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Information for health care professionals |
Pancreatic Enzyme Replacement Therapy (PERT)Approximately 10-15% of patients with Cystic Fibrosis have malabsorption. The commonest way to test for this is to send a stool for faecal elastase. Most patients with pancreatic-insufficient CF will have an elastase level of <15 mcg/g stool. By the time newborn screening results are available (usually at the age of 3-4 weeks) faecal elastase levels have reached their normal values and so stool can be sent at diagnosis. All pancreatic insufficient patients require PERT from diagnosis; PERT is given for all foods that contain fat. Importantly some patients are initially pancreatic sufficient but become insufficient over time. Any pancreatic sufficient patient who has had a change in gastrointestinal symptoms or problems with weight gain should have a stool sent for repeat elastase levels. There are several available PERT formulations but Creon is the commonest enzyme supplement used and comes as the following preparations
The usual starting dose for newborn screened infants is half a scoop of Creon micro (5000 lipase units per scoop) with each feed. The dose is then adjusted according to fat intake, weight gain, and signs of malabsorption (such as abdominal pain and bloating and/or loose, oily stools). There are no specific guidelines for enzyme dosing and this is decided on an individual basis according to reported symptoms and a dietary record. The dose required varies widely and most experienced parents and older children become very capable at judging their own enzyme requirements based on what they are eating (and specifically their fat intake). The usual dose range is one Creon 10,000 capsule for between 3 and 8 grams of fat. Inevitably the dose of Creon increases as children get older and their appetites increase. All PERT should be enteric coated and enzyme capsules should be swallowed whole and not chewed. Babies are usually given their Creon with a spoon of apple sauce. It is important that PERT is not mixed in with a bottle or meal as the enzymes will be activated before they reach the small intestine. Generally PERT is taken at the beginning of a meal but it will often need to be taken in the middle of a meal or between courses. Enzyme administration may need to be altered in some situations e.g. if a naso-gastric tube is being used or if a patient is nil by mouth. This should be discussed with the CF team. Doses of PERT ideally will not exceed 10,000 lipase units/kg/day (but often they will do). This is because high doses of PERT have historically been associated with fibrosing colonopathy, but this complication is rarely seen today. The clinical situation should be reviewed in all patients who require high PERT dosing; a proton pump inhibitor will often be started if there are ongoing signs of malabsorption. Things to consider if malabsorption persists:
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