When he was just one day old, Devin O'Bleanis had surgery at Long Island Jewish Hospital in New York to correct a twisting of his intestine. But when the problem persisted, an additional surgery was required to remove the damaged segment of his intestine altogether.
As a result, Devin developed short bowel syndrome (SBS), a condition in which patients are without a significant length of their small intestine and are usually deficient in a range of important nutrients, because their intestine cannot absorb enough nutrition from regular food.
Treatment consists primarily of nutritional management to help the remaining intestines adapt and take on the absorption functions that were lost. Many SBS patients get nutrients delivered directly into their stomachs or small intestines via enteral nutrition. But in some cases, this is not possible, so patients must get nutrition intravenously through parenteral nutrition (PN). Depending on the length and health of the remaining intestine, nutritional support becomes a lifelong necessity for some patients.
As a newborn with SBS, Devin was put on PN immediately and did well enough to go home after four months. A few days after discharge, however, Devin's parents noticed a yellow discoloration of his skin and eyes.
After consulting with their doctors at Long Island Jewish Hospital, the O'Bleanis's were referred to nearby Mount Sinai Hospital, where doctors ran tests and found damage to Devin's liver. But their recommendation of a liver and intestine transplant concerned the O'Bleanis's. "He was a vivacious and high-spirited child, and we didn't want to put him through that trauma," says Devin's mother, Yvette. "So we began to explore other options."
Their research led them to Children's Hospital Boston's pre-eminent program for treating SBS. A team of doctors evaluated Devin and concluded that the best course of action was not a transplant but a bowel lengthening procedure called a serial transverse enteroplasty, or STEP.
Developed at Children's by Heung Bae Kim, MD, and Tom Jaksic, MD, PhD, the STEP procedure is based on the idea that stapling v-shapes into alternating sides of the bowel will decrease its width and increase its length. Thus, the intestine eventually stretches out and becomes a normal tube, and food is propelled down the tube like a normal piece of intestine.
The O'Bleanis's agreed to the surgery, and just weeks later, Devin was back in Boston to undergo a STEP procedure. Following the STEP, his condition improved almost immediately. Devin's bilirubin level, which was causing his yellow discoloration, began to recede. And while a biopsy taken during surgery revealed some damage to his liver, it was determined that proper nutritional care would be able to reverse it.
"Patients like Devin are complex, but they can be helped if they get the right kind of care," says Dr. Jaksic, the Short Bowel Syndrome Program's surgical director. "They present a number of challenges, primarily because their care involves so many different specialties."
Delivering effective nutrition to patients like Devin demands important contributions from a multidisciplinary team, including gastroenterologists, dieticians, pharmacists and nurses. Christopher Duggan, MD, MPH, medical director of the Short Bowel Syndrome Program, helps direct how to best to keep kids with SBS growing.
"Devin was pretty malnourished when he got here," says Dr. Duggan. "The first thing we did was an intensive assessment of his nutritional status and nutrient needs. We were also very aggressive in weaning him from parenteral to enteral nutrition." Other team members involved in his care include: Clifford Lo, MD, PhD; Sharon Collier, RD; Julie Iglesias, CPNP; Kathleen Gura, Pharm D; and Denise Richardson, RN.
Today, Devin, an energetic 4-year-old, is back home in New York. Since his STEP surgery in 2003, he has gradually been taken off PN and is now eating everything by mouth. Although Devin sometimes requires extra fluid through his G-tube to supplement his oral diet, it's a marked improvement for the O'Bleanis family. "The doctors at Children's gave us our life back—which is Devin," says Yvette.