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Initiating Pancreatic Enzyme Replacement Therapy (PERT) — Cystic Fibrosis (CF) — Clinical Pathway: All Settings

Clinical Pathway for Pancreatic Enzyme Replacement Therapy (PERT) in Children with or at Risk for Exocrine Pancreatic Insufficiency (EPI)

Cystic Fibrosis (CF)

EPI is common in children who have CF and affects approximately 85% of the CF population. EPI is common in patients who have CF and affects approximately 85% of the CF population.

Clinical Presentation

Infants/children with CF and EPI symptoms may be admitted to the hospital because they present with the following:

Symptoms/Signs PERT Initiation
  • Meconium ileus
  • Rectal prolapse
  • Recommended
  • Respiratory distress
  • Poor weight gain
  • Electrolyte disturbance
  • Strongly consider
  • Other:
    • Cardiac disease
    • Prematurity
    • Renal disease
  • Discuss with subspecialist

Considerations for PERT initiation

Testing

EPI testing is obtained based on clinical signs and symptoms or confirmed diagnosis of CF.

 

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