Caring for patients with short bowel syndrome (SBS) requires a multidisciplinary approach involving dietitians, nurses, surgeons, gastroenterologists or internists, and social workers experienced in SBS care, according to a clinical practice update expert review from the American Gastroenterological Association.
Kishore Iyer, MD, from Mount Sinai Hospital New York; John K. DiBaise, MD, from Mayo Clinic in Scottsdale, Ariz.; and Alberto Rubio-Tapia, MD, from the Cleveland Clinic, Ohio, developed 12 best practice advice statements based on available evidence. The items focus on adult patients with SBS; however, there was some overlap with the management of pediatric SBS. The review was published online in Clinical Gastroenterology and Hepatology (2022 Jun 11. doi: 10.1016/j.cgh.2022.05.032).
One update concerns defining SBS. The authors recommend that surgeons performing massive resections should report the residual bowel length, rather than the length of bowel resected.
"It is only the former that dictates outcome," they wrote.
There is general agreement that a residual small intestinal length of 200 cm or less meets criteria for SBS. Measurement should be taken from "along the antimesenteric border of unstretched bowel, from the duodenojejunal flexure to the ileocecal junction, the site of any small bowel–colon anastomosis, or to the end-ostomy."
Based on the residual bowel length, patients can be classified into three groups: end-jejunostomy, jejuno-colic, and jejuno-ileo-colic.
Assessing Nutritional Status
A dietitian experienced in SBS should perform a thorough nutritional assessment on all SBS patients. Long-term monitoring should include laboratory studies checking electrolytes and liver and kidney function, fluid balance, weight change, serum micronutrients, and bone density. Bone density should be repeated periodically, every 2-3 years.
Fluid and electrolyte problems may affect outcomes for SBS patients, particularly for those without a colon.
Most adult patients with SBS have significant malabsorption, so dietary intake "must be increased by at least 50% from their estimated needs," the authors wrote. It's best if the patient consumes the increased quantity throughout the day in 5-6 meals, they noted.
An experienced dietitian should counsel the patient based on the patient's eating preferences. Incorporating preferences can help increase compliance with the adjustments that may become necessary based on symptoms, stool output, and weight.
Using Pharmacologic Therapy
Using antisecretory medications, including proton pump inhibitors or histamine-2 receptor antagonists, helps reduce gastric secretions, the damage of acid on the upper gut mucosa, and the function of pancreatic exocrine enzymes.
Antidiarrheals reduce intestinal motility but also cause a slight reduction in intestinal secretion. Common agents include loperamide, diphenoxylate with atropine, codeine, and tincture of opium. The review authors say loperamide should get preference over opiate drugs because it is not addictive or sedative.
Use of antidiarrheals should be guided by their effect on stool output.
"Loperamide and codeine may have a synergistic effect when used together," the authors wrote.
Clonidine, which can be given transdermally, has also shown some benefit in treating high-output stool losses, presumably because of its effects on intestinal motility and secretion.
Weighing Risks and Benefits of Teduglutide
The glucagonlike peptide–2 teduglutide is of particular interest for its ability to help improve intestinal absorption and hopefully wean patients off parenteral nutrition and some will achieve enteral autonomy, the authors wrote. "The very short half-life of native GLP-2 has been extended to allow daily subcutaneous injection in the recombinant molecule, teduglutide."
However, because teduglutide is a growth factor and can boost the growth of polyps and cancer, it is contraindicated in patients with active gastrointestinal malignancies. Patients should undergo colonoscopy before treatment and periodically thereafter, the authors advised. The benefits of its use in patients with nongastrointestinal malignancy should be weighed carefully with these risks.
"The significant side effects of teduglutide and the cost mandate that teduglutide is employed only after optimizing diet and the more conventional SBS treatments described previously in carefully selected patients with [short bowel syndrome–intestinal failure]," the authors wrote.
Dosing Drugs Effectively
Medications in tablet form need to dissolve before being absorbed. Most oral medications are absorbed within the proximal jejunum, so they can be used in patients with SBS.
"However," the authors noted, "sustained- and delayed-release medications should be avoided."
They suggested that, when applicable, alternatives such as liquids and topical medications should be considered, as should the monitoring of medication levels in the blood.
If a patient does not respond, approaches to consider may include increasing a dose, changing dose frequency, or changing drug formulation or route of administration, such as intravenous, subcutaneous, or transdermal.
Including Parenteral Nutrition and Oral Rehydration
Almost all patients with SBS will need parenteral nutrition (PN) support following resection, and few will be able to stop it before discharge from the hospital.
"Although more than 50% of adults with SBS are able to be weaned completely from PN within 5 years of diagnosis, the probability of eliminating PN use is less than 6% if not successfully accomplished in the first 2 years following the individual's last bowel resection," the authors wrote.
For long-term PN, tunneled central venous catheters are preferred over peripherally inserted central venous catheters because of the higher risk of thrombosis and issues related to self-administration of PN with the central catheters. Also, tunneled catheters are preferred over totally implanted devices, or ports, for long-term patients because the main benefit of the port is not realized given that the device needs to be continually accessed and exchanged weekly.
"When calculating PN volume and content, changes in the patient's weight, laboratory results, stool or ostomy output, urine output, and complaints of thirst should be monitored," the authors noted.
The authors also discussed oral rehydration solution because patients lose more water and sodium from their stoma than they take in by mouth. Careful consideration of the glucose and sodium levels in oral fluids is important because inappropriate fluids will exacerbate fluid losses in SBS. For example, hypotonic (including water, tea, coffee, alcohol) and hypertonic (including fruit juices and sodas) solutions should be limited.
"A major misconception on the part of patients is that they should drink large quantities of water; however, this generally leads to an increase in ostomy output and creates a vicious cycle further exacerbating fluid and electrolyte disturbances," they wrote, instead advising glucose–electrolyte rehydration solution to enhance absorption and reduce secretion. These do not include available sports drinks.
"A knowledge of these complications is critical for those caring for these patients to be able to not only identify and treat them when they occur but also to prevent their occurrence whenever possible," the authors wrote. Although they considered it beyond the scope of the review to outline every complication, they indicated some complications and management strategies via an included table. These complications can include cirrhosis, osteoporosis, acute kidney disease, and central venous catheter–related infection or occlusion.
Considering Further Surgery or Intestinal Transplantation
The authors noted that any further surgery should be carefully considered, with the following three contexts having possible value: "(1) to recruit unused distal bowel, (2) to augment the function of residual bowel through specific lengthening and tapering operations, or (3) to slow intestinal transit."
Surgeons involved in managing SBS may need to confront complex intra-abdominal problems such as massive desmoid tumors, mesenteric ischemia, or complex enterocutaneous fistulae; a multidisciplinary intestinal rehabilitation team may be better able to help these patients. The authors noted that care for patients starts even before the first operation, by taking every measure to avoid massive bowel resection and the resulting SBS.
The authors noted the importance of early referral for intestinal transplantation consideration for patients with refractory dependency on parenteral nutrition or even onset of parenteral nutrition failure, which refers to complications such as intestinal failure–associated liver disease.
"At present, nearly 50% of patients being considered for ITX are also requiring simultaneous liver replacement, indicating late referral for ITX," they wrote, citing a data from a report by the Centers for Medicare and Medicaid (Fed Regist. 2007 Mar 30;72:15197-280).
They also noted that data have shown short- and medium-term outcomes are steadily improving (Am J Transplant. 2019 Jul;19:2077-2091); however, long-term outcomes have been challenged by opportunistic infections, long-term graft attrition, and other impediments that may be preventing early referral for intestinal transplantation.
Educating Patients, Caregivers
Long-term PN may restrict activity for patients, but patients and caregivers should know about some modifications.
One is to cycle the PN over 10-14 hours overnight to allow freedom from the infusion pump during the day. Infusion pumps can be programmable, and some can be carried in a backpack for infusing during the day.
Authors recommend patient support groups, such as the Oley Foundation, which can help with issues surrounding body image and travel.
Because of the relative rarity of SBS, nonspecialist physicians may care for patients without a dedicated multidisciplinary team and may need education support in managing patients with complex care needs. One source the authors recommend is the Learn Intestinal Failure Tele-ECHO (Expanding Community Healthcare Outcomes) (LIFT-ECHO) project. The LIFT-ECHO project has become an online educational community with case-based learning in SBS, intestinal failure, and PN.
The authors disclose relationships with Takeda, Zealand, VectivBio, Napo, and Hanmi.
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Lead Image: iStock/Getty Images
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Cite this: Managing Short Bowel Syndrome: Expert Review - Medscape - Sep 12, 2022.