Every day six teenagers are diagnosed with cancer. Out of these six, two will be diagnosed with Primary Bone Cancer (PBC), making it the most prevalent form of cancer for young adults. Indeed, 90 pre cent of all cases are found in adolescents.
Mike Francis, father of the late Guy Francis, has spent the last 10 years fighting to shift attitudes and alert us to the risks PBC poses. Government research funding is sorely lacking, leaving charities such the Guy Francis Bone Cancer Research Fund, based in Acomb, York, straining to pick up the tab. There is, however, cause for hope: scientists at the University of York are growing human bones from stem cells, possibly marking an important victory in the battle against bone cancer.
Guy Francis was 17 years old when a tumour growing in his left knee began to cause him discomfort. It would be five months until a correct diagnosis would be given of osteosarcoma, the most common form of PBC. At first, doctors attributed his pains to sports injuries or growing pains. “Because the cancer affects an age-group liable to these minor problems, the cancer can go undiagnosed for a dangerously long time,” explains Mike. “When at first everybody thought Guy’s discomfort was caused by a sports injury he was recommended to a physiotherapist. Unfortunately, for osteosarcoma sufferers, physiotherapy does more harm than good as the exercises prescribed cause parts of the tumour to break off and spread to other parts of the body.”
He was then re-diagnosed with “water on the knee” and underwent two exploratory operations. Eventually, an MRI was recommended and, at this point, Guy was in such high levels of pain he was unable to bend his knee during the scan. “We were ignorant of the cancer. We learnt the nurse began to cry during the MRI”.
The diagnosis was confirmed 135 miles away at an Oncology ward in Birmingham and a full body scan confirmed the spread of the cancer to Guy’s thigh, shin, pelvis and ankle, while his kidneys and lungs were also badly affected. With an assessment of his chances of survival beyond a month at only 8%, Guy began to fight the cancer.
In 2002, there wasn’t a single penny of government money spent on research into primary bone cancer
Mike remembers coming back to York from Birmingham with Guy. “It was the day before his 18th birthday and we should have been enjoying a party at the village hall. Instead Guy was in bed, feeling very un-birthday-ish. The doorbell rang and there were 28 of his friends standing there. Suddenly the party was on! I was sent to the village shop to buy the place out of alcohol (or so it seemed). When I got back, Guy had told his friends that, as the chemo would make his hair fall out, he wanted to pre-empt this and got each of them to take a turn in shaving his head.
“Actually, this was to became very significant in the future as his friends felt part owners of his cancer”.
Guy would go on to fight the cancer, his commitment to the battle making a mockery of doctors’ survival figures. But it continued to return, twice in the following five years, before it eventually overcame Guy in 2002, a week after being awarded his degree from Teesside University.
The charity, founded shortly before Guy’s death, aims to raise awareness, as well as much needed funds for research into bone cancer. When Guy was diagnosed, research was under-funded and the disease was virtually anonymous in the public eye. Mike explains: “there wasn’t a single penny of government money spent on research into primary bone cancer.”
For the last 25 years, the chances of surviving in Britain for more than five years with PBC have been static at 55%, whilst in Germany research has accelerated the chances of survival to around 68%, and in the USA, statistics show survival rates at 78%. And it is not only against other countries that bone cancer’s survival rates are shown up – in the UK, five year survival rates for breast and prostate cancer are 85% and 80% respectively, with the latter accelerating significantly in the last decade.
Mike, who has been involved in presenting expert evidence to the National Institute for Health and Clinical Excellence (NICE), believes that the organisation needs to focus more on a cure rather than treatment and palliative care. “NICE don’t support a drug which aims to cure, they support drugs which are used to treat the cancer. With regard to teenage cancer such as PBC, which never really occurs once a person is over 24, there should be more of a focus on drugs which cure. If a teenager is given the opportunity to grow they will go on, have a career, get married, pay taxes and contribute to society.”
PBC may face a shortage in research funding, but that does not mean there aren’t people committed to caring for sufferers. Mike is full of praise for those who cared for Guy. “We do not have a blame culture. Guy was really lucky to be on a teenage ward, and not an adult ward. The attitude of all the staff was so positive and showed they understood the needs of teenagers – they were brilliant.”
The University of York, as well as Guy’s charity are making headway in their attempts to change the UK statistics. Dr Paul Genever, a senior lecturer in biology at the University and one of the scientists leading the research, explains the kind of work York is doing. “Our research is based on adult bone marrow cells that we obtain mainly through the removed bone following hip replacement operations.”
The close examination of these cells is performed with the aim of discovering “markers” which could indicate the early stages of PBC. “We are working to find out how and why cells start behaving abnormally and develop into cancerous bone cells. One structure we are looking at in particular are telomeres, which are like little shoelace caps on the end of chromosomes which protect the genes. The more a cell divides, the weaker and shorter these caps become, until eventually the cell realises the protection is no longer strong enough and stops dividing. The limited lifespan of telomeres mean that cells cannot just go on dividing infinitely – they can therefore protect us against the cancer.” Cancerous cells, however, can divide without a limit. “Telomerase is an enzyme that can rebuild telomeres enabling the cells to become immortal and therefore cancerous. By researching further into this particular enzyme and its effects on the bone marrow cells, we hope to provide some way of identifying early markers which indicate PBC at its early stages.”
The University is also using bone marrow stem cells to grow three dimensional bone structures which could replace titanium implants. Early on in his treatment, Guy Francis was fitted with a titanium implant which replaced the cancerous bone in his leg without need for amputation. The implants are an effective alternative to amputation, but there are problems arising from the use of titanium.
“Titanium is a finite resource and it will run out. There are titanium implant companies in Sweden who send workers out to crematoriums in order to retrieve the implant from someone who was fitted with one. They desperately want to recycle them.” 3D bone structures can be grown from stem cells around a scaffolding, as opposed to a 2D structure in a petri-dish. “These fillers and structures could be used in five years time to help patients with bone defects like arthritis or PBC. They aren’t like organ transplants as they don’t carry high risks of rejection. These cells don’t work like that.”
Mike Francis explains that he wants the money raised by the “Guy Francis” charity to be fed into schemes such as the ones going on at York. Susie Fothergill, Head of Development and Alumni Relations and a good friend of Mike discusses how money raised could be spent. “We might be able to fund a PhD student in Biology who could further research into PBC. Money could also be used to fund students in social departments in order to improve the treatment of PBC sufferers by healthcare professionals. Mike is very keen on improving early diagnosis rates. Posters highlighting the symptoms of bone cancer in G.P. surgeries could really make a difference.”
Guy’s case is a perfect illustration of the importance of education. By the time the cancer has spread to the major organs the chances of survival are significantly reduced. Treatments such as chemotherapy and hormone replacement therapy are often in vain. Other types of cancer also cause further bone weakness, often forcing the use of Bisphosphonates, which like so many treatments for cancer comes equipped with its own package of unpleasant side effects.
2012 marks the 10 year anniversary of Guy’s death, and the Guy Francis Bone Cancer Research Fund continues to have ambitious fundraising plans. Amongst fun runs, bike rides and G-BrewFest, taking place on 4 August of this year, Mike, inspired by a social media workshop he recently attended in London, plans to run a UK-wide celebrity-fuelled “text-athon” for students across the country in October. Although the York term will only have just begun at this point, Mike hopes students from York and across the country will get involved. “The disease only really affects teenagers and young adults, [and] being a student means you have the time and energy to make a difference. The ultimate aim is to raise awareness and money so that premature deaths are prevented.”
For more information, or to donate to the Guy Francis Bone Cancer Research Fund, go to the website, www.gfbonecancer.org.uk