Cystic Fibrosis (CF) is the most common life-threatening recessive inherited disease in the Caucasian population. In the past 15 years, the mean survival of CF patients has increased from 20 to 37 years. A major factor contributing to this increased mean survival is greater emphasis on optimal nutrition. It has been shown that CF children with better nutritional status have improved linear growth and maintain better pulmonary function and exercise tolerance. According to the CF Foundation nutrition consensus guidelines, children and adolescents with CF are expected to experience typical growth when appropriate nutrition and pancreatic enzyme replacement therapy are given.

Importance of optimal nutritional support in CF

Several factors in CF contribute to the loss of muscle mass (see figure).

Picture1Nutritional strategies for patients with CF have focused primarily on energy and fat intakes. It is clear that oral supplementation of large amounts of calories is only partially successful in CF. Little study has been done on the effects of dietary protein and amino acid (building blocks of protein) in CF patients. We recently demonstrated at CTRAL that patients with CF respond highly anabolic to nutritional supplements with essential amino acid mixtures even when they are suffering from muscle wasting. This suggests that muscle mass and function can be better maintained with an increased intake of essential amino acids, thereby reducing mortality and quality of life of CF patients. More research is needed to prove this. Importance of adequate pancreatic enzyme intake in relation to nutrition in CF Exocrine pancreatic insufficiency (EPI) is the most important factor in CF leading to maldigestion of nutrients. EPI will impair digestion of fat and protein by the intestines which may lead to deficiencies of essential nutrients. Providing an appropriate dose and good timing of PERT is therefore of crucial importance to increase lipid and protein digestion in CF. We have recently developed at CTRAL a methodology that is able to measure the acute response of pancreatic enzyme intake on fat and protein digestion in CF. This method can be used to investigate whether intake of different nutritional supplements and medications will improve digestion. Furthermore, the optimal enzyme concentration, timing of administration and method of enzyme delivery can be determined in these patients so that we can derive the best possible treatment strategies for these patients.

Overall long-term objectives of CTRAL

The information gained from our research program will add to the body of knowledge regarding optimization of the nutritional status of patients with CF. We expect the results of this program to have a direct impact on the clinical management of these patients. The results will be the first step to induce studies leading to the reformulation of the use of pancreatic enzymes in relation to nutritional composition. This information is crucial in order to ameliorate or even prevent body wasting in these patients and improve their quality of life and survival rates.

If you would like to participate in one of our (upcoming) studies, click here for more information.

CTRAL publications on Cystic Fibrosis:

2012 Manuscript Clin Nutr CF FFM depletion and consequences.PDF

2011 Abstract NACFC CF EAA intake.PDF

2011 Abstract NACFC CF Protein turnover.PDF

2012 Abstract ATS CF Arginine NO.PDF