Everything to play for

Published on Saturday 5 July 2008 19:54

It was really difficult when he finished treatment. His whole life revolved around going to hospital

AARON Thomson was just five years old when his mother Barbara heard the words every parent dreads. Following a bout of headaches so severe they would leave him gripping his head in tears, doctors diagnosed leukaemia.

That was June 2004, during a trip to the emergency department of Edinburgh's Royal Hospital for Sick Children. Just a few decades earlier it would have been a death sentence for Aaron, from Dalgety Bay, Fife. But thanks to significant advances in the treatment of acute lymphoblastic leukaemia, the most common childhood leukaemia, doctors were able to reassure his mother that Aaron's chances were good.

"At first he was very sick with intensive treatment, oral and injected chemotherapy, and lost his hair," Barbara, a 35-year-old midwife, explains. "His immune system crashed and he suffered from joint pains. The treatment was so unpredictable that if he went to school for a morning he generally came home in the afternoon unwell. It was a difficult time for the family and I had to give up work. But now he is better, back to being like any other nine-year-old. He just gets on with things."

Four years after a gruelling chemotherapy regimen, Aaron has been free of the disease for almost a year. His only concern today is whether to become a goalie, a guitarist or the next Gordon Ramsay.

Aaron's success story is echoed by hundreds of children diagnosed each year with a disease that once offered no chance of survival. A report published in the British Journal of Cancer last week illustrated just how far scientific advances have come, with better drugs and new treatment strategies meaning that 73% of youngsters recently diagnosed with the cancer in the UK will be cured.

So just how is it that in the space of 70 years such dramatic improvements have been made? Could survival rates improve even further so that one day no child will die of this disease? And with death becoming an ever-decreasing possibility, what are the long-term effects of such radical and gruelling treatment on the lives of its survivors?

Barbara admits she knew virtually nothing of childhood leukaemia or chemotherapy until her son faced the disease.

Leukaemia is a cancer of the blood, caused when cells fail to replicate themselves properly. Normally, white blood cells grow and divide in an orderly and controlled way but in leukaemia the process gets out of control, cells divide too quickly and do not mature. Immature cells fill up the bone marrow and stop it making blood cells properly. As the leukaemia cells do not mature, they cannot do the work of normal white blood cells, which leads to an increased risk of infection. Because the bone marrow is overcrowded with immature white cells it cannot make enough healthy red cells and platelets.

Acute lymphoblastic leukaemia occurs more frequently in children than adults and usually occurs in children between the ages of two and five. Its cause is not known, but risk factors include genetic conditions, exposure to infections and exposure to chemicals. In the first half of last century, a diagnosis of the disease, like that of other cancers, meant death within a few weeks.

The first major advances in chemotherapy were made in 1948 when scientists researching drug therapies for cancer discovered the effects of folic acid on leukaemia patients – it appeared to allow the cancer cells to proliferate.

Working in the face of opposition, from those who believed leukaemia patients should be left to die in peace, scientists led by Sidney Farber, a pathologist at Harvard Medical School, developed a drug that would block the action of folic acid. Patients lived slightly longer, and similar drugs became a focus of intense investigation.

Over the next 10 years further breakthroughs were made, culminating in the discovery of 6-mercaptopurin, a powerful immunosuppressant. Combination therapies using anti-cancer drugs and steroids kept children in long-term remission but survival rates remained low at only 25%. During the 1980s, treatment regimens became more intensive and as a result more children were cured – around half.

Over the years, and under numerous drug trials, it became clear that some children needed less intensive treatment than others depending on the particular genetic code of their disease. This was important because immediate side effects of chemotherapy include infections and cold sores. Common diseases, like chickenpox, could be fatal. In addition, intensive therapy can have long-term adverse effects on the heart, future fertility and trigger secondary cancers.

Children diagnosed today will receive tailored treatment programmes, designed to rid the body of the disease using the least amount of chemotherapy possible. They will also receive donated blood to boost their own supplies and antiviral drugs, antifungal drugs and antibiotics to ward off potentially deadly infections. Many children do not require highly intensive treatment programmes. But modern methods of thorough analysis of their blood mean those who do are targeted within the first few days of treatment.

This means that delivering the diagnosis, though still difficult, is nowadays an entirely different scenario for doctors. Dr Angela Thomas, a consultant paediatric haematologist at the Royal Hospital for Sick Children in Edinburgh, explains: "It's a diagnosis that can be made rapidly and with a great deal of certainty, within 24 hours of the child being admitted to hospital. There's no point faffing around pretending nothing's wrong. I say 'It looks like leukaemia', and that's all parents remember. But then I say 'we know how to treat it, and this is the right place to be and the majority of children do very well'.

"Now, over the first week, we gather extra information about the leukaemia to tell us how to treat it. Some will need more intensive treatment than others. If you gave everyone the most intensive regimen there would be very good survival rates but more toxicity. And quite a lot of the children don't require that much."

This kind of treatment management has allowed many children to have less chemotherapy. Although everyone receives injections at hospital and pills at home, for a total of up to three years, some require fewer drugs than others. Those who do not respond well to chemotherapy – around 2% of all cases – are given the last-resort treatment of a bone marrow transplant.

Sadly, some children do still die from leukaemia. In Scotland, this amounts to a handful of the 50 or so cases diagnosed every year. But Thomson is optimistic these numbers will dwindle even further. She says. "I don't think this will ever be 100% curable, just as no infection is 100% incurable. The last few cases will become increasingly difficult and it's important to identify more intensive therapy. We are hoping the survival rate will be pushed up but I have no idea to what. The nearer you get to 100% the more difficult it is to show improvement. The biggest gain to be had will be among those children who appear to be doing well who relapse suddenly."

So what is the outlook for families who will, months or years from now, discover their child has leukaemia? Despite the leaps in science, many questions about the disease remain unanswered. In a sense the easiest work has been done. The hardest part is targeting the most difficult cases.

Like many cancers, it is not known why children are predisposed to leukaemia. Breastfeeding is thought to have a protective role, as is spending time in daycare or playgroups during the very early years, as exposure to infections at a young age boosts the immune system. But much is still to be learned. The nature of the disease has been key to its research. Its presence in the blood has allowed scientists immediate easy access to its cells, through samples, rather than going through solid tumours.

Barbara got through her ordeal by keeping a journal of the treatment, which she hopes Aaron will read when he is older. "It was really difficult when he finished treatment because his whole life revolved around going to hospital and the nurses were like extended family.

"It was difficult to shut down that part of my life – I had spent three years worrying. I had to return to whatever normality was."

This is a problem common to many survivors, according to CLIC Sargent, the UK's biggest children's cancer charity. With total recovery an increasing likelihood, returning a child to normality, school, exams, friendships, family, good health and a career without letting their shaky start cloud their prospects is a major challenge.

Months at a time in hospital mean many children miss out on schooling. Survivors then struggle to catch up, leaving some with gaps in their education that can affect employment prospects. The treatments themselves can also take their toll.

Ellen Finlayson, CLIC Sargent's Head of Services for Scotland explains: "Survival often comes at a price, leaving many children and young people with social and emotional difficulties, as well as long-term medical problems. It is vital they receive the support they need in order to maintain a good quality of life."

Survivor Ruth Buchanan, 20, underwent a bone marrow transplant, chemotherapy and radiation for acute lymphoblastic leukaemia two years ago. It saved her life, but left her infertile and with an increased risk of developing skin, breast and cervical cancer and cataracts.

Her spleen was damaged during the treatment so she has to take medication and vitamins every day. Despite these problems, Ruth remains upbeat about the future. She was supported through her treatment by CLIC Sargent and hopes to help others in the future.

"When they first told me I wouldn't be able to have children it really upset me," she admits. "It still does but I try not to let it get me down. I don't worry about the other risks but take each day as it comes. Everything happens for a reason. Before getting ill, I used to hold back from doing things. Now I go out and enjoy myself more."

A mother's diary

Barbara Thomson kept a journal of her son Aaron's cancer treatment. Here, some extracts reveal just how tough it was.

WEEK ONE

The chemotherapy was brought to the room by two nurses who wore plastic eye protectors, white aprons, gloves and forearm protectors. The chemo itself was wrapped up in black plastic and had bright yellow stickers on it saying "CYTOTOXIC".

WEEK TWO

We were warned about food cravings, a side-effect of the steroids, but I don't think we took it seriously until I found myself making hot dogs at 3am and making an average of three breakfasts a day for him. When cousins or friends from nursery came it was good to see Aaron get up and try to play. He had lost weight and his little legs were all skinny, mainly from the knee down, and sometimes I wondered how they managed to hold him up as he hobbled down the ward to the playroom.

WEEK THREE

Aaron's mouth started to break down even though we had been so careful to follow the strict mouth care regime. His tongue and bottom lip were starting to get ulcerated and he was in a horrible position of wanting to eat, because of the steroids, but his mouth was causing him too much pain. He had a thing for salt and vinegar crisps and there was just no way he was able to eat them with a sore mouth.

WEEK FOUR

Aaron gets to about 6pm and gives up on the day. Some nights he disappears to my bed without telling me. I would go and look for him and he is there, he is sore and exhausted and has had enough in general. This really upsets me because he just looks so miserable and it is like the daytime is just a drag he has to get through before he can sleep again.

WEEK SEVEN

The next couple of weeks seemed to go well and as we approached the end of July we were really seeing the effects of the chemotherapy on Aaron. He needed constant topping up with platelets and red blood cells. When Aaron needed blood you could really tell because he would go so terribly white. Then at the end of the transfusion his little cheeks would be really pink and he would be so full of energy, like someone had pumped him full of sugar.