Intestinal Failure During Transition: An Overview

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Intestinal Failure During Transition An Overview

• Leah Gramlich, MD, FRCP
• University of Alberta

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Outline

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

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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

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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

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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

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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

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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

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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

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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

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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

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OK135S057
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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.

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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

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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

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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.
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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!

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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)

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Intestinal adaptation

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

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Nutrient absorption in jejunum

• Macronutrients
• Glucose other monosaccharides
• Fatty Acids
• Cholesterol
• Amino Acids Peptides
• Water soluble vitamins
• Electrolytes
• Minerals
• Water

• Iron
• Calcium
• Magnesium

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Nutrient absorption in ileum

• Bile Acids
• Fat Cholesterol
• Fat Soluble Vitamins
• Vitamin B12
• Electrolytes
• Water

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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

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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

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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

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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.

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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

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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

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Micronutrients

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

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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

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Modes of Nutrition Therapy

• Oral Diet (/-supplements)
• Enteral
• Enteral IV Fluid
• Enteral TPN
• TPN

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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

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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

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TPN complications

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

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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

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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

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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

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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

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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

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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

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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

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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

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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

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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.

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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

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Acknowledgements

• CAG
• CSCN
• Dietitians of Canada
• Nestlé Canada