Radical surgery for killer gene
Inherited: Deborah, Joseph and Rebekah Baptista and Jessica Paiva. Picture: Ian Munro/The West Australian

Three Perth sisters have taken the extraordinary step of having most of their large intestine removed to avoid the cancer time bomb running in their family.

Even more remarkably, they now enjoy near-normal lives, thanks to minimally invasive surgery at St John of God Hospital in Subiaco where they had their colons removed in a race against time to beat bowel cancer.

Like their father Joseph, student Rebekah Baptista, 18, and sisters Deborah Baptista, 25, and Jessica Paiva, 31, have familial adenomatous polyposis, an inherited bowel cancer syndrome that causes hundreds of tiny polyps to form in the large bowel by the late teens.

If untreated, inevitably some of the polyps turn into cancer, usually by age 40.

Fourteen years ago, doctors discovered Mr Baptista carried the gene mutation that causes FAP and did radical bowel surgery. His daughters had a 50 per cent chance of having the same abnormality.

Each tested positive and started having regular colonoscopies from age 17 as doctors weighed up the best time for surgery.

They did not want to operate when the girls were too young but the longer they held off, the more likely the girls would get cancer.

Deborah had surgery in 2009, Jessica in 2011 and Rebekah two weeks ago.

Under the careful eye of colorectal surgeon Greg Makin and gastroenterologist Luca Crostella, most of their large intestines were removed, leaving only the final section, the rectum, which was cleared of polyps and reattached to the small intestine.

Mr Makin used keyhole surgery that needed only four small cuts in and around their belly buttons.

"Most nutrients are absorbed by the small intestine, so you can function pretty well without the colon, you just go to the toilet a bit more often," he said.

"It's still a big step but we know that without preventive surgery they had virtually a 100 per cent chance of getting cancer."

Though the sisters will have to be monitored and face another operation when they finish having children, they are hugely relieved.

Jessica, who has three young sons, said the hardest time was watching Rebekah go into hospital a few weeks ago.

"We have all stressed for each other but she's only 18, so we worried even more," she said.

Deborah said that when they looked at the risk of getting cancer, it made surgery seem a no-brainer.

"You don't want to get cancer, so it was a pretty simple choice for all of us," she said.

The West Australian

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