P056: Improving care for patients with hyperammonemia: Use of quality and safety frameworks and creation of a clinical care pathway

Introduction: Hyperammonemia is an urgent metabolic condition that can lead to cerebral edema, coma, seizures, and death.Prompt diagnosis of the underlying cause, appropriate management, and careful monitoring are critical for reducing acute and chronic morbidity following a hyperammonemic crisis.However, lack of familiarity with these diagnoses and the necessary treatments among healthcare providers can result in delays in therapeutic intervention even at tertiary centers with higher volumes of urea cycle disorders (UCDs) and other hyperammonemia cases, demonstrating a need for standardized and optimized processes.Quality improvement (QI) frameworks have proven invaluable for improving patient outcomes in pediatric medicine but have not yet been broadly applied to the care of metabolic diseases.Methods: A multidisciplinary team was assembled including pharmacists, nutritionists, nurses, and providers from intensive care, metabolism, and the emergency department to assess the current state of care for patients with hyperammonemia.Baseline data was gathered via a retrospective analysis of hyperammonemic crises at a single center, tracking timing of interventions and laboratory values from presentation to recovery.This data was used to inform the design of a clinical care pathway for patients with hyperammonemia that served both as a guide for patient care as well as a centralized educational resource.Employing QI frameworks, interventions were designed with the aim to decrease time to Ammonul administration and decrease the area under the curve where ammonia remained elevated to >100 umol/L.Results: The initial retrospective analysis found substantial variation in time to initial Ammonul administration and advancement to full protein diet, which resulted in prolonged elevated ammonia levels.The analysis also found that many patients developed hypokalemia during Ammonul therapy, prompting a pharmacy review which confirmed few adverse effects upon co-administration of Ammonul and IV fluids.This review led to pharmacy approval of coadministration of Ammonul with potassium-containing IV fluids.Based on the retrospective analysis, the multidisciplinary team designed a clinical care pathway to address provider education, formula preparation, and gaps in pharmacy, physician and nursing workflows.Statistical process control chart analysis demonstrated that implementation of this clinical care pathway resulted in a mean shift in time to Ammonul administration from triggering ammonia level from 3.3 hours to 2.2 hours.Additionally, time patients remained hyperammonemic also saw a mean shift from 11.88 hours to 5.9 hours.Conclusion: Using a QI framework, development and implementation of a novel clinical care pathway led to a meaningful reduction in time to Ammonul administration and time spent in a hyperammonemic state.Standardization of care facilitated hospital-wide implementation and allowed broad dissemination of recommendations for optimal treatment of UCDs and other, non-UCD hyperammonemic crises.Broadly, this work demonstrates that QI initiatives and standardized clinical care pathways represent powerful tools for improving outcomes for patients with inborn errors of metabolism and other rare disorders.