How should I initiate and monitor enzyme replacement therapy in patients with exocrine pancreatic insufficiency?

Initiation of Enzyme Replacement Therapy (ERT) in Exocrine Pancreatic Insufficiency:

• Offer oral pancreatic enzyme replacement therapy to patients diagnosed with exocrine pancreatic insufficiency to manage malabsorption symptoms.
• Use non-invasive testing such as stool elastase estimation to confirm exocrine pancreatic insufficiency before initiating therapy.
• Adjust the enzyme dose based on clinical response to minimise symptoms or signs of malabsorption.
• Consider adding an acid suppression agent (e.g., H2 receptor antagonist or proton pump inhibitor) if symptoms persist despite optimal enzyme therapy, noting this is an off-label use.

Monitoring of Enzyme Replacement Therapy:

• Regularly assess nutritional status, including total energy intake and weight, ideally with input from a specialist dietitian.
• At least annually, perform clinical and biochemical assessments for pancreatic exocrine function and malnutrition, adjusting treatment accordingly.
• In children under 16, consider more frequent monitoring (every 6 months).
• Monitor for complications such as distal intestinal obstruction syndrome and manage hydration and bowel function accordingly.
• Use available resources such as the Specialist Pharmacy Service to identify equivalent licensed pancreatic enzyme products, especially during supply disruptions.

These steps ensure effective initiation and ongoing management of enzyme replacement therapy in patients with exocrine pancreatic insufficiency.

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