Clinical Pathway for Children with/at Risk for Exocrine Pancreatic Insufficiency (EPI) Who May Need Pancreatic Enzyme Replacement Therapy (PERT)
Symptoms Suggestive of EPI
  • Poor weight gain/linear growth
  • Steatorrhea
  • Loose/frequent stools after meals or snacks
  • Foul smelling stools
  • Flatulence
  • Abdominal pain associated with eating
  • Voracious appetite
  • Rectal prolapse
Front-line Ordering Clinician (FLOC)/RN Team Assessment
  • History & Physical Exam
    • Assess prenatal testing, newborn screen, sweat test
    • Feeding history, growth pattern
    • Surgical history suggestive for acquired EPI
    • Conditions with high risk for EPI
    • Symptoms of fat malabsorption
    • Previous enzyme replacement therapy
Do not initiate PERT
  • Child presents with any of the following:
    • 2 positive PI CF mutations
    • Meconium ileus
    • Rectal prolapse
    • Poor weight gain
    • Steatorrhea
Initiate PERT at First Visit

Formulations | Dosing Recommendations | Examples

 Confirm EPI
Normal Fecal Elastase
> 500 mcg/g
Borderline Fecal Elastase
200-500 mcg/g
Abnormal Fecal Elastase
< 200 mcg/g
  • PERT not indicated; discontinue if previously initiated
  • Consider subspecialist for evaluation
    • Pulmonary/CF Center
    • GI
  • Refer to subspecialist for evaluation
    • Pulmonary/CF Center
    • GI
  • Refer to clinical nutrition as indicated
  • Additional testing by subspecialist
  • Consider PERT while awaiting subspecialist
    evaluation
  • Refer to subspecialist for evaluation
    • Pulmonary/CF Center
    • GI
  • Refer to clinical nutrition as indicated
  • Additional testing by subspecialist
Monitor
Monitor
Monitor
Consider Titrating PERT Dose
Refer to clinical nutrition as indicated
Monitor
  • Growth
  • Fat malabsorption
  • Growth
  • Fat malabsorption
  • Repeat fecal elastase
    • 1 yr
    • Sooner if clinical symptoms
  • Growth
  • Fat malabsorption
  • Annually as clinically indicated
    • Fecal elastase
    • Fat soluble vitamin levels
  • Assess adherence to PERT
  • Titrate PERT dose
  • Consider formulation change
  • Evaluate other causes of malabsorption
Posted: March 2019
Revised: November 2019
Authors: Padula, L. RD; Brownell, J. MD; Reid, E. RD; Jansma, B. CRNP; Mascarenhas, M. MD; Sadgwar, S. RN;
Jones, L. PharmD; McKnight-Menci, H. CRNP; Hanna, M. RD