“Why Me?” – These were the first words uttered by Guy after he had been told he had bone cancer. There was no answer then – nor is there now.
Given that the lack of an answer is unacceptable, it is not unreasonable to ask more broadly “Why Guy?”. and “Why Anyone?”. The answer is that – at present –there is no clear answer – largely because not enough people have cared.
However, Guy and other sufferers have sparked a public conscience by asking the question “Why”. Guy only asked it once – challenging issues around bone cancer, particularly as it affects young people.
From this, he founded the “Why Guy? Campaign”.
His first concern, in 2002, was “Why?” there was absolutely no Government funding into research into Why? it was mostly teenagers who were the target of this fatal disease. The answer may be a combination of indifference and a recognition that the numbers involved – about 550 per year – were too small to care about, compared with the “big 5” cancers.
To some extent this is understandable. The big difference is that, when every day 6 teenagers will be told that they have some kind of cancer – and that 2 of the 6 will be told that it is bone cancer – then bone cancer stops just being one of the 53% of all cancer that are classified as being rare, into being the predominant cancer for teenagers.
That fact alone should make any British Government give bone cancer a high priority – but it hasn’t. After all, with an increasingly aging population (who will go on to attract a variety of illnesses that will require public health expenditure support), the falling birth rate pressurises the younger generation to contribute more tax revenue. With poor employment rate amongst this younger working generation, the figures don’t add up.
It therefore makes far more sense to save the lives of teenagers, in the expectation that their extended lives will produce greater tax revenue to support the retired and aging population.
THIS IS SOCIAL VALUE.
This argument was at last seen in 2011 to have a great influence on the decision by NICE (National Institution for Health & Clinical Excellence) to approve the osteosarcoma related drug – Mifamurtide (MEPACT) – that such a drug might extend a teenager’s life by 60 years.
ASKING THE QUESTIONS.
Causes? Lifestyle? Environment? Detection? Treatment? Survival?
A sure cause of bone cancer is not, as yet, known. As the vast majority of cases of osteosarcoma and Ewing’s sarcoma develop in teenager’s growing bones, it suggests that something is affecting the cells of bones as they mature. But why should the disease be so selective? Is there a hereditary factor to be considered, or is it just that the cells are susceptible to genetic mutation and genetic defects in their own right?
An interesting area to study may be in comparing the cells of siblings, particularly twins.
Having a healthy immune system is a basic requirement for healthy living generally, so regular exercise and a balanced diet are recommended. A sedentary lifestyle, obesity, lack of exercise, over intake of growth hormones is known to increase the possibility of cancer.
Smoking, tobacco, betel nut chewing etc., are known to cause lung and mouth cancer; these can spread to the surrounding bone and cartilage.
Exposure to radiation is a known factor to induce bone cancer. Radiologists have to take special precautions when using medical imaging devices but is the same urgency considered in prolonged exposure to radiation, albeit low level, when sitting in front of the VDU screens of a computer? This is thought to be a factor in the increase of breast cancer amongst men over the last 20 years – so, as the use of computers is very actively encouraged in education, is increased technology by young people putting them at risk?.
Of course there is natural radiation particularly from certain rock strata such as granite. Could this be why in Cornwall, a county where granite is predominant, there appears to be a disproportionate number of teenage bone cancer cases than elsewhere in Britain. In 2006, the “Helston Cluster” was headlined (see http://news.bbc.co.uk/1/hi/england/cornwall/3133539.stm) with the suggestion that it warranted fuller epidemiological study. Another anomaly is being securitised in Penzance.
Water fluoridation and the siting of residential housing in close proximity to high voltage overhead power cables (pylons) are other areas requiring further study and debate, as does work on the exposure to carcinogens such as asbestos, cobalt, nickel, quartz or crystalline silica, charcoal and tar.
Delayed and misdiagnosis by healthcare professionals is a key issue. Raising awareness amongst this group is essential if the percentage of those surviving beyond 5 years is to be dramatically improved. Our S.I.G.N.S™ Patient’s Checklist is a very simple, no cost way of prompting discussion between Doctor and Patient.
There may be variations of the type of treatment offered depending whether the bone cancer is osteosarcoma or Ewing’s sarcoma – and every patient is assessed as an individual anyway. Some basic protocols are followed but it is acknowledged that “One size does not fit all!”.
Osteosarcoma will usually be treated with a course of high-dose chemotherapy to reduce the size of the tumour, prior to surgery. Often the chemotherapy will kill off any other small areas of bone cancer and any metastasis. Surgeons will always try for limb-sparing - perhaps replacing the diseased bone with a titanium implant (prosthesis). But in some cases, amputation will be necessary. Further chemotherapy may be necessary after surgery.
Ewing’s sarcoma responds to radiotherapy and treatment may consist of a combination of radiotherapy, chemotherapy and surgery according to the extent and location of the disease.
This is the crucial area of concern for all those involved in bone cancer. The data provided by NHS Choices highlights the need for strategies which will eliminate delayed and misdiagnosis.
NHS Choices offers the following advice:-
“The most important factor in determining the likely outlook for cases of bone cancer is whether the cancer has spread from the bone to other parts of the body (metastasis).
Health professionals use a general measurement of a "five-year survival rate" when describing cancer statistics. However, it's important to make clear that the five-year measurement is not an absolute measurement and it does not mean that people with bone cancer only have a five-year life expectancy.
Osteosarcoma : Six out of 10 people with localised osteosarcoma will live for at least five years after diagnosis and most of these people will be completely cured*.
The outlook for metastatic osteosarcoma is much poorer as only 1 in 10 people will live for at least five years after being diagnosed.
Ewing's sarcoma : Seven out of 10 people with localised Ewing’s sarcoma will live for at least five years after diagnosis, and again, most of them will be completely cured*”.
Only 3 out of 10 people with metastatic Ewing’s sarcoma will live for at least five years after diagnosis.”
· Whilst Guy’s Fund acknowledges the statements from such eminent organisation as NHS Choices, we feel compelled to state that we are unaware of published research that categorically supports the assertion that *“ patients with either osteosarcoma or Ewing’s sarcoma will be “completely cured” after 5 years”.
In general terms, the overall 5-year survival rate for people with bone cancer in the UK is considered to be 55%.
As found in research carried out by the University of Newcastle, this rate has not improved in the UK in 25 years – largely because there has been no investment in bone cancer research by the Government. During the same period, the most common childhood cancer – leukaemia – has seen its survival rate improve from 53% to 80% - thanks to Government funding into research.
BUT THERE ARE SOME POSITIVE STEPS.
Approval of Mifamurtide (MEPACT) for certain osteosarcoma patients. The approval in October 2011 by the National Institute for Health and Clinical Excellence (NICE) of the drug Mifamurtide (MEPACT), manufactured by Takeda UK Ltd., for use in appropriate cases of osteosarcoma, could be of major significance. Improved survival rates in the USA are very encouraging – but it will, of course, be several years before the extent and percentage gain beyond 5 years will be available for analysis.
However, the conclusions by NICE – whichafter over 3 years consideration of the drug, now accepts that there is an argument for including evidence of Social Value into health economics statistics – is a major development. The NICE press release can be found at http://www.nice.org.uk/newsroom/pressreleases/NICERecommendsMifamurtideForBoneCancerChildren.jsp
England Multidiscipline Team for Ewing’s sarcoma. For Ewing’s sarcoma patients and families, the establishment of an England Virtual Multidiscipline Team (MDT) is equally encouraging. Under the auspices of National Specialist Commissioning it will provide a national reference centre of expertise for the treatment of Ewing's Sarcoma of bone.
The aim of the National Ewing's MDT is that all new cases of Ewing's sarcoma of bone should be discussed by a National MDT with at least two surgeons, two radiation oncologists and the patient's own treating clinician in attendance.
A similar initiative for an osteosarcoma MDT would seem appropriate.
National Sarcoma Nurse Forum. Good news too for general sarcoma patient care with the establishment, in 2011, of a National Sarcoma Nurse Forum by a number of UK Clinical Nurse Specialists.
Improving outcomes for people with sarcomas Another NICE webpage which will be of interest is that on “Improving outcomes for people with sarcomas” at http://www.nice.org.uk/guidance/index.jsp?action=byID&r=true&o=10903
GUY’S AIMS – AND HOW WE ARE DEVELOPING THEM.
The “Why Guy? Campaign” will never be static. At times it may be controversial – but never discourteous.
We shall continue to ask challenging questions in order to raise awareness of the many aspects of bone cancer which need attention.
What we do know is that, in Britain, we are not as magnificent in providing healthcare as the Government would like to convince us that we are. It is false to say that the NHS is the envy of the World. In Europe, many of the things that the British medical establishment dismiss as “Secondary Healthcare” are regarded in Western Europe as “Primary Healthcare” – and go on to show that 5-year survival rates for bone cancer patients exceed the achievements of the United Kingdom – as well as suggesting greatly reduced costs to the taxpayer. The “International Consensus” meeting in the UK in 2006 on the “Localised Treatment of Ewing’s sarcoma”, initiated by the Bone Cancer Research Trust, proved that - and groups are continuing with this development.
So our aims for the coming years are:-
- Reminding General Practitioners, Accident & Emergency front-liners, and general hospital departmental specialists that, when a young person presents with a sports injury or growing pains,they should look beyond the safe option.
- Asking can bone be grown artificially and in sufficient amounts, as it is known that titanium is at a world high price because natural stock are depleting. Plus, monitoring the search for alternatives for constructing prosthetic implants.
- Encouraging epidemiological studies where concentrations of bone cancer incidents are reported.
- Developing a campaign of advocacy under the title “Patient Voice”. This will include lobbying Government and other Agencies on patients’ rights, national research funding inadequacies and important welfare reform issues associated with long-term treatment and physical disability.
Guy Francis Bone Cancer Research Fund is committed to asking questions and raising awareness of the many aspects of bone cancer where research could make a difference to the lives of patients and their families.