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 pts 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 / 56 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 pts 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 pts 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 addl 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?