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“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 meetings—attended by as many as 100 members—Guilford explained that his motive was the same as McLeod’s: to find the cause of so much death. They ultimately agreed to a contract where only the family, not Guilford, could directly approach members and gather their DNA samples. From there, the gene mapping went quickly, and scientists homed in on CDH1. The gene encodes a protein that normally orients and aligns cells in the stomach; without it, the cells become lopsided, rogue, and possibly cancerous. Any parent with a mutation in the gene has a 50 percent chance of passing it on their children.
This breakthrough meant that a genetic test could now reveal who was at risk of diffuse gastric cancer; the family would no longer have to live in fear of where cancer would strike next. McLeod herself tested negative for the mutation. She was in the clear.
But those who tested positive for the mutation now faced an agonizing new dilemma. Doctors could not guarantee that endoscopies, even annual ones, would reliably catch such an aggressive cancer in time. Total gastrectomies had been performed before, in patients whose stomachs were already ridden with tumors—but never routinely in healthy people who did not have cancer and may ultimately never have cancer at all. So now they had to choose: 70 percent chance of deadly cancer or surgery with a 100 percent chance of significant side effects?
Rangi McLeod, who worked alongside Maybelle in urging relatives to join the study, was the first of the family to test positive for the CDH1 mutation. Not long after, doctors found a tumor in his stomach. It’s not all bad news, Guilford recalls Rangi saying. “I can lead my family to the next stage.” His gastrectomy would no longer be strictly prophylactic, but he’d have his stomach out, he’d recover, and he’d show anyone who still feared the surgery that it was safe. Rangi did not recover, though. He fell into a coma after the new connection between his esophagus and intestine grew weak. He died a few weeks later. “The whole project almost fell apart immediately on the spot,” Guilford told me.
In time, the family decided that Rangi would not want them to stop—he would not want for their children and grandchildren to continue to succumb to cancer. The family found a more experienced surgeon in a bigger hospital in Auckland; the next 10 gastrectomies were a success. Since then, stomach cancers in the family have plummeted. And the hill where the quarry once operated is green again. The land, it turned out, had not been sold but taken by the government, and was returned to the family. In any case, the curse, some in the family said, had finally lifted.
A successful gastrectomy looks like this: For at least the first six months, your life revolves completely around food, and not in a fun way. You eat tiny portions 10 times a day. You have to chew, chew, chew food like gum to make up for the lack of a stomach. Your digestive system spews from both ends. Your blood sugar rises and crashes unpredictably; you faint at the worst times. You are tired all the time. You lose a lot of weight, which might feel welcome at first but then feels scary. You are unable to work for a couple of months—longer if your job is physical or your recovery is difficult. About one in 10 patients has complications serious enough to warrant hospitalization, according to studies done in U.S. hospitals.
Gradually, the upper part of the intestine adapts into a sort of stomach. You start eating larger portions, less often. You gain weight. You still need regular shots of B12, which you cannot absorb without a stomach. But several people more than 10 years out from surgery told me they eat almost normally—with only small, lingering quirks. Plain water, for example, can be strangely hard to drink, possibly because of its surface tension, while flavored water goes down fine. Young women who have had their stomach out routinely go on to get pregnant and have healthy children.
Still, those who ultimately recovered well knew of family members who continue to struggle years later with nausea or reflux or fatigue. One of Paringatai’s cousins left her teaching position because she could no longer physically keep up in the classroom. Another cousin, Isaia Piho, was a firefighter. He, too, switched to a less demanding job afterward. Isaia and his younger brother, James, told me they had watched their mother die of stomach cancer. They are fathers themselves now, and they did not want their children to experience the same.
But not everyone who weighs the odds decides on surgery. Guilford knows another guy, also a firefighter, who decided to keep his stomach. “I run into burning buildings every second day,” he told Guilford. “I’m good with risk.” He’s chosen to have regular endoscopies instead. Still others might prefer not to know that they carry a CDH1 mutation. In the McLeod family, a young man in his 30s who went untested recently died of cancer, Guilford said. Diffuse gastric cancer has remained stubbornly difficult to treat over the three decades he’s been studying it. At the stage when it can be easily detected, the survival rate is just 20 percent.
Doctors are still trying to fully grasp the long-term consequences of losing a stomach, which makes it more difficult for the body to take up nutrients. “We’re learning that gastrectomy severely impacts bone health in the long run,” Daniel Coit, a surgical oncologist who recently retired from Memorial Sloan Kettering Cancer Center, told me. The loss of stomach acid may make calcium harder to absorb. As these patients age and continue to lose bone density, they will be particularly vulnerable to fractures.
Coit, who performed numerous prophylactic gastrectomies in his career, thinks the social and psychological consequences of losing a stomach deserve more attention too. He had one family in which multiple people died prematurely of suicide or alcohol use after their surgeries. Did the procedure lead directly to their struggles, or unmask a previous predisposition? His example is only anecdotal, he said, but the issue should be studied.
This is anecdotal as well, but alcohol also came up repeatedly in conversations I had with people who have been through gastrectomies—without me asking. Either they themselves started drinking too much, or they had family members who started drinking too much.
James Piho told me he drank to numb his fear of cancer and then he drank to numb his depression after his surgery, when he was unable to provide for his daughter. James actually works in a drug-and-alcohol rehab center, and his experience had him wondering about a link between total gastrectomy and alcohol. Could it be psychological or biological, or both? For people who find drinking plain water uncomfortable, alcohol seems to literally go down easier. And bariatric surgery, in which the stomach is shrunk but not entirely removed, is correlated with a well-documented increase in alcohol-use disorder. Losing even part of a stomach may make patients’ bodies more sensitive to alcohol—two drinks, according to one study, might feel like four. Haupiua Steventon, a member of McLeod’s family who had her stomach removed at 18, got a job at a bar after the long recovery derailed her university studies. “I fell into alcoholism very easily,” she told me. She eventually found her footing and has two kids now, but looking back, she wishes she had been warned about alcohol post-surgery. She wouldn’t have chosen to work in a bar.
In interviews that Paringatai conducted with different generations of McLeod’s family, she observed that some of the younger members struggled more, mentally, post-surgery. The older generation, she told me, had witnessed the deaths of so many “mothers, sisters, fathers, first cousins, children, grandchildren.” Today, young people in the family have not experienced those tragedies firsthand—a sign of progress that nevertheless made the sacrifice of a stomach harder to bear.
“I think we’ll look back one day and we’ll go, ‘Man, I can’t believe that we did such draconian surgery on those people,’” Guilford said. His lab continues to investigate diffuse gastric cancer, with the hope of developing a treatment or drug that makes a total gastrectomy obsolete. In recent years, doctors have become more open to patients choosing surveillance over surgery, especially as it’s become clear that CDH1 carriers with no family history are at lower risk for diffuse gastric cancer—perhaps a lifetime risk of about 10 to 40 percent rather than 70 percent. But even Coit, who is among the more skeptical of surgery, recommends prophylactic gastrectomies for people with a strong family history. Their risk is high. Surgery is the best solution we’ve got. But of course, Guilford said, “people would love to keep their stomachs.”
Paringatai found that some of the Māori took their stomachs home, rather than allow the hospital to discard the organ as medical waste. In Māori culture, she explained, the body is sacrosanct. They wanted to honor the stomach, thanking it for its service. Several buried theirs on their family land. In a way, this is how they can, for now, keep their stomachs.
