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Intestinal Failure During Transition: An Overview

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Title: Intestinal Failure During Transition: An Overview


1
Intestinal Failure During Transition An Overview
  • Leah Gramlich, MD, FRCP
  • University of Alberta

2
Outline
  • Definitions
  • Transition from Adolescence to Adulthood
  • Intestinal Failure optimizing care

3
Definitions Transition of Care
  • The purposeful, planned movement of adolescents
    and young adults with chronic physical and
    medical conditions from child-centered to
    adult-oriented health care system
  • AAP Committee on Children with Disabilities and
    Committee on Adolescence Transition of Care.
    Pediatrics 1996 98(6) 1203-1205

4
Transition Setting the Stage
  • Chronic illness / disability affect 2-4 of
    population
  • US 1/3 children/youth have some form of chronic
    illness
  • Conference Proceedings Transition to adult
    health care setting the stage. J Adolesc Health
    1995173-5
  • Cerebral palsy / Spina bifida
  • Cystic fibrosis
  • Diabetes

5
Overview Setting the Stage
  • Young Adults in Childrens Hospitals Why are
    they there?
  • Disease complexity
  • 57 with gt 3 medical/surgical services involved
  • Failure of transition planning
  • 28 no documented plan for transition
  • Concern of lack of appropriate services in adult
    sector
  • Lam et al Med J Austral 2001 521

6
Overview Setting the Stage
  • IBD
  • NASPGHAN Position Statement
  • J Ped Gastro Nutr 2002 245-248
  • Chronic liver disease
  • Liver transplant patients
  • Celiac disease
  • Intestinal failure/Home TPN therapy
  • Rare GI / Metabolic diseases

7
Principles of Transitional Care
  • Transition process should be considered at the
    day of diagnosis
  • Plan developed in which goals for independence /
    self-management are outlined
  • Approximate time schedule
  • Revisions made throughout treatment

8
BS 18 yo male
  • CC Transition of Care
  • HPI - Multiple intestinal atresisas - TPN
    dependent since birth - HEN initiated
    12/04 - Metabolic complications Macrophage
    activation syndrome, TPN cholestasis, Acidosis,
    metabolic bone disease

9
BS
  • Social History - Grade 12 - Parents
    divorced - non-smoker - no family doctor
  • Meds Pantoloc 40 mg bid Bicitra 50 meq
    qd calcium, Vitamin D Immodium

10
BS
  • Oral Intake- 3500-7000 kcal/d, high fat -
    Peptamen 1.5, 4 cans/d via (mickey PEG)
  • GI Symptoms - 5-6 liquid BMs/d
  • ROS -recurrent and chronic sinusitus
  • P/E 166 cm 47.5 Kg (IBW60-66 kg) - thin,
    good lean tissue stores - G-tube site OK

11
OK135S057
12
Intestinal FailureDefinition
  • Gastrointestinal function which is insufficient
    to satisfy body nutrient and fluid requirements
  • American Society of Transplantation 2001
  • Dependence on parenteral nutrition to achieve
    maintenance in adults and growth in
    children. Goulet J Pediatr Gastroenterol Nutr.
    2004 38(3)250-69.

13
Children and adolescents medical and nutritional
needs
  • Implications of IF in children are different than
    those in adults
  • Increased risk for
  • Growth failure
  • Delayed pubertal development
  • Compromised bone mineral density
  • Medical, surgical, nutritional management are
    affected by needs for growth and maturation

14
Challenges in Pediatric Intestinal Failure
  • Provision of sufficient nutrients to promote
    growth and development
  • Prevention and treatment of TPN-related
    complications
  • TPN induced liver disease
  • Infection
  • Vascular access complications
  • Osteopenia

15
Intestinal failure requiring transplantation
  • Short bowel syndrome
  • Volvulus
  • NEC
  • Gastroschisis
  • Enterocyte disorders
  • Epithelial dysplasia
  • Microvillus inclusion disease
  • Neuromuscular disorders
  • Hirschprungs
  • Pseudo-obstruction

Grant D et al Ann Surg. 2005241(4)607-13.
16
Prognosis for intestinal autonomy
  • Residual small bowel length
  • More important for adults than children
  • Presence of IC valve
  • Bacterial overgrowth
  • Presence of colon
  • Affects intestinal transit
  • Fluid, electrolyte, energy absorption
  • Motility
  • Bacterial overgrowth
  • Gut adaptation critical!

17
Normal bowel length
  • lt 27 weeks gestation 115 cm
  • Term infants 250 cm
  • Adults 600 cm
  • Change in mucosal surface area with age
  • 0.95 m2 (newborn)
  • 7.5 m2 (adult)

18
Intestinal adaptation
  • Structural and Functional changes
  • Hyperplasiagthypertrophy
  • Absorption/secretion
  • Motility
  • Luminal Nutrients
  • Hormones
  • Growth Factors
  • Pancreatico-biliary secretions

19
Nutrient absorption in jejunum
  • Macronutrients
  • Glucose other monosaccharides
  • Fatty Acids
  • Cholesterol
  • Amino Acids Peptides
  • Water soluble vitamins
  • Electrolytes
  • Minerals
  • Water
  • Iron
  • Calcium
  • Magnesium

20
Nutrient absorption in ileum
  • Bile Acids
  • Fat Cholesterol
  • Fat Soluble Vitamins
  • Vitamin B12
  • Electrolytes
  • Water

21
Nutrient balancerole of the colon
  • Significantly improves with presence of colon
  • Undigested CHO-converted to SCFA colonocyte
    energy substrate
  • Steatorrhea may affect colonic recycling of
    nitrogen

22
Nutritional Factors Affecting Gut Adaptation
  • Trophic feeding
  • Specific nutrients
  • Long Chain Triglycerides
  • SCFA-butyric acid from fermentable fibre
  • Protein (complex vs hydrolysates)
  • Glutamine / Growth Hormone
  • Pre-biotics/Pro-biotics

23
Nutritional Issues in Intestinal Failure
  • Energy consider fractional absorption of macro
    and micronutrients, depending upon anatomy
  • Protein 1.5 gm/kg modifications with liver
    disease and renal disease
  • Fat MCT vs LCT
  • Water consider what the patient is drinking

24
Energy Requirements
  • Assumption fractional absorption of nutrient
    intake (50)
  • 120-150 of the RDA
  • Relative increase in nutrient requirements to
    compensate for malabsorption - Potential for
    increased needs related to increased cytokine
    production, changes in portal blood flow,
    hormones
  • Potential for significant growth failure in
    children and malnutrition in adults from
    inadequate dietary intake.

25
Protein Requirements in Intestinal Failure
  • Protein requirements
  • 3-3.5 g/kg in neonates
  • 1-1.5 g/kg in adolescents
  • 1-1.5 g/kg in adults
  • May have ? protein/BCAA needs with chronic liver
    failure
  • May need reduction in protein intake in renal
    insufficiency

26
Fats LCT vs. MCT
  • Absorption
  • MCT better absorbed
  • Increased benefit if colon intact
  • MCT may be associated with ? jejunostomy output
  • Trophism
  • LCFA trophic to gut
  • Energy utilization

27
Micronutrients
  • Vitamins B12, ADEK
  • Minerals Magnesium, Calcium
  • Trace Elements Zinc, Selenium

28
Dietary Management of IF
  • High Calorie/High Protein
  • Frequent small meals
  • Consider fibre - slow transit - water
    holding capabilities -
    fermentation and transport of scfas in colon
    with salt and water con-transport
  • Oral vitamin and mineral supplementation

29
Modes of Nutrition Therapy
  • Oral Diet (/-supplements)
  • Enteral
  • Enteral IV Fluid
  • Enteral TPN
  • TPN

30
Medical management of short bowel
syndromepharmacological
  • Gastric Acid Suppression
  • H2-receptor antagonists
  • Proton pump inhibitors
  • Motility
  • Prokinetics
  • Bacterial Overgrowth
  • Cyclical antibiotic therapy
  • Probiotics
  • Diarrhea/High Ostomy Output
  • Loperamide
  • Octreotide
  • Clonidine
  • Cholestyramine

31
Surgical Management
  • Restoration of intestinal continuity
  • Stricture resection/dilation
  • Tapering procedures
  • Bowel Lengthening
  • Serial Transverse Enteroplasty (STEP)
  • Bianchi Procedure
  • Bowel resection
  • Intestinal /multi-visceral transplantation

32
TPN complications
  • Hepatobiliary
  • Infants gt adolescents/adults
  • Venous Thrombosis
  • Catheter Infection, thrombosis
  • Renal
  • Bone

33
PN Associated Hepatotoxicity
  • TPN Cholestasis ggt, bili, Alk Phos gt1.5 x uln
    (require 2/3) Cavicchi AIM 2000. 132525-32
  • Mild lfts lt2x uln Moderate 2-5 x uln
    Severe gt5x uln seidner D. ASPEN 03

34
Risk Factors for TPN-Related Liver Disease
  • Patient factors age, low birth weight,
    preexisting liver disease, microsepsis
  • GIT factors bowel remnant lt50cm
  • Nutritional Factors
  • limited oral or enteral intake
  • increased reliance on TPN
  • increased duration of TPN
  • composition of TPN lipid, CHO, Pro, other

35
Optimizing the Nutrient Prescription to Obviate
TPN Toxicity
  • Avoid overfeeding
  • 25-35kcal/kg IC if in doubt
  • Limit CHO to lt 4mg/kg/min
  • Limit fat to lt 1gm/kg if cholestasis develops,
    consider 0.5gm/kg Cavicchi. AIM. 2000

36
Vascular Access Considerations
  • Infection 0.5catheter/yr BC - 3.2/-1.2
    /1000 patient days Ont - 2.2/-0.4/1000
    patient days
  • Thrombosis
  • Breakage
  • Air embolus

37
Survival and PN Dependence in Adults With SBS
  • Messing et al.Gastro, 19991171043
  • 124 adult patients with nonmalignant SBS
  • Survival (2, 5 yrs) 86, 75
  • PN Dependence (2, 5 yrs) 49, 45
  • After 2 yrs probability of permanent intestinal
    failure is 94

38
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39
SBS Mortality
  • Death is related to - underlying
    disease - TPN related liver disease (15 long
    term TPN) - Sepsis (catheter related),
    related to residual bowel length -
    malnutrition Messing et al,Gastro
    1999117, ,Chan et al , Surgery 199912628

40
TPN Failure
  • Impending or overt liver failure - Increased
    LFTs/bleeding/cirrhosis
  • Thrombosis of central veins gt2 of subclavian,
    jugular or femoral veins
  • Frequent CVL Sepsis (gt2 episodes/yr)
  • Frequent severe dehydration

41
SBS Intestinal Transplantation
  • Indication Intestinal Failure and complications
    of long-term TPN
  • Pediatric gt Adult (over 500 pt. worldwide)
  • Survival 1yr - 65 3yr - 55
  • Complications rejection (1.5episodes/graft)
  • Nutritional autonomy 70 grafts surviving gt30d
    Faarmer et l. Arch
    Surg.20011361027

42
Outcome With Intestinal Transplantation
  • gt1000 done at 60 centers www.intestinaltransplantr
    egistry.com
  • 60 lt 18 yrs old
  • Indications - Peds - volvulus, atresia,
    aganglionosis, NEC, pseudoobsruction - Adults -
    ischemia, chrons, trauma, volvulus

43
Nutritional Issues in Transition of Care
  • Nocturnal enteral and TPN support usually
    accepted more readily with an emphasis on
    cycling of IV TPN (lt 7 days/week)
  • Complexity of regimen may be related to increased
    needs due to growth-need to be innovative in
    approach to meet these needs
  • Need to consider the psychological and
    developmental needs when designing a medical and
    nutrition support therapy in children and
    adolescents
  • Emphasis should be on development of skills for
    adolescent to self-manage medical and nutritional
    therapy.

44
BS Transition Considerations
  • Reassure patient, family and pediatric care
    providers
  • Ongoing realignment of care and evaluation
  • Foster independence in activity and in decisions
    around healthcare

45
Acknowledgements
  • CAG
  • CSCN
  • Dietitians of Canada
  • Nestlé Canada
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