DESENSITIZATION TO PANCREATIC-ENZYME INTOLERANCE IN A CHILD WITH CYSTIC-FIBROSIS

Objective. Pancreatic enzyme is essential in the treatment of cystic f ibrosis (CF), but intolerance to it occasionally occurs. We encountere d a child who was intolerant to multiple commercially available prepar ations of pancreatic enzymes and, hence, desensitization was attempted , with success. Case Presentation. A 33-month-old girl was diagnosed w ith CF at 6 months of age. initially, she was started on Pancrease MT 16, which was subsequently discontinued because fecal fat studies were normal and she seemed to do well on Nutramigen and vitamin supplement s. At 29 months of age, she developed diarrhea with bulky stools and w eight loss. A fecal fat 72-hour study revealed a coefficient of absorp tion of 50%. She was treated with Pancrease MT 16, but had consistent vomiting 1 to 2 hours after administration of enzymes. The vomiting oc curred on switching to different pancreatic enzymes preparations, ie, Creon 10, Viokase, and Pancrease MT 16. Vomiting.occurred even with sm all doses of enzymes disguised in food. She had no history suggestive of gastroesophageal reflux, peptic ulcer, or pork allergy, and no vomi ting on days when enzymes were not given. This was suggestive of type I hypersensitivity reaction. Pancreatic enzymes were discontinued, and she was given a low-fat, high-carbohydrate diet with satisfactory wei ght gain. Methods. Double-blind, placebo-controlled titrated oral chal lenges with pancreatic enzymes resulted in definite vomiting within 1 to 1.5 hours after challenges with Viokase and Pancrease MT 16, but no t with placebo. Rush oral desensitization with Viokase solution was at tempted, starting with 5 mg, and the dose was doubled every 20 minutes , aiming to reach a cumulative dose of 700 mg. However, the child vomi ted when a cumulative dose of 315 mg was reached. Another trial of slo wer desensitization was done using Pancrease MT 16 (1 capsule: 16 000 U of lipase, 48 000 U of amylase, and 48 000 U of protease), starting with 1/4 capsule per day, with increments of 1/4 capsule every 3 days, until an entire capsule was reached by day 10, then increased by simi lar to 1/2 capsule every 4 days until reaching the therapeutic dose of 1 capsule with each meal by day 25. Results. The patient tolerated th is fairly well and has been on this treatment and regular diet for >1 year, without any adverse reaction. This illustrates a rare case of ga strointestinal adverse reaction to pancreatic enzymes that was treated successfully with desensitization. Conclusion. Pancreatic enzyme into lerance, although rare, would be a major problem in the management of patients with CF. Hence, desensitization would be essential and may be accomplished successfully using the protocol described in this report .