LAUSR

Individuals with pancreatic insufficiency (PI) as a result of conditions such as cystic fibrosis (CF) require pancreatic enzyme replacement therapy (PERT) to assist macro and micronutrient digestion and absorption. Patients with PI receiving enteral feeds require PERT to digest their enteral formula. Standard polymeric feeds administered with PERT have been shown to be well tolerated and absorbed and are generally the first-line option for enteral feeding in CF [1]. An elemental formula high in medium chain triglycerides (MCT) may be an alternative if a patient continues to show signs of malabsorption or poor growth despite administering PERT or if no options for PERT administration are appropriate [1]. One study however demonstrated CF infants on a MCT-based formula had improved fat absorption with PERT vs no PERT, suggesting that even with high MCT elemental feeds, addition of PERT may be optimal [2]. There are several options for dosing enzymes with enteral feeds. The method chosen will depend on the feeding regimen (i.e. continuous or bolus feeds), the patient’s ability to take enzymes orally and the size and type of the patient’s enteral feeding tube. According to current national guidelines [1], PERT should be taken orally with enteral feeds wherever possible with the dose based on fat content of the enteral formula. If oral administration is not possible, there are two previously described alternatives. Ferrie et al. [3] describe dissolving crushed or whole enzyme beads in sodium bicarbonate to form an activated solution ready to be delivered directly into the feeding tube or mixed into the enteral formula. Alternately, the whole enzyme beads can be suspended in thickened fruit juice or pureed baby food and administered directly into the feeding tube [3, 4]. Crushing enzyme beads and adding directly to enteral formula has not been previously detailed in the literature. We describe the case of an extremely premature infant with CF where this method proved to be effective when alternative methods were contraindicated. It should be noted that a novel in-line digestive cartridge containing immobilized lipase [5], which digests fat as the enteral feed passes through the cartridge, is available in North America but not internationally. This is a useful option for PERT with enteral feeds where it is available. Extremely premature (24-week gestation) female dichorionic diamniotic twins diagnosed with F508del homozygous CF on newborn screening were referred to our CF team from the neonatal intensive care unit at our CF centre [6, 7]. Twin 2 (birthweight 660 g) passed away on day 40 after withdrawal of intensive care due to respiratory failure and significant neonatal complications. Twin 1 (birthweight 510 g, birth length 29 cm) also had a difficult neonatal course, requiring ventilator support for 60 days before extubation to CPAP, and an initial period of total parenteral nutrition (TPN). Enteral feeds of breast milk with human milk fortifier (HMF) were introduced at 26.5-week corrected gestational age (CGA) via 5-Fr NGT. By 28-weeks CGA, twin 1 was weaned from TPN to full continuous nasogastric feeds of breast milk with additions of HMF and protein powder. PERT was commenced at 30-weeks CGA due to insufficient weight gain and clinical symptoms of malabsorption (including fat globules on stool microscopy) despite enteral feeds providing 100% estimated energy requirements (EER). The patient was unable to take enzymes orally as she was intubated and ventilated, and had immature oral skills. Dissolution of enzymes in * Jodi Grunert [email protected]