Case Study Dystrophic EB - Debra€¦ · …where children’s health comes first. 1 year post SLD...
Transcript of Case Study Dystrophic EB - Debra€¦ · …where children’s health comes first. 1 year post SLD...
…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
…where children’s health comes first
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