Pediatric surgeon Sam Alaish recalls this decade-old case like it happened yesterday. The 2-year-old was in shock and being rushed into the OR for an emergency laparotomy. Opening the abdominal cavity, Alaish found one of the worst cases of intestinal malrotation with midgut volvulus, he would ever see. In such cases, he knew, patients have only six to eight hours before the twisted intestine chokes off its own blood supply, and, by the sight of it, necrosis had already started spreading. He saved what he could but it was too little—the child would require complex, multispecialty care for most of his life.
“You take out what’s dead, leave in what’s borderline, and come back at a later time to see if it survived to give him his best chance to have the most intestine possible, but we knew he would need TPN (total parenteral nutrition) for a long, long time,” says Alaish. “These are kids with severe disease, who need a lot of follow up and multidisciplinary care to optimize their outcomes.”
Unfortunately, Alaish adds, there are too few multidisciplinary centers to treat children with short bowel and intestinal failure secondary to congenital conditions like Hirschsprung’s disease, gastroschisis, intestinal atresia and necrotizing enterocolitis (NEC), all of which can lead to disabling and even life-threatening complications. The four-year survival rate for newborn infants on TPN was 70 percent; in newborns with less than 10 percent of expected intestinal length, the five-year survival was 20 percent (Ann. Surg. 2005;242(3):403–9). Those numbers have improved with the advent of multidisciplinary coordinated care, but the mortality still remains high at 7 to 28 percent. Children fortunate enough to survive continue to suffer greater morbidity from both infections and liver failure (J Surg Res 2011;170:27-31).
To further improve such outcomes, Alaish and pediatric gastroenterologist Darla Shores have led development of a new clinic at Hopkins that brings multiple specialists together under one roof to treat such complex patients.
“We do know that if you put these very challenging patients in a multi-disciplinary clinic they do better, but not every state has such a clinic for kids,” says Alaish.
“We’re taking all the bits and pieces that were happening randomly at different clinics and getting everyone in the same area to see patients in a more efficient manner—that way you’re getting everyone’s opinion all at once, so you don’t have to rely on notes and conversations after the fact,” adds Shores. “All of the specialists come together, agree on one course of action and present a uniform plan to the family, which should improve patient care and patients’ quality of life.”
In addition to pediatric surgeons, gastroenterologists and gastro-intestinal nurses, staff at the monthly clinic include a nutritionist, occupational therapist, pharmacist, psychologist and speech pathologist. The strength of Hopkins long-standing pediatric nutrition program is a value-added feature of the clinic, says Shores, citing the myriad nutritional complications and needs of patients with short bowel.
“After surgery, nutrition is the biggest piece in caring for these children,” says Shores, who oversees the day-to-day management of patients’ feeds and TPNs. “They’re hooked up to an IV every day, oftentimes for 12 to 18 hours, so the idea of liking food and eating normal meals is not something they grow up with. Getting them transitioned to eating by mouth is definitely challenging.”
Another value is having pediatric surgeons like Alaish and David Hackam, Hopkins’ new pediatric surgeon-in-chief who specializes in treating patients with NEC. In ways, notes Shores, surgeons know the patients better than their medical colleagues—they know what they took out and left in—and can be quickly summoned for emergency issues like gastrostomy tube repairs and line infections. Also, Alaish and Hackam, along with Shores, bring another major benefit to patients in that they’re aggressively pursuing research to improve existing treatment protocols and to develop new, more effective therapies for short bowel. In fact, to enhance the impact of research for these fragile patients they’ve created the Center for Intestinal Rehabilitation and Cure Using Science, or CIRCUS.
“The reason I came here,” says Alaish, “was to put some science behind our treatments, to measure our outcomes, to see how we’re doing and what can we do better.”
That 2-year old Alaish had operated on? After receiving multidisciplinary care he was able to graduate from IV nutrition to full enteral feeds after five years. Now he’s 10 years old, eats a normal diet, including an occasional burger, plays baseball and rides his bicycle—“just like any normal 10-year old boy would do,” says Alaish.