Case Report Describes Unconventional Method for Delivering PERT in Premature CF Baby

Case Report Describes Unconventional Method for Delivering PERT in Premature CF Baby
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Adding pancreatic enzymes to “pre-digest” food was found to be an effective way to ensure nutrition in an infant with cystic fibrosis who was being fed through a feeding tube, according to a recent case report.

The report, “Crushing pancreatic enzymes with enteral feeds in an extremely premature infant with cystic fibrosis—a novel and effective technique,” was published in the European Journal of Clinical Nutrition.

Cystic fibrosis can result in pancreatic insufficiency, when the pancreas doesn’t make enough digestive enzymes. In such instances, proper digestion and nutrient absorption often require pancreatic enzyme replacement therapy (PERT) — essentially, treatment with the missing enzymes.

The new report details the case of a baby with cystic fibrosis who was born extremely premature, after 24 weeks of gestation. The baby had “a difficult neonatal course,” requiring mechanical assistance breathing, and needing a feeding tube to receive food.

About 45 weeks after birth, the baby was started on PERT due to poor weight gain and other symptoms of insufficient nutrition absorption.

Typically, PERT is administered by mouth, but this is not feasible when a feeding tube is being used. Two methods to administer PERT via a feeding tube have been described previously: dissolving the enzyme beads in sodium bicarbonate, or suspending the beads in fruit juice.

However, neither of these methods was deemed suitable for this baby.

“Dissolution of enzymes in sodium bicarbonate was contraindicated as the patient was being treated intermittently with sodium bicarbonate for metabolic acidosis,” the investigators wrote. “It was also not considered appropriate to introduce fruit juice to the gut of an extremely premature infant.”

Instead, the researchers crushed the PERT beads and added them directly to the infant’s feed. Rather than trying to get the enzymes to the baby’s small intestine (which is where they normally would digest food), the enzymes were added straight to the food, such that the baby was given food that had been “pre-digested.”

“This method was trialled because no other option of enzyme administration was practically or medically suitable,” the researchers wrote.

The treatment proved effective, as the baby was able to gain more weight and had improved clinical symptoms. She was treated for more than seven months before being discharged from the hospital.

“This case outlines the successful implementation of an alternative method of administering PERT for patients receiving enteral feeds. The addition of crushed enzyme beads to pre-digest enteral formula has been shown to be safe and effective in this patient over a long-term hospital admission, with no known adverse outcomes,” the authors concluded.

Marisa, a science writer, holds an MS in Cellular and Molecular Pathology from the University of Pittsburgh, where she studied novel genetic drivers of ovarian cancer. She specializes in cancer biology, immunology, and genetics. Marisa began working with BioNews in 2018, and has written about science and health for SelfHacked and the Genetics Society of America. She also writes/composes musicals and coaches the University of Pittsburgh fencing club.
Total Posts: 34

Inês holds a PhD in Biomedical Sciences from the University of Lisbon, Portugal, where she specialized in blood vessel biology, blood stem cells, and cancer. Before that, she studied Cell and Molecular Biology at Universidade Nova de Lisboa and worked as a research fellow at Faculdade de Ciências e Tecnologias and Instituto Gulbenkian de Ciência.

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Marisa, a science writer, holds an MS in Cellular and Molecular Pathology from the University of Pittsburgh, where she studied novel genetic drivers of ovarian cancer. She specializes in cancer biology, immunology, and genetics. Marisa began working with BioNews in 2018, and has written about science and health for SelfHacked and the Genetics Society of America. She also writes/composes musicals and coaches the University of Pittsburgh fencing club.
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