Clinical Pathway for Pancreatic Enzyme Replacement Therapy (PERT) in Children with or at Risk for Exocrine Pancreatic Insufficiency (EPI)
- Conditions with High Risk of EPI
- Cystic Fibrosis
- Acquired Pancreatic Insufficiency
- Chronic Pancreatitis
- HI Status-post Pancreatectomy
- Chemotherapy
- Congenital Pancreatic Insufficiency
- Shwachman-Diamond Syndrome
- Johanson-Blizzard Syndrome
- Pearson Syndrome
- Jeune Syndrome
- Pancreatic Aplasia
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
- PERT
- Enzyme Formulations
- Dosing Recommendations
- Initiation Examples
- Refer to Clinical Nutrition as clinically indicated
- Child with Confirmed EPI
- Initiate PERT
- Titrate PERT dose
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
Confirm EPI
- Assess Pancreatic Exocrine Function
- Obtain Fecal Elastase, Interpret Fecal Elastase Results
- Additional Lab testing based on Suspected Condition
Normal Fecal Elastase
> 500 mcg/g
> 500 mcg/g
Borderline Fecal Elastase
200-500 mcg/g
200-500 mcg/g
Abnormal Fecal Elastase
< 200 mcg/g
< 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
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: May 2021
Authors: Padula, L. RD; Brownell, J. MD; Reid, E. RD; Jansma, B. CRNP; Mascarenhas, M. MD; Sadgwar, S. RN;
Shanley, L. PharmD; McKnight-Menci, H. CRNP; Maqbool, A. MD
Revised: May 2021
Authors: Padula, L. RD; Brownell, J. MD; Reid, E. RD; Jansma, B. CRNP; Mascarenhas, M. MD; Sadgwar, S. RN;
Shanley, L. PharmD; McKnight-Menci, H. CRNP; Maqbool, A. MD
Evidence
- CF Diagnosis Clinical Care Guidelines
- Pancreatic insufficiency in Cystic Fibrosis
- Enteral Tube Feeding in Cystic Fibrosis Clinical Care Guidelines
- Pancreatic Enzymes in Cystic Fibrosis Clinical Care Guidelines
- Pancreatic Enzyme Replacement Therapy for Pancreatic Exocrine Insufficiency: When Is It Indicated, What Is the Goal and How to Do It?
- A Primer on Exocrine Pancreatic Insufficiency, Fat Malabsorption, and Fatty Acid Abnormalities
- Practical Guide to Exocrine Pancreatic Insufficiency - Breaking the Myths