All babies spit up.
That’s what Nolan Miller’s parents thought after they brought him home Sept. 20, 2003, two days after he was born. But Nolan wasn’t just spitting up a little milk. Nolan was vomiting. Constantly. Then he stopped having bowel movements.
“He took a bottle and it all came right back up,” says Nolan’s father, Matthew. “Then it kept coming.”
A systems engineer for Methodist Hospital, Matthew telephoned a local hospital. The service’s on-call pediatrician advised that he and his wife, Amy, take Nolan to the emergency room. Still vomiting after they arrived, Nolan was kept in the emergency room while physicians conducted a series of tests.
“That was one of the longest nights in our lives,” Matthew says.
Nolan was admitted and the battery of tests continued, yet still no cause was found. Various diagnoses were considered but none was a clear-cut answer. Nolan was placed in full isolation at the hospital’s Neonatal Intensive Care Unit. Two days and more tests passed and still no answer.
“They told us the tests showed nothing wrong,” Matthew says. “They told us to prepare ourselves … that we were going to lose him.”
Matthew and Amy refused to give up hope.
Acting on the advice of a friend, they requested a call be put in to a local pediatric neurologist, who spent all night reviewing Nolan’s test results before suggesting the attending physicians reevaluate exploratory surgery. A pediatric surgeon was brought in, and the operation revealed that Nolan was suffering from Necrotizing Entrocolitis, a rare condition that most often affects premature babies (Nolan was full-term) and had escaped detection on the X-rays and previous tests.
With Necrotizing Entrocolitis (NEC), bacteria had begun to eat away at Nolan’s small bowel. The formula he was fed would go only so far into his bowel, then be forced back up, causing him to continuously vomit.
The diseased bowel was removed and the Millers were advised a second surgery in six to eight weeks would be needed to see if the bowel portion that remained had healed. During that second surgery, doctors found the bowel had not healed and additional small bowel was removed. Nolan was left with approximately 34 cm of his small bowel – approximately one-fifth of the 150 cm he had at birth.
The resulting condition, known as Short Bowel (Gut) Syndrome, required that Nolan be fed 24/7 via a gastrostomy-tube and a second central line for Total Parenteral Nutrition – a fluid mixture that can include a combination of sugar and carbohydrates, proteins, lipids, electrolytes and trace elements. Because TPN bypasses the stomach and liver, dependence upon it for a prolonged period of time can lead to liver damage or failure.
Though his vomiting continued, the rate of his feedings was gradually increased, and Nolan was released from the hospital December 22, 2003.
At 9 a.m. the following day, Nolan and the Millers, exhausted from the first night at home dealing with feeding pumps and continued vomiting, met with the Intestinal Rehabilitation Program team at The Nebraska Medical Center.
The Millers found that everyone in the room shared a common goal – to one day wean Nolan off TPN and the feedings.
And that gave them hope.
Whether the cause is disease or massive surgical resections due to any of a number of conditions, children and adults with small bowel syndrome or intestinal failure are more than uncomfortable.
They can’t eat.
TPN and gastrostomy or G-tube feedings are treatments but they aren’t to be considered permanent solutions – unless there is no alternative.
Determining and implementing the options is the role of the Intestinal Rehabilitation Program team at The Nebraska Medical Center.
The program is among the few comprehensive centers in the United States and the only one in the Omaha area to offer a complete range of medical and surgical interventions for the management of adult and pediatric patients with complex intestinal disorders.
The goal of the program is to restore enteral autonomy and to decrease dependence on TPN through diet, medicine and/or surgery.
Debra Sudan, MD, transplant surgeon, is a professor of surgery at the University of Nebraska Medical Center (UNMC). She also serves as director of the Living Related Transplant Program and as surgical director of the Intestinal Rehabilitation Program at The Nebraska Medical Center.
Dr. Sudan is considered to be an expert in the Bianchi and serial transverse enteroplasty procedures (STEP), two surgical procedures designed to lengthen intestines when diet and medications are not successful.Bianchi is an intestinal lengthening procedure that involves dividing the bowel in two halves then reconnecting the halves in series with the rest of the small intestine.
STEP involves stapling and dividing the bowel transversely on alternating sides to decrease its width and increase its length. A resulting dilatation or widening of the lengthened bowel may help enhance the intestine’s ability to absorb nutrients naturally.
Dr. Sudan says that of the 77 lengthening procedures performed at The Nebraska Medical Center, 37 have been STEPs. That represents quite an accomplishment, as fewer than 50 such procedures have been reported in the English literature to date. The patient survival rate is over 90 percent, she says.
“We’ve published more regarding these procedures,” she says, “and I am not aware of anyone who has done more.”
Small bowel transplantation is considered a final option for patients who require life-long use of parenteral nutrition and have developed complications. The Nebraska Medical Center is recognized internationally for its Small Bowel Transplant Program and is one of the busiest programs in the country. The program was the second of its kind when created in 1990 and today is among only 10 in the nation. In 2006, surgeons conducted approximately eight lengthenings and 25 transplants – for a total of more than 200 transplants since the program began, Dr. Sudan says.
A member of the liver transplant team since 1996, Dr. Sudan says approximately two-thirds of all bowel transplants also require a liver transplant. The organs must come from the same donor, and in the case of children, most of the available donors are larger than the infant or the very young patients.
While it has been difficult to locate donors in the past, the numbers are improving because of changes made to the way donated organs are allocated.
“It’s been shown that there is a higher mortality rate for those suffering from intestinal failure than for those on the liver transplant waiting list,” Dr. Sudan says. “People are beginning to place organs off the intestinal transplant list, and there is a greater effort to place more organs out of each donor, because we know there are more options available for those on the liver waiting list than for those awaiting a bowel transplant.”
The Intestinal Rehabilitation Program at The Nebraska Medical Center provides options to those who clearly are not in need of a transplant yet must cope with the inability to absorb nutrients through means other than TPN and feeding tubes.
Dr. Sudan says that of the approximately 90 people – nearly three-quarters of them children – who have been treated by the Intestinal Rehabilitation Program, roughly half have been weaned from TPN completely. “And the long-term survival of those we have weaned has been excellent.”
The intestinal rehabilitation team includes gastroenterologists, surgeons, nurses, dietitians and infectious disease specialists who work closely to limit complications of TPN, reduce the risk of infections, improve absorption of nutrients and end dependence on TPN.
The family-centered care that is the core of the program has helped attract parents of infants and small children from around the country. “The care they receive here makes a huge impact on the program,” she says. “Patients repeatedly tell me they visited other programs in other cities, then they came here and there was no doubt. They feel more confident here because of the team we have in place.”
Dr. Sudan says intestinal rehabilitation is a demanding field but one where the successes are tremendous for everyone involved. “In one week we stopped TPN on four kids,” she says. “All four had come to us likely to need transplants, and yet here they were, completely weaned from TPN without surgery.
“For me, that was one of the best things. It’s incredibly rewarding.”
The very first meeting with the intestinal rehabilitation team, the Millers knew they were in the right place.
“They were really optimistic about Nolan,” Amy says, “and we needed to hear that.”
In an attempt to reduce the vomiting, an adjustment was made to Nolan’s feedings and the Millers were sent home, told to come back again the next morning.
“They asked us right away how the night had been and we said, ‘Better,’” Matthew recalls. “They said, ‘Good. We didn’t have you back as much for Nolan today as we had you back for you. If you hadn’t said it had gotten better, we were going to admit all of you to the Nebraska House so you could get the nursing care and rest you need to get you all back on your feet.’”
Matthew still marvels at the memory. “That showed us they had all of our best interests at heart and had truly taken an interest in our case.”
The team’s personal care and concern persisted as Nolan was slowly weaned from TPN.
“The greatest thing about the team is that they’ve always been available to us,” Amy says. “We’ve called them at all hours and they’re always there for us. They showed us they trusted us to take him home and take care of him.”
The team gradually increased the feeds though the g-line or “button” into Nolan’s stomach, weaning him from the nutrients provided by the TPN until it was “nothing but saline.” Matthew says.
Initially told to expect their son to be on TPN for at least five years, the team removed the TPN line when he was 13 months old.
“It just blew us away,” Matthew says. “It gave us a greater sense of hope. This team knew us, they knew him and they had a plan.”
Nolan started eating solid foods when he was about 2-1/2 years old and hasn’t stopped since, preferring fruits and vegetables over candy, his parents say.
Today, he looks no different from any boy approaching his fourth birthday. The only signs that remain are hidden beneath his T-shirt – a horizontal scar and the g-button through which he receives feedings each day.
That, too, will one day change – if his parents have their wish. “Even though his hydration will always be an issue,” Amy says, “our goal is to have him off his feeds at age 5.”
They’ve started a website, www.nolanmiller.com, to document their son’s story and to provide encouragement and inspiration to other parents of children who suffer from NEC and short bowel syndrome. And they do their best to spread the word about the care and treatment they experienced through the Intestinal Rehabilitation Program.
Matthew tells the story of a recent visit he and Nolan made to The Nebraska Medical Center. There in the clinic waiting room were a couple and two mothers. Their babies were being fed through tubes and TPN lines.
“They looked tired and scared,” Matthew says. “I had to think, they must look like how Amy and I looked when all this started. So I told them, ‘You’re in the right place.’
“They asked me how I knew, so I lifted Nolan’s shirt and showed them his feeding button. They were amazed and went on and on about how he looked healthy and happy just like any other child.
“Right when they were thinking everything is bad and going downhill fast, here they could see what this team could do for them. They could see there really is hope.”
That’s important. Because, for the Millers and the dozens of parents like them, seeing is truly believing.