Roughly 30,000 women in the UK today are running an abnormally high risk of breast or ovarian cancer. They may suspect it, but most of them do not know for sure because they have not, whether through anxiety or lack of awareness, undergone genetic testing. A morbid fear has been handed down the generations; many of them have great-grandmothers, grandmothers, mothers, sisters and aunts who developed cancer. Last week, two male doctors told these women that the best way to prevent themselves from succumbing to the disease was to have both their breasts and their ovaries removed.
What other parts of the body physically define a woman's gender in the way that the breasts and the ovaries do? And yet many healthy women have already had this mutilating surgery, and said unreservedly that it is a huge relief not to have to live with the threat of cancer any more. They say they have no regrets.
Tracey Barraclough, 42, is one. Four years ago she found she had inherited the mutation in the BRCA1 gene that caused her mother's - and probably her grandmother's, and her great-grandmother's - deaths from ovarian cancer. It meant that she had up to a 60% chance of getting ovarian cancer herself and a 50-80% chance of breast cancer. She wasn't prepared to live with those odds. "My mother suffered dreadfully," she says.
Having her ovaries removed was not so hard, she says. She already has a child, seven-year-old Joshua. But then the doctors told her about her chances of developing breast cancer - still very high, despite being reduced by the ovarian operation. It took her a year to make up her mind.
"It was very lonely," she says. "Only I could make that choice. They were telling me statistics of what might be and it was up to me."
In the end, Barraclough chose to have her breasts removed and reconstructed - and she's glad she did. There has been no backward glance, no crisis of identity. Although there were a few dark days, being a woman, she says, "is about more than boobs".
Improvements in breast reconstruction techniques have helped make going under the knife less daunting - after all, large numbers of healthy women have had successful cosmetic implants. On the ovarian side, ovaries cannot be seen, and hormone replacement therapy exists. Yet nobody could call this an easy choice. It is hardly surprising if some women push the idea of a gene test into a dark, unvisited corner of their mind: surgery to remove healthy parts of your body for fear that they may later become diseased is a shocking thing to consider.
"It is a huge thing for a woman to go through and you need an awful lot of counselling," says Michelle Barclay, policy manager at Breakthrough Breast Cancer. "I met one woman who didn't have enough counselling and was being wheeled into the operating theatre when she had a massive panic attack. She didn't go through with it."
Drs Paul Harkin and Richard Kennedy, from Queen's University, Belfast, issued their advice on the basis of sound science and statistical calculation. The removal of both breasts decreases a woman's chances of breast cancer by 90% - although not 100%, because some breast tissue remains. Removal of the ovaries decreases the risk of ovarian cancer 24-fold. The bald facts sound convincing for the small proportion of women carrying a mutated BRCA1 gene, but in practice there is more to mull over.
This, for instance. While women with the mutated gene stand a high chance of getting cancer, they are no more likely to die of it than any other woman who gets cancer, and the death rate from breast cancer has been steadily dropping.
And this. Screening methods in some countries are more advanced than in the UK. Having more than one mammogram a year is not generally regarded as a good idea, because the radiation emitted can itself cause potentially cancerous DNA damage. But in the US and some parts of Europe it is now possible to have MRI scans instead of mammography, and a study in the UK is looking into this possibility.
Siroos Mirzaei, of the Institute of Nuclear Medicine at Wilhelminenspital, Vienna, and three other doctors from Texas, wrote to the British Medical Journal last year, arguing that breast removal to prevent cancer should be a last resort. "We do not recommend prophylactic mastectomy, because with new developments in the field of mammosonography and new devices such as the Mammotome [which enables a precise biopsy of suspected breast lesions] and nuclear medicine techniques, the detection of breast cancer in its early stages is becoming increasingly possible," says Mirzaei.
"Greater efforts should be taken to find new techniques as an alternative to prophylactic surgery, especially in young women who are often faced with this serious decision."
Unfortunately, regular trips to Vienna or the US are not an option for most. Michelle Barclay says the latest screening technology is very expensive and will not be quickly available to those who rely on the NHS. On the other hand, "Breast surgery is very expensive too," she says - and not only the removal, but also reconstruction and aftercare.
In the meantime, those women in families with a history of breast or ovarian cancer need the best possible information and support to deal with what has become one of the 21st century's greatest dreads. Gerald Gui, consultant breast surgeon at the Royal Marsden hospital in London, says it is "a very difficult area for both clinicians and their patients".
He recommends that women with a family history of the disease should consult a multidisciplinary team of experts before they make up their minds - geneticists, psychologists, specialist nurse counsellors, specialist surgeons. And because the condition is rare - affecting only 5% of women - they should be cared for in specialist centres where all of this help is available. Surgery, if it is the final choice - and Gui is at pains to point out that it is not always the right choice for everyone - should be carried out "by specialist surgeons who have the experience and the understanding of the condition and problems that arise".
