Oral Supplementation with a High-fat, High-energy Product Improves Nutritional Status and Alters Serum Lipids in Patients with Cystic Fibrosis

Objective: To assess the tolerance and acceptability of a nutrition supplement in patients with cystic fibrosis (CF), to monitor changes in dietary intake, and to evaluate nutritional status.

Design: Subjects were their own controls for this 3-month, prospective, open study. Acceptability and tolerance questionnaires and 3-day food records were completed at baseline and monthly intervals. Compliance and nutritional status were also assessed.

Setting: This study was conducted at the University of Wisconsin Hospital and Clinics Cystic Fibrosis Center, Madison.

Subjects: Patients with CF older than 4 years of age were recruited during clinic or hospital visits if they met specific weight or growth criteria (n = 19).

Intervention: Subjects were asked to consume the supplement at a maximum of 30% their estimated daily energy requirements.

Main Outcome Measures: Responses to acceptability ratings of and tolerance questions about the supplement were obtained along with anthropometric data and biochemical measurements of serum albumin, plasma retinol, alpha-tocopherol, and fatty acid levels.

Statistical Analyses Performed: Data were analyzed using Minitab and Statistical Analysis Software. Paired and unpaired t tests and nonparametric sign tests were used, as well as regression and Pearson correlations. A significance level of .05 was used for all tests.

Results: All subjects tolerated the supplement, although 12 reported mild symptoms of fullness, nausea, and/or bloating, which were resolved when intake was distributed throughout the day. Mean compliance was 69% of recommended intake. Weight gain in children was strongly correlated with compliance (r = .98). Linoleic acid intake increased significantly (P = .0003) as did plasma linoleic acid in the phospholipid fraction (P = .03).

Conclusion: The supplement studied would be a beneficial addition to the supplementation choices available to patients with CF.