Question
What is pancreatic enzyme replacement therapy (PERT)?
Answer
The first and probably the most common issue is pancreatic insufficiency. Typically, there is thin mucus around the normal pancreas. However, in Cystic Fibrosis, this mucus is very thick, and it blocks the passage of pancreatic digestive enzymes from reaching the small intestine. Without these enzymes, patients will have malabsorption of fats, proteins, carbs, and fat-soluble vitamins. How can you tell if your patient is pancreatic insufficient? Well, they will have big bulky, oily, foul-smelling, and frequent stools. They will also have abdominal discomfort, pain, and even weight loss.
How do we fix this? With pancreatic enzyme replacement therapy (PERT), enzymes help digest and absorb fats, proteins, and carbs. Each enzyme capsule contains lipase to break down fats, amylase to break down carbs, and protease to break down proteins. Besides the history of greasy stools, we will send off stool specimens to check for fecal elastase to officially diagnose pancreatic insufficiency. If the fecal elastase is less than 200, the patient is pancreatic insufficient, meaning they need enzymes. If the fecal elastase is greater than 200, they are pancreatic sufficient and do not need enzymes. Some patients with CF mutations that are not as severe or disease-causing will be pancreatic sufficient, and they do not need to be on enzyme therapy.
As mentioned before, pancreatic enzymes are released into the small intestine. Therefore the enzyme capsules are enteric-coated to withstand stomach acid and will only get activated in the small intestine, which is not as acidic. Patients have to take these enzymes before every meal and every snack. The goal is not to exceed 2,500 units of lipase per kilo per meal.
This Ask the Expert is an edited excerpt from the course, Cystic Fibrosis: A Comprehensive Overview, presented by Prigi Varghese, MSN, APRN, CPNP-PC.
