The operations were unpleasant and often painful, but they were necessary. And they have eased some of the tremendous burden of worry that had dominated the lives of these women for more than 10 years. Put quite simply, the sisters want to see their children grow up, and this radical surgery will improve their chances.
'As long as I am around for them, walking about, and OK, then it will have been worth it. If it means that I don't have ovaries, a uterus or breasts, then I don't mind,' Mrs Matheson says. She has three children, aged 16, 14 and 13.
Mrs Matheson and her sisters all live in the Grampian region in north-east Scotland and they are members of a 'cancer family'. There is a one in two chance that they - and many of their female relatives - carry a gene that predisposes them to breast and ovarian cancer, increasing the risk to between 60 and 80 per cent.
In their aunts' generation - their father's sisters - five of ten blood-related women died from ovarian cancer; in their own generation, three women have developed pre-menopausal breast cancer. There is evidence that the disease is occurring at an earlier age in succeeding generations of the family.
It is largely due to the determined efforts of Dr Anderson, Mrs Matheson and her late husband, Dr Allan Matheson, that the 33 female members of the family are now under the scrutiny of doctors at the Aberdeen Royal Hospital's NHS Trust. Dr Marion Hall, a gynaecologist, and Dr Alan Johnston, a clinical geneticist (who has now retired), masterminded management programmes specific to the women's needs. Since Dr Johnston's retirement, Dr Hall and Dr Neva Haites, also a clinical geneticist, have continued to develop the work.
For the younger family members counselling begins at 18, when the risks are outlined. Gynaecological follow-ups start at 28, with yearly pelvic examinations, blood tests and scans. Such screening is not foolproof and removal of the ovaries is advised between 35 and 40 for those who have completed their families. Twenty women in the sisters' generation have had this operation.
Annual breast checks, starting at 25, include ultrasound and mammography, but so far the three sisters are the only ones who have decided to undergo preventive, or prophylactic, mastectomies.
These women are lucky because their chance of survival is being maximised. But there are many more like them in Britain. While some may be aware of the inherited risk, others will be ignorant that a preponderance of female deaths from cancer in their families are linked. Radical prophylactic surgery, particularly a mastectomy, is rarely considered by doctors or the women themselves.
Earlier this year, the Independent reported the views of James Drife, professor of obstetrics and gynaecology at Leeds General Infirmary. He argued that society placed too high a premium on breasts so that people viewed mastectomy as 'unacceptably mutilating'. As a result, many women, particularly those with a family history of breast cancer, are being denied an option that could save their lives. The article prompted outrage from women and some doctors who condemned it as 'offensive'. But Professor Drife's comments were directed at women such as these sisters. 'It is an issue that I want to see women discussing. I want to know if it would be an acceptable action for a woman who is depressed and anxious about breast cancer because her relatives have it,' he says.
A prophylactic mastectomy had been idly discussed but not seriously considered by the sisters or those caring for them. Then in 1988, a cousin aged 38 was diagnosed as having breast cancer. Until this point, ovarian cancer had been the overwhelming worry for the family and they had no reason to suspect that the genetic defect also predisposed them to a second type of cancer.
'We tried to keep it all in perspective - after all, the disease is very common in the population. But at the back of our minds was the thought 'Oh God, is this the next thing to worry about?' ' Dr Anderson says. It was. Within the year a fourth sister developed a breast tumour at the age of 35, and then a month or two later, another cousin was diagnosed with the disease.
Suddenly, mastectomy seemed the only option. Breast examinations and mammograms were not enough to reassure the sisters. Dr Anderson and Mrs Matheson had already had suspicious lumps investigated in the past. 'It got to the stage where we could not get through six months without something cropping up. We just wanted to get away from all that perpetual worry,' Dr Anderson says.
There was a strange sense of fear and fascination, she recalls. 'In our grandmother's generation the diseases were occurring in women in their sixties; in the next, in their fifties and forties; in our generation, it was the thirties. The stress of living with it was terrible. The knowledge about ovarian cancer had taken its toll, but we had dealt with that and given each other lots of support. But could we deal with this?'
With the full approval of their families, the three sisters decided to have mastectomies. This major operation required a week's stay in hospital and caused severe pain at times, Dr Anderson says. The surgeon opened the flap of skin at the side of the breast and shelled out the contents. A silicone implant was then inserted through the flap and muscle brought around to support it. 'The nipples were not removed and they do pose a risk, but it is very small,' she says.
The sisters are a remarkable trio, who have lived with the threat of cancer and with illness and death among their close relatives for more than a decade, yet appear untouched by it. They agree that being a medical family has enabled them to talk openly about it. 'We tried to adopt a practical approach, that this was a problem which had to be dealt with as best we could,' Mrs Matheson says.
It was in 1978 that they first became aware of a cancer risk. 'Three aunts in their mid-forties - all married with children - developed ovarian cancer in the space of eight years. It seemed too much of a coincidence,' Dr Anderson says. 'Although there had been a background of cancers in the family before that, they had tended to affect older people and hadn't seemed unusual.' She set about learning what she could about familial ovarian cancer.
At the same time Mrs Matheson and her husband tried to find out what various female members of the family had died of.
To draw up a family tree is, for most people, an enjoyable task which reinforces a sense of continuity of family. For Hazel Matheson it became a macabre venture, as she searched out the cancer deaths. She and her husband delved back three generations, their task made a little easier by the fact that most of the family had remained in the Grampian region.
During this time, another cousin developed the disease and died. 'What we were finding out was very alarming, and all the time people were ill or becoming ill all around us. It was scary and interesting at the same time, but it did provide the impetus,' Mrs Matheson says.
Eventually, she set up a regular newsletter for the family to keep them informed. The reaction of their female relatives - mainly mothers with young children - was mixed. At first they were understandably scared but anxious to learn more. That was followed by anger and denial of any problem, Patricia Fraser, a farmer's wife, remembers. 'One cousin felt she could not go through with an operation to remove her ovaries. She kept putting it off. She developed ovarian cancer and died when she was 42.'
The sisters pray that their efforts will prevent any more unnecessary deaths from cancers in their own family - and perhaps others. It was one of their reasons for agreeing to the interview with the Independent. They urge all family doctors to consider the possibility of familial cancer when they see a patient with suspected breast or ovarian cancer.
'In our case, five members of the family were treated in the same hospital within eight years; two were patients from the same general practice and died in the same year, but nobody was able to make the link outside the family,' Dr Anderson says. Confusion over maiden and married names was a key factor.
Mrs Matheson says another reason for speaking publicly about their cancer family is that it is vital that all women who are at risk know that prophylactic surgery is now an option that could save their lives. 'What we have gone through has been a very positive thing, really,' she says.
SCIENTISTS CLOSE IN ON THE GENE
THE GENE responsible for inherited (familial) breast and ovarian cancer is one of the most sought-after in cancer gene mapping. Now British geneticists are hopefully 'just months away' from the goal, according to Dr Neva Haites, a senior lecturer in clinical genetics at Aberdeen University, and one of the doctors responsible for caring for the sisters.
The defect, which can be carried by men and women, occurs on chromosome 17 and almost certainly involves a tumour-supressor gene, she says. 'Carriers will have one healthy gene and one damaged copy that doesn't function. If their healthy gene is also damaged - something that can happen at any time - their risk of cancer is increased.'
In Hazel Matheson's family, the disease is occurring at a younger age with each succeeding generation, a phenomenon known as genetic anticipation. 'We are not sure why this happens, but it may be an environmental influence which is changing the character of the gene. Their grandmothers had larger families and could not have taken the Pill, for example,' Dr Haites says.
Once the gene is identified, it will be possible, with a blood test, to distinguish with certainty those family members who are carriers and need high-risk management, from those who do not have the gene.
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