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Apr i l 21

s t
, 2014 ! Lar ys s a Gr gur i c
Introduction
• Immune response to
drugs:
– 100+ meds
– Sulfonamide abx,
NSAIDS

• Steven Johnson
Syndrome - <10%
TSBA
• TENS - >30% TSBA
• TENS/SJS Overlap –
15-30% TSBA
Source: Telegraph.co.uk
Mortality & Prevalence
• Mortality
– 7.5% - children
– 20-25% - adults
– Early interventions ! survival
rate – 90%
• Delayed intervention !
mortality – 30-51%
– ! mortality compared to
thermal burns – related to
inflammatory disease process
• Prevalence – Rare
– 1.0-1.3 cases per 1 million –
TENS
– 2.9-6.1 cases per 1 million –
SJS
– Higher incidence in HIV/
AIDs – 1000x
Blistering of TENS
Pathophysiology & Diagnosis
• Keratinocyte apoptosis
– Infiltration of MAC
– Mechanism?
• T-Cell Mediated
response? NK-Cell?
• Fas cell-surface receptor?
• Drug directly toxic to
cells?
• Identified by histology
– Necrosis of epithelium
– Staphylococcal scaled
skin syndrome – abx
– No universal accepted
model to dx TENS
H&E Stain, Skin Biopsy
SC
Dermis
Epidermis
Signs & Symptoms
• Sx: Fever, rash, blisters,
sloughing off of skin
• Can include ulceration of
mucosal tissues
– Oral, pharynx, ocular,
genitalia
– ! time to heal (1-3 weeks+)
– Pt. becomes unable or
unwilling to eat/drink due to
oral mucosal involvement
• Can progress to sepsis and
multi-organ failure
Mucosal Involvement of TENS
Treatment
• Aseptic technique –transfer to Burn Center
• Withdrawal of offending drug
– May have been taken 3-4 weeks prior, but usually
identifiable
• Supportive Tx: Pain management, nutritional
support – fluid/electrolyte balance, isolation,
wound dressings: e.g. ActiCoat (silver; anti-
microbial)
• No consensus on further Tx – not enough
incidence for randomized controlled studies to
be performed; case study observations
– Glucocorticoid steroids not used anymore
Nutritional Implications
• ! needs due to hypermetabolic response; catabolism of
macronutrients " wt loss
– Negative N Balance can remain ~2 weeks after initial
response
• Fluid needs: cannot use std calculations
– Wounds do not exude @ same rate as thermal burn
• Peds pt?
– ! basal metabolic rate, " endogenous energy reserve, growth/
development period
• Odynophagia (painful swallowing) / Dysphagia
– Thickened liquids/pureed consistency tolerated by most;
progression to regular diet: ~57 days
– Smaller utensils / syringes
• EN Feeding tube can cause perforations, bowel necrosis,
PN has high risk of sepsis – varies case by case
• Gout – allopurinol can trigger: low purine diet
Presentation of Case
Case Study Patient
# Miss S.L.

# 16 y/o ♀
# African American.
# Attends high school; no part-time job.
# Lives with mother in single-family house. No
brothers or sisters.
# Uses marijuana recreationally; no cigarettes.
Timeline
02/02/14
• Symptoms began with oral lesions which then spread to face/body
• Went to ED @ NSLIJ
• Per pt’s mother pt. was kept NPO, received IVF
02/06/14
• Transfer to NUMC Burn Center
• TBSA 80-90%; considered 2
nd
degree burns
• Initiating drug thought to be Motrin (?)
• Presented with intact blisters, tachycardia, fever.
• Fluid/electrolyte management started.
02/07/14
• Placement of NJT using Cortrak Device to initiate EN
• Systemic Inflammatory Response Syndrome (SIRS)
• Initial Nutritional Assessment
Initial – 02/07/14
$ Dx: s/p TENS episode involving 80-90% TSBA
$ Past Medical/Surgical History:
# Not significant
$ Diet History:
# Not available at this time; pt. had difficulty talking
due to oral mucosa involvement
A N T H R O P O M E T R I C S
Ht 167.6 cm / 5’6” UBW 79.5 kg / 177 lbs
Wt 82.1 kg / 181 lbs. %UBW 103%
BMI 29.4; overweight IBW 130 lbs ±10%

%IBW 139%
Wt for
Age
97
th
Percentile Ht/Length
for Age
Between 75
th
and 90
th

Percentiles
Initial: Meds / lab values
• 02/06/14 " 02/07/14
• Medications should be limited TENS RXN
– Reglan as part of PEP uP



Alb Na K Cl Glu BUN Creat Ca Hgb Hct MCV
2.0" 136
WNL
4.1
WNL
102 94
WNL
6" 0.6
WNL
7.9
WNL
9.7" 29.2" 70.8"
Pre-Alb Alb
2.9" 2.0"
F
e
b
-
0
6
-
1
4

F
e
b
-
0
7
-
1
4

Initial Diet rx
Vital AF 1.2, 1600 mL Daily PEP uP
with IVF D5W @ 40 ml/hour (48 kcal/24 hr)

Total Kcals: 1970
Total Pro: 120 g
PEP up protocol
The Enhanced Protein-Energy Provision via the
Enteral Route in Critically Ill Patients
• Developed by Dr. Daren Heyland and colleagues
• 24 hour volume based EN protocol
– Designed to increase protein and calorie delivery to help
improve clinical outcomes in ICU
• Interdisciplinary Protocol:
– MD orders based on criteria outlined in protocol
• Initial volume determined by pt’s admission weight or trophic feeds
initiated (15 mL/hour).
• Contraindications: bowel perforation or obstruction, proximal high
output fistula, active GI bleed or DKA " otherwise pt. must be fed!
– RN implements – adjusts run rate to assure full EN dose is
provided over 24 hours
– RD assesses the patient and adjusts formula/volume
CORTRAK DEVICE
Enteral Access System
• Allows placement of FT without x-ray confirmation
• Used primarily for NJT @ NUMC
Jejunal Placement
AMT BRIDLE DEVICE
Reduces incidence of feeding tube removal by pulling
Initial assessment continued
$ Energy Needs:
# RDA for pediatric pt. aged 15-18, 40-45 kcal/kg:
= 2560-2880 kcal/day
# Burned pt.: can use Curreri Formula
# (25 kcal ! kg usual body wt.) + (40 ! TBSA%)
= 2086 kcal/d
Decided to go with higher estimation considering age
$ Protein Needs:
# 120:1 N to 100:1 N ratio
o 148-178 g PRO/d (~1.8-2.2 g/kg)
o Indicated for catabolic patient, >50% “burn” involvement
" High Nutritional Complexity
$ Recommended ! TF to
Pivot 1.5 1900 ml daily
PEPuP
• Pivot 1.5:
# Higher protein content (25%);
peptide based
# Indicated for critically ill,
hyper metabolic patient
# Enrichment of ! 3 fatty acids
indicated for immune support
and anti-inflammatory
properties
% 2850 kcal
% 168.8 g PRO
o Would successfully
meet pt’s needs
• Supplements recommended:
– Cerovite (liq) vit+min: 15 ml/day
– For Wound Healing:
• Vitamin C (liq) 500 mg/day
• Zinc SO
4
220 mg Q 48 hours
– Vitamin D 1000 units/day
– FeSO
4
(liq) 300 mg q 12 hours (Fe def anemia)

*Chose liquid formulations to prevent TF clog
Timeline Continued
02/08/14
• Seen by OB-GYN Service for Vaginal Involvement
• Bladder CT Scan
• Acticoat Silver applied to affected areas on back
02/09/14
• Prognosis improving – only 2 quarter sized areas have
sloughed off
• Dx with reactive tachycardia " no tx for this age group
2/10/14
• Status: Improved
• Foley removed
• Blisters remaining intact
• Nutritional Follow Up – seen by RD
Follow Up: 2/10/14
• Pt. remains on Pivot 1.5 1900 mL/day with 200
ml free H
2
0 q 6 h
– TF Study: 5581 ml; average of 1860 ml/day: ~98%
delivery
• PO intake: sips of cranberry juice and Ensure
• Continued to remain at high nutritional
complexity

Labs 02/10/14:
14.05!
9.5"
28.9"
541
WNL
131" 96"

17
WNL
4.8
WNL
27
WNL
0.4
WNL
113!
MCV: 70.4"
Mg: 1.8 WNL
Phos: 4.1 WNL
Timeline continued
2/11/14
• Per MD Progress Note:
• No Acute Issues – “pt looks better”: blisters thick and stable
without weeping
• Beginning to tolerate PO feeds – mucosal surfaces improving
• Plan: Commence PT/OT, advance diet, continue wound care
2/13/14
• Nutrition Follow Up
• Pt. up and walking around unit with PT
2/14/14
• Feeds held overnight to encourage PO intake during the day
Follow Up: 2/13/14
• Subjective: “I would eat more if you took this tube out of my nose.”
24 Hour Recall of PO Intake:
Breakfast – 2/12/14:
4 oz. hot cereal
" of large roll
8 oz. cranberry juice
6 oz. Ensure Plus shake
(bottle is 8 oz.)
Lunch – 2/12/14:
“Late Lunch” brought into
Burn Center by mom (White
Castle)
2 slider cheeseburgers with
everything on them
~8 onion rings
16 oz. Pepsi
No Dinner on 2/12/14
Breakfast – 2/13/14
1 egg
6 oz. Ensure Plus Shake

Calorie Breakdown
(30.5 g pro)
Follow Up Continued
• Explained to pt. the need for the NJT –discussed with MD about
initiation of nocturnal feedings
– Pt. understood rationale and was happy feeds would be held
• Meds: Magic Mouth Wash containing abx
• Updated Diet Rx: Regular Diet with Pivot 1900 mL Daily PEP uP
– TF Study: Pt. received an average of 1166 mL/day: 67% of assessed
calorie needs, 81% protein needs
• Although volume goal was not met, with PO intake based on 24-
hour recall pt. was likely meeting estimated needs.




15.49!
9.3"
28.8"
533
WNL
132" 97"
18
WNL
4.3
WNL
29
WNL
0.4
WNL
137!
MCV: 69.8"
Mg: 1.9 WNL
Labs 02/13/14:
Follow Up Continued
• Care Plan Recommendations:
– 1. " TF to Pivot 1.5 @ 85 mL/hr from 2000 hrs "
0600 hours
• (850 mL total: providing 1275 kcals, 80 g PRO)
– 2. " Regular Diet to Pediatric Diet to better meet pt.
preferences
– 3. Continue Supplements as Ordered (Ensure Plus
TID)

• Goal:
– PO #75% meals served

• High Nutritional Complexity

SERUM LAB VALUES INTERPRETED
DATE LAB VALUES & INTERPRETATION
02/06/14 Pre-Alb: " acute catabolic state, stress
Alb: " with burns/trauma
02/07/14 "
02/13/14
WBC: ! trauma/tissue injury
H&H, MCV: " Fe def anemia
Na: " burns, hyperglycemia
Cl: " fever
Glu: !physical inactivity, infection, stress
Timeline continued
On 2/14/14:
Pt. discharged to home
with excellent prognosis!
Class discussion
Nutrition Care Process
Assessment
Diagnosis
Intervention
Monitoring
& Evaluation
Nutrition Diagnosis
• Self feeding difficulty (NB-2.6) related to
physiological difficulty caused by swollen mucosa
causing inability to close lips, swallow or chew foods
evidenced by mucosal membranes affected by TENS
and associated dysphagia.

- and -

• Increased protein and calorie needs (NI-5.1) related
to increased demand for nutrients evidenced by
albumin of 2.0 and pre-albumin of 2.9 and 90%
TSBA involvement of TENS inducing a
hypermetabolic response.
Goals & Interventions
Goals Interventions/Monitoring
1. Maintain current wt of
177# (±1-2#)
# Initiate enteral feedings with Pivot 1.5 1900 ml
daily PEPuP to meet assessed needs: 2560-2880
kcal & 148-178 g protein daily

# Once PO is re-initiated, encourage intake of
supplemental nutrition in the form of Ensure, other
palatable menu items e.g. pudding, yogurt

Monitoring:
# Q 48 Hours – collect pump data and assess delivery
rate

# Weekly Weights

Goals Intervention
2. Intake #75% of meals served when
PO diet initiated
# Initiate PO diet as tolerated. Provide
pt with prefs, encouragement of
intake. Continued supplementation
with Ensure Plus.
# Monitor trays to assess intake
3. Increase H&H, MCV to WNL # Provide Fe supplementation: FeSO
4

(liq) 300 mg q 12 hours

# Monitor A.M. labs for improvement
Goals & Interventions
Journal Article
Title Management of Dysphagia in Toxic Epidermal Necrolysis and
Steven-Johnson Syndrome
Introduction • Mucosal involvement of TENS is common which can lead to
extreme pain while swallowing " poor PO intake
• Inflammation & severe ulceration of conjunctiva, oral cavity,
pharynx, nasal cavity, esophagus and genitalia
• Causes odynophagia/dysphagia
• Aim: to describe swallow function in TEN/SJS
Methods • Burns Unit Database Reviewed (between 1999-2004) to obtain
medical records of 14 patients, 6 male & 8 female: 8 TENS, 2
TENS/SJS overlap, 4 SJS; confirmed by biopsy
• Most had involvement causing odynophagia, poor oral
intake, ability to tolerate liquids more easily than solids and
an increased aspiration risk
Results • 12 out of 14 (85.7%) had mucosal involvement
• 12 of 13 pts (92.3%) on oral diet suffered odynophagia and poor
PO intake
• 11 pts (84.6%) tolerated fluids better than solids
Journal Article
Title Management of Dysphagia in Toxic Epidermal Necrolysis and
Steven-Johnson Syndrome
Results
Continued
• 1 pt (not on oral diet) had increased ICU stay & tracheostomy;
required mechanical ventilation still after TENS had resolved
• 1 death due to severe sepsis
• 6 pts referred to SLP to assess swallowing:
• 6 pts (16.7%) presented with oral dysphagia only
• 83.3% presented with oral and pharyngeal dysphagia
• Dysphagic Features: Poor mouth opening and lip seal
(66.7%), poor bolus control (66.7%) and impaired oral
clearance (66.7%)
• Pharyngeal Features: delayed swallow initiation
(83.3%), reduced hyolaryngeal excursion (83.3%) and
laryngeal penetration or aspiration of thin fluids
(66.7%)
• Pharyngeal phase dysphagic features were apparent
in those intubated earlier in admission
• Patients were commenced on thickened fluids, puree consistency
most frequently described – 78.6% required diet/fluid
modification in early stages of feeding
Journal Article
Title Management of Dysphagia in Toxic Epidermal Necrolysis and
Steven-Johnson Syndrome
Results
Continued
• EN feeding required in 8 pts (57%); 7 were TENS, 1 was SJS
• Required due to poor intake/dehydration
• Complications: difficult insertion, refusal (pain), tube
removal by pt
• 90% of pts tolerated full diet and thin fluids by discharge
Discussion • Optimal levels of nutritional intake via PO may be difficult to
achieve & maintain
• Sloughing of oral, nasal and pharyngeal mucosa may potentially
affect dynamics of swallow mechanism
• Food/fluids at “ambient” temperature was better tolerated
• Pts avoid foods with high acidity, abrasiveness – preferring
smooth/moist foods
• Mean duration until return to full diet without restriction was 57
days
Conclusions • Insufficient PO intake and increased metabolic needs indicate
need for EN
• Tube insertion may be painful and require add’l pain relief
Clayton, N.A., & Kennedy, P.J. (2007). Management of dysphagia in
toxic epidermal necrolysis (TEN) and Steven-Johnson syndrome
(SJS). Dysphagia, 22, 187-192.

Questions?