Two weeks ago, Dr. Dan Hall looked around his intensive-care unit at Children’s Hospital and realized there were four babies born with their intestines outside their bodies.
When Hall started in medicine three decades ago, so many babies with that condition – gastroschisis – would have been unheard of. And unthinkable.
Now, it is hardly unusual.
Researchers are still putting together definitive numbers on how many babies are born with the condition.
But doctors in Colorado report they have seen a frightening and baffling increase in a condition that, while not fatal to about 90 percent of babies who get it, has no known cause.
One of the doctors, at the University of Colorado Health Sciences Center, recently received a federal grant to investigate what could be behind the increase.
So far, researchers can say with certainty only that the malady is not genetic, that most babies can lead normal lives if treated and that it is more than three times as likely to occur in mothers 20 years old or younger.
At the same time, pediatricians and obstetricians from Washington state to South Carolina, from British Columbia to Australia are reporting similar increases in the number of cases.
In North Carolina, a study in the early 1990s found that out of every 10,000 babies born in that state, 1.4 had gastroschisis. A decade later, the number was 4.49 out of every 10,000, said Shilpi Chapra, a pediatrician at the University of Washington in Seattle.
In Canada, the rate grew from 1.85 per 10,000 between 1986 and 1990 to 4 per 10,000 between 1996 and 2000, Chapra said.
“People are calling this an epidemic,” she said.
She doesn’t disagree.
In the past couple of years, Chapra has crossed the country presenting papers on the rise of gastroschisis. The response is universal: “People come up to me and tell me, ‘We need to do something about this. We have five cases right now.”‘
Chapra returned to her Seattle office after one presentation and found a phone message from the Centers for Disease Control and Prevention.
CDC researchers are trying to measure whether gastroschisis has become more common – and wanted her help. A number of states, including Colorado, are contributing data.
Chapra’s interest in the condition began during a fellowship at the University of Kentucky.
“I had never seen so many gastroschisis cases – over 15 months, I saw 19.”
Nurses at the university hospital wrote it off as a rural phenomenon, she said.
But when Chapra looked deeper, she found that wasn’t the case.
Searching for reasons
Researchers are convinced gastroschisis is not genetic – but they’re struggling to determine its cause.
Instead, babies with gastroschisis had been born to mothers from 24 different counties, urban and rural.
Counting the cases is one thing. Explaining the apparent increase is quite another.
Researchers are convinced the condition is not genetic. It seems to strike male babies and female babies in equal proportions.
Researchers theorize that gastroschisis happens because something inhibits growth of the baby’s blood vessels, and they have compiled a long list of possible culprits. Cigarettes, pesticides and methamphetamine are on the list, but so are such seemingly innocuous things as aspirin and Sudafed.
“It’s not something biological. It has to do with environmental factors – either drugs or nutritional issues or both,” said Hall.
Chapra believes that whatever the environmental trigger may be, the timing of exposure to it is critical.
She points to one study in which mice were given aspirin on day eight of pregnancy. There was no increase in gastroschisis among their babies.
But when mice moms-to-be were given aspirin at nine days, there was an increase, she said.
The only characteristic that links most babies with gastroschisis is each mother’s age. “The incidence is much higher in mothers under 20,” said Regina Reynolds, a CU neonatologist.
Reynolds, who started noticing the increase in gastroschisis cases at CU several years ago, has been awarded a grant to study what might be causing the increase.
Onieda Lohman, 20, is one of those young moms.
Lohman’s baby, MaKenzie Loh man Herdt, was born June 9 at University of Colorado Hospital. That she came eight weeks early and weighed a little over 2 pounds was a surprise. That she had gastroschisis was not.
Lohman was 12 weeks pregnant when her doctor said an ultrasound showed something was not quite right: gastroschisis.She went home and cried. Then she got on the Internet, and much of what she found reassured her: Roughly 90 percent of babies born with the condition survive.
“Obviously, it’s a tough diagnosis,” said Dr. Henry Galan, director of obstetrics at CU, who works with high-risk pregnancies.
Galan said it’s important to emotionally prepare parents who are expecting a baby with gastroschisis, because the condition is so visually disturbing and the parents tend to be very young.
He even shows them a medical-textbook picture of a gastroschisis baby – “if I think they can handle it.”
And he emphasizes the positive: “It’s not without risks, but it’s fixable,” he said.
That’s what doctors told Loh man. “They said, ‘Of all the birth defects, this is the one to get,”‘ she said, holding MaKenzie in a Children’s Hospital room as MaKenzie’s dad, Adam Herdt, looked on.
Usually, prenatal tests alert doctors when a woman is expecting a baby with gastroschisis, which allows doctors to closely monitor the pregnancy.
Intestines form outside the body in all babies. Then, at about 10 weeks’ gestation, the abdominal wall closes around the gut. In babies with gastroschisis, that doesn’t happen.
The intestines can function while the baby is in the womb, although not as efficiently as they should, and gastroschisis babies often are small, Galan said.
Once the baby is born, the exposed intestines need to be protected, not only from infection but from twisting that could cut off the blood supply.
“In a significant percentage of cases, the kids are born and we can take them right to the (operating room) and put the intestines back in and close it right up,” said Michael Alls house, a pediatric surgeon who operates on babies with gastroschisis at Children’s Hospital.
But in some babies, especially premature babies, putting the entire intestine in at once would cause too much pressure, making it hard for the baby to breathe, he said.
For those babies, there is another option – a silo. The silo is a cylindrical covering applied to the exposed intestine. Over time, it helps ease the gut inside the baby.
Babies typically stay in the hospital four to six weeks, depending on how well they are able to digest food, Allshouse said.
At five weeks – and after two surgeries – MaKenzie is improving steadily, taking milk through a tube in her nose and actually eating three times a day, Lohman said.
Once the babies go home, the long-term outlook is good, Galan said.
“If there aren’t any significant bowel complications, they probably end up leading a normal life,” he said.
Allshouse said he warns parents that for the first year, their babies may be fussier than normal, because their stomachs aren’t always working smoothly.
Lohman has no idea what might have caused MaKenzie to have gastroschisis. She just knows that her baby is getting better, and she can’t wait to take her home.
“We’re lucky,” Lohman said, smiling at her daughter. “And we’re happy with what we got.”
