Summer 2009

Intestinal Rehab for Short Bowel Syndrome

Sue Rhee, M.D.

Patients diagnosed with short bowel syndrome have traditionally faced disheartening survival rates and painful quality-of-life compromises. Today, however, evolving clinical approaches have improved the outlook, especially when patients arrive at intestinal rehabilitation centers before they experience severe complications.

Such centers see a disproportionate number of these rare syndromes, and engage multidisciplinary teams that include pediatric gastroenterologists, pediatric surgeons and transplant surgeons, pediatric hepatologists, dietitians, pharmacists, and social workers. This ideally positions them to balance considerations of total parenteral nutrition (TPN), medications and potential surgeries — including serial transverse enteroplasty (STEP), the latest tool in the armamentarium.

"We tailor the therapy to each individual child, depending on their underlying condition, and coordinate care with the patient's pediatrician," says Sue Rhee, M.D., medical director of the Pediatric Intestinal Rehabilitation and Transplantation Program at UCSF Benioff Children's Hospital.

Managing Complications

The causes of short bowel syndrome range from congenital conditions (e.g., intestinal atresias and gastroschisis) to acquired causes such as necrotizing enterocolitis. "Regardless of cause, though, our first concern is to support growth and nutrition, using TPN, which we can administer both as an inpatient and at home," says Rhee.

One challenge is managing TPN's two major risks: infection and liver disease. "To prevent line infections, one strategy we use is antibiotic lock therapy for our catheters," says Rhee. Managing the risks of infection and liver disease also tends to involve complex adjustments of medications and TPN content. That is why Rhee's program is part of a National Institute of Health funded consortium researching how to better identify those at risk for complications — and devise better ways to prevent and treat those complications.

Ultimately, however, the goal in all cases is to wean patients from TPN, using their remaining bowel and a specialized diet. And sometimes, when a patient is not progressing or complications become severe, there are surgical options.

Serial Transverse Enteroplasty and Beyond

One recent surgical option is the bowel lengthening procedure known as serial transverse enteroplasty, or STEP. This novel surgery, which has become standard procedure for pediatric intestinal rehabilitation programs, involves simultaneously cutting and stapling sections of the intestine to hasten intestinal growth and improve motility. Surgeons Tippi MacKenzie, M.D., Doug Miniati, M.D., and Kerilyn Nobuhara, M.D., at UCSF Benioff Children's Hospital have completed a number of STEP procedures, including one on a 6-week old.

It's a real improvement over the past and can improve the chances of weaning patients from TPN," says Rhee.

Transplantation is another surgical option. Typical indicators include liver failure, frequent sepsis or running out of places for a central line.

"We have top transplant surgeons here and a renowned abdominal transplant program, but our goal is to avoid transplant because, though it can be lifesaving, it also can have many potential complications," says Rhee, who is triple board-certified in pediatrics, transplant hepatology and pediatric gastroenterology.

The transplant hepatology certification is especially important for management of intestinal transplant patients with concurrent liver disease. It is yet another example of why quick referral to an expert center is such a crucial consideration for young people with gastrointestinal concerns.

For more information, contact Sue Rhee, M.D., at (415) 476–5892.

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