Appeal type: Formulary Exception
Decision: Overturned

Diagnosis: Chronic Intestinal Pseudo-Obstruction (CIPO)

Treatment: Motegrity (prucalopride)

The insurer denied coverage for Motegrity (prucalopride)

The denial is overturned. 

The patient is an infant with a past medical history notable for intestinal failure, hypoganglionosis, intestinal perforation, intestinal failure associated liver disease, total parenteral nutrition (TPN) dependent, chronic intestinal pseudo-obstruction (CIPO) and feeding intolerance. His feeding intolerance is felt to be secondary to oral aversions, emesis and intestinal dysmotility. His bowel is surgically altered with 165 centimeters (cm) of small bowel ending in an end ileostomy. He receives the majority of his calories and hydration via TPN cycled x 12 hours. In addition, he receives partial enteral nutrition as Elecare 30 at 14 cubic centimeters (cc)/hour x 10 hours via a jejunostomy tube (JT). His current medications include calcium carbonate via gastrostomy tube (GT), IV (Intravenous) dextran, prucalopride via GT, ursodiol via GT and IV Pepcid. His last weight is 10.64 kilograms and his height is 72.5 centimeters. His ostomy output is reported as 200-500 cc per day on his current regimen. 

The insurer is denying coverage for the prucalopride as not medically necessary given that the child has not tried and failed 2 formulary alternatives including Linzess and Amitiza. His treating team is appealing the decision since he is clinically better when the prucalopride was started. The issue for consideration is the medical necessity of prucalopride for the treatment of this patient's intestinal dysmotility. 

This patient suffers from chronic intestinal pseudo-obstruction leading to intestinal failure related to small bowel dysmotility. He is TPN dependent with partial tube feeds via JT to provide enteral stimulation and to minimize liver injury.  

This patient demonstrates the clinical manifestations of CIPO as detailed in a review from UptoDate (see attached pdf file) in which the authors note the following:
Clinical features - Abdominal pain, bloating, and distension are the most common clinical features of chronic intestinal pseudo-obstruction (CIPO). In one series that included 59 patients, symptoms included abdominal distension (75 percent), abdominal pain (58 percent), nausea (49 percent), constipation (48 percent), heartburn/regurgitation (46 percent), fullness (44 percent), epigastric pain/burning (34 percent), early satiety (37 percent), and vomiting (36 percent). These symptoms may be acute, recurrent, or chronic. 

Acute episodes are characterized by abrupt onset of intense, cramping pain, abdominal distention, nausea, and vomiting. After the acute episode, patients may be asymptomatic or, more often, continue to experience symptoms due to delayed transit in the proximal (eg, anorexia, early satiety, nausea and vomiting) and/or distal (constipation) gastrointestinal tract. Patients may have diarrhea due to small bowel bacterial overgrowth. Weight loss results from impaired intestinal transit, malabsorption due to bacterial overgrowth, and inadequate intake due to exacerbation of symptoms with food ingestion. 

In this case, all of his symptoms are related to dysmotility of his upper intestinal tract (stomach and small intestine). Constipation is irrelevant since his colon is not even in circuit (end ileostomy).

Based on his symptom complex, anatomy and diagnosis, prucalopride is the promotility agent of choice supported by the pediatric expert opinion as above. The suggested formulary alternatives are not appropriate for his condition. The clinical response to this agent in improved enteral tolerance is critical to his overall well-being and to protect against bacterial translocation and progressive liver injury. This decision was based on the information submitted for review and the 2 reviews of CIPO diagnosis and management from Uptodate cited above.

The insurer's denial of Formulary Exception for Motegrity (prucalopride) is overturned. The requested medication is likely to be more effective than the formulary options and will produce a more clinically beneficial outcome.