The Nebraska Medical Center, a leader in the nation in intestinal transplants, is succeeding at finding ways to avoid them.
Children from around the nation and even other countries are using the Nebraska Medical Center’s intestinal rehabilitation clinic to get enough nutrition, escape transplantation and in many cases lead fairly normal lives.
Take Gabe Ferguson, a 13-year-old boy who lost much of his small intestine 10 years ago from surgery for an abdominal tumor. Gabe, whose family moved from Georgia to Papillion to be closer to the clinic, is free of the tube feedings and intravenous line through which he had been nourished for years.
“I would have probably died if I hadn’t come here,” Gabe said during a recent appointment at the intestinal rehab clinic.
Cases like his are complicated and difficult. Many of the children who are born with short small intestines or who have lost intestines to disease and surgery will have challenges all of their lives to get enough nutrition or to get off medical support. But the med center’s clinic has made progress in the past 10 years in helping many of the children survive and enjoy quality lives.
Improvements have come gradually with accumulated findings. The strategies range from surgeries to improve the small intestine’s performance and changes in philosophy to nutritional advances and infection control.
Only a few years ago, some clinic patients might have died, or been moved immediately to intestinal transplantation, which has mixed results.
The clinic doesn’t compete with the Nebraska Medical Center’s transplant program. The head of the pediatric intestinal rehab program is a transplant surgeon, Dr. Dave Mercer. The med center still does many intestinal transplants — more in 2013 and 2014 than any hospital in the nation.
But the intestinal rehab clinic offers a fresh approach, making it less likely that transplant will be the first option. The clinic’s caseload is growing and has included children from Saudi Arabia, Kuwait and Peru.
Among the young patients at the clinic on a recent Tuesday was Logan Hartstack, an energetic 6-year-old.
“How’s the eating going?” Mercer asked Logan’s mom, Phyllis Coverdell. She and her husband, Brandon Coverdell, moved their family of five to Omaha from New Market, Iowa, to have better access to the clinic’s staff.
“Pretty well,” she said of Logan, a patient of the intestinal rehab clinic since about 6 months of age. Logan was born with gastroschisis, which means part of his intestine was outside his body at birth. Much of his small intestine was dead. Logan’s skin became yellowish over time.
Today he’s a spunky, hyperactive boy. He still gets part of his nutrition through a central line in his upper chest, receiving nourishment directly into his bloodstream. This is hard on his liver, because many nutrients have a toxic effect when given intravenously.
He also has a tube into his belly, a conduit for nutrition that is easier on his liver, because those nutrients go into his intestines, and his natural secretions work on them. And he eats. This combination pushes enough calories and nutrients into his body despite his compromised small intestine.
Logan wore black-and-green glasses — which he calls his “Ninja Turtle glasses” — and black Converse basketball shoes, jeans and a T-shirt with the word “Sublime” on it for a recent visit to the clinic. He was soon immersed in a smartphone and hardly listened to what was going on.
“I need one of your poops, OK?” Mercer said to him.
“Why?” Logan asked.
“Because I need it,” Mercer said. “It’s for science!”
Mercer, a 44-year-old Canadian-American, has a relaxed manner, rolling a ball back and forth with a baby, talking about video games with kids and joking with their parents.
He has headed the pediatric intestinal rehab clinic for seven years. Mercer travels around the country, telling intensive-care personnel and physicians that short-bowel syndrome shouldn’t be viewed as a death sentence and that the Nebraska Medical Center can help many kids with it.
When Gabe Ferguson’s “G-tube” was removed a couple of years ago, Mercer let Gabe take a hammer to it in the clinic. At a recent checkup, Gabe and Mercer sat knee to knee and talked about video games titled “Call of Duty: Zombies” and “Star Wars Battlefront.”
Mercer, who also performs pancreas, liver, kidney and small intestine transplants, said that 20 years ago, many of the children getting care in the clinic would have died.
“The change has been dramatic, especially in the last decade,” Mercer said. “It’s a philosophical change right upfront.”
Although the Nebraska Medical Center did a total of 40 intestinal transplants in 2013 and 2014, its numbers have declined in the 5-and-under age group, when many intestinal transplants are typically done.
In the three-year stretch from 2012 through 2014, the med center performed 25 intestinal transplants in children 5 and under. Before that, the last three-year increment in which the med center did so few on young children was 1994 through 1996. The hospital performed a high of 26 in that age group in 2008 and only seven last year. And the med center is seeing far more children in its intestinal rehab clinic than before.
The clinic got its start 15 years ago and saw only 10 to 15 kids. The clinic treated 35 to 40 new children last year, said Brandi Gerhardt, clinical nurse coordinator for the pediatric intestinal rehab program.
The advances have been gradual and have been made on multiple fronts. Dr. Alan Langnas, chief of transplantation at the med center, describes them as “baby steps” that push the science ahead.
Med center doctors and staffers published a paper in the November 2013 issue of The Journal of Pediatrics showing that even children with “ultrashort small bowels” could be treated and saved with appropriate therapies.
The advances in their care include:
Patience — Many children with diseased and short bowels immediately went on the list for transplantation only a few years ago. Now the med center waits, works with them through various strategies of nutrition intake and puts them on the transplant list as a last resort.
Surgery — A form of surgery called the STEP procedure cuts and staples the small intestine to lengthen and narrow it, so that nutrients are more likely to caress the interior wall of the gut and be digested and absorbed in the process. Med center officials believe that they may do more STEP surgeries than any hospital in the country.
Diet and nutrition — Although the clinic uses the central line through the chest for many children, staffers try to get them off that method of delivering nutrition, called total parenteral nutrition, whenever possible. It can damage the liver.
Alternatively, the clinic pushes formula or other nutrients through a G-tube placed in the stomach. Increasingly, the clinic encourages pushing “blenderized” diets with a meaty and starchy base into the G-tube. The nutrients may include chicken, quinoa, oatmeal, safflower oil or other items that the patient can tolerate.
And the clinic pushes feeding by mouth (even bacon and Cheetos, if the child wants them), which can be challenging in those who have had multiple abdominal surgeries as babies and know nutrition as a thing that leads to diarrhea or vomiting. The more a child can consume by mouth, the less reliant he will be on the intravenous feedings and G-tube nutrition.
Avoiding infection — Infections through the central line in the chest are perhaps the greatest threat to the children. Strategies to avoid such infections — including an “ethanol lock” that uses an ethanol solution to prevent such infections — have been devised over the past few years.
The clinic has developed a reputation nationwide.
“I would say that the Omaha program is one of the few on the forefront of offering both intestinal rehabilitation and successful intestinal transplant,” said Dr. Sherilyn Gordon Burroughs, a transplant surgeon at Houston Methodist in Texas. “And that’s because they have a long experience in both.”
Mercer said other hospitals with solid pediatric intestinal rehab programs include Boston Children’s Hospital, Cincinnati Children’s and UCLA.
Avoiding transplantation saves organs for other patients who need them more. Also, intestinal transplants have a lower five-year survival rate — about 55 percent — than heart, kidney, liver and pancreas transplants.
Kaylip Franks brought his baby son, Anakin, to the rehab clinic from Oklahoma and fully expected transplantation. “It was kind of like a mind-blowing thing when they told me all these different things they could do,” Franks said. Anakin underwent a STEP surgery, among other therapies.
Anakin, now 1, no longer has the central line in his chest but does have the G-tube in his belly. “He’s doing wonderful,” Franks said. “I thought we were on our last limb.”
Desmond Brown, a 4-year-old from Louisiana, was born premature with only a tiny length of functional small intestine. His grandmother Sandra Ryan says staffers at a hospital in their home state suggested that Desmond would not make it. They asked her to sign a do-not-resuscitate order and sent in a pastor.
Desmond was treated at the Nebraska Medical Center, and now he’s back home in Shreveport and doing well.
Many young patients, however, endure roller-coaster rides of hope and disappointment. Braxton Anderson, 6, still has the central line in his chest and the G-tube into his stomach.
At a recent appointment, his mother, Cassie, said Braxton, who wore a black cowboy hat, had eaten two sausage burritos at McDonald’s the day before.
“That is glorious!” Gerhardt said.
But Braxton, the smallest kid in his kindergarten class, had lost 4 pounds in the previous few weeks. That’s a lot of weight for a 42-pound boy.
As it turned out, Braxton, of Callaway, Nebraska, had a central line infection, which was treated at the med center. Soon he was putting on weight. “His appetite has really taken off in the past couple weeks,” his mother said.
The clinic helps show parents how to care for their children’s central lines and G-tubes. It tells them the warning signs of infection and orders them to take a child showing those signs to a hospital immediately. Some families also use home care services.
Khloe Morris, 4, had a bowel disease and lost most of her small intestine shortly after birth. The girl, from Banks County, Georgia, was referred to the clinic about three years ago. Mercer said her liver function has improved and her nutrient intake is good enough to consider taking the central line out of her chest this summer.
“If this was the early 2000s, you would have said, ‘We have to do a transplant,’ ” Mercer said of Khloe. “Even in the mid-2000s, we probably would have transplanted just based on the anatomy.”
Gerhardt said private insurance is generally reliable when it comes to the clinic’s services, although families with commercial insurance often must pay for meals and lodging. Medicaid reimburses the clinic for its work, but approval from some out-of-state Medicaid programs can take months, Gerhardt said.
For Sandra Ryan, the med center’s intestinal rehab program was the godsend she longed for when she was asked, along with her daughter, to sign do-not-resuscitate orders for Desmond. The boy’s progress through visits to the clinic has been incredible, Ryan said by phone.
“Honey, it’s the greatest,” Ryan said of the clinic. “It it wasn’t for them, I don’t think Desmond would be here to this day.”
At his fourth birthday party a couple of weeks ago, Desmond ate a chili dog and a cupcake.