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Metabolic Emergency in Flight

Irene A Hurst1, Amy Calhoun2, Ryan Mehren3

  • 1Department of Emergency Medicine, University of Wisconsin-Madison, Madison, WI; Division of Pediatric Emergency Medicine, University of Wisconsin-Madison, Madison, WI; School of Medicine and Public Health, University of Wisconsin-Madison, Madison, WI.

Air Medical Journal
|March 6, 2022
PubMed

Insights

Inborn errors of metabolism can present with nonspecific symptoms in children. Specialized pediatric flight teams are crucial for stabilizing critically ill infants with these rare genetic disorders during transport.

Area of Science:

  • Biochemistry
  • Pediatrics
  • Genetics

Background:

  • Inborn errors of metabolism (IEM) are genetic disorders that can lead to severe illness in infants and children.
  • Symptoms of IEM are often nonspecific, leading to delayed diagnosis as they are not always included on newborn screening panels.
  • Prompt recognition and specialized care are vital for managing acute presentations of IEM.

Observation:

  • Young children with undiagnosed IEM can present with critical, nonspecific symptoms like vomiting, altered mental status, seizures, coma, or death.
  • These critically ill patients may not respond typically to standard resuscitation protocols.
  • A case involving the aeromedical transport of a pediatric patient with a rare, undifferentiated IEM is detailed.

Findings:

  • Specialized pediatric flight teams can provide critical care during transport for infants with inborn errors of metabolism.
  • Careful consideration of resuscitation and treatment strategies is necessary for critically ill pediatric patients with IEM during flight.
  • Swift transport to a pediatric specialty center is lifesaving and prevents neurological damage.

Implications:

  • Highlights the importance of considering IEM in critically ill children with nonspecific symptoms.
  • Underscores the necessity of specialized aeromedical transport protocols for pediatric patients with rare metabolic disorders.
  • Emphasizes the role of early intervention and specialized care in improving outcomes for children with inborn errors of metabolism.

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