About EPI in Patients With CF

EPI is present in up to
90% of patients with CF

EPI is characterized by deficiencies in the amount
or activity of exocrine pancreatic enzymes. This can
result in the inability to digest food properly, or maldigestion.1-3

EPI is associated with a number of conditions

Early diagnosis and treatment can help your patients eat with fewer uncomfortable EPI symptoms1,4

Untreated EPI has been linked to4:

  • Impaired height and weight
  • Malabsorption leading to vitamin and nutrient deficiencies

Vitamin deficiency risks include:

Graphic vitamin D
Graphic vitamin A
Graphic vitamin E
Graphic vitamin K

EPI can make life with CF even more difficult

Symptoms of EPI include4-7:

  • Abdominal pain
  • Bloating
  • Flatulence
  • Nausea
  • Malaise
  • Frequent stools
  • Steatorrhea
    (foul-smelling, bulky, oily stools)
  • Diarrhea
  • Weight loss due to malabsorption

Poor nutritional status associated with untreated or insufficiently treated EPI may lead to poorer outcomes in patients with CF.5

Starting patients on PERT

According to the Cystic Fibrosis Foundation guidelines, patients with laboratory evidence of EPI should be started on a PERT even in the absence of signs or symptoms of fat malabsorption, since the dangers of nutritional deficiency and negative consequences of the association of feedings with abdominal pain argue strongly in favor of proactive treatment.8

The food-driven GI symptoms of EPI can make mealtime difficult for patients and caregivers4

Managing EPI includes9:

Pill icon

Pancreatic enzyme replacement therapy

Vitamins and bottle icon

Maintaining adequate nutritional status with vitamin/nutrient supplements

Bottle and weight icon

Dietary modifications

CF=cystic fibrosis; EPI=exocrine pancreatic insufficiency; GI=gastrointestinal; PERT=pancreatic enzyme replacement therapy.

References: 1. Perbtani Y, Forsmark CE. Update on the diagnosis and management of exocrine pancreatic insufficiency. F1000Res. 2019;8:F1000 Faculty Rev-1991. doi:10.12688/f1000research.20779.1 2. Brennan GT, Saif MW. Pancreatic enzyme replacement therapy: a concise review. JOP. 2019;20(5):121-125. 3. Capurso G, Traini M, Piciucchi M, Signoretti M, Arcidiacono PG. Exocrine pancreatic insufficiency: prevalence, diagnosis, and management. Clin Exp Gastroenterol. 2019;12:129-139. doi:10.2147/CEG.S168266 4. Othman MO, Harb D, Barkin JA. Introduction and practical approach to exocrine pancreatic insufficiency for the practicing clinician. Int J Clin Pract. 2018;72(2):e13066. doi:10.1111/ijcp.13066 5. Wooldridge JL, Heubi JE, Amaro-Galvez R, et al. EUR-1008 pancreatic enzyme replacement is safe and effective in patients with cystic fibrosis and pancreatic insufficiency. J Cyst Fibros. 2009;8(6):405-417. doi:10.1016/j.jcf.2009.07.006 6. Domínguez-Muñoz JE. Chronic pancreatitis and persistent steatorrhea: what is the correct dose of enzymes? Clin Gastroenterol Hepatol. 2011;9(7):541-546. doi:10.1016/j.cgh.2011.02.027 7. Ockenga J. Importance of nutritional management in diseases with exocrine pancreatic insufficiency. HPB (Oxford). 2009;11(suppl 3):11-15. doi:10.1111/j.1477-2574.2009.00134.x 8. Borowitz D, Robinson KA, Rosenfeld M, et al; Cystic Fibrosis Foundation. Cystic Fibrosis Foundation evidence-based guidelines for management of infants with cystic fibrosis. J Pediatr. 2009;155(suppl 6):S73-S93. doi:10.1016/j.jpeds.2009.09.001 9. Pancreatic enzymes clinical care guidelines. Cystic Fibrosis Foundation. Accessed July 1, 2021. https://www.cff.org/Care/Clinical-Care-Guidelines/Nutrition-and-GI-Clinical-Care-Guidelines/Pancreatic-Enzymes-Clinical-Care-Guidelines/