Good Morning 
MORNING REPORT
JULY 23, 2012
Illness Script
 Predisposing Conditions
 Age, gender, preceding events (trauma, viral illness, etc),
medication use, past medical history (diagnoses,
surgeries, etc)
 Pathophysiological Insult
 What is physically happening in the body, organisms
involved, etc.
 Clinical Manifestations
 Signs and symptoms
 Labs and imaging
Semantic Qualifiers
Symptoms
Acute /subacute
Chronic
Localized
Diffuse
Single
Multiple
Static
Progressive
Constant
Intermittent
Single Episode
Recurrent
Abrupt
Gradual
Severe
Mild
Painful
Nonpainful
Bilious
Nonbilious
Sharp/Stabbing
Dull/Vague
Problem Characteristics
Ill-appearing/
Toxic
Well-appearing/
Non-toxic
Localized problem
Systemic problem
Acquired
Congenital
New problem
Recurrence of old
problem
CXR 1:
LUL
consolidation
CXR 2:
Worsening of
the LUL
consolidation
with
development of
a small pleural
effusion
What Happens in SCD?**
 Autosomal recessive
 Chromosome 11
 Glutamine  Valine
 Polymerization of HgbS
on de-oxygenation
 Crescent shaped RBCs
 Vascular occlusion
 Organ ischemia
 End-organ damage
Early Diagnosis**
 Can be detected at birth
on the NBS
 Early detection = better
outcome
 Decreased
bacteremia/sepsis (by
84%)
 PenVK started by 3 months
 PCV13 at 2, 4, 6mo
 PCV23 at 2 & 5yo
ACS Predisposing Conditions
 Peak age 2-4 years
 Winter months
 Recurrence higher if first episode of ACS is before
the age of 3yo
 Opioid usage (PO > IV) with preceding VOC
Decreased inspiratory effort 
 Areas of atelectasis 
 Predisposition to development of ACS

 Bacteremia (in young children)
 Over-hydration during another illness
ACS Pathophysiology
 Infectious (at least 30% associated with + sputum
or BAL cultures)
Strep pneumo (most common in younger children)
 Mycoplasma, chlamydia
 Staph aureus, Hib, Salmonella, Enterobacter

 Fat embolus to the lungs
 Arises from micro-infarction to the bone marrow
 If large, can be life threatening
 Other vascular occlusions from the sickling process
 COMBINATION of ABOVE
ACS Clinical Manifestations
 Fever, cough, chest pain = most common
 SOB, wheeze, hemoptysis, chills
 Hypoxia and respiratory distress
 New infiltrate on CXR
 Upper lobe more common in children
 Can be multi-lobar
 Associated pleural effusion
 Hgb decreased from baseline
 Leukocytosis
 + blood cultures and/or sputum or BAL cultures
2nd leading
cause of
admissions
after VOC**
More
common in
children but
more severe
in adults
Acute Chest Syndrome
Acute Chest Syndrome
 Definition
 The
radiologic appearance of new pulmonary
infiltrate involving at least one complete lung
segment plus one of the following
 Fever
>38.5
 Hypoxia
 Chest pain
 Signs of respiratory distress (tachypnea, wheezing,
cough, retractions)
Acute Chest Syndrome
 Treatment
 Broad spectrum antibiotics
 Cephalosporin
(Rocephin)
 Macrolide (Azithromycin)
 +/- Vancomycin (often used here at CHNOLA)
 Hydration
(2/3 to 3/4 MIVF)
 Oxygen (goal sats >92%)
 Incentive spirometry and CPT
 Bronchodilators +/- steroids
 If
patient has a history of asthma
 Pain
control
Acute Chest Syndrome
 Treatment
 Simple
transfusion
 Goal
Hgb close to 10g/dL
 EARLY!!
 Exchange
transfusion
 Progressive
illness despite
treatment
 Significant hypoxia
 Multi-lobe infiltrates
Acute Chest Syndrome
 Importance
 About
50% of SCD patients experience at least 1
episode of ACS
 Significant
morbiditiy and mortality
 Multiple ACS episodes may lead to
 Chronic,
restrictive pulmonary disease
 Pulmonary HTN
 Children
with recurrent episodes should be evaluated
with PFTs by a pediatric pulmonologist
Thanks for your attention 
NOON CONFERENCE:
HEME/ONC EMERGENCIES
DR. VELEZ