Year: 2002
Type: article
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 and 3rd) for more precise evaluation at the extremes of the growth distribution. In addition, charts are provided for body mass index (BMI =(weight in kg)/(height in meters)2) with percentiles for boys and girls, age 2 to 20 years. Reference values are available for tracking head circumference in children beyond 36 months of age (12). The 2000 edition growth charts should be used for all CF clinical care, and have been used in calculation of the CFF Patient Registry data beginning with the 1999 annual report. For optimal care, it is suggested that patients less than 24 months of age have all previously collected growth data (weight, length, head circumference) completely transferred to a 2000 edition growth chart. For patients older than 24 months, it is suggested that previously collected growth data be transferred to the 2000 growth charts for each six-month interval. Based upon clinical judgment of the pattern of growth and related clinical events (i.e., prolonged illness, use of a feeding tube, lung transplantation), more extensive growth data should be transferred to the 2000 edition growth chart. One strong indicator of global nutritional sufficiency is if patients are growing in height to their full genetic potential (13). The genetic potential for height of each patient can be estimated by a variety of methods (14–16). This should be determined for each patient and the target height range (genetic potential related to biologic parental height) should be noted on the growth chart. Catch-up linear growth may take up to four years in children with CF diagnosed in infancy (17). A steady increase in percentile height towards the target height range indicates adequate nutritional status. Evaluation of Weight for Height Proportion Assessment of the weight for height proportion for an individual patient is of clinical importance. The 1992 CFF Consensus Report (7) contained some inconsistencies in how to use the recommended weight-for-height index. In practice, some CF Centers adopted the Moore modification (18) while others used the original McLaren and Read method (19). To standardize the approach to assessing relative proportion of weight for height, the committee now recommends that the term `percent ideal body weight' (abbreviated as `%IBW') and the modification of Moore et al. be used. This recommendation will be followed in subsequent discussions (Fig. 1). Users of software that calculates %IBW must know what method the program employs, since not all programs use the Moore method. Although somewhat cumbersome to calculate, %IBW remains a very sensitive index of body weight allowing for gender, age, and height. Weight-for-height percentile from NCHS/CDC growth charts is less valuable because the age of the child is ignored.FIG. 1.: Calculation of percent ideal body weight (% IBW).BMI is an estimate of adiposity, which should remain relatively constant throughout adulthood. However, BMI is not constant across the pediatric age range; therefore, BMI percentiles available on the 2000 CDC growth charts, rather than BMI, should be used to evaluate the pediatric patient. Guidelines for classifying underweight by BMI have been reported in the general population for adults but have not been validated in children with CF (20,21). Although the cut-off values for BMI percentiles that relate to health status are not yet well established, low BMI has been shown to be associated with children are growing in weight and the Consensus Committee recommends that BMI percentile should be used for clinical as with percentile height and weight charts, an individual BMI percentile genetic as well as health sequential values when the pattern from a For it is suggested that %IBW and BMI percentile be and used for clinical care BMI percentiles are not available for children age 2 percentile should be used. Evaluation of BMI and percentiles the of patients at risk for nutritional (Table However, not all patients in the will have nutritional of by the of growth in a will to be used to requires evaluation and to prevent nutritional failure. Patients the of nutritional as in should be and of nutritional in patients with CF and those at of Body Composition Measurements The two body circumference and triceps skinfold are to and percentiles data from values are from the circumference and triceps and provide more assessments of and fat These are the and fat with age and data also provided by Clinical Evaluation of development is in patients with CF. This is related to growth and nutritional rather than to a pubertal development is an of growth as well as health of the child and In addition, now that the period of pubertal growth is also very to mass and health A method of or or or registered should be at in beginning at age 9 years and boys at age should be a of nutritional (Table BONE health is of for patients with CF, as have in children and adults with CF mass is of the of health and is by early CF with potential risk for to or pubertal of and and and use of for lung is also a risk for and may and linear growth health can be by growth, and by and that are of of mass or of normal of is not as sensitive or as energy an and standard for the or for may be to of low are standard methods for these of from children or with data is not years and older have of the risk for health should have an assessment of mass by for lung associated with (i.e., with a from use of pubertal and nutritional failure. In to the children at risk for health should have annual in to routine annual the of and should be determined by values or suboptimal should be by of or in children with CF is limited to general health growth by adequate general nutrition by and to that the recommendations for age and by as remains but may be used in lung patients and may appropriate for patients with a of to a in health in children may be to to of patients with CF have The of function must be of are to of and other status is a strong of and has a on nutritional therefore, the is not in nutritional management but as a are associated with sufficiency in a an from this in with an associated with function and the diagnosis of CF has been established, is by clinical and such the of or to document (1) with (2) fat (3) after years of and other as more such as and to low low the of and It is to the function of patients as as the diagnosis of CF is may be the of other is and (Table as well as nutritional management should be for In to a age appropriate of the should be patients that are patients should be for the to if genetic two that are associated with This evaluation can of growth, nutrition, and or more of the for assessing function may be needed for more for is has been are with all and and less than for is needed or are to because the of the and are not associated with with may the and to remain of the until the have a of This can be by the of a or a best when each and For prolonged such as at a or the may be more if distributed throughout the and should to to the of fat in the or based on from their CF and should be of the for with all and are not to it is recommended that be and that the be or an The of can be by growth and the best available is a fat with calculation of a of fat or patients must have analyzed for fat the method to A variety of methods are to the of but are not yet available for clinical use This is an such as and are measures of function but not establish the of A more of can be in the of the Consensus Conference on and To it is recommended that should be less than or less than fat to Nutritional of is recommended for most infants as the of nutrition for the first of can also be used. to from or is more in patients with CF than in the general a than the standard 20 may be needed and can be by by or by the of fat or feeding should continue for the first 12 months of can be used in the should be at to months developmental age to the recommendations of the American of should be with or not or of and should be within the first in to the recommended for children with CF (Table and to be if the is may in the or with CF. with the is For infants are but not their of growth, can be to with the of Ross such as or As infants are to it is that the that children with CF should a that is to in fat and and should be that this is to the usual nutritional for children CF. to this age, and of from to to may with growth at this The should for the child with CF and fat for other members two years of should their children with CF low fat or low the of children establish and is a social as well as a nutritional for patients with CF should about feeding to and to prevent should be This is a period for of growth in children with CF in to limited for and and of may be responsible for this in growth interventions should be in this age if are children must have a of and aspects of This is associated with nutrient to growth, pubertal and of CF development and the general are more in this also nutritional are at risk for nutritional This is an age when such as or may nutrition Growth and pubertal may and at a of social and Nutritional will be more if the patient as well as the may be more to to and body as a for nutrition than weight and status. for and is an essential of nutritional care of the patient with CF in the of Patients with CF a fat to of than that recommended for the general population should be based on the pattern of weight and of fat is method to estimate the of an individual with CF. The clinical is a steady of weight in growing and can to the of that are with Patients with CF are with continue to also are for of fat and patients with CF have of as well as Patients with CF have or of the are at risk for is evidence that patients with CF of for and of these are in and of nutritional is a of A and may also function as an It is a in patients with CF or A of have low of in patients with CF with can be One a of on for patients that it may a evidence for a clinical is A A is for and is to In between and of CF patients have been to be A In of CF patients were to have in The of these that A in CF is A is a may low should not be at the of to the for to increase on because of the of and patients with CF to percent of patients with CF have been to be in children and adults and those in are more likely to have because of limited to is an to as well as and has been reported to be low in patients with CF, in those and and have been reported to percent of CF patients continue to have low in the of and with as well as in measurement of is not has been used as a Although it is not by or is a more sensitive of of to the of will of a while of suggested that while the more sensitive it to be very if and are are a of K. of the by use can In a of adults with CF were at of four times a not to This that recommendations for may be and essential is in patients with CF, and can be seen in patients as well as in those are However, clinical and are should be in infants with to The in patients with are that can be to and is to and is to of to may be a in the seen in in patients with CF. has been that is a in CF (i.e., is not to fat is in patients with CF is the of recommendations can be at the such as and and are in are a of energy and can be and should be for and recommendations for the general population have been has also been an of the of and an risk in children and adults with CF. have that and low mass are issues of in the pediatric CF population To of in children and at a by the is an of for children with CF is used as an index of status. However, is an and may be in patients with CF to are a more sensitive indicator of because are not by but at this is not available are it is recommended that status be monitored in children and with CF by and have reported and in children and infants with CF in CF is to because may be when is in the normal as a for a period of months can be for CF patients are to or have is to A is also in CF patients with suboptimal A status or in those that not to A and children with CF are at risk of because of the The evidence in infants is limited to while in older patients with CF has been in more Patients with CF are to take in a This recommendation should be the months and for those in CF of infants with CF likely are at the of this range when not to the of or when to with CF should have have been This method has the of and has the potential for are available and can be more and It is estimated that between and of CF patients have used to of these patients from these of or is in the range of to are for two of or is by and is of or Patients with CF and their may be to with The American of Committee on with published guidelines on that best the of it is to maintain a provide about against and to establish and maintain a with It is to patients with CF and their about in a AND of Nutritional and Patients at for patients to have nutritional and to be at risk for nutritional are in growth is patients should be seen more than the every in the Guidelines for routine should be seen every two to four and children age two years should be seen every four to These should and nutritional and intervention as in intervention should at the target for weight-for-height proportion and genetic height for CF patients with weight or of weight of as well as nutritional and behavioral should be in the patient to pulmonary or are these should be with in patients with CF of all and can pulmonary and growth extensive review of the literature and recommendations for diagnosis and of in infants and children has been published CF related with or can growth. should be in all patients with growth. The is to the Consensus Conference for management of of may also to growth. These infectious of the or such as or with which may for can fat and in essential status and growth should be in patients have at than to a may be for patients with growth. and of growth and nutritional status in the patient with is In addition, of by an may an appropriate Evaluation of feeding and strategies should be early in the evaluation of patients with nutritional failure. The Assessment is a of It is to use and to have adequate to be used to that can be to on in with CF has focused on assessment of or of body and of these has been However, should be in the with CF with very low body Evaluation can be to if patients are at are by or or if the are and are not should be about the of and as well as the fat and of should be about or which may be used the when are may be if the child is to or has an which with A a assessment of and is in the A 3 to must be collected for assessment of energy and nutrient such as or should be in children with for Patients Nutritional as described behavioral assessment are behavioral intervention may be used in with nutritional intervention to interventions have been to be in feeding for children 3 to 12 years and their in a and can be used in the clinical The first behavioral is to increase by on at a A of to their child or what or will A is to appropriate for the of at each for more behavioral should be nutritional interventions with of to the and use of nutritional energy weight by and of nutritional and available to nutritional status of energy is but is to that are not used as for normal The of may be for patients with to and should be when to in weight The and of should be to the patient and and their and to this intervention should be should be as a not as a or beginning of the should be as a to of and A of that and patients this is to the of at diagnosis or early in the of CF, nutritional is should be as of to nutritional status and of The should be provided with information on the of feeding and and how feeding data to the of of the of and for or should be based on the of the that the of should be of and if is an should be at the of is needed to the and of this health should be of all and a plan for management of should be the and Standard are well are for of adequate The data are as to with are in patients have or is to normal the to of estimated energy should be provided a of to may be needed in infants to growth and optimal lung growth. The of should be based on the of weight fat stores, and growth. may be used for patient have in and should be by to of are data on the appropriate of with One that with and after and were well a towards less of fat and in the One that fat in infants of three and of as fat to fat when the usual of and The Consensus Committee recommends that be in the usual all the of very as may to be or at the of a is needed to the optimal method to provide with may be associated with Patients on should be monitored for the full has been should be two to three the feeding and at the of the feeding on two should be if these are than This of should be when a patient is or if the patient is not Patients with feeding should have their for Patients with may from the of or use of a as well as is in patients with CF than but those with CF to the of weight in patients with and It is weight and mass in patients have but of these patients with should be on a One of children with growth height and weight lean body and pulmonary function and However, since height and weight patients underweight at the of of that this intervention is not a for nutritional Growth can also and are needed to if the are may be a for growth in children with CF. have and weight in patients with such as development of and have been not to be after the is more data available that the the be recommended for routine use in CF. is to have every child with CF normal growth and This requires and adequate and and a plan for intervention when growth is This is best with a team approach at an CF This document is to best recommendations based on current However, cystic fibrosis and nutrition is needed in guidelines can be based on evidence and not opinion.
Language: English
Subfield: Pulmonary and Respiratory Medicine
Field: Medicine
Domain: Health Sciences
Open Access status: bronze
OpenAlex ID: W1983928112