From the Guidelines
Management of short bowel syndrome requires a comprehensive approach focusing on nutritional support, medication therapy, and sometimes surgical intervention, with the goal of reducing morbidity, mortality, and improving quality of life, as emphasized in the most recent guidelines 1. The approach should be tailored to the patient's specific anatomy, remaining bowel length, and presence of colon, as these factors significantly influence absorption capacity and treatment strategies. Initially, patients should receive parenteral nutrition to maintain hydration and nutritional status while the remaining bowel adapts. As intestinal adaptation occurs, enteral nutrition should be gradually introduced with small, frequent meals that are low in fat, high in protein, and contain complex carbohydrates.
Medications play a crucial role in management, including:
- Anti-diarrheals like loperamide (2-4mg before meals) or diphenoxylate with atropine to reduce intestinal transit time
- Acid suppressors such as proton pump inhibitors (e.g., omeprazole 20-40mg daily) to reduce gastric hypersecretion
- Growth factors like teduglutide (0.05 mg/kg subcutaneously once daily) to promote intestinal adaptation by increasing villous height and crypt depth
- Bile acid sequestrants like cholestyramine (4g with meals) to manage bile acid-induced diarrhea, particularly in patients with less than 100cm of terminal ileum
Vitamin and mineral supplementation is essential, including:
- Fat-soluble vitamins (A, D, E, K)
- B12 injections (1000mcg monthly)
- Zinc, magnesium, and calcium
For patients with severe disease, intestinal rehabilitation programs offer specialized care, and surgical options like intestinal lengthening procedures or transplantation may be considered for those with permanent intestinal failure, as recommended in recent guidelines 1. A multidisciplinary team approach, including dietitians, nurses, surgeons, gastroenterologists, and social workers, is essential for the successful management of these patients, as highlighted in the AGA Clinical Practice Update on Management of Short Bowel Syndrome: Expert Review 1.
From the FDA Drug Label
GATTEX® is indicated for the treatment of adults and pediatric patients 1 year of age and older with Short Bowel Syndrome (SBS) who are dependent on parenteral support.
Study 3 was a randomized, double-blind, placebo-controlled, three parallel-group, multinational study in adults with SBS who were dependent on parenteral nutrition/intravenous (PN/I.V.) support for at least 12 months and required PN at least 3 times per week.
Patients on GATTEX in both dose groups experienced a 2.5 L/week reduction in PS requirements versus 0.9 L/week for placebo at 24 weeks.
Study 5 was a 24-week, multicenter study conducted in 59 pediatric patients aged 1 year through 17 years with SBS who were dependent on PS.
Results described in Table 3 correspond to the recommended GATTEX dosage of 0. 05 mg/kg subcutaneously once daily.
Reduction in PS volume of at least 20%, n/N (%)18/26 (69%)
Achieved enteral autonomy, n/N (%)3/26 (12%)
Reduction in PS infusion of ≥1 day/week, n/N (%)10/26 (38%)
Management of Short Bowel Syndrome: The management of short bowel syndrome involves the use of teduglutide (GATTEX), which is indicated for the treatment of adults and pediatric patients 1 year of age and older with Short Bowel Syndrome (SBS) who are dependent on parenteral support 2.
- Reduction in Parenteral Support: Studies have shown that patients on GATTEX experienced a reduction in parenteral support requirements, with a 2.5 L/week reduction in PS requirements versus 0.9 L/week for placebo at 24 weeks 2.
- Enteral Autonomy: Some patients achieved enteral autonomy, with 3/26 (12%) of pediatric patients achieving enteral autonomy 2.
- Reduction in PS Infusion: Patients also experienced a reduction in PS infusion, with 10/26 (38%) of pediatric patients experiencing a reduction of ≥1 day/week 2.
- Dosage: The recommended dosage of GATTEX is 0.05 mg/kg subcutaneously once daily 2.
From the Research
Management of Short Bowel Syndrome
The management of short bowel syndrome (SBS) involves a multidisciplinary approach to enhance intestinal rehabilitation and reduce the need for parenteral support.
- Teduglutide, a glucagon-like peptide 2 (GLP-2) analog, has been shown to improve intestinal absorption and reduce parenteral support requirements in patients with SBS-IF 3, 4, 5, 6.
- The use of teduglutide has been associated with significant reductions in parenteral volume and infusions, as well as improvements in stool frequency and consistency 3, 5.
- Long-term clinical nutrition, combining total parenteral nutrition (TPN) and enteral nutrition, is required for the adaptation process in patients with SBS 7.
- Parenteral nutrition-associated cholestasis (PNAC) and liver disease (PNALD) are potential complications of long-term TPN, but the use of n-3 fatty acids in parenteral nutrition may help reduce the risk of these complications 7.
Treatment Options
- Teduglutide has been approved for the treatment of adults with SBS who are dependent on parenteral support 4, 6.
- The safety and efficacy of teduglutide have been demonstrated in clinical trials, with a reduction in parenteral support requirements and a low risk of adverse events 3, 5, 6.
- Intestinal adaptation, which results in morphologic and functional changes that increase the performance of the remnant bowel, can be enhanced with nutrition and pharmaceutical approaches, including the use of prebiotics and GLP-2 analogs 4.
Patient Outcomes
- Patients with SBS who receive teduglutide treatment may achieve parenteral nutrition independence or a significant reduction in parenteral support requirements 3, 5.
- The use of teduglutide has been associated with improvements in nutritional status, including long-term stability in body weight, albumin levels, and body composition 3.
- Patients with SBS who receive long-term teduglutide treatment may experience a sustained reduction in parenteral support requirements and a low risk of adverse events 5, 6.