Consensus Report on Nutrition for Pediatric Patients With Cystic Fibrosis

Abstract

2001 CONSENSUS COMMITTEE MEMBERS Laura K. Bachrach, M.D. Robert J. Beall, Ph.D. Preston W. Campbell, III, M.D. Susan C. Casey, B.S., R.D. Mitchell B. Cohen, M.D. Mary Corey, Ph.D. W. Hobart Davies, Ph.D. Judy A. Fulton, R.D. Richard J. Grand, M.D. John E. Grunow, M.D. Dana S. Hardin, M.D. Lesles Hendeles, Pharm.D. James E. Heubi, M.D. Van S. Hubbard, M.D. Hui-Chuan Kai, Ph.D. Sheila Innis, Ph.D. Elisabeth Luder, Ph.D., R.D. Karen MacGuiness, R.D. Richard K. Mathis, M.D. Annie McKenna, M.S., R.D., C.N.S. Antoinette Moran, M.D. Laurie Moyer-Mileur, Ph.D., R.D. Kimberly O. O'Brien, Ph.D. Hebe Quinton, M.S. Lynne M. Quittell, M.D. Ross W. Shepherd, M.D., FRACP Ronald J. Sokol, M.D. Lori J. Stark, Ph.D. John N. Udall, Jr., M.D., Ph.D. Babette Zemel, Ph.D. INTRODUCTION Patients with cystic fibrosis should have normal growth. Growth requires appropriate energy and nutrient intake, but gastrointestinal and pulmonary function and genetic potential are also important. Poor clinical outcomes are associated with undernutrition in patients with CF (1–6). The Cystic Fibrosis Foundation recognizes that attention to nutrition is an integral part of CF care. In March 2001 a Consensus Committee, co-sponsored by the Cystic Fibrosis Foundation and the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, met to review the literature and update a Consensus Conference Statement published in 1992 addressing these issues (7). The CF care team should monitor growth, provide anticipatory counseling, and plan intervention strategies when patients are at risk for undernutrition or are diagnosed with nutritional failure. A registered dietitian must be a part of the team to provide the discipline-specific expertise needed for optimal nutritional management and may, along with other team members such as the physician, nurse, social worker or a psychologist, provide expertise concerning developmental and behavioral aspects of eating. Prevention and early intervention are most successful in combating nutritional failure. This document outlines the current recommendations of the Consensus Committee on how best to monitor growth and nutrition, strategies to prevent undernutrition, and interventions for patients with nutritional failure. It is based on the limited evidence available in the literature and also represents expert opinion. As with all such documents, these recommendations are likely to change as new data becomes available. MONITORING GROWTH, BODY COMPOSITION, PUBERTAL AND BONE STATUS Early detection of suboptimal growth allows for appropriate intervention and timely rehabilitation. Present Cystic Fibrosis Foundation (CFF) guidelines suggest that patients be seen on a routine basis, every 3 months (8). Growth and nutritional status should be monitored at these intervals (Table 1).TABLE 1: Nutritional assessment in routine CF center careThere are three specific times when special attention should be focused on growth and nutritional status within the scope of usual clinical care. These are: (1) the first 12 months after the diagnosis of CF for each patient; (2) birth to 12 months of age for infants diagnosed prenatally or at birth, until a normal pattern of growth (head circumference, weight, and length) is clearly established; and (3) the peripubertal growth period (girls about 9 to 16, and boys about 12 to 18 years of age). By establishing a pattern of normal growth and development after the diagnosis of CF, patients enter mid-childhood and pubertal growth well nourished, and are more likely to continue this pattern of normal growth. Surveillance of Growth and Body Composition Accurate, sequential measurement, plotting and interpretation of head circumference, weight, length, and height are essential to the care of children with CF. Standard anthropometric measurement techniques are well described in the literature (9–11). Mid-arm circumference and triceps skinfold thickness measurements provide clinical information about lean body mass (muscle and organ) development and subcutaneous fat (energy) stores, respectively. Each center should select and maintain anthropometric instruments, establish a detailed measurement protocol, and train those responsible for obtaining anthropometric measurements. Each clinic should also have standard protocols for cleaning the instruments between patient assessments to protect against the spread of infectious agents. Clinical Evaluation of Measures of Growth and Body Composition Evaluation of Growth Measurements Weight and length (measured supine) or height (measured standing), as well as head circumference in infants, should be monitored and analyzed as growth parameters. In 2000, new NCHS/CDC growth charts were distributed for plotting weight, head circumference, length, and height (CDC web site, http://www.cdc.gov/growthcharts to download charts). The percentiles for all growth measures are expanded, compared to the 1977 charts, with two new percentile curves (97th an