1. In Cystic Fibrosis Patients Receiving Pancreatic Enzymes
The Effect of Proton Pump Inhibitors and H2 Blockers on BMI and Pulmonary Function Tests
In Cystic Fibrosis Patients Receiving Pancreatic Enzymes
Hani Fanous, MBBCh1; Guanghai Wang, MA1,2; Folasade Ogunlesi, MD1; Iman Sami, MD1
1Division of Pulmonary Medicine & Sleep, Children’s National Medical Center, 111 Michigan Avenue NW, Washington, DC, USA
2School of Psychology & Cognitive Science, East China Normal University, Shanghai , China
Cystic Fibrosis (CF) is an autosomal recessive condition that results in absent or dysfunctional Cystic Fibrosis Transmembrane Regulator (CFTR). More than 85% of CF patients have exocrine pancreatic insufficiency causing malabsorption of fat and fat-soluble vitamins, that impacts their weight and body mass index (BMI). There is a positive correlation between a BMI > 50% and pulmonary function assessed by FEV1.
In addition to pancreatic enzymes replacement therapy (PERT), some patients require Proton Pump Inhibitors (PPI) or H2 blockers not only to treat symptoms of gastroesophageal reflux, but also to decrease gastric acidity, and optimize pancreatic enzyme activity.
Background
Effect of H2 blockers and Proton Pump Inhibitors on Body Mass Index in Cystic Fibrosis patients receiving PERT 
Treatment with PPIs or H2 blockers puts patients at risk of poor calcium absorption and increased osteoporosis. We therefore sought to determine whether they added any benefit to patients in maintaining BMI and thereby improving their pulmonary function. Patients prescribed antacids either had symptoms of gastroesophageal reflux or suboptimal nutritional status and malabsorption necessitating a high dose of PERT. This may have introduced a bias since untreated patients were doing well from a nutritional standpoint. Patients on antacids maintained their BMI (difference of in group 2, and +0.4 in group 3).
Although there was no statistically significant difference between treated and untreated groups, without treatment groups 2 and 3 may have had a much lower BMI than the untreated group (1). The small sample size in each group was also a limitation.
Although the difference in mean FEV1 did not reach statistical significance due to the small sample size, it was felt to be clinically significant. Despite the fact that the treated groups had a comparable BMI to the untreated group this did not translate into improved lung function since their mean FEV1 was actually lower. This may reflect the fact that this group of patients had more problems with reflux which can impact lung function.
Discussion
To Determine:
The effect of H2 Blockers or PPI on mean BMI as a primary outcome.
The effect of H2 Blockers or PPI on mean FEV1 as a secondary outcome.
Objectives
Cross-sectional study of CF patients at Children’s National Medical Center CF center from January 2014 through December 2014 ages 2-25 years.
Indications for prescribing a PPI or H2 blocker:
Symptoms of gastroesophageal reflux.
Symptoms of malabsorption.
Poor growth that was not adequately treated despite a high dose of PERT.
Study Design & Methods
Effect of H2 blockers and Proton Pump Inhibitors on Pulmonary Function in Cystic Fibrosis patients receiving PERT
Treatment with PPIs or H2 blockers to reduce gastric acidity, to achieve an optimal environment for pancreatic enzyme functioning, maintained BMI but did not improve FEV1 in our cohort of CF patients.
Conclusion
Analysis of a larger sample size from the Cystic Fibrosis Foundation Registry.
Future Directions
Group 1 received pancreatic enzymes only (n=19, Mean age 9.9 years); Group 2, pancreatic enzymes plus a PPI (n=44, Mean age 12.2 years); Group 3, enzymes and an H2 blocker (n=23, Mean age 9.3 years). Total eligible patients n=86.
Mean BMI in Group 1 was 17.7 ± 3.4 (SD), 20.1 ± 11.8 (SD) in group 2, and 18.1 ± 3.7 (SD) in group 3, p=0.887    Pulmonary function testing showed a mean FEV1 of 90.1 ± 22.5 (SD) in group 1, 78.3 ± 23.9 (SD) in group 2 and 81.7 ± 28.4 (SD) in group 3, p=0.454.  There was no gender effect in any of the groups. 
There was no statistically significant difference in mean BMI or FEV1 in patients on PPI or H2 blockers when compared to those only on PERT supplements.
Results
1.Haupt ME, Kwasny MJ, Schechter MS, McColley SA. Pancreatic enzyme replacement therapy dosing and nutritional outcomes in children with cystic fibrosis. The Journal of pediatrics. May 2014;164(5): e1111.
2. CF Foundation . Cystic Fibrosis Foundation Annual Patient Registry Report, 2013.
3.Gelfond D, Ma C, Semler J, Borowitz D. Intestinal pH and gastrointestinal transit profiles in cystic fibrosis patients measured by wireless motility capsule. Digestive diseases and sciences. Aug 2013;58(8):
4.US Food and Drug Administration. Proton pump inhibitor drugs: drug safety communication – low magnesium levels can be associated with long-term use. March2, May 21,2013
References