Pancreatic enzyme replacement therapy (PERT) is a major factor associated with achieving optimum growth and nutritional status in cystic fibrosis (CF) patients with pancreatic insufficiency and consequent malabsorption. Currently in Australian CF clinics policies for the usage of PERT vary considerably. This paper highlights the current issues related to fat absorption and use of PERT in CF. It also provides evidence to support the recommendation of a PERT dose based on a standard ratio of lipase units per gram of dietary fat consumed for individual patients. A consistent approach to PERT doses will facilitate identification of patients whose PERT intake increases their risk of fibrosing colonopathy and for whom gastroenterological review is warranted. Recent reports indicate that PERT intake can be reduced with a secondary improvement in growth and nutrition status as a consequence of increased dietetic input. These Australian guidelines for the judicious use of PERT in CF should lead not only to a refinement in nutritional management of patients with CF but should also facilitate an improvement in compliance with therapy due to sophistication in patient education materials. The Australian guidelines for the use of PERT in CF if correctly applied, will also provide patients and their families with a better understanding of the relationship between PERT and nutritional status.
Journal of Paediatrics and Child Health Vol. 35, Issue 2, p. 125-129