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Cystic fibrosis (CF) is a common, severe autosomal recessive disorder. The incidence of CF varies among different populations. It is most common among Caucasians of northern European ancestry. The incidence [...], Because of the multiple systems involved in cystic fibrosis, the variability and chronicity of the disease, and the increased survival of this population, a specialty team of experts for care has evolved. A multidisciplinary approach is essential to assist patients and their families in adjusting to the disease and to optimize treatment interventions. The dietitian is responsible for assessment of nutritional status, including the determination of energy requirements and eating habits, interpretation of anthropometric data, and evaluation of nutritional adequacy. The nutrition care plan forms an integral part of the overall treatment objectives and is reported to other team members as it is devised, implemented, and monitored. A consensus report issued in April 1990 by the Cystic Fibrosis Foundation includes both general nutrition guidelines and detailed recommended treatment standards aimed at providing optimal nutrition care.

Children with cystic fibrosis may consume more calories but still be smaller than healthy children. Researchers compared eating habits and parents' perceptions of eating habits of 28 school-age children with cystic fibrosis to those of 28 healthy children. A majority (54%) of the children with cystic fibrosis consumed the recommended diet of 120% of the recommended daily allowance (RDA). These children were significantly shorter and lighter than healthy children. Children with cystic fibrosis spent an average of seven minutes more eating dinner than did healthy children, and their parents reported more intense eating behavior problems., Study Objective. To investigate calorie intake, behavioral eating styles, and parent perception of eating behavior of school-age children with cystic fibrosis (CF) compared with healthy peers. Design. A two-group comparison study. Setting. A clinical sample of 28 school-age children with CF and a community sample of 28 healthy peers matched for age (6 to 12 years) and socioeconomic status. Measurements and Main Results. The children with CF consumed more calories per day (2175 cal/d) than the control children (1875 cal/d) and achieved a significantly higher recommended daily allowance (RDA) of energy (128% of the RDA) than the control children (91.61% of the RDA). Fifty-four percent of the CF sample were achieving the CF dietary recommendations of 120% of the RDA. Despite this energy intake, the CF sample was significantly below the control sample on weight (24.56 vs 31.23 kg), height (125.48 vs 133.06 cm), and z score for weight (-0.811 vs 0.528) and height (-0.797 vs 0.371). On measures of behavioral eating style, the CF sample had significantly longer meals (23.90 min) than the control sample (17.34 min) and had a significantly slower pace of eating (43.27% 10-second intervals with bites) than the control sample (51.29% 10-second intervals with bites) but did not differ significantly on the number of calories consumed during dinner. On a measure of parent report of mealtime behaviors, parents of the children with CF rated mealtime behavior problems of "dawdles" and "refuses food" as more intense (mean, 3.46) than did the parents of control children (mean, 2.67). For the CF sample, a significant correlation was found between the parent intensity ratings of problem behavior in general and meal duration (r = .48), and a significant negative correlation was found between the parent intensity ratings of problem mealtime behaviors and the percentage of intervals with bites (pace of meal) (r = -.533). Conclusions. Although the school-age children with CF were consuming more calories per day than their healthy peers, and more than 50% of the children in the CF sample were at or above the CF dietary recommendations, the children in the CF sample were significantly below the control children on measures of weight and height. The behavioral data suggest that increased caloric intake is not without cost, because the CF sample spent an additional 7 minutes per day at dinner and ate their meals at a slower pace than their healthy peers. These data were associated with higher intensity ratings of mealtime behaviors by parents of children with CF. These findings point to the need for individualized assessment of energy needs for school-age children with CF and comprehensive programs that teach parents behavioral strategies to motivate their children to meet these higher energy requirements in an adaptive manner. Pediatrics 1997;99:665-671; cystic fibrosis, oral intake, behavior, eating., ABBREVIATIONS. CF, fibrosis; RDA, recommended daily allowance; ECBI, Eyberg Child Behavior Inventory. Despite advances in medical treatment, approximately 34.4% of patients with cystic fibrosis (CF) continue to be below the [...]

ABBREVIATIONS. CF, cystic fibrosis; RDA, recommended daily allowance; SES, socioeconomic status; ECBI, Eyberg Child Behavior Inventory. Poor growth and inadequate nutrition continue to be a significant problem for cystic fibrosis [...]