MORNING REPORT JULY 23, 2012

Good Morning

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 ChronicLocalized Diffuse

Single MultipleStatic Progressive

Constant IntermittentSingle Episode Recurrent

Abrupt GradualSevere MildPainful NonpainfulBilious 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 recessiveChromosome 11

Glutamine ValinePolymerization of

HgbS on de-oxygenation

Crescent shaped RBCsVascular occlusionOrgan ischemiaEnd-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

monthsPCV13 at 2, 4, 6moPCV23 at 2 & 5yo

ACS Predisposing Conditions

Peak age 2-4 yearsWinter monthsRecurrence higher if first episode of ACS is

before the age of 3yoOpioid 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 commonSOB, wheeze, hemoptysis, chillsHypoxia and respiratory distressNew infiltrate on CXR

Upper lobe more common in children Can be multi-lobar Associated pleural effusion

Hgb decreased from baselineLeukocytosis+ blood cultures and/or sputum or BAL cultures

Acute Chest Syndrome

2nd leading cause of admissions after VOC**More common in children but more severe in adults

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

NOON CONFERENCE: HEME/ONC EMERGENCIES

DR. VELEZ

Thanks for your attention