Clinical Pathway for Pancreatic Enzyme Replacement Therapy (PERT) in Children with or at Risk for Exocrine Pancreatic Insufficiency (EPI)
Clinical Pathway for Pancreatic Enzyme Replacement Therapy (PERT) in Children with or at Risk for Exocrine Pancreatic Insufficiency (EPI)
Child with/at Risk for Exocrine Pancreatic Insufficiency (EPI)
This pathway provides guidance to providers when considering the need for the initiation or escalation of pancreatic enzyme replacement therapy (PERT) to treat exocrine pancreatic insufficiency (EPI).
Definitions
- Exocrine pancreatic insufficiency (EPI): also called pancreatic insufficiency, this is the condition describing impaired digestive and buffering functions (exocrine functions) of the pancreas. The term EPI is generally applicable to children of all ages.
- Children with EPI have reduced macronutrient (primarily fat) and micronutrient (fat-soluble vitamin) absorption.
- In the short term, this can cause distressing gastrointestinal symptoms; in the longer term, untreated EPI may result in malnutrition.
- Pancreatic enzyme replacement therapy (PERT) is medication containing enzymes that provide the digestive function absent in 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
Exclusions
Children with normal exocrine pancreatic function
- Fecal elastase-1 > 500 mcg/g