Author ORCID Identifier

0000-0003-2841-7660

Date of Completion

5-2025

Document Type

Open Access Project

Degree Name

Doctor of Nursing Practice (DNP)

Faculty Advisor

Dr. Eileen Stuart-Shor

Practice Site Mentor

Dr. Mary Poyner Reed and Dr. Amy Kritzer

Committee Mentor

Dr. Ellen Benjamin

Abstract

BACKGROUND: Children with medical complexity are a distinct patient population that includes children with inborn errors of metabolism (IEM). The medical complexity of this patient population can lead to gaps in care, which is amplified by poorly coordinated care. Emergency care encounters are inevitable for patients with an IEM. The medical fragility of these patients can be further intensified during these encounters, where these patients are at an increased risk of adverse events, morbidity, and mortality without prompt recognition and care. Patients with an IEM followed by the Metabolism Program at the project hospital often seek emergency care at the project hospital’s Emergency Department. Given the limited number of patients with these diagnoses, these patients often receive initial emergency care from medical professionals who are unfamiliar with their diagnosis. This increases the risk of negative outcomes for these patients.

METHODS: A systematic review of the literature was conducted to identify strategies to address this problem. The overall aim of the project was to design, implement, and evaluate the implementation of evidence-based enhanced and standardized emergency planning documentation to improve and standardize emergency planning and initial emergency care for patients with an IEM.

INTERVENTION: The implementation of updated emergency planning documentation included pre-implementation design and integration of the documentation during the chronic care phase of the care continuum for utilization during the acute-on-chronic and acute care phases of care. Evaluation of the project was based on six specific aims (process and outcome), which were analyzed using both quantitative and qualitative descriptive statistics.

RESULTS: The quality improvement project was implemented for 17 distinct IEM diagnoses. During implementation, the number of patients with emergency planning documentation increased from 92% to 94%. The time to critical action steps for laboratory studies collected and intravenous fluid started improved by 22% and 13%, respectively. The involved care team members indicated a positive impact on emergency care encounters. Caregivers were satisfied, comfortable, and prepared for their child’s emergency care.

CONCLUSION: The implementation of the updated emergency planning documentation improved the emergency care for patients with an IEM by facilitating timely treatment initiation and enhancing interdisciplinary communication.

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