass clinical trials, they will allow doctors to stop prescribing expensive drugs to patients much sooner and to potentially switch them to different, hopefully more effective, medication. The new technology is expected to drive the costs of treating cancer down, through cuts in spending on the Pharmaceutical Benefits Scheme and lessening the need for scans.
Christmas, 2010: It should have been one of the best times in Brett Burgess's life. The physical education teacher, then 44, had just been told by the Queensland Education Department he'd been promoted to principal at Bribie Island State High School, north of Brisbane.
But the excitement of his promotion was shattered when he was diagnosed with melanoma in his lungs and told by one cancer specialist: "There's nothing we can do for you."
In the September school holidays of that year, he had fallen off his surfboard on a weekend away at Palm Beach, Currumbin, on the southern Gold Coast. An X-ray showed he had a shadow on his lung, doctors suggesting he might have pneumonia. But when he was still in pain two months later, and a follow-up X-ray showed the shadow was still there, a biopsy was ordered, revealing he had advanced melanoma. "Initially, it's an absolute shock," Brett says. "There were tears. You worry about your family, your finances, you worry that you're worried." Although he had undergone regular skin checks for sun cancer over the years and doctors had removed less serious basal cell carcinomas, the origin of the melanoma remains a mystery.
Brett bravely fronted up to Bribie High to begin his career as a principal at the start of the 2011 school year. At a meeting of about 80 teachers, groundsmen and other staff, most of them strangers, he "welcomed everyone, told them some nice things and, trying not to cry, said: 'Unfortunately, I've got a bit of a medical issue and I can't be here, so I introduce the acting principal'." He was in the job no more than an hour and a half before handing the school to his deputy. The happily married father of two children - Jordan, then 17, and Georgia, 12 - took the rest of the year off, not knowing whether he would still be alive to return to the school and the career he loved.
After being given a year to live by one of the first doctors he consulted, Brett was referred to Paul Mainwaring, who at that stage had few treatment options for advanced melanoma. Mainwaring started Brett on chemotherapy and about six months later threw him a lifeline. An experimental trial was under way for a new melanoma drug, generically known as ipiluminab and marketed as Yervoy. Brett was still taking the drug when he returned to Bribie High in 2012. The breakthrough immunotherapy - the first drug of its kind to work in melanoma patients - was shrinking his cancer and allowed him to resume life as a high-school principal. "The Yervoy did great things," he says. "It dissolved almost all of the cancer except for a very small amount. It was a great relief." But under trial protocols, Brett was unable to stay on the drug.
He was off all treatment through 2013 but continued to have regular scans and in mid-2014, when tumours started to regrow in his lungs, he had radiotherapy. Two months later, surgeons operated to remove the lower third of his right lung. By then, Yervoy was subsidised on the PBS. Brett went back on the Yervoy after surgery but when it failed to work as it had during the trial, he was then prescribed Opdivo. The cancer has since disappeared and he stopped immunotherapy in June. "That's as good as you get," Brett says. "Part of me expects at some stage that there'll be more cancer and we'll have to deal with it. Hopefully by then, there'll be something even better."
Brett, now 51, is stoic as he recounts his cancer experiences but chokes back the emotion at the first mention of his wife of 27 years, Anne-Marie. Almost seven years since his diagnosis, he says he hasn't always appreciated the trauma she endured on his melanoma journey. "It's pretty hard for her," he says, struggling to stay composed.
The pair met as 20-somethings in the central west Queensland town of Longreach, where they were both working as teachers. "He was really kind and you could tell he came from a good family," Anne-Marie says. "He was just a really decent person and I liked the look of him. Even at his sickest, I always found him so attractive. I probably love him more now than I have ever loved him. I just admire him so much. He's more compassionate."
Brisbane surgeon, Professor Mark Smithers, says while the emergence of new drugs is undoubtedly a breakthrough for metastatic melanoma patients, he's concerned the focus has been diverted away from early detection and prevention. "The drugs are fantastic," says Smithers, who has been involved with the melanoma clinic at the Princess Alexandra Hospital, in Brisbane's south, for 30 years. "I didn't think I'd see it in my career that we would see people having this great response. However, the number of people who have a complete response so that their disease goes away, and might even stay away, is a small percentage. We're going to have a much greater impact on melanoma if we can diagnose it early and, more importantly, if we can prevent it."
Smithers says recent Queensland research has found the incidence of invasive melanomas - those capable of spreading beyond the skin - is declining in the under-40s and has plateaued in the 40-59-year age group. But rates continue to increase in those aged 60-plus. The turnaround in the younger generations has been attributed to the success of Australia's skin-cancer awareness campaigns in the 1980s and '90s. But Smithers says Australia is in danger of losing momentum, and new generations are at risk of becoming complacent without ongoing public health campaigns, such as the iconic "Slip, Slop, Slap" message of last century. He says the last big Federal Government-funded sun-safe campaign was in 2007. "We're worried that governments are dropping the ball," Smithers says.
As another Queensland summer dawns, the state retains the mantle of world melanoma capital with an incidence of 72 cases per 100,000 people per annum, on average, in the five years between 2010 and 2014. Queensland's rates are far higher than those for Australia as a whole - 48 cases per 100,000 people.
The latest Queensland Health statistics, based on telephone surveys of 25,000 adults in 2015-16 and 2500 parents and carers in 2014, also reveal alarming levels of sunburn, increasing the risk of developing skin cancer. More than half of Queensland adults surveyed and 64 per cent of children aged five and older had been sunburnt in the previous 12 months. Men were much more likely than women to be sunburnt. In 2014, of the 3666 people diagnosed with invasive melanoma in Queensland, 2179 were men - almost 60 per cent. In the same year, Queensland men were more than twice as likely to die of melanoma as women. Of the 315 deaths, 220 were men.
Dermatologist Professor H. Peter Soyer, based at the University of Queensland's Diamantina Institute, is working on using imaging technology and artificial intelligence to detect melanomas earlier in people identified as being at high risk through genetic and environmental factors, such as jobs that increase the amount of time they spend in the sun. The hope is to develop a targeted screening program for melanoma, selecting only people likely to develop the deadly skin cancer, for regular surveillance. Using a new Vectra machine at the PA Hospital, one of only three in the world, Soyer is creating avatars of patients already diagnosed with melanomas to map the spots on their skin and detect changes over time. "It's all about detecting and removing melanomas as early as possible at a stage when it's not yet a melanoma, or so early that it will not advance and do harm," he says. Dermatologists are viewing the images and identifying suspicious spots for removal. In future, with more research, Soyer expects this role to be taken over by artificial intelligence, with computer software programs analysing moles and blemishes.
Brisbane union official SÚn Etheridge, a MasterChef finalist in 2011, has enrolled in Soyer's research. Mum to five-year-old Jack and two-and-a-half-year-old Vivienne, she considers herself fortunate to be alive after a doctor at a suburban skin cancer clinic in 2009 dismissed her concerns about a mole on her left thigh. "I'm very aware of my melanoma risk," she says. "My great-uncle died of melanoma, so I have a family history. I've got the 'ginge-tinge', reddish hair, pale skin and green eyes. I probably had at least five, maybe even more, instances of blisters and peeling after being in the sun as a child.
"Almost 10 years ago now, I developed a new mole that was unusually coloured and itchy and it didn't feel right. But the doctor told me I was being paranoid. All the signs should have pointed to getting it out but we want to believe medical people, and I listened to the voice of authority."
For months, Sún didn't get a second opinion despite it being "constantly on my mind". At one point, she vividly recalls slapping the mole, saying, 'Don't you be killing me', when it felt itchy as she was changing her clothes. It wasn't until a colleague came into work one day, devastated about the pending death of her 27-year-old best friend from melanoma, that Sún was convinced to have her mole rechecked.
"My friend made me make an appointment that afternoon," she says. "The doctor took one look at it and 24 hours later, it was being cut out." The next day, Sún was told it was a melanoma. "I've got a seriously decent scar but because it hadn't gone into the bloodstream, touch wood, I didn't get a death sentence," she says. "I'm very lucky. If I hadn't worked next to that woman and she hadn't had that conversation with me about her best friend dying from melanoma, I wouldn't have gone for a second opinion when I did. I would have missed it." About 12 months later, she had a second melanoma removed from her back after it was picked up during a three-monthly skin check. This one was caught at a much earlier stage.
Sún, 39, married to Brentley Frazer, a Griffith University lecturer and author, tells her story to encourage other Queenslanders to seek a second, and if necessary, a third, medical opinion if they remain concerned about a health issue. "Back your instincts," she says. "If something doesn't feel right with your body, even if a doctor tells you that you're being paranoid, keep pushing. I didn't push. I was saved by incredible luck."
Not knowing what the future holds, Brett and Anne-Marie Burgess have worked hard on creating new memories during the past seven years, taking overseas trips with good friends and their children - Jordan, now 24, a civil engineer, and Georgia, 19, who is studying to be a primary school teacher. "When Brett was first diagnosed, we decided that if he's going to die, let's see as much of the world as possible," Anne-Marie says. "People told us not to go, you won't get travel insurance. We just said: 'Stuff it. We've been through worse'. It turned out to be the best thing ever."
For Nicole Johnson, melanoma ended her dreams of having a baby. But she has bought a caravan with her husband and they hope to see more of Australia. She's grateful new drugs are giving them more time together. ■
To donate to skin cancer research: pafoundation.org.au