About EPI in Patients With CF

Up to 90% of patients
with CF have EPI1

EPI is associated with a number of conditions

EPI can make life with CF even more difficult for patients and their caregivers2

Left untreated, EPI can compromise a growing child in many ways2

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

According to the Cystic Fibrosis Foundation Guidelines3:

  • The dangers of nutritional deficiency and negative consequences of eating associated with abdominal symptoms argue strongly in favor of proactive treatment

Early diagnosis and treatment of EPI can help patients absorb the nutrients they need4,5

EPI may cause the following symptoms2,6,7:

  • Abdominal pain
  • Bloating
  • Excessive flatulence
  • Frequent stools
  • Nausea
  • Malaise
  • Diarrhea
  • Steatorrhea

Poor nutritional status associated with untreated EPI may lead to poorer outcomes in patients with CF8

Effective management of EPI depends on9:

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Pancreatic enzyme replacement therapy, such as ZENPEP®

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Dietary modifications

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Regular nutritional assessment as a component of routine care

The Cystic Fibrosis Foundation states that patients with laboratory evidence of EPI should be started on PERT even in the absence of signs of fat malabsorption.3

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

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References: 1. 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
2. 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 3. Borowitz D, Robinson KA, Rosenfeld M, et al. 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 4. 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 5. Johnson CD, Williamson N, Janssen-van Solingen G, et al. Psychometric evaluation of a patient-reported outcome measure in pancreatic exocrine insufficiency (PEI). Pancreatology. 2019;19(1):182-190. doi:10.1016/j.pan.2018.11.013 6. 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 7. Johnson CD, Arbuckle R, Bonner N, et al. Qualitative assessment of the symptoms and impact of pancreatic exocrine insufficiency (PEI) to inform the development of a patient-reported outcome (PRO) instrument. Patient. 2017;10(5):615-628. doi:10.1007/s40271-017-0233-0 8. 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 9. Cystic Fibrosis Foundation. Pancreatic Enzymes Clinical Care Guidelines. Accessed November 2, 2022. https://www.cff.org/pancreatic-enzymes-clinical-care-guidelines#pancreatic-enzymes-clinical-care-guidelines-executive-summary