Worth every mile: Short bowel syndrome brings family to Boston

short-bowel-syndrome

During his most recent visit to Boston Children’s Hospital, 3-year-old Konrad Schienke resembles a tiny tornado, gleefully scampering around the room as he mugs for the camera and shouts, “Cheese!” Later, he smiles as a doctor gently felt his abdomen, giggling as if he was being tickled.

“It’s hard to believe what a sick little kid he has been,” says his father, Erich.

Yet, just a few years ago, this energetic boy resided in the neonatal intensive care unit at his local hospital in Pennsylvania, struggling with a diagnosis of short bowel syndrome. This rare but serious condition can occur when a child either loses or is born without enough small intestine, preventing the body from extracting the nutrients it needs to survive. Untreated, short bowel syndrome can lead to severe dehydration and malnutrition.

Although Konrad’s physicians recommended an intestinal transplant, his parents believed there had to be a less extreme approach. “While we are very fortunate that intestinal transplants are a possibility, we discovered that the rate of complications remains high compared with results from programs doing intestinal rehabilitation,” says his mom, Neyda.

“We’re both university professors, so our inclination is always to research things,” she explains. “As we tried to understand the best route to take, it became obvious that we wanted to go to Boston Children’s to see Dr. Mark Puder and his colleagues. The hospital has a record of success in intestinal rehabilitation as well as preventing adhesions in large abdominal surgeries, which Konrad would certainly need.”

Specialized care for short bowel syndrome

The team at the Center for Advanced Intestinal Rehabilitation at Boston Children’s recommended that Konrad enroll in the center’s intestinal rehabilitation program and undergo ostomy takedown surgery. This procedure reconnects a patient’s two remaining segments of intestine. Over time — typically a period of two years — the small intestine adapts and grows, eventually increasing its ability to absorb nutrients. In the meantime, Konrad would have to remain on total parenteral nutrition, which delivered vital fluids and nutrients intravenously.

Although Neyda and Erich admit they were nervous about what the future might hold, they say they immediately felt relieved upon arriving in Boston.

“We were especially impressed by the multidisciplinary approach,” says Erich. “There’s no one chief expert — they work collectively for the patient. There’s something really magical about it that we haven’t experienced anywhere else.”

short-bowel-syndrome

After a successful surgery, Konrad remained in the hospital to recover, an experience Neyda recalls fondly. “The level of care, concern and compassion that all the doctors and nurses at Boston Children’s showed us carried our family through some very stressful times.”

Today, Konrad still receives tube feedings, while his intestine grows and heals. In between busy days learning to count and read, obsessing over trucks and tractors, and playing near his family’s rural home, he still makes regular trips back to Boston for checkups — journeys made easier by the Center’s team approach.

“We really like that it’s a one-stop visit at Boston Children’s,” says Erich. “We don’t have to schedule eight appointments with eight different doctors and hope that they talk to each other.”

Still, the family doesn’t mind hopping in the car and driving north when necessary. “In the end, it’s worth it,” says Neyda. “It’s worth every mile for Konrad.”

Learn more about the Center for Advanced Intestinal Rehabilitation at Boston Children’s Hospital.