…where children’s health comes first

Case Study Dystrophic EB

Ita SaulDietitian ManagerOur Lady’s Children’s HospitalCrumlinDublin, Ireland

Patients’ photographs have been removedfrom this presentation

…where children’s health comes first

Adam dob 09/05/1993Dx RDEB

Bt. Weight: 3.52kg Social History: 1 sister unaffected

…where children’s health comes first

Initial Feeding

Aim:150% Req: Protein & Kcals

Feed: Concentrated Infant Formulawith Energy Enhancer

…where children’s health comes first

FEED COMPARISONFeed/100ml

EBM Formula13%

Formula 16%

Formula 16%+Cals

Protein 1.3 1.5 2.0 2.0

Kcals 66 67 84 100

P:E ratio 7.8 8.9 9.5 8.0

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1st year of life

Spoon feeds introduced at 4 monthsInitial weight gain appropriateContinuing Follow On Formula and pureed spoon feeds

Main Concern:Constipation

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Early childhood

Continues Concentrated Formula from bottle with small amounts of smooth foodse.g. Dairy Products/ bread/sloppy foodsNumerous attempts to replace with Paediatric Fibre Rich Feed

FAILED

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Age 6 . 5 years

Weight 16.2kgHeight 1.09m

Growth FalteringDiscuss possibility of Gastronomy

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Age 6.8years

Weight 15.9kg

Admission for placement of Skin Level Device

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Age 7.2 years

Weight 17.8kgHeight 1.15m

Age 7.6 yearsWeight 20.1kgAdmitted for IV Iron

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1 year post SLD insertion Weight 20.3kg Height 1.17m

Weight increase of 4kg since device fittedEstimated Intake:1800kCals(Feed and Oral) 88/kg60g Protein/day RNI Protein 28.5g/day

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AGE 8.5 years

Weight 20kg

Admitted Leaking ButtonIntroduce Oral Wound Healing Feed via

tube (500ml)in addition to Fibre Rich Enteral Feed

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FEED COMPARISONPer 100ml Wound healing

Feed (oral)Standard FibreFeed (enteral)

Protein g 10.0 4.0

Calories 125 100

Fibre g 0.3 1.5

Zinc mg 4.5 1.2

Arginine g 1.5

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Constipated

Change to 800ml Fibre Rich FeedWound Healing Feed 200ml bolus/day

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Age 9.1 years

Weight 20.2kgINTAKE800ml Fibre Rich Feed Providing 48g Protein 1200Kcals+Dairy products + soft foods

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CASE CONFERENCE

Deterioration over past 2 yearsGrowth FalteringDeep ulcerated infected wounds

‘Make a Wish ‘trip to USA No enteral feeds during holiday

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Age 9 .7 years

Admitted for reviewWeight 16.9kgButton Out alternate nightsTolerating 400 – 500ml Energy Dense Fibre Rich Feed/day

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Following Week

GJ tube fittedChange to Wound Specific Enteral Feed

However1 kCal/ml

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Age 9.7years

Tolerating 1000ml overnightMother reports better mood and skin improvement(? Subjective)

c/o Occasional leakage

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Age 9.8 years - AdmissionWeight 16.9kgBilateral pneumoniaSerum Albumin 15g/LSerum Sodium 130mmol/L

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GI Review

Advise increasing feed densitySuggest Fat Emulsion as medicine

FAT EMULSION50% fat 30ml = 135 kCals

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Tolerated well initiallyDiscontinued at home

Age 9.9 years ADMISSIONSerum Albumin 11g/LSerum Sodium 127mmol/L

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Age 10.1 years ADMISSIONPrescribed change of feed ratio

Energy dense Fibre Rich Feed/Wound Healing Feed

…where children’s health comes first

…where children’s health comes first

…where children’s health comes first

Enteral Feed ComparisonPer 100ml Standard

Multifibre EnergyMultifibre

Wound Healing

Protein g 4.0 6.0 5.5Calories 100 150 100Iron mg 1.6 1.6 1.6Fibre g 1.5 2.4 1.5Zinc mg 1.2 1.8 2.0Vit. C mg 10 15 38Arginine g 0.85

…where children’s health comes first

Age 10.3 years

ADMISSION

Severe pneumonia

RIP