The Family That Decided to Have Their Stomachs Removed - The Atlantic

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This article was featured in the One Story to Read Today newsletter. Sign up for it here.<br>“What do you mean, you just take the stomach out?” Karyn Paringatai wondered, when doctors first said her stomach had to be surgically removed. Could she still eat? Yes, but differently. What would replace it? Nothing. She would have to live the rest of her life missing a major organ.

Paringatai was not actually sick, not yet. Her stomach was fine. But her cousin, just a few years older, had recently died of an aggressive stomach cancer at age 33, leaving behind three children. In a video recorded after her diagnosis turned terminal, the cousin told her little kids to be good for their father. “Please don’t be too mean to the lady that he meets,” she added, anticipating how the void left by her death might be filled. But she must have known that this void could not be filled, not ever. The cousin’s own mother had died young of stomach cancer. So had her grandmother. So had her sister.

To the doctors who saw Paringatai’s cousin in Tauranga, New Zealand, this pattern was hauntingly familiar. Her cancer was an unusual and distinct kind called diffuse gastric cancer, in which cancerous cells percolate undetected through the stomach, forming obvious masses only in advanced stages—usually too late to treat. The doctors had witnessed the same rare cancer run through a large Māori family near Tauranga. In that family, one woman lost six of her siblings to stomach cancer; a boy had died at 14. The family now reached out to Paringatai’s. It’s genetic, they said. You have to get tested.

Paringatai, whose father was also Māori, got tested. And indeed, she carried a mutation in the same gene, known as CDH1, as the other family. This gave her a 70 percent lifetime risk of developing advanced diffuse gastric cancer. Because this form of cancer can metastasize so quickly and unpredictably, the only surefire method of prevention is a complete removal of the stomach, or total gastrectomy. It’s analogous to a preventive mastectomy for breast cancer—but far more physically taxing. A number of women with a CDH1 mutation have actually had both their stomach and breasts removed because this mutation can also confer a 40 percent risk of breast cancer. One of them told me, about her gastrectomy, “If you can do that, you can do anything.”

Paringatai’s surgeon could not answer all of her questions about living without a stomach—her total gastrectomy was the first he would ever perform in a healthy person. But she went through with the procedure in 2010, and she credits it with saving her life. In the operating room, her surgeon made a long incision down her abdomen, cut out the fist-size pouch of her stomach, and stitched her esophagus to her small intestine. She was the first in her family to have her stomach removed prophylactically. Others followed. On a recent trip to visit her father’s family, Paringatai found herself sitting on a porch with her aunties and cousins. Of the eight people there, she realized, only one still had a stomach: her partner. “You’re the odd one out,” she teased.

Mutations in CDH1 seem to be unusually prevalent in Māori families, where they arose multiple times, possibly because they once conferred some evolutionary advantage. But mutated versions of CDH1 have been found around the world too, and thousands of patients have likely now had gastrectomies to head off cancer.

In New Zealand, “we’re coming up to nearly 30 years of people living with no stomachs,” says Paringatai, who is now a Māori-studies professor at the University of Otago. For the past several years, she has been documenting the experiences of Māori with CDH1. That people can live this long without a stomach is a testament to the adaptability and resilience of the human body. That doctors resort to such radical measures exposes the limits of what modern medicine can offer.

The first Māori to undergo prophylactic gastrectomies were the family that warned Paringatai’s about the cancer gene. They knew all about the gene because they had helped find it. Back in 1994, Maybelle McLeod contacted a genetics lab at the University of Otago about the premature cancer deaths stalking her relatives. Among themselves, she told me, “nobody even talked about it.” The family believed they lived under a curse for letting their land be sold for a quarry. McLeod grew up listening to the quarry’s warning sirens, learning to take cover indoors before the blasting began. She watched as the hill near her home was stripped bare.

McLeod eventually moved away, became a nurse, and learned of the then-nascent field of cancer genetics. This, she thought, explained the so-called curse. The geneticist she contacted, Parry Guilford, agreed to take the case. But her family still had to be persuaded to trust this pakeha, this white man, with their DNA. Over a series of...