Nutrition in EB A Case Study Approach

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Nutrition in EBA Case Study Approach

• Jennifer Phillips MS, RD, LD

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Objectives

• Will be able to determine nutritional needs in a
  pediatric EB patient
• Will be able to provide example of
  multidisciplinary team approach
• Will be able to develop a care plan that improves
  nutritional outcome

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Patient

• 6 year old white female with Recessive Dystrophic
  EB
• Full term infant, uncomplicated pregnancy
• Blistering at birth and aplasia cutis of right
  leg
• Diagnosed at 3 days of age

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Review of Past Medical History

• Presented to clinic at 2 years of age
• Presented with extensive skin erosions, nail
  loss, dysphagia, milia and digit webbing
• Barium swallow done showing mild stricture
• Known concerns Food refusal, vomiting, oral
  lesions, behavioral concerns.

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Anthropometric Measurementsat Presentation

• Wt 13.18 kg (50th percentile) NCHS growth charts
• Length 92.1 cm (25-50th percentile)
• Body Mass Index (BMI) 15.5 kg/m2 (25-50th
  percentile)

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Nutritional Assessment

• No known food allergies
• Fair appetite
• Consumes mostly soft foods d/t dysphagia, oral
  lesions
• Constipation a problem, takes MiraLax daily PRN
• 4-5 cans Pediasure with Fiber per day

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Nutritional assessment, cont.

• Bites of pudding, yogurt or soup 2-3X daily
• Intake provides 85 kcal/kg, 2.5 gm protein/kg
• Chewable multivitamin and zinc daily

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Nutritional Intake Compared to Estimated Needs

• Calories 125-150 kcal/kg (125-150 RDA)
• 85 kcal/kg (currently)
• Protein 2.5-3 gm/kg (200-250 RDA)
• 2.5 gm/kg (currently)

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Interventions

• Higher calorie supplement Boost Plus with goal
  of 4 cans/day to provide additional 480 kcal, 28
  gm protein.
• Try Boost pudding, high fat yogurts
• Polycose powder
• Esophageal dilatation

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Suggestions to Increase Calories and Protein

• Add cheese (100 kcal/ounce) to eggs, sandwiches,
  potatoes, chips, vegetables etc.
• Add peanut butter (95 kcal/tbsp) to toast,
  crackers, bananas, apples etc.
• Add butter to foods (45 kcal/tsp)
• Add powdered milk (33kcal/tbsp) or Carnation
  Instant Breakfast (130kcal/packet) to all milk

• Sweetened condensed milk (60kcal/tbsp)
• No calorie free fluids
• Add meats to soups and casseroles
• Add ice cream to supplements
• Use Pediasure or other supplement in place of
  milk in preparing oatmeal, pancakes, etc.

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Initial Follow Up(4 years 11 months old)

• Weight up 3 kg
• Increased tolerance of textured foods
• Majority of intake from Boost Plus (4-5 cans per
  day)
• Refusal of iron supplement

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8 Month Return Visit(5 years 7 months)

• Anthropometrics
• -Weight 16.3 kg (10th percentile)
• -Height 111.2 cm (25-50th percentile)
• -BMI 13.1 kg/m2 (lt3rd percentile)

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Diet History

• Refusing favorite foods
• Mealtimes source of conflict
• Only consuming 4-5 cans of Boost Plus

• Inadequate fluid intake
• Inadequate caloric intake 80 kcal/kg

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Issues Surrounding Food Refusal

• 5 years old wanting independence
• Control issues
• Tube being used as a threat to get patient to eat

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Interventions

• Add ice cream to Boost Plus
• Offer small meals throughout the day
• Do not let meal times linger as tiring for the
  patient and adds frustration for all involved

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Admission

• 6 years old
• Admit for PEG placement, multiple tooth
  extraction

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Diet History

• Significant decline in oral intake
• - Only consuming 1 can Boost plus per
• day, 1 bowl of cereal and milk and
• ½ bowl of pasta, drinks water,
• milk and juice with meals

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Anthropometric MeasurementsUpon Admit

• Weight down 400 grams X 3 ½ months
• Weight 15.9 kg (3rd percentile)
• Height 113.5 cm (10-25th percentile)
• BMI 12.3 kg/m2 (lt3rd percentile)
• Suggested weight for height 18.5 kg-19.6 kg
• Now weight age of a 4 year old

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Laboratory Values

• Iron 17 mcg/dL Low
• Zinc 598 mcg/dL Low
• Albumin 3.8 g/dL Normal
• Vitamin D 25-Hydroxy 12.8 ng/mL normal

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Difficulty in Determining Visceral Protein Status

• Albumin affected by fluid status, can look
  falsely elevated secondary to dehydration
• Prealbumin falsely elevated secondary to
  inflammation

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Indications for PEG placement

• Patient with 1.2 kg weight loss over the past
  year despite growing 7 cm taller
• BMI fallen from 50th percentile to less than the
  3rd over the past year
• 84 of suggested weight/height indicative of a
  moderate degree of malnutrition

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Consequences of Malnutrition

• Growth failure
• Poor wound healing
• Anemia
• Increased susceptibility to infection

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Initiating Nutrition Support

• Estimate calorie, protein and fluid needs
• Select enteral formula
• Determine feeding regimen that can be executed at
  home
• Determine if patient at risk for refeeding

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Calorie, Protein and Fluid Needs

• Calorie needs 110-120 kcal/kg (120-130 RDA)
• Protein needs gt 3 grams/kg (250 RDA)
• Fluid needs at least 1300 mL/day

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Formula Selection

• Adult Formulas
• Boost Plus (1.52 kcal/mL, .043 gm protein/mL,
  DRIs met in 1000 mL )
• Ensure Plus (1.5 kcal/mL, .055 gm protein/mL,
  DRIs met in 1000 mL)
• Jevity 1, 1.2, 1.5 per mL (fiber, .044 .056 .064
  gm protein/mL, DRIs met in 1000 mL )

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Formulas continued..

• Pediatric Formulas (ages 1-10 yrs)
• Pediasure with Fiber (1 kcal/mL, .03 gm
  protein/mL, DRIs met in 1000mL for 1-8 y.o. and
  1500 mL 9-10 yrs)
• Kindercal with Fiber (1 kcal/mL, .03 gm
  protein/mL, DRIs met in 946 mL)
• Resource Just for Kids 1.5 with Fiber (1.5
  kcal/mL, .042 gm/mL, DRIs met in 870 mL)

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Formula Choice

• Boost Plus
• - Covered by insurance
• - Can use for enteral feedings and will drink by
  mouth
• - Intact formula, patient with functioning GI
  tract

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Risk for Refeeding

• Gradual increase in caloric intake
• 15 mL/hour for 2 days
• 30 mL/hour for 2 days
• 45 mL/hour for 3 days
• 60 mL/hour for 3 days
• 75 mL/hour (goal)

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Feeding Plan

• Goal feeds Boost Plus at 75 mL per hour for 10
  hours overnight. Also needs to drink 2 cans
  during the day. Bolus any remaining formula
  unable to take by mouth.
• Needs additional 12-16 ounces of fluid/day to
  meet fluid needs.

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6 Month Follow up

• Weight gain 5.2 kg (28gms/day)
• Tolerating feeds without difficulty
• Feeds _at_75 mL per hour for 10 hours not consuming
  extra 2 cans, refusing supplements by mouth
• Formula changed to Nutren 1.5 for insurance
  purposes