ESPEN Congress Geneva 2014 NUTRITION IN PAEDIATRIC PATIENTS 2. Nutrition treatment in children with intestinal failure S.

Transcription

1 ESPEN Congress Geneva 2014 NUTRITION IN PAEDIATRIC PATIENTS 2 Nutrition treatment in children with intestinal failure S. Kolaček (HR)

2 Nutrition treatment in children with intestinal failure - IF special emphasis on Short Bowel Syndrome Module 4.3 Topic 4 Sanja Kolaček CHILDREN S HOSPITAL ZAGREB, Croatia

3 Nutritional Treatment of Hyperlipidemia in Children Lecture Objectives Introduction to the subject Screening for hyperlipidemia in childhood Diagnosis and evaluation of hyperlipidemia in children CHILD1 and CHILD2 nutritional intervention Familial hypercholesterolemia (FH)

4 NUTRITION IN IF /SBS Lecture objectives Definition & aetiopathogenesis Clinical presentation Enteral / oral nutrition support (EN) Parenteral nutrition support (PN) & complications Role of surgery Role of team approach

5 CHRONIC INTESTINAL FAILURE Reduction of gut mass/function below minimum for absorption & digestion to support life & growth Causes: Short Bowel Syndrome (50% 60%) Intractable diarrhoea congenital enteropathies (30 41%) Intestinal motility disorders (9%)** D Antiga L & Goulet O. JPGN 2013; ** Diamanti A, et al. JPGN 2009

6 Etiology of intestinal failure The distribution of primary etiologies for intestinal failure Javid PJ et al. Am J Surg 2010; 199:

7 SHORT BOWEL SYNDROME -SBS INTESTINAL FAILURE = reduction of gut mass & function below minimum for absorption & digestion to support life and growth AFTER MASSIVE SURGICAL RESECTION

8 SBS causes Conditions leading to extensive intestinal resection PRENATAL NEONATAL CHILDREN & ADULTS Defects of abd. wall (gastroschisis) Apple peel syndrome Midgut volvulus Multiple atresias Extensive aganglionosis Necrotising enterocolitis Midgut volvulus Meconium ileus Vascular accidents Primary motility disorders Vascular accidents Invagination Polyposis syndrome Multiple hemangioma Trauma, Tumours Bypass surgery in obesity

9 IF & SBS: how common IF prevalence: 13.7/ children Beath S et al. Clin Nutr 2011;30: SBS incidence: 2 3/ /year Grant O et al. J Pediatr Gastroenterol Nutr 2004;38:250 69

10 SBS: factors relevant for pathophysiology AGE At birth cm of small intestine In adults cm of small intestine SEGMENT OF THE BOWEL Jejunum: leaky tight junctions rapid absorption, fast loss of water & Na low ability for adaptation Ileum: specific transport for bile acids & vit B12 huge capacity for adaptation Ileocoecal valve: prevents small bowel bacterial overgrowth Colon: conservation of water & electrolytes + extra starch digestion INTEGRITY OF THE RESIDUAL INTESTINE

11 Prognostic determinants of IF caused by SBS POSITIVE NEGATIVE Ileum as residual segment Ileocecal valve present Colon in continuity Early age at resection Healthy residual GUT Jejunum as residual segment High jejunostomy Resection > 1-2 y of age Dysfunctional residual segment

12 SBS: pathogenesis MAJOR DETERMINANTS extent of resection age at resection type & integrity of bowel left HOW SHORT IS STILL ENOUGH? <40 cm or <20% Life possible: full blown SBS >15 20 cm of small intestine with ileocoecal valve >30 40 cm of small intstine without the valve After adaptation: cm enough for life on peroral intake (+ colon in continuity)

13 INTESTINAL ADAPTATION Warner et al. J Pediatr Surg. 2013; 48:

14 INTESTINAL ADAPTATION Hypertrophy villus elongated crypts deeper mucosa ticker Functional adjustments more transport proteins more enzymes slower motility Increase in absorptive surface Faster, more efficient metabolism

15 Control of adaptation Luminal nutrients!!! Saliva, bile, pancreatic secretion... Blood irrigation & Motility Trophic factors/hormons glucagon like peptide 2 GLP2, IGF, epidermal GF, GH, insulin, polyamines...

16 SBS: Clinical presentation Wales PW et al. Seminars in Pediatric Surgery 2010 Early phase (weeks after resection) Intermediate phase (months after resection) Late phase (steady state after adaptation) Huge loss of water and electrolytes (jejunum vs.ileum vs. ileum + colon) Malabsorption of nutrients & loss of nutrients requiring specific transport Dependence on PN complete / partial and / or Special nutr. requirments and/ or Develop. of complications (IFALD, loss of venous access, lithiasis, bone mineralization, D-lactacidosis...)

17 IF/SBS: major determinants of treatment 1. Provide nutrition to sustain life and support growth: PN+EN 2. Maximize intestinal adaptation 3. Prevent major complications of PN CVC related sepsis / loss of venous access IFALD (Intestinal Failure Associated Liver Disease)

18 SBS: Enteral nutrition Olieman JF, et al. J Am Diet Assoc 2010 D Antiga L, Goulet O. JPGN 2013, Hojsak I, Kolacek S. Liječ Vjesn 2013 ROLE promotion of intestinal adaptation prevention of IFALD RECOMMENDATION GUT to be used to a maximum extent but without worsening diarrhoea avoid overfeeding!! OPTIONS??? breast feeding; standard formula extensivelly hydrolyzed (ehf), amino acid based (AA) MODE??? bolus versus continuous???

19 EN in SBS: type of formula What to recommend?? Hojsak I, Kolacek S. Liječ Vjesn 2013 Only expert opinions!! Breast milk best option At start use ehf or AA, particularly in infants with dilated short segment prone to cow s milk protein allergy In older infants / children switch to polymeric Start with weaning as age appropriate

20 EN in SBS: mode?? Benefits of continuous Better utilization of reduced surface & transport proteins Osmotic load better tolerate Improved growth & intestinal adaptation Benefits of boluses Physiologic hormonal surge & gallblader emptying Improved mobility & QL Promote normal feeding, habits & skills Recommendations Combine small boluses over day & night continuous Switch to boluses when appropriate

21 Intestinale failure: PN 1. Provide balanced iv intake 2. Save central veins (prevent CVC related infections) 3. Prevent other major complications IFALD (Intestinal Failure Associated Liver Disease)

22 Balanced PN Energy, amino acid solutions balanced energy intake, avoid excess energy age adjusted composition with semi essential amino acids arginine, cystein, taurin, proline, tyrosine Carbohydrate only glucose provide 70% 75% of non protein energy introduce stepwise Lipids source of essential amino aids provide 25% 30% of non protein energy pathogenic role of quantity and quality in IFALD??

23 Paediatric PN: types of solutions Ready made - cheaper, available - not suitable for small children & changing requirements Tailor-made - adapted to requirements of the individual child - more appropriate for changing requirements of children with intestinal failure

24 Intestinale failure: PN Save venous access prevent CVC-related sepsis 1. Aseptic approach 2. Use of ethanol & taurolidin lock 3. Early discharge home

25 Paediatric PN: compounding

26 Aseptic approach: how we do it

27 Oliviera et al. Ethanol locks to prevent CRBSI in parenteral nutrition: A meta analysis. Pediatrics 2012

28 Central venous catheters and catheter locks in children with cancer: A prospective randomized trial of taurolidine vs heparin. Handrup MM et al. Pediatr Blood Cancer 2013;60: Kaplan Meier analysis of time to first catheter related blood stream infection (CRBSI) CVC with taurolock (solid line) associated with significantly lower risk of CRBSI than CVC locked with heparin. N=129 CVC; P=0.001

29 Paediatric PN in IF: prevention of common complications Strict aseptic procedures on insertion & during CVC maintenance!!! - Team approach - Home parenteral nutrition O Grady N et al. CDC Guideines for prevention of intravascular catheter-related infections. CID 2011;52

30 Role of multi-disciplinary intestinal rehabilitation programs on outcome in children with IF (Stanger JD, et al. J Pediatr Surg 2013;48:983 92) A. Pooled RR of survival from IF C. Pooled RR for development of liver failure D. Pooled RR for enteral autonomy

31 Paediatric IF: Home PN - The best option for long-term PN : - better quality of life - lower rate of CVK-related infections - lower costs by 50% - 60% - To provide Home PN the following is required - stable patient on cyclic PN - structured training of caregivers - back-up of experienced center & nutrition support team available 7 days a week for 24 h ESPGAN / ESPEN Paediatric PN Guidelines, JPGN 2005

32 HOME PARENTERAL NUTRITION & Nutritional Care Team

33 PATHOGENESIS OF IFALD Empty GUT Imature liver (prematurity) Early & recurrent sepsis Intestinal stasis & bacterial overgrowth Lipids: quantity & quality Phytosterols (from soya) Enteral feeding Balanced macro&micronutrient i.v. supply Cyclic PN & Home PN & Team approach Balanced omega 3/ omega 6 (fish oil) High alphatocopherol Liver injury Liver protection Adapted from: D Antiga L, Goulet O. JPGN 2013

34 PN in IF: Role of fish oil Seida JC, et al. JPEN 2013;37:44 55

35 PN: Pure fish oil vs fish oil in combination D Antiga L, Goulet O. JPGN 2013 Pure fish oil is effective, but as a sole source may not meet EFAs requirements on long term basis Emulsion with fish oil as 1 component useful, safe major advancement for long term PN???

36 Drug therapy Dehmer JJ, et al. Advances in Pediarics 2011;58 To slow transit time To bind bile acids To lower secretion To lower acid secretion To prevent bacterial overgrowth To stimulate intestinal adaptation loperamid cholestiramin somatostatin PPI, H2 blockers Trimetoprim, rifaximin metronidazol gentamycin per os... GH, insulin, glutamin pectins GLP 2!!

37 SBS: Role of GLP-2 Uko V, et al. Pediatr Drugs 2012;14:

38 SBS: nontransplant surgery Rege AS, et al. Nutr Clin Pract 2013, Weih S et al. Langenbecks Arch Surg 2012 ROLE bowel lengthening (Biancchi, STEP) promotion of motility in dilated segment (tailoring, plication) slowing motility (antiperistaltic segment, valves...) INDICATIONS for intestinal reconstruction PN dependent patient time for adaptation lapsed deterioration of intestinal function due to dilatation & SBBO ANATOMIC REQUIREMENTS for lengthening dilatated intestine >3 cm >20 cm of small intestine >40 cm of total bowel

39 LILT Bianchi procedure Taken from: Sudan D. Autologous reconstruction of the GI tract. In: Langnas AN et al (eds). Intestinal failure: Diagnosis, Management and Transplantation. Malden. Blackwell Publishing 2008;

40 Serial transverse enteroplasty STEP Taken from: Sudan D. Autologous reconstruction of the GI tract. In: Langnas AN et al (eds). Intestinal failure: Diagnosis, Management and Transplantation. Malden. Blackwell Publishing 2008;

41 intestinal transplantation When to do it? D Antiga L, Goulet O. JPGN 2013 Prolonged PN versus Early transplantation (ITx) Prognosis after 5y, 10y: 85%, 75% for PN versus 50%, 30% for ITx Morbidity & mortality significantly improved in centres with multidisciplinary intestinal rehabilitation programs CURRENT RECOMMENDATIONS Long term PN is the treatment of choice for children with IF including irreversible Intestinal Failure Children with IF should be treated in centres with multidisciplinary teams and intestinal rehabilitation programs ITx is reserved for irreversible IF with life treatening complications: loss of venous access severe recurrent sepsis end stage IFALD...

42 Nutrition Treatment of IF in children Conclusions Long term PN is Conditio sine qua non for IF age adjusted PN composition aseptic approach cyclic home PN prevention of IFALD promising role for fish oil based emulsions Support intestinal adaptation use EN in SBS use GUT as early as possible & to maximum extent tolerated promising role of GLP 2 promising role of surgical intestinal lengthening procedures Treat in centres with multidisciplinary intestinal rehabilitation team & program ITx is a life saving procedure, NOT a routine