Initiating Pancreatic Enzyme Replacement Therapy (PERT) — Adequate Response to PERT — Clinical Pathway: All Settings

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

Adequate Response to PERT

Clinical Improvement or Resolution of Symptoms suggestive of EPI

Short Term (≤ 72 hours) Long Term (> 72 hours)
  • Resolution of
    • Steatorrhea
    • Rectal prolapse
  • Decreased
    • Frequency of stools after meals/snacks
    • Malodorous stools
    • Flatulence
    • Abdominal pain
    • Voracious appetite
  • Infants
    • Within 1 week resume/maintain median weight gain for age
      • Age Median g/day
          Females Males
        0-4 wks 29 34
        4-8 wks 34 40
        2-3 mos 24 27
        3-4 mos 20 21
        4-5 mos 16 17
        5-6 mos 13 14
        6-8 mos 11 11
        8-10 mos 9 9
        10-12 mos 8 8
        12-18 mos 7 7
        18-24 mos 7 6
  • Children
    • Within 1 month resume/maintain median weight gain for age
      • Age Median g/day
          Females Males
        2-3 yrs 6 5.5
        3-4 yrs 5.2 5.5
        4-5 yrs 4.7 5.5
        5-6 yrs 5 5.5
        6-7 yrs 6.3 6
        7-8 yrs 8.2 6.6
        8-9 yrs 10 7.7
        9-10 yrs 11 9
        10-11 yrs 12.3 10.7
        11-12 yrs 12.3 12.3
        12-13 yrs 12.6 14.2
        13-14 yrs 11.5 16
        14-15 yrs 9.3 16.2
        15-16 yrs 6 15
  • Adults
    • Sustained improvement in symptoms and nutritional parameters
      • (e.g., fat-soluble vitamin or essential fatty acid status, anthropometrics)
      • Note: due to other comorbidities, adults may not reach “normal” values
Charts in right column adapted from the WHO Child Growth Standards July 2010 and CDC Data Tables