Custom parenteral nutrition for critically ill patients with aminoacidopathies

ABSTRACT

Background: Nutrition therapy is crucial in the management of aminoacidopathies. The goal during critical illness is to reverse catabolism by providing sufficient energy and non-offending amino acids (AAs). If the patient's condition is unstable, tolerance of adequate enteral nutrition (EN) to promote anabolism may not be feasible. Parental nutrition (PN) may be necessary to meet nutrition goals, however standard preparations of PN are contraindicated. Integrity Compounding Pharmacy [Sandy Springs, GA] offers specialty compounding options tailored to provide PN to critically ill patients with aminoacidopathies void of offending AAs. Method(s) Retrospective chart review was performed. Patient Awas a 4-day old twin female born at 31-weeks gestation with phenylketonuria (PKU) hospitalized for prematurity and respiratory failure. Patient B was a 4-day old female, sibling of patient A, also with PKU hospitalized due to prematurity, respiratory failure and ductal dependent pulmonic stenosis. Patient C was a 26 year old male with maple syrup urine disease (MSUD) admitted for metabolic decompensation and respiratory failure in the setting of novel Covid-19 virus. Patient Dwas an 8 year old female with MSUD presenting with nausea and vomiting in the setting of novel Covid-19 virus. All four patients experienced elevated blood levels of offending AAs and inadequate EN intake. Custom PN from Integrity Compounding Pharmacy was utilized in all four patients ranging from 6 to 11 days. Patient A, B and D received custom PN as sole source nutrition for a period of time while transitioning to EN. Patient C tolerated a small amount of EN as well as custom PN to meet nutrition goals. Result(s) The Integrity custom PN provided appropriate AAs to optimize nutrition until full EN could be tolerated. This essential nutrition therapy helped reverse catabolism, achieve metabolic control and prevent further sequelae. Conclusion(s) Custom PN should be considered in critically ill patients with aminoacidopathies that have significant EN intolerance.Copyright © 2022 Elsevier Inc. All rights reserved.