This article was sponsored by AbbVie, Inc.
Underdosing.1 Noncompliance.2,3 Unclear treatment expectations.4 These are some of the ongoing challenges associated with pancreatic enzyme replacement therapy (PERT) that highlight the clear need for increased pharmacist-to-patient engagement. In this article, we will discuss appropriate medication administration and the role of pharmacists in managing PERT (TABLE 12-7).2,5-7
DIGESTIVE ENZYME SECRETION ORIGINATES IN THE PANCREAS
In healthy adults, normal pancreatic functioning enables the body to properly digest and absorb nutrients.3 The primary role of the pancreas is exocrine function, with 85% of the organ responsible for the secretion of digestive enzymes, water, and bicarbonate into the duodenum.8 The release of enzymes helps people properly digest fats, carbohydrates, and proteins.3,7,9
WHEN EXOCRINE PANCREATIC FUNCTION IS INSUFFICIENT
Exocrine pancreatic insufficiency (EPI) occurs when the pancreatic enzyme activity in the intestinal lumen is reduced below the minimum level needed to maintain normal digestion.7,10 Certain underlying conditions that affect the pancreas are associated with EPI (TABLE 29,11-17).7,9,11,12 Two major effects of EPI include maldigestion of fat and malabsorption of nutrients, such as fat-soluble vitamins, including A, D, E, and K.7,9 Patients with EPI may experience symptoms resulting from undigested fat passing through the gastrointestinal tract.7,9 These symptoms can vary, but may include steatorrhea (bulky, oily, foul-smelling stools), diarrhea, unexplained weight loss, flatulence, bloating, and abdominal pain.3,4,7,9,18
PERT: THE STANDARD OF CARE FOR EPI3
From March 1, 2010, to February 1, 2016, more than 7 million PERT prescriptions were filled in the United States (FIGURE19).19 PERT is prescribed to help mimic the physiological pattern of pancreatic exocrine secretion, delivering enzymes into the duodenum together with food.3 PERT includes 3 types of enzymes: lipases, amylases, and proteases, which help the body break down and absorb fats, carbohydrates, and proteins, respectively.3,20
To be effective in managing EPI, PERT must be taken appropriately to ensure that both enzymes and food reach the duodenum at the same time.3,5,21
ACCURATE DOSING OF PERT IS ESSENTIAL FOR EPI MANAGEMENT
The prescribed PERT dose should be individualized and adjusted based on weight, fat content of the patient’s diet, degree of steatorrhea, and clinical symptoms.4 These criteria should also be used for titrating patients to the appropriate dose.4
According to the Cystic Fibrosis Foundation (CFF) Consensus Conference Guidelines, weight-based enzyme dosing for patients 4 years or older should begin at 500 lipase units/kg/meal, adjusting to a maximum of 2500 lipase units/kg/meal, if needed (TABLE 35).5 The total daily dose should not exceed 10,000 lipase units/kg.3,5 Using a fat-content-of-the-diet approach, patients will typically need between 500 lipase units and 4000 lipase units per gram of fat ingested per day.4,5,22
The total daily PERT dose should reflect consumption of approximately 3 meals and 2 or 3 snacks per day.4,5,22 Typically, only half the prescribed dose is needed for snacks.5
PERT UNDERDOSING: AN ONGOING PROBLEM
Although PERT has been proven efficacious in the treatment of EPI, nutrient and fat absorption depend on appropriate weight-based dosing.3,4 It is important to continue to assess a patient’s response to therapy to help ensure accurate dosing.5
If patients are still experiencing symptoms after PERT initiation, they should be encouraged to have a follow-up visit with their physician.
THE REALITY OF PATIENT EXPERIENCE ON PERT
Despite PERT being the standard of care for EPI treatment, a review of prescription data shows that nearly 37% of new PERT patients (not including those with cystic fibrosis) did not refill their prescription.1
The CFF Consensus Conference Guidelines define poor response to therapy as patients continuing to experience abdominal complaints (such as pain, bloating, and flatus), abnormal stool movement (such as loose or frequent stools, overt diarrhea, and symptomatic steatorrhea), and/or poor development in children (caused by malnutrition) despite treatment with PERT.5,6 If patients are experiencing any of these symptoms, there may be possible factors that are causing them to have a poor response to treatment (TABLE 45,9,20,21).5
COUNSELING PATIENTS PRIOR TO PERT USE
Consumption of PERT with food contributes to the effectiveness of managing EPI.9 PERT has been proven to be effective when taken in tandem with high-fat diets compared with low-fat diets.9 Low-fat diets can also be connected to insufficient intake of fat-soluble vitamins.9 Therefore, it is important to note that fat reduction is no longer considered a part of the management plan for EPI due to chronic pancreatitis.9
It is essential that patients understand the importance of taking PERT with milk or snacks that contain fat, at a dose that is typically half of their prescribed dose.5
Here is what patients should know before starting PERT2,5,21:
Patients who are prescribed PERT should also consider seeing a dietitian to ensure they are consuming the recommended amounts of fats and calories.10 For example, in patients with cystic fibrosis, the CFF Consensus Conference Guidelines recommend that “patients should consume a high-calorie diet with unrestricted fat which is appropriate for age and clinical status.”5
In addition to PERT, various elements can factor into a patient’s comprehensive EPI management plan.3,9,10 Patients with EPI may be advised to eat a nutritionally well-balanced diet and take vitamin and mineral supplements, including fat-soluble vitamins A, D, E, and K. Smoking cessation and alcohol cessation are also recommended to help manage EPI.3,9,10
ROLE OF THE PHARMACIST
PERT may be an unfamiliar type of treatment for patients; thus, there is an opportunity for pharmacists to play an active role in EPI management. Before dispensing PERT, it is imperative that patients understand both the dosing schedule and what to expect with treatment. Pharmacists should also instruct patients to speak with their physician before discontinuing therapy. Pharmacist-specific goals for patients with EPI should include dose verification, proper expectation setting, appropriate treatment administration, and comprehensive PERT counseling.2,5-7
INITIATING THE PHARMACIST-TO-PATIENT DISCUSSION ABOUT PERT
Many factors can contribute to the success of PERT treatment. Pharmacists should have ongoing communication with patients to ensure they are comfortable taking PERT, understand how it is different from other medications, and have all the knowledge they need throughout their course of treatment.
Here are some questions to help start the discussion with patients:
Pharmacists can get more information about EPI and PERT from professional organizations (TABLE 5). Additionally, pharmacists can help patients have a more informed discussion with their doctor by referring them to IdentifyEPI.com.