Nutritional Interventions for Children with Cystic Fibrosis Prepared by: Scottish CF Paediatric Dietitians Group Lead Author: Elsie Thomson, Royal Aberdeen Childrens Hospital SPCF MCN Dietetic Protocols Co-ordinator/Editor: Alison Coates, Royal Hospital for Sick Children, Edinburgh Date: October 2012 Review date : October 2014
Scottish Paediatric Cystic Fibrosis Managed Clinical Network Protocols and Guidelines Introduction Nutritional Intervention Protocol This protocol has been devised on behalf of the Scottish Paediatric Cystic Fibrosis MCN for use by dietitians and other health professionals working in Cystic Fibrosis (CF) care across Scotland. It is based on current evidence and best practice. It should be used in conjunction with the published CF Trust consensus documents: Standards for the Clinical Care of Children and Adults with CF in the UK, 2 nd Edition, Dec 2011 Nutritional Management of CF, April 2002 Individuals with CF who are well nourished have a better outcome (Steinkamp & Wiedemann 2002) and there is a strong link between lung function and nutritional status. Wasting (poor weight gain) and stunting (poor height gain) are independent predictors of mortality (Sharma et al 2001, Beker et al 2001). For these reasons effective and timely nutritional intervention is essential. Summary of main points All children with CF in Scotland should aim to achieve optimal nutritional status and growth. Practical advice and support from a dietitian on nutritional interventions should be an integral part of ongoing care. Nutritional interventions should be tailored to the individual and family circumstances. A number of nutritional characteristics of CF are associated with poor nutrition: Increased stool energy losses due to fat malabsorption Increased energy demands of CF Anorexia and poor dietary intake Faddy eating and behavioural eating problems Frequent chest infections, CF related diabetes (CFRD), CF related liver disease (CFLD) and gastro oesophageal reflux (G) The majority of children with CF should achieve a normal nutritional status and rate of growth by following a high energy diet and taking adequate pancreatic supplements if pancreatic insufficient (PI). However for some children, nutrition will be a problem and additional nutritional support will be required using oral supplements and / or enteral feeds (Steinkamp & Weidermann 2002, Efrati et al 2006).
Aims of nutritional intervention To achieve and maintain good nutritional status and adequate growth from diagnosis To meet energy requirements in patients with CF (estimated 100-150% EAR but can be very variable between individuals) To initiate and monitor nutritional support if required All children with CF should have regular dietetic assessment (refer to protocol for dietetic assessment of children with CF), education and advice from a specialist paediatric dietitian with support from a specialist CF dietitian via the Scottish Paediatric CF MCN. Nutritional support should be an integral part of overall care. Frequent chest infections, and possible diagnosis of CFRD, CFRLD and G must also be considered as contributing to poor nutritional status. Oral nutritional supplements and enteral nutritional support should only be implemented after optimising routine nutritional management as follows:. Provision of age appropriate practical and achievable advice regarding a high-energy diet (Table1) Assessment and optimisation of PERT therapy (see PERT protocol) Management of food dislikes and behavioural eating problems (with advice from or referral to a Clinical Psychologist if necessary) Refer to Table 2 for anthropometric criteria indicating nutritional interventions which may be required. Oral Supplements Nutritional supplements have been found most useful for short-term use around the time of infections when appetite may be poor (Cochrane review 2007). A range of oral nutritional supplements can be used depending on age, requirements and taste preferences. Energy dense supplements are most useful. Oral supplements should not replace meals and timing and quantity need careful monitoring. They should be used as an adjunct to a high energy diet. Advice re PERT must be given when recommending supplements. All supplements require PERT except glucose polymers (for example,. Maxijul, Vitajoule ). Children on oral nutritional supplements should be closely monitored to optimise tolerance, adherence and progress.
Enteral Tube Feeds Before initiation, consideration should be given to the family s lifestyle and the additional treatment demand of enteral feeding. Most children with CF tolerate whole protein enteral feeds but occasionally Peptide or Elemental feeds may be required due to intolerance, slow gut motility or poor adherence to PERT. Most children will tolerate 1.5 2 kcal per ml feeds. Nasogastric and gastrostomy feeding are both suitable but for long term use consider gastrostomy route. Children and families should be given information regarding different methods of feeding and be able to choose feeding route and tube type. Most children with CF feed overnight and continue on a high energy diet during the day but in severe cases daytime bolus feeds may be required. Advice re PERT must be given when initiating enteral feeding and regularly monitored (see PERT protocol for more details). Blood glucose levels should be monitored when initiating enteral feeding as CFRD may develop. A glucose profile is useful. Children and families should have training in administering feeds and looking after tubes. Contact numbers to access support should be provided. Children on enteral feeds should be closely monitored to optimise tolerance, adherence and progress. Parenteral nutrition Exceptionally, some children with surgical gut issues may require parenteral nutrition. Close liaison between the pharmacist and the dietitian is required, particularly in the stages of weaning from PN onto enteral feeds or oral diet. Table 1 : Suggestions for Improving Energy and Protein Intakes Promote regular high-energy meals. Encourage frequent use of energy-dense snacks (eg. crisps, biscuits, nuts, cakes) between meals but not at the expense of intake at meals. Encourage high fat foods e.g. cheese, fried foods, pastry, spreads, chips. Use full fat dairy products e.g. milk, cheese, yoghurts, cream. Promote generous use of spreads and oils (mono and polyunsaturated). Encourage high energy drinks e.g. full fat milk, home-made milkshakes. Encourage desserts, e.g. crumbles/fruit pies & custard, milk puddings, icecream.
Table 2: Anthropometric criteria linked with nutritional interventions (Adapted from the Australasian Clinical Practice Guidelines For Nutrition in CF) CATEGY INFANTS <2 CHILDREN 2-18 INTERVENTIONS Acceptable/normal nutritional status Reduced nutritional status / At risk of nutritional failure Nutritional failure Weight and length tracking along percentiles and within 1 centile bands of each other Weight and height percentiles decreasing with time** No weight gain over 6 months <90%Wt /Ht Weight 2 or more centile bands below length <85% Wt /Ht Failure of above nutritional interventions to improve nutritional status BMI percentile 25 th 91 st AND Weight and height percentiles tracking along previous percentiles AND No weight loss BMI percentile 9 th -25 th Weight loss over 3 months Plateau in weight gain over 6 months BMI percentile < 9th Weight falling 2 or more percentile positions WITH Failure of non invasive nutritional interventions to improve nutritional status Routine nutritional care, assessment, education and preventative counselling Nutritional, medical and psychological evaluation to investigate contributing factors eg behavioural eating problems, CFRD Some patients may be at risk of nutritional failure. Consider goal setting, oral supplements and discussion re enteral tube feeding if this fails Further nutritional, medical and psychological evaluation to investigate contributing factors eg behavioural eating problems, CFRD Recommendation for enteral feeding Overweight N/A BMI>98 th Nutritional counselling: Dietary and activity advice Weight maintenance may be appropriate Consider body image issues ** Falling height centiles may indicate stunting despite acceptable height for weight. Compare with mid parental height range
References and Further Reading CF Trust consensus document: Standards for the Clinical Care of Children and Adults with CF in the UK, 2nd Edition, Dec 2011 http://www.cftrust.org.uk/aboutcf/publications/consensusdoc/cf_trust_standards_of _Care_2011_web.pdf CF Trust consensus document: Nutritional Management of CF, April 2002 http://www.cftrust.org.uk/aboutcf/publications/consensusdoc/c_3500nutritional_man agement.pdf Clinical Paediatric Dietetics, 3rd Edition. Shaw & Lawson (2007) Australasian Clinical Practice Guidelines for Nutrition In Cystic Fibrosis (2006) http://www.cysticfibrosis.org.au/pdf/cf_nutrition_guidelines.pdf SteinkampG, Weidemann B. Relationship between nutritional status and lung function in CF Thorax 2002; 57; 596-601 Sharma R et al. Wasting as an independent predictor of mortality in patients with CF Thorax 2001; 56; 746-750 Beker et al. Stature as a prognostic factor in CF survival. J Am Diet Assoc 2001; 101; 438-442 Efrati et al. Long term rehabilitation by gastrostomy in Israeli patients with CF. J Pediat Gastroenterology Nutr 2006; 42; 222-228 Smythe R & Walters S. Oral Nutritional Supplements for CF. Cochrane Review 2007 Resources CF Trust Fact Sheets: Nutrition A guide for children and parents http://www.cftrust.org.uk/aboutcf/publications/factsheets/fs_eating_well_children_a nd_parents_%28web%29_sept_2010.pdf Nutrition : A guide for feeding infants http://www.cftrust.org.uk/aboutcf/publications/factsheets/fs_eating_well_- _Infants_%28WEB%29_Sept_2010.pdf