Appeal type: Medical necessity
Decision: Overturned

Diagnosis: Cystic Fibrosis, Exocrine Pancreatic Insufficiency

Treatment: Relizorb

The insurer denied coverage for Relizorb.

The denial is overturned.

This is a female child with a past medical history notable for cystic fibrosis (CF), chronic lung disease, exocrine pancreatic insufficiency, and poor weight gain status post gastrostomy tube placement for supplemental nighttime tube feeds. As part of her nutritional intervention, the patient has been receiving pancreatic enzyme replacement therapy (PERT) with Creon capsules. In order to optimize her nutritional state, the patient's treating team prescribed Relizorb in line digestive enzyme cartridges during her continuous nighttime feeds that she had been using with reported improved weight gain and overall clinical condition. 

This patient has advanced CF. She has high nutritional needs given the energy requirements associated with her condition, her pulmonary infections, and her maldigestion associated with exocrine pancreatic insufficiency. The mainstay of treatment for pancreatic insufficiency in CF is PERT. PERT clearly improves fecal fat absorption in most patients with pancreatic insufficiency as evidenced by increasing the coefficient of fat absorption, decreased stool frequency, and improved stool consistency in both adult and pediatric patients with CF. The effectiveness of PERT in patients with CF may be limited by variations in the pH of the gastrointestinal tract. Several studies suggest that suppression of gastric acid improves the efficiency of PERT in CF, but most of the evidence is low quality. 

Evidence-based guidelines for administration of PERT for enteral tube feeding have not been established. Current consensus on PERT management includes oral administration of PERT prior to the bolus feeds; however, at the time the guidelines were written, there were no recommended methods for enzyme administration with nocturnal tube feedings. CF patients receiving nocturnal feeds are encouraged to take enzymes orally at the beginning of nocturnal feed and at the end of the feeding; also, if possible, PERT is provided in the middle of the feed. For patients who are not able to take PERT orally, the microspheres can be administered via the feeding tube. The practice of mixing enzymes with the enteral feeds can potentially lead to tube occlusion and inconsistent enzyme delivery.

A novel delivery system (Relizorb) consisting of lipase immobilized onto polymeric carrier beads is available for children who receive overnight tube feedings, usually by gastrostomy with a feeding pump. The beads are packaged into a cartridge that can be placed in line with the feeding tube, releasing small amounts of lipase in steady dosing into the formula overnight. The evidence of improved benefit with the use of this product comes from the results of the ASSURE trial published in JPGN 2018. The results demonstrated that the device use was found to be safe, and well tolerated. This was the first prospective study to show enteral nutrition can improve fatty acid abnormalities in CF. Relizorb may have important long-term therapeutic benefits in patients with CF.

This patient would benefit from the use of Relizorb during her nighttime tube feeds as supported by literature. The use of Relizorb is approved by the FDA in children, is medically necessary for this patient's condition, and is supported by studies to be safe and effective. 

The health plan did not act reasonably with sound medical judgment, and in the best interest of the patient.

Based on the above, the medical necessity for Relizorb is substantiated. The insurer's denial should be overturned.