NHD CPD eArticle Vol 7.16 by NH Publishing Ltd

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Volume 7.16 - 5th October 2017

CROHN’S DISEASE: NUTRITIONAL MANAGEMENT IN CHILDREN Rachel Wood Specialist Paediatric Dietitian in Allergy and Gastroenterology Therapy and Dietetics Rachel is Clinical Lead for Dietetic Allergy and Gastroenterology services in a tertiary centre for Paediatrics based at Central Manchester University Hospitals NHS Foundation Trust Royal Manchester Children's Hospital.

Inflammatory bowel disease (IBD) can be defined as Crohn’s disease (CD) or Ulcerative Colitis (UC). Both conditions are of chronic inflammation and of unknown aetiology, although the causes still trigger much debate, as does whether or not the changing environment plays some part in the development of these diseases, including dietary changes, processed foods and the microbiome. However, in paediatric CD, the genetic component is much more dominant than in adults.1,2 The incidence of CD in children is on the rise worldwide, with an estimated prevalence of 58 in 100,000.3 The role of nutritional therapy as treatment is becoming even more popular. CD can affect any part of the gastrointestinal (GI) tract; from the mouth to the bottom. Orofacial Granulomatosis (OFG) is linked to CD, as well as symptoms of frequent mouth ulcers, but the most common area to be affected by CD is the terminal ileum and right colon. CD is characterised by full thickness acute and chronic inflammation anywhere in the gastrointestinal tract; it can be patchy as well as having granulomas.2 This, as you can imagine, comes with a whole host of symptoms. In paediatric CD, symptoms can often be non-specific which can delay diagnosis. As well as the increasing prevalence, it appears that the classic ‘triad’ symptoms of abdominal pain, weight loss and diarrhoea are also changing. DIAGNOSIS

Other symptoms can include lethargy, anaemia, poor appetite, weight loss and nausea/vomiting associated with

eating; patients can become very low in mood. One of the most important factors in paediatrics is linear growth; not receiving the correct nutrition can impair this, as well as affect development if nutritional intake is not corrected. Diagnosis is often around or during puberty, so ensuring the correct nutrition and treatment is vital to optimise growth and to ensure puberty is not delayed. TREATMENT

Most paediatric centres are now using Exclusive Enteral Nutrition (EEN) as first line treatment in newly diagnosed Crohn’s patients.4 This comes with variation in the name as it is also known as Liquid Diet Therapy (LDT), or the Polymeric Diet (PMD). There are three main consensus guidelines for the management of CD in Paediatrics: NICE clinical guideline for CD: management CG152, (2012), BSPGHAN guidelines produced in 2010 and the ECCO/ESPGHAN produced in 2014 all state that enteral nutrition should be used as first line treatment to induce remission.5,6,1

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Remission induced by Exclusive Enteral Nutrition (EEN) has been demonstrated in 80% of newly diagnosed children with CD and 58% in previously diagnosed children in a flare up . . .

EEN is the complete avoidance of food for a period of six to eight weeks. The patient receives all of their nutritional requirements in the form of a ‘liquid’ diet. The aim of this is to induce remission, aid mucosal healing, reduce inflammation and provide optimum nutrition to promote growth and development. There are many positive outcomes in using EEN as first line treatment for CD, but this does not come without its own challenges for patients and their families. We know as clinicians that EEN is highly effective at inducing remission; 8090% of children on EEN as seen in the systematic review by Day et al2 and in Knight et al.17 EEN also does not come with the complications of affecting bone density and delayed growth that steroids bring. However, many children just cannot adhere to EEN for the six- to eight-week period that is required, despite nasogastric tube (NGT) placement, which after the oral route would always be the next step before steroids. Others try meticulously, but symptoms just don’t resolve, even with the combination of Azathioprine and, therefore, there is no choice than to escalate treatment. Remission induced by EEN has been demonstrated in 80% of newly diagnosed children with CD and 58% in previously diagnosed children in a flare up and we know there are other positive factors including improved energy and mood. However, in

practice this can be one of the hardest things you can ask a child or young person to do, mainly affecting their quality of life and social aspects around eating that they have missed out on for so long because of being unwell.16 A child presented at age 12 years with a variety of symptoms; loose stools up to five to seven times per day with mucous, but no blood, general lethargy with some aches and pains, low in mood and severe weight loss over a period of six months. Weight at assessment was 27.5kg, plotting just under the 2nd centile and height was 148cm plotting on the 50th centile.7 There was a reported 16% loss of body weight over a period of six months. This is significant weight loss and means that instead of tracking along the 25th centile and the 50th centile for height, which would be proportional, he dropped down across three centile lines, indicating a risk of refeeding syndrome. STARTING TREATMENT

EEN will be introduced slowly over a number of days, being mindful not to overfeed and daily monitoring of blood levels including phosphate, magnesium and potassium levels. This requires intense input from the dietitian and may delay how quickly the patient can reach their target volume of nutritional drinks and, hence, how quickly they can go home from the ward.

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Table 1: The composition of feeds available to use with CD Feed name

Emsogen (EF)

Elemental EO28 extra (EF)

Modulen (PF)

Alicalm (PF)

Ensure Plus/ Fortisip (PF)

Protein source

Amino acid

Whole protein

17% LCT/83% MCT

65% LCT/35% MCT

74% LCT/26% MCT

52% LCT/48% MCT

100% LCT

Fat source

EEN commonly is done for six to eight weeks, although it is shown to have had improvements on mood and energy levels within the first seven to 10 days and inflammatory markers within 14 days, all of which continue to improve for over six to eight weeks.4 Although the initial improvements in symptoms and mood are seen within the first few weeks, it is important to inform the patients and families about the ongoing benefits of continuing with the EEN for the full six to eight weeks with the aim of complete remission. The consensus guidelines of ECCO/ESPGHAN1 advise that if a clinical response has not been induced by two weeks of EEN, then an alternative treatment should be commenced, indicating that if EEN is going to work, you will see this within two weeks. A review half way into a patient’s treatment of EEN is a must to ensure that this is working for them and encouragement to call if no improvements are seen following discharge from the ward on their target volume. This prevents further delays to growth and development. CHOICE OF FEED

Many years ago, Elemental Feeds (EF) were used as the EEN in treatment of CD. It was felt that the amino acid based elemental feed gave the gut good nutrition whilst resting it from the whole proteins that could aggravate the mucosal lining. Since then, it has been shown that a polymeric Feed (PF) is just as effective and more palatable which improves compliance; the mechanism we are not entirely sure of yet. A double blind randomised controlled trial comparing EF versus PF found that in 34 children who completed it, there was no difference in inducing remission of CD between the two choices of EEN, meaning that the earlier theory of avoiding certain food allergens was not correct, particularly when there had been no studies highlighting specific food as triggers.8

The Cochrane review9 also shows that there was no statistical difference found between EF and PF in any of the trials that were reviewed, which led to the ECCO/ESPGHAN guidelines stating that EEN feeds should be given orally and as whole protein feeds; unless there is a clinical reason not to use these, for example in cows’ milk protein allergy.1 Modulen and Alicalm are whole protein feeds that are specifically for use in CD. They are marketed to contain the optimum amount of nutrients including lipids, with Modulen containing a natural anti-inflammatory factor. Both these feeds come in powder forms and require making up to the required concentration, which can take time and energy. The benefits of the PF being whole protein are that they are much more palatable than the EF and you can add different flavours to the Modulen and Alicalm to add variety and improve compliance with taking the drinks. Modulen is a 1kcal/ml feed, which can mean large volumes of feeds are needed to be taken to meet full requirements compared to Alicalm (1.35kcal/ml) and Ensure Plus (1.5kcal/ml). REQUIREMENTS

Estimated Average Requirements (EAR) should always be used for an EEN plan for a child. This is the daily nutrient level estimated to meet the requirements of half the healthy individuals in a particular life stage and gender group.10 This is more effective than using the requirements per kilogram as you are aiming to get the individual back to a healthy weight for height and age. Most studies fail to show that there is an increase in resting energy expenditure with active CD unless there is fever, so it is difficult to quantify increased requirements above base line level. BSPGHAN guidelines recommend EEN to meet 120% of EAR, but this is unrealistic and

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often unachievable.6 In clinical practice you have to be practical about the volumes of EEN you are advising a patient to drink: if on a daily basis it is too high, the child/young person is less likely to continue the treatment. The 12-year-old boy mentioned earlier had an EAR of 2,247kcals/day.10 This equates to seven of the whole protein, ready to feed 1.5kcal/ml drinks (220mls) each day, whereas 120% of EAR would be over eight of the drinks per day. Due to this child’s potential risk of refeeding and with all patients starting on the polymeric diet, the aim would be to reach the target volume of drinks on day three to four. Patients should be given the opportunity to take the EEN orally with the aim that the PF is much more palatable

and negates the need for a NGT to meet targets. NGT placement can be distressing for the child, it also requires much more support and training and can be linked with more time off school, difficulties socialising and can affect quality of life. However, it can take the pressure off having to drink the entire target volume.11 The possibility of an NGT should always be discussed at the first review as even the unlikeliest of patients can often not tolerate the volumes expected with EEN. One study placed the NGT during endoscopy and feeds started after 12 hours, building up to target by day three, and then permitting oral intake as tolerated.12 There is much debate and no consensus on what other fluids are allowed during the

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NHD CPD eArticle period of EEN; one study reported 68% of respondents were allowed clear fluids (fizzy drinks, ice and clear soup), 48% allowed boiled sweets, 39% allowed chewing gum and tea and coffee.4 One centre permitted 10% of intake as food, this is as suggested from Johnson’s study of partial enteral nutrition (PEE) and EEN that the anti-inflammatory effects aren’t seen if intake of food is continued and that although supplements are beneficial, it doesn’t suppress intestinal inflammation which is the key to remission.13 FOOD REINTRODUCTION AND REMISSION

Food reintroduction after EEN is another controversial area and every centre appears to introduce food slightly differently. Faiman’s retrospective cohort study in 201414 was the first to compare standard versus rapid food reintroduction with standard practice being a slow elimination diet to ensure remission is established for as long as possible. Faiman’s study was small and was unable to identify exact timings of relapse; despite this, it did show that there was no statistical significance between the five-week introduction and the three-day

Volume 7.16 - 5th October 2017

introduction.14 The rapid introduction means that the diet isn’t unnecessarily restricted for long periods which could be detrimental to the nutritional adequacy of the diet and hence the growth of the child.14 Following food, it is advised to continue with nutritional supplements alongside diet to aid continued remission. A retrospective study primarily focusing on Maintenance Enteral Nutrition (MEN) showed that remission rates at one year were 60% with MEN compared to 15% with no treatment. This study aimed for 25% of EEN intake per day for MEN and again found it effective compared to medications such as Azathioprine.15 This equates to one to two supplement drinks per day alongside diet. CONCLUSION

Nutrition and enteral feeding plays a key role in the management of CD within Paediatrics, not only in inducing remission, but in maintaining remission as well, without the risks that are associated with other medications used within the disease management. The dietitian is therefore an important part of the gastroenterology multidisciplinary team.

References 1 Ruemmele FM, Veres G, Kolho KL, Griffiths A, Levine A, Escher JC, Amil Dias J, Barabino A, Braegger CP, Bronksky J et al (2014). Consensus guideline of ECCO/ ESPGHAN on the medical management of paediatric Crohn’s disease’ Journal of Crohn’s and Colitis, 8, 1179-1207 2 Day AS, Whitten KE, Sidler M, Lemberg DA (2008). Systematic review: nutritional therapy in paediatric Crohn’s disease, Alimentary Pharmacology and Therapeutics, 27, 293-307 3 Kappelman MD, Rifas-Shiman SL, Kleinman L, Ollendorf D, Bousvaros A, Grand RJ et al (2007). The prevalence and geographical distribution of Crohn’s disease and ulcerative colitis in the United states, Clinical Gastroenterology Hepatology, 5 (12), 1424-9 4 Whitten KE, Roger P, YOOI C, Day AS (2012). International survey of enteral nutrition protocols used in children with Crohn’s disease, Journal of Digestive Diseases; 13; 107-112 5 National Institute for Health and Care Excellence (NICE) (2012). Crohn’s disease: management. NICE clinical guideline 152 available at www.nice.org.uk/guidance/ cg152 [NICE guideline] 6 Sandhu BK, Fell JME, Beattie RM, Mitton SG (2010). Guidelines for the Management of Inflammatory Bowel Disease (IBD) in Children in the United Kingdom, Journal of Paediatric Gastroenterology and Nutrition, 50 (Supp 1): S1-S13 7 RCPCH (2012). UK-WHO growth charts, 0-18 years, available from www.rcpch.ac.uk/improving-child-health/public-health/uk-who-growth-charts 8 Fell JME, Paintin M, Arnaud-Battandier F et al (2000). Mucosal healing and a fall in mucosal pro inflammatory cytokine mRNA induced by a specific oral polymeric diet in paediatric Crohn’s disease, Alimentary Pharmacology Therapeutics: 14: 281-289 9 Zachos M, Tondeur M, Griffiths AM (2007). Enteral nutritional therapy for induction of remission in Crohn’s disease, Cochrane Database of Systematic Reviews, Issue 1:Cd000542 10 SACN (2011). Dietary reference values for energy, available at www.gov.uk/government/publications/sacn-dietary-reference-values-for-energy 11 Rodrigues AF, Johnson T, Davies P, Murphy MS (2007). Does polymeric formula improve adherence to liquid diet therapy in children with active Crohn’s disease? Archis Dis Child; 92: 767-770 12 Grogan JL, Cassan D, Terry A et al (2012). Enteral feeding therapy for newly diagnosed paediatric Crohn’s disease: A double blind randomised controlled trial with two years follow-up, Inflammatory Bowel Disease: 18(2) 246-253 13 Johnson T, MacDonald S, Hill SM, Thomas A Murphy MS (2006). Treatment of active Crohn’s disease in children using partial enteral nutrition with liquid formula: a randomised controlled trial, Gut: available at British Medical Journal: 55; 356-361 14 Faiman A, Mutalib M, Moylan A et al (2014). ‘Standard versus rapid food reintroduction after exclusive enteral nutritional therapy in paediatric Crohn’s disease, European Journal of Gastroenterology and Hepatology: 26; 276-281 15 Duncan H, Buchanan E, Cardigan T et al (2014). A retrospective study showing maintenance treatment options for paediatric CD in the first year following diagnosis after induction of remission with EEN: supplemental enteral nutrition is better than nothing! BMC Gastroenterology; 14:50 16 Day AS, Whitten KE, Lemberg DA et al (2006). Exclusive enteral feeding as primary therapy for Crohn’s disease in Australian children and adolescents: a feasible and effective approach, Journal of Gastroenterology and Hepatology: 21: 1609-14 17 Knight C, El-Matary W, Spray C, Sandhu B (2005). Long-term outcome of nutritional therapy in paediatric Crohn’s disease, Journal of Clinical Nutrition; 24: 775-779 Copyright © 2017 NH Publishing Ltd - All rights reserved. Available for printing and sharing for the use of CPD activities for personal use. Not for reproduction for publishing purposes without written permission from NH Publishing Ltd.

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Questions relating to: Crohn’s disease: nutritional management in children. Type your answers below, download and save or print for your records, or print and complete by hand. Q.1

Explain the characteristics of Crohn’s disease (CD).

Q.2

What are the difficulties of diagnosing paediatric CD?

Q.3

Why is linear growth an important factor in diagnosis of paediatric CD?

Q.4

Describe why Exclusive Enteral Feeding EEN is recommended as the first line treatment in the management of CD.

Q.5

What are the challenges of EEN in CD?

Q.6

Why are polymeric feeds considered just as effective as amino acid feeds?

Q.7

Explain the benefits of whole protein feeds.

Q.8

What are the difficulties of NGT placement?

Q.9

What controversy surrounds food reintroduction?

Please type additional notes here . . .