Favorable Outcome With Teduglutide Treatment of a Young Woman With Long-Standing Intestinal Failure/Short Bowel Syndrome (SBS) With No Remaining Central Venous Access

Abstract

Introduction: Describe treatment with teduglutide (rDNA origin) in a 23-year-old patient with longstanding intestinal failure/SBS due to congenital jejunal atresia Case Report: Our patient is a 23-year-old female born with jejunal atresia, who experienced intestinal failure due to SBS as a neonate. She has absence of the corpus callosum, seizure disorder, and neurodevelopmental delay. Her gastrointestinal history is significant for eosinophilic colitis, enteritis, gastro-esophageal reflux disease, and malabsorption due to SBS. She had a history of multiple prior central venous catheters. One year prior to this report, due to feeding intolerance and partial bowel obstruction, she underwent surgery with extensive lysis of adhesions, Ladd’s procedure, and serial transverse enteroplasty procedure (STEP). After surgery, we attempted to wean her off parenteral nutrition (PN). Following a catheter-associated central venous thrombosis, she was placed on treatment with fondaparinux to prevent progression of thrombosis. However, the catheter would no longer infuse, and she had no further central venous access for PN. Enteral feedings were increased with elemental formula to meet her estimated energy needs. Her daily fluid needs were partially met with ORS and additional peripheral intravenous fluids (IVF) to compensate for high fecal losses and electrolyte and mineral deficiencies. She developed pneumatosis intestinalis (PI) on this regimen. Once the PI resolved, she was placed on a low-carbohydrate enteral formula and oral rehydration solution (ORS) via G-tube with supplemental fluid, electrolyte, magnesium (Mg), and phosphorus (Phos.) administered via peripheral vein, based on stool output, serum electrolyte, and mineral levels. Peripheral IVs were changed frequently and with great difficulty by the hospital IV team. Due to lack of central venous access and inability to transition the patient from IVF, electrolytes, and minerals, she was started on teduglutide 0.05 mg/kg daily subcutaneously. She was monitored in the hospital for adverse effects, daily weights, fluid intake and output, serum electrolytes, and minerals. She experienced recurrence of PI and elevated lipase levels that resolved within 4-5 days with conservative management. Six weeks after starting teduglutide, the patient no longer required additional IVF, IV electrolyte, or mineral supplementation. After 9 weeks of teduglutide, she continues to tolerate G-tube feeds and ORS with normal serum electrolytes, Mg, and Phos. without IV supplementation at home.Table 1: Results