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

Goals and Metrics

Patient Education

Provider Resources

Child with Confirmed EPI

Initiate PERT
Titrate PERT dose

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

Child with Suspected or Confirmed
Exocrine Pancreatic Insufficiency (EPI)

Team Assessment

History and 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

Acquired Pancreatic Insufficiency
and/or
Congenital Pancreatic Insufficiency

Cystic Fibrosis (CF)

Child presents with any of the following:
- 2 positive PI CF variants
- Meconium ileus
- Rectal prolapse
- Poor weight gain
- Steatorrhea

Initiate PERT at First Visit

Formulations | Dosing Recommendations | Examples

Do not initiate PERT

Screen for Exocrine Pancreatic Insufficiency

Obtain Fecal Elastase
- Requires a single spot stool sample
- Perform on solid stool
- Results inaccurate if:
  - Stool sample is loose/watery
  - Test is performed in term infants < 72 hrs of age or preterm infants < 2 wks of age
Low result must be confirmed with repeat testing

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

Repeat fecal elastase to confirm diagnosis
Refer to subspecialist for evaluation
- Pulmonary/CF Center
- GI
Refer to clinical nutrition as indicated
Consider RUQ ultrasound
Additional testing by subspecialist
Consider PERT while awaiting
subspecialist evaluation

Repeat fecal elastase unless genetic variants consistent with CF and EPI
Refer to subspecialist for evaluation
- Pulmonary/CF Center
- GI
Refer to clinical nutrition as indicated
Consider RUQ ultrasound
Additional testing by subspecialist

Monitor
- Growth
- Fat malabsorption

Monitor
- Growth
- Fat malabsorption
- Repeat fecal elastase in 1 yr or sooner if clinical symptoms

Initiate PERT
Formulations | Dosing Recommendations | Examples

Adequate Response to PERT

Inadequate Response to PERT

Monitor
- Growth
- Fat malabsorption
- Annually as clinically indicated
  - Fecal elastase
  - Fat-soluble vitamin levels
    - Vitamin A
    - 25-OH vitamin D
    - Vitamin E
    - PT/INR as a measure of vitamin K

Consider Titrating PERT Dose
Refer to clinical nutrition as indicated

Posted: March 2019
Last Revised: December 2024

Authors:
- L. Padula, RD;
- J. Brownell, MD;
- E. Reid, RD;
- M. Mascarenhas, MD;
- S. Sadgwar, RN;
- L. Shanley, PharmD;
- H. McKnight, CRNP;
- A. Maqbool, MD

Editors: Clinical Pathways Team

Evidence

CHOP Programs

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The clinical pathways are based upon publicly available medical evidence and/or a consensus of medical practitioners at The Children’s Hospital of Philadelphia (“CHOP”) and are current at the time of publication. These clinical pathways are intended to be a guide for practitioners and may need to be adapted for each specific patient based on the practitioner’s professional judgment, consideration of any unique circumstances, the needs of each patient and their family, and/or the availability of various resources at the health care institution where the patient is located.

Accordingly, these clinical pathways are not intended to constitute medical advice or treatment, or to create a doctor-patient relationship between/among The Children’s Hospital of Philadelphia (“CHOP”), its physicians and the individual patients in question. CHOP does not represent or warrant that the clinical pathways are in every respect accurate or complete, or that one or more of them apply to a particular patient or medical condition. CHOP is not responsible for any errors or omissions in the clinical pathways, or for any outcomes a patient might experience where a clinician consulted one or more such pathways in connection with providing care for that patient.

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

Evidence

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