Home Parenteral Nutrition

Question 1

Examples of primary digestive disorders and primary non-digestive disorders

Answer 1

• primary digestive disorders: intestinal surgery, SBS (volvulus, gastroschisis, nectrotizing enterocolitis), enteropathies, dysmotility/pseudobstruction
• primary non-digestive disorders: prematurity (immature gut), multi-organ failure (extensive burns/trauma), chemotherapy, bone marrow transplant

Question 2

Factors to consider when commencing PN

Answer 2

• clinical status, fluid requirements, venous access, electrolyte levels, liver function, weight, drugs and other IV infusions
• urgency of PN: in severe gut failure use within 24 hours for very premature baby, use within 5 days for a term neonate, in <7 days for an older child

Question 3

Management of PN: feeding

Answer 3

• need to make sure feeding as per actual weight not expected weight
• monitor weight and height using z scores

Question 4

Management of PN: metabolic

Answer 4

• need to do nutritional monitoring for refeeding syndrome
• look out for low phosphate, potassium and magnesium in bloods
• vitamins too: deficiencies common and cannot put in bag as unstable so need to give orally (with trace elements)

Question 5

Management of PN: liver disease

Answer 5

• PN can precipitate cholestasis due to CHO load
• occurs in 40-60% of children
• phytosterols may be connected
• in long term patients can cycle PN to reduce the risk of this (aim for 12 hours a day), give lipids 2-3x per week, give variety of feeds with different lipid (soya, olive, coconut, MCTs, fish oil)

Question 6

Management of PN: thromboembolism

Answer 6

• should be inserting PN under radiological control to minimise damage

Question 7

Management of PN: infection

Answer 7

• use 2% clorhexidene to clean catheter site
• taurolidine can also be used as a catheter lock solution which causes bacterial and fungal lysis. Effective if inserted in the catheter hub after infusion

Question 8

GLP-2 therapy for SBS

Answer 8

• improves intestinal structure and functional integrity
• reduces requirements for PN
• causes mucosal hypertrophy, lengthens the villi, inhibits gastric secretion, stimulates intestinal blood flow, increases the barrier function and enhances the absorption of nutrients and fluids
• allows for weaning of PN
• however, needle phobia is a potential concern with teduglutide (GLP2)
• need to monitor weekly for 3 weeks, then 2 weekly, then monthly

Question 9

Principles of weaning from PN

Answer 9

• reduce in 10-20% increments as a guide
• aim for a night off with 40-50% kcals PN
• allow child to enjoy food
• ensure appropriate dietary restrictions: limit fructose and refined sugar

Question 10

Long term/ home PN complications

Answer 10

• life threatening complications: catheter-related bloodstream infections, intestinal failure associated liver disease, thrombosis
• long term problems: small bowel bacterial overgrowth, D-lactic acidosis, growth retardation, metabolic bone disease

Question 11

ESPEN guidelines for paediatric PN

Answer 11

1) stabilise
2) weight gain
3) normalise
4) wean off