Young cancer patients are ‘falling through the gap’

Cancer survival rates are rising all the time but among one age group of patients – adolescents and young adults – the gains have been slower.

The reasons are complex, ranging from biology and treatment protocols to psychological and social issues, but consultant paediatric haematologist Prof Owen Smith is passionate about how more could be done in Ireland to improve the outcome of adolescent and young adult (AYA) cancer patients. He reckons the cure rate can be pushed up by as much as 20 per cent, depending on the type of tumour.

On a corridor wall outside his office, on the third-floor of Medical Tower One in the children’s hospital now known as CHI@Crumlin, hangs an array of framed newspaper articles, the more faded ones dating back to 2007. They are testimony to just how long he has been championing the creation of dedicated services in Ireland to prevent AYA patients “falling through the gap” between paediatric and adult treatment services.

He started “knocking on doors” about this issue after returning to Ireland in the mid-1990s from nine years of post-graduate training in University College London Teaching Hospitals. There, they had designated services for this age group of cancer patients.

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“What struck me was that there was nothing in the system [here] looking at the needs of adolescents/young adults,” he says. “So, I got things moving and got people interested in things. There was a lot of data coming through that if you treat this sub-group of patients differently, their outcomes are massively different.”

After more than two decades, Smith is finally seeing his vision for a national adolescent and young adult haemato-oncology programme taking shape. Last year, he was appointed by the National Cancer Control Programme (NCCP) to be the national clinical lead for children and adolescents – a post recommended by the National Cancer Strategy (2017-2026), which, for the first time, recognised AYA cancer patients as a distinct group.

Here, we do cancer treatment for the under 16s extremely well, he says. By 2005-2007, Ireland had the lowest mortality rate of 31 European countries for children aged 0-14 with leukaemia, the most common childhood cancer. The five-year survival rate for all childhood cancer is 81 per cent, according to figures published by the National Cancer Registry in 2017.

However, the paediatric cancer programme that is working so well, says Smith, needs to be rolled out for the older youngsters “with their unmet needs”.

Just what those needs are will be the focus of an inaugural AYA haemato-oncology study day at the Royal College of Physicians of Ireland in Dublin on March 18th, hosted by the NCCP. It will bring multi-disciplinary health professionals together to look at measures that can improve outcomes for adolescents and young adults.

In the run-up to this event, the rapid-talking Smith brings up graphics on the computer screen in his office to indicate how, internationally, survival rates of those diagnosed with cancer between the age of 16 and 24 lag behind younger patients. And improvements in survival rates have been significantly greater for children and older adults, than for the AYA cohort.

This is “staggering” he says of a bar chart illustrating the average annual percentage change in cancer survival rates across all ages in the US, 1975-1997.  The coloured bars for those aged 0-15 poke above the line, indicating the 1.46 per cent average increase year on year, but then they shrink dramatically for adolescents and young adults before rising sharply again for older adults.

While there are several strands in the new national AYA programme to improve outcomes, what’s key, he says, is getting these young people on to clinical trials, as has been done so successfully within the paediatric services.

“Clinical trials protect patients – they do better,” he says simply. UK data from 2005-2010 shows how participation in such trials drops sharply for the AYA group.

“We are hell-bent that all children and adolescents go on clinical trials,” he says. But it is “difficult getting this across” to colleagues in the adult services who are treating everybody over 16.

Clinical trials

He points to data on 15-24-year-olds that shows those on clinical trials had 18 per cent better overall survival at two years – and half the risk of death – when compared with those of the same age not on trials.  And research on blood cancer patients aged 15-17 has indicated they do significantly better on paediatric clinical trials versus adult clinical trials.

Another element in the worse prognosis for AYA cancer is changing biology at that age. “Today’s research is tomorrow’s cures and the only way for this country to address the biological issues is to get into bed with basic scientific discovery,” says Smith. “We are trying to completely unravel the genomic landscape of these cancers.”

A €12 million collaborative project between Systems Biology Ireland in UCD and the European Molecular Biology Laboratory will look at these aspects of the disease in this age group. So far, €9 million funding has been secured and they hope to kick off the research later this year.

As for the psycho-social dimension to AYA treatment, Smith admits that, early in his career, he found it hard to believe this could be a factor in cancer outcomes. “I trained as a bio-chemist before I did medicine; I was into problem-solving and this stuff felt ‘fluffy’.”

However, research findings convinced him of its importance. He cites one study in Texas on patients aged 16-21 with a sarcoma, where one group was treated in a children/adolescent facility and the other in an adult hospital. All were given the same cocktail of drugs and they were administered intravenously, “so it wasn’t a question of compliance”, yet the outcome was statistically significant for the patients who went into the age-appropriate facility.

The cancer strategy recommends that the HSE ensures a dedicated AYA facility is included in the new children’s hospital, now due to open in 2023. Bed numbers for cancer patients are to rise from the current 19 for under-16s in Crumlin, to 28 in the new hospital, with the age limit increased to 20 years.

Meanwhile, four of the country’s cancer centre hospitals will soon be earmarked to become the first specialised AYA centres, operating as “spokes” off the CHI hub. With staffing appointments for these due to start within months, they will be modelled on the UK’s Teenage Cancer Trust units, of which there are 31.

The idea is to provide a comprehensive cancer care package for adolescents, including psychology services, nursing care and dentistry. Fertility preservation and counselling is another vital service for this age group.

From these AYA centres, cancer patients and survivors can be eased into the adult services. Currently, the transition in Ireland from paediatric to adult cancer care is “like getting somebody to jump out of a plane without a parachute – it’s unbelievable”, says Smith.

A death sentence

Leukaemia was virtually a death sentence in the 1960s but now there is up to a 90 per cent cure rate. While the medical profession has become very good at treating the acute toxicity of cancer treatment, there are long-lasting side-effects.

Cancer has become a chronic disease – up to 70 per cent of children who live for more than 30 years after their diagnosis will have at least two co-morbidities, such as necrosis of the bone, which necessitates joint replacements, or cardiac issues. “At the moment, there is no programme in place to look after this,” he says. “Cure comes at a huge cost for these patients.”

Part of a new trial known as ALLTogether01, being conducted primarily by paediatric haematologists in 15 countries across the EU, is looking at the use of less toxic drugs. It will also be using CAR T-Cell therapy, which collects and uses patients’ own immune cells to treat their cancer, in leukaemia cases where other treatments have failed to achieve remission. And rather than being confined to paediatric patients, this trial, due to start by the summer, will be open to those aged up to 44 years.

‘Very lucky’ to be treated in Crumlin

Karen O’Neill believes she was “very lucky, if you can use that word” to be treated for her cancer as a teenager in Crumlin children’s hospital.

Diagnosed in an adult hospital, the Connolly in Blanchardstown, shortly after she had turned 16, O’Neill says the haematologist who came to see her promised “he would try to pull as many strings as he could” to get her into the paediatric setting.

“He said if I was treated in St James’s, where I would have had to go, it would be a bit miserable for a 16-year-old.” That was 10 years ago and today they are stricter about the cut-off age of 16 for paediatric services.

Although being treated around children meant it was a “much happier and positive environment”, it was still not appropriate for a 16-year-old, says O’Neill, now aged 27. However, it was far better than the alternative.

“Coincidentally, one of my good friends from school was diagnosed with cancer just after I had cancer. She had just turned 16 as well but she ended up being treated in Tallaght, in the adult unit. We had worlds apart different experiences of our journey.

“She subsequently passed away but even in the 2½ years she was on treatment, it was just so age inappropriate.” O’Neill realised that in Crumlin she took it “as a given, the support mechanisms you have around you. I got such an insight at a young age into the disparities in the services in the country.”

She went on to study medicine in Trinity and is now training to be a paediatrician at University Hospital Galway. A passionate advocate of dedicated services for adolescent and young adult cancer patients, she says: “I think I am in quite a privileged position in that I know the health service as an employee and also as a patient. I have seen the gaps but I can be realistic about the services and what you can and cannot implement.”

Prof Owen Smith was her doctor in Crumlin and she is now a member of the clinical leads committee within the National Cancer Control Programme. “I think we still have miles to go in terms of adolescent oncology treatment in this country.” It is a unique time of life to be faced with a serious illness, even in terms of transition through education.

“I was lucky, I was in transition year but it still had a massive impact on my study through fifth and sixth year. With appointments and stuff, you miss a lot of school.” It is also a huge disruption to growing up.

“You are just about to get your own independence in the world and then it’s taken away from you. You go from trying to be independent from your parents to them having to help you to the toilet every time you try to get out of bed. And socially everything is taken away from you, just as you gain that independence. It had a massive impact.”

O’Neill underwent chemotherapy over five months, spending longer in St John’s ward in Crumlin during that time than at home in Celbridge, Co Kildare. “You think when the chemo is over that everything is cured and you are all better but you still have all the side-effects of treatment and the infections to deal with before your body fully gets strong enough.”

However, she is very grateful that she came out of it “relatively unscathed” and has been in remission for 11 years. Cardiology is the only physical issue she has had to follow up on; she is on medication and attends a cardiologist once a year.

“I am very lucky because I have friends who have five or six different specialists they have to attend and so many different [body] systems affected.”

She still carries a psychological burden, although that has decreased with the passing years. “Initially, there is always the fear of relapse.” And there’s the added trauma of some people who have been in treatment at the same time passing away.

It’s no wonder O’Neill has joined the drive for adolescent cancer patients to be treated “in an age-appropriate environment with proper services that they deserve – and, hopefully, maximise survival rates and minimise survivorship issues”.

‘I was the youngest woman in that ward’

Sorcha Lavelle was only 22 years old when she was diagnosed with breast cancer and was plunged into 22 rounds of chemotherapy, a double mastectomy and 20 sessions of radiotherapy.

“It was scary” she says, being one of the youngest breast cancer patients to be treated at St James’s Hospital in Dublin, with “no real stats” on likely outcome.

Getting chemotherapy is intimidating enough, she points out, but on top of that, being in the company of people a lot older, “it’s very intense”.

“I was the youngest woman in that ward. There were maybe one or two women in their 30s but they were still 10 years older than me.”

She believes if she had been in a young adult scenario, she could have connected better with those around her and chatted more about what they were all going through. “I very much kept to myself – me and my mother.”

Lavelle describes being on a clinical trial as “the best thing, if you can have a best thing, of treatment”. Living at home in Virginia, Co Cavan, it was very comforting to be able to log in every morning with her temperature and tell them how she was feeling. She would be phoned back by staff on the chemotherapy ward at St James’s if there was any adverse sign at all.

“Personally, that made my experience easier for me and, probably, for my mum as well because it was like peace of mind.”

While Lavelle, a former air hostess and now staffing manager with a marketing company, found the social isolation of treatment and being off work for 18 months hard, she says she had great support from family. But coming to terms with having a mastectomy so young was difficult, and “not really having your peers understanding how you are coping with is quite challenging.

“People would say ‘chemo must be very tough, losing your hair’,” while she was thinking “yeah, but hair grows back . . .” However, “I would rather have no boobs and still be alive”, she says wryly.

“Not a lot of people are aware this can happen to younger girls,” she adds, which is why she is intent on getting the message across to her peers – and the wider female community – “check your boobs”.