Pancreatic enzyme replacement therapy (PERT)

What it is

In about 85% of people with CF, thick secretions block the pancreatic ducts so digestive enzymes can’t reach the gut (pancreatic insufficiency). Without them, food — especially fat — isn’t absorbed, causing poor growth, greasy stools, and vitamin deficiency. PERT replaces those enzymes: capsules containing lipase, protease, and amylase (Creon, Zenpep, Pancreaze, Pertzye, Viokace) are taken with every meal and snack so nutrients can be absorbed. Dosing is individualized and paired with fat-soluble vitamin supplements (A, D, E, K).

How it’s taken

Taken by mouth with every meal and snack that contains fat. Dose is based on body weight and fat intake and adjusted by the CF care team and dietitian. Capsules are usually swallowed whole (or the beads sprinkled on soft acidic food for infants), not crushed or chewed.

What to expect

PERT is lifelong for those with pancreatic insufficiency. When dosed well it normalizes digestion, growth, and weight. The CF team monitors nutrition and symptoms and adjusts the dose; fat-soluble vitamins are given alongside.

Risks & side effects

• Too little: ongoing malabsorption, poor growth, greasy stools, vitamin deficiency
• Very high doses have been linked to fibrosing colonopathy (a bowel-narrowing condition)
• Abdominal discomfort, constipation, or bloating
• Mouth or skin irritation if capsules are chewed or left in the mouth

Alternatives