Electrolyte Derangement Due To Medication Shortage In An Ultra Short Bowel Syndrome

INTRODUCTION: Short bowel syndrome (SBS) is a life-threatening condition associated with failure of the bowel to maintain electrolyte, fluid, and nutritional balance due to impaired functioning or loss of absorptive capacity due to disease. Anatomically, SBS per an AGA technical review is defined as less than 200cm of small bowel. Though the length of the bowel is important in defining intestinal failure, the remaining anatomy and health of the remaining bowel should also be taken into consideration. Given the complexity in defining and assessing short bowel is it no surprise that treatment is equally complex and required careful individualization. We present a case of a patient with short bowel syndrome whose treatment is complicated by medication shortages resulting in hypochloremic metabolic alkalosis. CASE DESCRIPTION/METHODS: 26-year-old male patient with a history of vascular Ehlers-Danlos syndrome resulting in catastrophic bowel infarction leading to multiple small and large bowel resections. He presented to the hospital with acute kidney injury and hypochloremic metabolic alkalosis. He has a history of failed small bowel transplant resulting ultimately in end duodenostomy with large midline entero-cutaneous fistula with large output and has been TPN dependent since 2016. His course has been complicated by parenteral nutrition-associated liver disease (PNALD) treated with SMOFFlipids and cycled TPN. Due to his high output, he was also maintained on intravenous proton pump inhibitors (PPI) to decrease gastric output and limit his fluid and electrolyte loss. He has had no recent changes in parenteral nutrition formula and denies any nausea/vomiting or an increase in output. He denies missing any parenteral nutrition infusions and reports no recent medication changes other than missed doses of his PPI due to a national medication shortage. He was started back on his intravenous PPI and fluid resuscitated with rapid normalization of his electrolytes. DISCUSSION: SBS is a complex medical condition requiring individualization of care based on patient-specific characteristics. Our patient was unique due to his ultra-short bowel syndrome, effectively an end duodenostomy resulted in large electrolyte losses including excessive chloride losses. Large outputs in SBS can result in either metabolic alkalosis or metabolic acidosis depending on patient-specific anatomy. In most cases shortage of PPIs is of little consequence, however, in our patient, it resulted in alkalosis and kidney injury due to volume depletion.