Recently Kipp Ellsworth, with input from members of the nutritional team, developed our first institutional Short Gut Diet.
Per Kipp, this diet is “designed to facilitate digestion while minimizing abdominal pain and ostomy/stool output in our inpatients with truncated intestinal anatomy. Previously, clinicians ordered a regular diet for our short gut patients, with parents and nurses providing oversight of the ordering process based on their knowledge of short gut diet precepts. Obviously this non-standardized approach resulted in significant noncompliance, another onerous daily task for nursing, and a failure of inpatient short gut diet principles reinforcement. I anticipate the new diet serving as an omnipresent education tool, reinforcing short gut diet precepts for patients and parents during their inpatient stays.”
Related blog posts:
Reference from Kipp Ellsworth:
Abstract: Due to concerns related primarily to allergic response and malabsorption, enteral nutrition therapy has traditionally relied on the use of elemental formulas in children with intestinal failure (IF). Blended food diets via a gastrostomy tube have been reported to improve feeding tolerance in pediatric populations receiving long-term enteral nutrition therapy. Complex macronutrients have been shown to stimulate intestinal adaptation in animal models. We report on our experience in children with IF who had an overall improvement in stool output when transitioned from an elemental formula to a tube feeding formula with real food ingredients (TFRF). Data were collected in a retrospective chart review of children with IF, >1 year of age, who were receiving enteral nutrition via continuous infusion, bolus feeding, or both. Indications for the TFRF trial were diarrhea or inconsistent stooling patterns. Ten children with a mean small bowel length of 48.3 cm were trialed on TFRF. Nine of 10 (90%) children tolerated the transition to 100% TFRF, of which 7 of 9 (78%) had their entire colon in continuity. The average age at successful transition was 29.2 months, and the average length of time to transition to 100% TFRF was 67.3 days. TFRF is well tolerated in children >1 year of age with IF; it also improves their stooling patterns. A commercially available TFRF is a cost-effective and nutritionally adequate means of providing nutrition to this patient population.
More data on teduglutide indicate its potential for short bowel syndrome (SBS) (Gastroenterol 2012; 143: 1473-81, editorial 1416-20). Treatments for SBS are needed. One year of parenteral nutrition often costs the health care system in excess of $100,000 per year. This cost does not account for laboratory studies, health care visits, complications, and hospitalizations. Treatment of intestinal failure with transplantation “may cost upwards of $1 million.”
In this study of adult patients with an average of 50 years, teduglutide was given in a prospective randomized double-blind study to 42 patients and another 43 patients received placebo. The dose of 0.05 mg/kg/day via subcutaneous injection was chosen based on a previous trial which showed that a higher dose was less effective. Among these patients, the most common reasons for SBS were vascular disease (34%), Crohn’s disease (21%), volvulus (11%), and injury ((9%).
- Teduglutide over a 24-week study was more effective than placebo. 63% of study patients had a drop in parenteral nutrition requirement of more than 20% compared with only 30% of the placebo group.
- The mean reduction in parenteral nutrition support of teduglutide-treated patients was 4.4 L/week compared with 2.3 L/week for placebo-treated patients.
- Citrulline, a biomarker of mucosal mass, was increased in the teduglutide group. In the treatment group, citrulline increased by 20.6 μmol/L compared with 0.7 μmol/L for the placebo group.
How does teduglutide work? Teduglutide is a much more stable analog of glucagon-like peptide-2 (GLP-2). The latter is released by the distal small bowel and colon. GLP-2 promotes intestinal epithelial growth and increases transit time.
What are the adverse effects of teduglutide? First, there is a concern that teduglutide could promote colonic adenomas based on studies in mice. GLP-2 receptors are present in the lung, and brain (including hypothalamus); its effects in these areas is poorly understood. In addition, abdominal pain, distention, nausea, peripheral edema, and nasopharyngitis were more common in the treatment group. The long-term consequences of teduglutide therapy are not known.
CLMP stands for Coxsackie- and adenovirus receptor-like membrane protein. It is required for intestinal development (Gastroenterology 2012; 142: 453-62).
In this study of seven patients from five families with congenital short-bowel syndrome (SBS), the authors identified a loss-of-function of CLMP in five of the patients. CLMP is a tight-junction-associated protein that is expressed in the intestine of human embryos throughout development.
To study the effect of CLMP expression, the authors created a zebrafish model with compromised CLMP activity. This lead to offspring with a foreshortened body and intestine, strengthening the evidence that CLMP dysfunction is responsible for congenital short-bowel syndrome.
Additional references/previous related posts: