It truly takes a village to ensure children with short bowel syndrome have the best outcomes possible. The Intestinal Rehabilitation Program (IRP) at MUSC’s Shawn Jenkin's Children's Hospital is a multidisciplinary clinic dedicated to the care of these patients. “We bring together all of the expertise these patients require in one interprofessional team,” says Kathy Chessman, a PharmD with the program. “We work together to take care of these children in a single clinic where everyone contributes their expertise to making decisions about the best treatment for each patient.”
The plan of care is complex because intestinal failure has wide ranging impacts on multiple organ systems and patients need long-term, at-home monitoring after discharge. “Our typical new patient is a premature infant in the neonatal intensive care unit (NICU) with necrotizing enterocolitis that has required resection of part of their bowel, or with congenital intestinal atresia, a genetic anomaly that causes a lack of bowel formation,” says Candi Jump, DO, a pediatric gastroenterologist. “When a child’s bowel length or function does not allow them to grow by taking bottles or eating food like other children, it’s our job to help them thrive using a combination of intravenous (IV) parenteral nutrition and feeding tubes. We usually start seeing new patients in the NICU and follow them through their inpatient journey and into the outpatient world.” Also, older children, who were previously followed by other physicians, can have their care transitioned to the multidisciplinary IRP clinic.
Because intestinal failure is relatively uncommon, some pediatricians may never see a case and, when they do, finding the long-term specialized care to manage the child’s medical needs can be difficult. “It’s not like asthma. There aren’t that many children who have this,” says Chessman. “If you only take care of one or two, it’s hard to do. It really takes an interprofessional team of intestinal failure experts because there are so many nuances with their nutrition and medical care,” Chessman says. “The parenteral solution has to be adjusted to match their growth rate and to manage anything we see on their labs. Every week I communicate with the infusion companies about whether to change each patient’s nutrition or not. Central lines can break or become infected. G-tubes can have issues. Patients can develop diarrhea or small bowel bacterial overgrowth that has to be treated. There’s a lot to caring for this population.”
The MUSC IRP team includes a pediatric gastroenterologist, surgeon, pharmacist, dietitian, and nurse practitioner who have experience in treating these patients. In addition, when a child needs additional care, they have access to the full array of pediatric services at MUSC Children’s Health. “These patients are complicated,” says Jump. “When they get sick, they need pediatric hospitalists and a pediatric intensive care unit. They may need a hematologist to help with clots, a surgeon to fix or replace intravenous lines, or a nephrologist because of their risk for kidney stones. These children need access to the whole spectrum of pediatric care.”
The MUSC IRP sees approximately 100 patients, of whom, 15 are managed on parenteral nutrition at home. The team ensures caregivers are trained to care for their child’s central line and administer daily IV nutrition infusions along with any tube feeding or oral nutrition included in their treatment regimen. “We aim to promote adaptation of their bowel. Even though a child may leave the NICU on IV nutrition, our goal, where possible, is to help them get totally off IV feeds. Sometimes it takes weeks or months or years but that’s always our goal,” says Jump. The team accomplishes this by optimizing IV nutrition with close monitoring for nutrient, vitamin, and mineral deficiencies and incorporating as much oral feeding as the child can tolerate with gradual increases. “There’s a lot of waiting as the gut adapts to different feed schedules and formulas,” says Jump. “But relying completely on IV nutrition and not using your gut can harm the liver. We also want to avoid having a child develop oral aversion if we can help it. Our behavioral and feeding therapists help with that. Ultimately, our goal is to get them off IV nutrition completely. Some won’t be able to, but many of our patients can.”
Because the team follows these patients so closely and for so long, they form deep bonds with them and their families. In part, this is what drew both Jump and Chessman to the field. “When I was starting out in this area, I saw a patient with intestinal atresia who was born 6 weeks after my own daughter,” Chessman says. “She became my patient the first day I came back from maternity leave, so she was like my second daughter. She grew up on parenteral nutrition and, even though she had a difficult life, we worked closely with her family to keep her growing. We saw her all the way through her first two years of college. I’ll never forget her. For me, she really defines what we’re here to do.”
For Jump, it was a patient she saw during training. “The first patient I saw had undergone an intestinal transplant and he wasn’t doing well afterwards. He was in and out of the hospital all the time and I got to know him very well. I remember being on call as an intern and seeing some of the residents watching the World Cup with him and thinking, ‘How can I make this kid’s life better?’ I’ve loved this field ever since. These patients just occupy a special place in your heart.”
