PPIs and Fat Absorption in CF and EPI

Fat malabsorption contributes to poor nutritional status in people with cystic fibrosis (CF)
and exocrine pancreatic insufficiency (EPI). Prescribing gastric acid-reducing agents such as
proton pump inhibitors (PPIs) and histamine receptor antagonists (H2RAs) as an adjunct to
pancreatic enzyme replacement therapy (PERT) to improve PERT efficacy and dietary fat
absorption has become accepted clinical practice in CF, despite limited evidence to support
the practice. Establishing the efficacy and true health benefit of acid suppression for
nutritional status and outcomes in CF is particularly important in light of potential health
risks and cost associated with long-term or even lifetime use of these medications.

This study aims to characterize changes in fat malabsorption using the coefficient of fat
absorption (CFA) as the primary endpoint in subjects who are on and off acid suppression with
a PPI in addition to PERT. Additionally, the SmartPill® will be used to evaluate duodenal
power of hydrogen (pH) while on and off acid suppression, and the malabsorption blood test
(MBT) will be used to characterize changes in fat absorption. Associations will be explored
between changes in nutritional status (weight, height, BMI), clinical GI symptoms, and
quality of life in subjects treated with PPI vs. placebo.

Inclusion Criteria:

- Cystic fibrosis and pancreatic insufficiency (Fecal elastase <200 ug/g stool)

- Age ≥12 years

- In usual state of good health

- Willing to participate in a four-month study with three visits

Exclusion Criteria:

- Forced expiratory vital capacity at one second (FEV1) <40% predicted

- Pregnancy or breast feeding

- Other illness affecting growth or nutritional status

- Unwillingness to continue their clinically established PERT dose for the duration of
the study

- Use of other medication that affects dietary fat absorption

- Allergy to soy products

- Allergy to safflower products

- For subjects ≥18 years, celiac disease or allergy to gluten