Effect of Enteral Nutrition on the Course of Cystic Fibrosis

Abstract 2079 Pulmonary: Cystic Fibrosis Poster Symposium, Tuesday, 5/4

The use of enteral nutrition(EN)in cystic fibrosis(CF)patients is increasing but definitive efficacy studies have not been completed. The aim of this study was to evaluate whether(EN),i.e., tube-feeding, could slow the rate of decline of weight and pulmonary function and improve survival by preventing the undernutrition which often occurs in CF patients. Longitudinal data on a cohort of 664 patients (314 male/350 female), ages 6-18 years, in whom FEV₁, height and weight measurements were available was selected from the 1989-95 Cystic Fibrosis Foundation Patient Registry. We excluded patients who were on TPN, on EN before this period, pregnant, had heart, lung or liver transplants, and those with B.cepacia. Based on data from 3 years prior to EN and the two years following EN, we found that the rates of decline in body mass index and weight Z-scores were reduced significantly (p=0.0001) with the use of EN. The rate of decline of FEV₁ was lower in the 2 years after starting EN compared to the 3 years prior to EN, especially for females with ΔF508 homozygous genotype (p=0.04). Over the two-year period on EN, the mortality rates in this cohort were 60% for FEV₁<20% predicted, 39.7% for FEV₁ 20-30% predicted, and 26.8% for FEV₁30-40% predicted. Mortality rates were higher for females than males (20.3% vs 14%, p=0.03). These mortality rates are lower than previously reported (Kerem et al. N Engl J Med 1992; 326: 1187-1191). Conclusion: EN appears to have a beneficial effect on weight and pulmonary function. The effects are most pronounced in females and those with severe lung disease. The data would support the use of EN in severely affected patients, including those awaiting lung transplantation.

Supported in part by Grant #MCJ 369165 from MCHB, DHHS.