Clinical Paediatric Dietetics EDITED BY
Vanessa Shaw and
Margaret Lawson
Third edition
Clinical Paediatric Dietetics
Clinical Paediatric Dietetics EDITED BY
Vanessa Shaw and
Margaret Lawson
Third edition
© 1994, 2001, 2007 by Blackwell Publishing Blackwell Publishing editorial offices: Blackwell Publishing Ltd, 9600 Garsington Road, Oxford OX4 2DQ, UK Tel: +44 (0)1865 776868 Blackwell Publishing Professional, 2121 State Avenue, Ames, Iowa 50014-8300, USA Tel: +1 515 292 0140 Blackwell Publishing Asia Pty Ltd, 550 Swanston Street, Carlton, Victoria 3053, Australia Tel: +61 (0)3 8359 1011 The right of the Authors to be identified as the Authors of this Work has been asserted in accordance with the Copyright, Designs and Patents Act 1988. All rights reserved. No part of this publication may be reproduced, stored in a retrieval system, or transmitted, in any form or by any means, electronic, mechanical, photocopying, recording or otherwise, except as permitted by the UK Copyright, Designs and Patents Act 1988, without the prior permission of the publisher. First edition published 1994 Second edition published 2001 Third edition published 2007 1
2007
ISBN: 978-14051-3493-4 Library of Congress Cataloging-in-Publication Data Clinical paediatric dietetics / edited by Vanessa Shaw and Margaret Lawson. — 3rd ed. p. cm. Includes bibliographical references and index. ISBN-13: 978-1-4051-3493-4 (hardback : alk. paper) ISBN-10: 1-4051-3493-3 (hardback : alk. paper) 1. Diet therapy for children. I. Shaw, Vanessa. II. Lawson, Margaret, MSc. RJ53.D53C58 2007 615.8′54083—dc22 2006034442 A catalogue record for this title is available from the British Library Set in 9.5/11.5pt Palatino by Graphicraft Limited, Hong Kong Printed and bound in Singapore by COS Printers Pte, Ltd The publisher’s policy is to use permanent paper from mills that operate a sustainable forestry policy, and which has been manufactured from pulp processed using acid-free and elementary chlorine-free practices. Furthermore, the publisher ensures that the text paper and cover board used have met acceptable environmental accreditation standards. For further information on Blackwell Publishing, visit our website: www.blackwellpublishing.com
Contents
Contributors, vii Foreword, x Preface, xi Acknowledgements, xii
Part 1 Principles of Paediatric Dietetics 1 Nutritional Assessment, Dietary Requirements, Feed Supplementation, 3 Vanessa Shaw & Margaret Lawson 2 Provision of Nutrition in a Hospital Setting, 21 Ruth Watling
Part 2 Nutrition Support and Intensive Care
Part 3 Clinical Dietetics 6 Preterm Infants, 73 Caroline King 7 Gastroenterology, 90 Sarah Macdonald 8 Surgery in the Gastrointestinal Tract, 125 Vanessa Shaw Short Bowel Syndrome, 134 Tracey Johnson 9 The Liver and Pancreas, 142 Stephanie France 10 Diabetes Mellitus, 163 Alison Johnston
3 Enteral Nutrition, 33 Tracey Johnson
11 Cystic Fibrosis, 178 Carolyn Patchell
4 Parenteral Nutrition, 46 Joanne Grogan
12 The Kidney, 203 Julie Royle
5 Nutrition in Critically Ill Children, 60 Rosan Meyer & Katie Elwig
13 The Cardiothoracic System, 239 David Hopkins
vi
Contents
14 Food Hypersensitivity, 259 Kate Grimshaw 15 Immunodeficiency Syndromes, HIV and AIDS, 278 Immunodeficiency Syndromes, 278 Marian Sewell & Vivien Wigg HIV and AIDS, 282 Julie Lanigan 16 Ketogenic Diets, 295 Liz Neal & Gwynneth McGrath 17 Disorders of Amino Acid Metabolism, Organic Acidaemias and Urea Cycle Defects, 309 Phenylketonuria, 309 Anita MacDonald Maple Syrup Urine Disease, 332 Marjorie Dixon
21 Peroxisomal Disorders, 442 Refsum’s Disease, 442 Eleanor Baldwin X-linked Adrenoleukodystrophy, 450 Anita MacDonald 22 Childhood Cancers, 461 Evelyn Ward 23 Eating Disorders, 473 Dasha Nicholls 24 Epidermolysis Bullosa, 482 Lesley Haynes 25 Burns, 497 Helen McCarthy & Claire Gurry 26 Autistic Spectrum Disorders, 504 Zoe Connor
Tyrosinaemias, 343 Marjorie Dixon
Part 4 Community Nutrition
Homocystinuria, 349 Fiona White
27 Healthy Eating, 523 Judy More
Organic Acidaemias and Urea Cycle Disorders, 357 Marjorie Dixon
28 Children from Ethnic Groups and those following Cultural Diets, 540 Sue Wolfe
Emergency Regimens, 375 Marjorie Dixon 18 Disorders of Carbohydrate Metabolism, 390
29 Faltering Growth, 556 Zofia Smith
Glycogen Storage Diseases, 390 Marjorie Dixon
30 Feeding Children with Neurodisabilities, 566 Sarah Almond, Liz Allott & Kate Hall
Disorders of Galactose Metabolism, 400 Anita MacDonald
31 Obesity, 588 Laura Stewart
Disorders of Fructose Metabolism, 410 Marjorie Dixon 19 Disorders of Fatty Acid Oxidation and Ketogenesis, 421 Marjorie Dixon 20 Lipid Disorders, 434 Patricia Rutherford
32 Prevention of Food Allergy, 597 Kate Grimshaw & Carina Venter
Appendix I Manufacturers of Dietetic Products, 605 Appendix II Dietetic Products, 607 Index, 611
Contributors
Liz Allott Senior Lecturer in Dietetic Practice, Faculty of Health and Social Work, University of Plymouth, Peninsula Allied Health Centre, College of St Mark and St John, Derriford Road, Plymouth PL6 9BH Sarah Almond Senior Paediatric Dietitian, Chailey Heritage Clinical Services, Beggars Wood Road, North Chailey, East Sussex BN8 4JN Eleanor Baldwin Refsum’s Research Dietitian, Nutrition & Dietetic Department, Chelsea & Westminster Hospital, 369 Fulham Road, London SW10 9NH Zoe Connor Community Paediatric Dietitian, Bromley NHS PCT, St Paul’s Cray Clinic, Mickleham Road, Orpington, Kent BR5 2RJ
Stephanie France Former Chief Paediatric Dietitian, Kings College Hospital, Denmark Hill, London SE5 9RS Kate Grimshaw Research Dietitian, University Child Health, Southampton General Hospital, Tremona Road, Southampton SO16 6YD Joanne Grogan Senior Dietitian, Royal Liverpool Children’s NHS Trust, Eaton Road, Liverpool L2 2AP Claire Gurry Senior Paediatric Dietitian, Central Manchester and Manchester Children’s University Hospitals NHS Trust, Charlestown Road, Blackley, Manchester M9 7AA Kate Hall Freelance Dietitian,
[email protected] Lesley Haynes Specialist Dietitian, Great Ormond Street Hospital for Children NHS Trust, London WC1N 3JH
Katie Elwig Chief Paediatric Dietitian, St Mary’s Hospital, Praed Street, London W2 1NY
David Hopkins Specialist Dietitian, Bristol Royal Hospital for Children, Paul O’Gorman Building, Bristol BS2 8BJ
Marjorie Dixon Specialist Dietitian, Great Ormond Street Hospital for Children NHS Trust, London WC1N 3JH
Tracey Johnson Specialist Dietitian, The Birmingham Children’s Hospital NHS Trust, Steelhouse Lane, Birmingham B4 6NH
viii
Contributors
Alison Johnston Lead Clinical Specialist Dietitian (Diabetes), Royal Hospital for Sick Children, Yorkhill, Glasgow G3 8SJ Caroline King Clinical Lead and Specialist Neonatal Dietitian, Hammersmith Hospital, Du Cane Road, London W12 0HS Julie Lanigan Clinical Trials Co-ordinator/Specialist Research Dietitian, Institute of Child Health, 30 Guilford Street, London WC1N 1EH Margaret Lawson Former Nestlé Senior Research Fellow, Institute of Child Health, 30 Guilford Street, London WC1N 1EH Helen McCarthy Lecturer in Dietetics, School of Biomedical Sciences, University of Ulster, Cromore Road, Coleraine BT52 1SA Anita MacDonald Consultant Dietitian in Inherited Metabolic Disorders, The Birmingham Children’s Hospital NHS Trust, Steelhouse Lane, Birmingham B4 6NH Sarah Macdonald Specialist Dietitian, Great Ormond Street Hospital for Children NHS Trust, London WC1N 3JH Gwynneth McGrath Senior Dietitian, The Birmingham Children’s Hospital NHS Trust, Steelhouse Lane, Birmingham B4 6NH Rosan Meyer Paediatric Research Dietitian, Room 252 Norfolk Place, Imperial College St Mary’s Campus, London W2 1BG Judy More Freelance Paediatric Dietitian, London, www.child-nutrition.co.uk Liz Neal Research Dietitan, Institute of Child Health, 30 Guilford Street, London WC1N 1EH Dasha Nicholls Consultant Child and Adolescent Psychiatrist,
Head of Feeding and Eating Disorders Service, Great Ormond Street Hospital for Children NHS Trust, London WC1N 3JH. Carolyn Patchell Head of Dietetic Services, The Birmingham Children’s Hospital NHS Trust, Steelhouse Lane, Birmingham B4 6NH Julie Royle Chief Dietitian, Central Manchester and Manchester Children’s University Hospitals NHS Trust, Pendlebury, Manchester M27 4HA Patricia Rutherford Former Chief Dietitian, Royal Liverpool Children’s NHS Trust, Eaton Road, Liverpool, L12 2AP. Now Clinical Information Manager, Vitaflo International Ltd, 11–16 Century Building, Liverpool L3 4BL Marian Sewell Senior Dietitian, Great Ormond Street Hospital for Children NHS Trust, London WC1N 3JH Vanessa Shaw Head of Dietetics, Great Ormond Street Hospital for Children NHS Trust, London WC1N 3JH Zofia Smith Community Paediatric Dietitian, Parkside Community Health Centre, 311 Dewsbury Road, Beeston, Leeds LS11 5LQ Laura Stewart Community Paediatric Dietitian, Royal Hospital for Sick Children, Sciennes Road, Edinburgh EH9 1LF Carina Venter Senior Allergy Dietitian (Research and Clinical), The David Hide Asthma and Allergy Research Centre, St Mary’s Hospital, Newport, Isle of Wight Evelyn Ward Senior Paediatric Dietitian, St James University Hospital NHS Trust, Beckett Street, Leeds LS9 7TF Ruth Watling Head of Dietetics, Royal Liverpool Children’s NHS Trust, Eaton Road, Liverpool L2 2AP
Contributors
Fiona White Chief Metabolic Dietitian, Central Manchester and Manchester Children’s University Hospitals NHS Trust, Pendlebury, Manchester M27 4HA
ix
Vivien Wigg Senior Dietitian, Great Ormond Street Hospital for Children NHS Trust, London WC1N 3JH Sue Wolfe Chief Paediatric Dietitian, St James University Hospital NHS Trust, Beckett Street, Leeds LS9 7TF
Foreword
It gives me great pleasure to write the foreword for the third edition of Clinical Paediatric Dietetics. This edition clearly shows the diversity of areas paediatric dietitians work within, from clinical dietetics to community nutrition. The new structure recognises the important extent of the role dietitians play in the health care of sick infants and children. Recognition should be given to The Paediatric Group of the British Dietetic Association for their evidence based and practical approach. Vanessa and Margaret should once again be sincerely congratulated for managing contributions from almost
40 dietitians with valued input from a significant number of others – a task that would overwhelm many. The value of this book is recognised not only by dietitians but also by many other health professionals as an excellent source of reliable information to ensure optimal nutritional care is given to sick infants and children. Dame Barbara Clayton DBE Honorary Research Professor in Metabolism, University of Southampton Honorary President, The British Dietetic Association
Preface
The aim of this manual is to provide a very practical approach to the nutritional management of a wide range of paediatric nutritional disorders that may benefit from nutritional support or be ameliorated or resolved by dietary manipulation. The text will be of particular relevance to professional dietitians, dietetic students and their tutors, paediatricians, paediatric nurses and members of the community health team involved with children requiring therapeutic diets. The importance of nutritional support and dietary management in many paediatric conditions is increasingly recognised and is reflected in new text for this edition. The authors are largely drawn from practising paediatric dietitians around the United Kingdom, with additional contributions from academic research dietitians and a psychiatrist. The text has been reorganised for this edition and includes a thorough review of the scientific and medical literature to support practice wherever available. The major part of the text concentrates on nutritional requirements of sick infants and children in the clinical setting. Normal dietary constituents are used alongside special dietetic products to provide a prescription that will control progression and symptoms of disease whilst maintaining the growth potential of the child. There is a new section on community nutrition including healthy eating throughout childhood. We acknowledge that the
distinction between clinical dietetics and nutrition in the community is rather arbitrary since many clinical conditions are dealt with in the community, and the principles of healthy eating underpin many clinical interventions. New topics have been added: nutrition support in critical care, autistic spectrum disorders, prevention of food allergy. There has been an expansion of the range of disorders and treatments described in many chapters, e.g. gastroenterology, liver disease, gut transplantation, cardiac conditions, fatty acid oxidation defects; and new recommendations and guidelines for parenteral nutrition, feeding preterm infants and assessing children with neurodisabilities. Arranged under headings of disorders of organ systems rather than type of diet, and with much information presented in tabular form and with worked examples, the manual is easy to use. Appendices list the many and varied special products described in the text, together with details of their manufacturers. The appendices are not exhaustive, but include the products most commonly used in the UK. The most recent information and data has been used in the preparation of this edition, but no guarantee can be given of the validity or availability at the time of going to press. Vanessa Shaw Margaret Lawson
Acknowledgements
We would like to thank a number of the contributors to the first edition, who were unable to contribute to the second edition, but whose work formed the basis for the following chapters:
We would like to thank a number of the contributors to the second edition, who were unable to contribute to this third edition, but whose work has formed the basis for the following chapters:
Chapter 2, Provision of Nutrition in a Hospital Setting: Christine Clothier Chapter 3, Enteral Feeding: Debra Woodward Chapter 4, Parenteral Nutrition: Alison Macleod Chapter 6, The Gastrointestinal Tract: Sheena Laing Chapter 8, The Liver and Pancreas: Jane Ely Chapter 14, Ketogenic Diet for Epilepsy: Jane Eaton Chapter 18, Lipid Disorders: Anne Maclean Chapter 22, Eating Disorders: Bernadette Wren and Bryan Lask Chapter 25, Burns: Judith Martin
Chapter 4, Parenteral Nutrition: Janice Glynn Chapter 12, The Kidney: Janet Coleman Chapter 13, The Cardiothoracic System: Marion Noble Chapter 14, Food hypersensitivity: Christine Carter Chapter 15, HIV and AIDS: Jayne Butler Chapter 15, Immunodeficiency Syndromes: Dona Hileti-Telfer Chapter 21, Refsum’s Disease: June Brown Chapter 25, Burns: Dearbhla Hunt Chapter 30, Feeding Children with Neurodisabilities: Karen Jeffereys Chapter 31, Obesity: Mary Deane
Part 1 Principles of Paediatric Dietetics
Chapter 1
Nutritional Assessment, Dietary Requirements, Feed Supplementation
Chapter 2
Provision of Nutrition in a Hospital Setting
1
Nutritional Assessment, Dietary Requirements, Feed Supplementation
Vanessa Shaw & Margaret Lawson
Introduction This text provides a practical approach to the dietary management of a range of paediatric disorders. The therapies outlined in Parts 2 and 3 describe dietetic manipulations and the nutritional requirements of the infant and child in a clinical setting, illustrating how normal dietary constituents are used alongside special dietetic products to allow for the continued growth of the child while controlling the progression and symptoms of disease. Nutrition for the healthy child and nutritional care most often provided in the community setting is addressed in Part 4.
Dietary principles The following principles are relevant to the treatment of all infants and children and provide the basis for many of the therapies described later in the text.
Assessment of nutritional status Assessment and monitoring of nutritional status should be included in any dietary regimen, audit procedure or research project where a modified diet has a role. There are a number of methods of assessing specific aspects of nutritional status, but no one measurement will give an overall picture of status for all nutrients. There are several
assessment techniques, some of which should be used routinely in all centres while others are still in a developmental stage or are suitable only for research. Figure 1.1 outlines the techniques that can be used for nutritional assessment.
Dietary intake For children over the age of 2 years food intake is assessed in the same way as for adults: using a recall diet history, a quantitative food diary over a number of days, a weighed food intake over a number of days or a food frequency questionnaire [1]. For most clinical purposes an oral history from the usual carers (or from the child if appropriate) will provide sufficient information on which to base recommendations. As well as assessing the range and quantity of foods eaten it is also useful to assess whether the texture and presentation of food is appropriate for the age and developmental level of the child. For instance, does the child use an infant feeding bottle or a cup; is the child able to self-feed or need a lot of help; is the food of normal consistency, as eaten by the family, or is it a smooth purée, chopped or mashed? The assessment of milk intake for breast fed infants is difficult and only very general estimations can be made. Infants can be test-weighed before and after a breast feed and the amount of milk consumed can be calculated. This requires the use of very accurate scales (±1–2 g) and should be
4 Clinical Paediatric Dietetics
First line or basic assessment
Nutrient intake
Clinical assessment
Anthropometry
Second line tests, as indicated by results from basic assessment
Biochemistry
Haematology
Research tools or unsuitable for paediatrics
Body composition studies • Immunology • Functional tests • Isotope studies Figure 1.1 Nutritional assessment methods.
carried out for all feeds over a 24-hour period as the volume consumed varies throughout the day. Testweighing should be avoided if at all possible as it is disturbing for the infant, engenders anxiety in the mother and is likely to compromise breast feeding. Studies have shown that the volume of breast milk consumed is approximately 770 mL at 5 weeks and 870 mL at 11 weeks [2]. In general, an intake of 850 mL is assumed for infants who are fully breast fed and over the age of 6 weeks, with additional intake from food at the appropriate weaning age. Estimation of food intake is particularly difficult in infants, as it is not possible to assess accurately the amount of food wasted through, for example, spitting or drooling. Conversion of food intake into nutrient values for young children may involve the use of manufacturers’ data if the child is taking proprietary infant foods and/or infant formula. The composition of breast milk varies and food table values may be inaccurate by up to 20% because of individual variation. Assessment of the adequacy of an individual calculated nutrient intake for sick and for healthy infants and children is discussed in the section on Dietary Reference Values (see p. 10).
Anthropometry Measurement of weight and height or length is critical as the basis for calculating dietary requirements as well as monitoring the effects of dietary intervention. Other anthropometric measurements are summarised in Table 1.1.
Head circumference Head circumference is a useful measurement in children under the age of 2 years, particularly where it is difficult to obtain an accurate length measurement. After this age head growth slows and is a less useful indicator of somatic growth. A number of genetic and acquired conditions will affect head growth (e.g. neurodevelopmental delay) and measurement of head circumference will not be a useful indicator of nutritional status in these conditions. Head circumference is measured using a narrow, flexible, non-stretch tape. Details of a suitable disposable paper tape are available from the Child Growth Foundation (see p. 20). Measurement should be made just above the eyes to include the maximum circumference of the head, with the child supported in an upright position and looking straight ahead. Weight Measurement of weight is an easy and routine procedure using an electronic digital scale or a beam balance. Ideally, infants should be weighed nude and children wearing just a dry nappy or pants, but if this is not possible it is important to record whether the infant is weighed wearing a nappy, and the amount and type of clothing worn by older children. For weighing infants up to 10 kg, scales should be accurate to 10 g; for children up to 20 kg, accuracy should be ±20 g and over 20 kg it should be about 50–200 g. A higher degree of accuracy is required for the assessment of sick children than for routine measurements in the community. Frequent weight monitoring is important for the sick infant
Nutritional Assessment, Dietary Requirements, Feed Supplementation
5
Table 1.1 Anthropometric measurements. Measurement
Derived indices
Comments
Head circumference
Head circumference for age
Easy to measure; useful up to the age of 2 years; useful as proxy for length increase; does not normally change rapidly Affected by medical condition; may not indicate nutritional status
Weight
Weight for age Weight for age z score
Easy to measure; useful on a day to day basis Does not differentiate between lean tissue, oedema and body fat
Length/height
Length/height for age Length/height for age z score Height age
Not easy to measure accurately – needs more than one person to measure; does not change rapidly Best overall indicator of nutritional wellbeing
Weight for stature Body mass index (W/H2) Body mass index z score
Useful for children with low height age BMI indicates relative weight for height, but does not differentiate between lean and fat tissue
Mid arm circumference
Mid arm circumference for age Mid arm circumference z score
Easy to measure; useful up to the age of 5 years Less likely to be affected by water retention or fat deposition than body weight
Skinfold thickness (SFT)
Triceps SFT for age
Difficult to measure accurately; unpleasant procedure for children Usually triceps only are used in children Distinguishes between lean and fat tissue
Waist circumference
Waist circumference for age
Easy to measure, although requires removal of clothes Distinguishes between high weight due to muscle bulk and that due to fat
Hip circumference
Waist : hip ratio
Easy to measure No standards for children
or child and hospitalised infants should be weighed daily if there are problems with fluid balance, otherwise on alternate days; children over the age of 2 years in hospital should be weighed at least weekly. Recommendations for the routine measurement of healthy infants where there are no concerns about growth are given in Table 1.2 [3]. If there are concerns about weight gain that is too slow or too rapid, measurement of weight should be carried out more frequently.
Height Height or length measurement requires a stadiometer or length-board. Details of suitable equipment, which may be fixed or portable, are available from the Child Growth Foundation. Measurement of length using a tape measure is too inaccurate to be of use for longitudinal monitoring of growth, although an approximate length may be useful as a single measure (e.g. for calculating body mass index). Under the age of about 2 years supine length is measured; standing height is usually measured
Table 1.2 Recommendations for routine measurements for healthy infants and children. Head Circumference
Weight
Length/Height
Birth 2 months
Birth 6–8 weeks if birthweight
