1. MORNING REPORT JULY 23, 2012 Good Morning

2. 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

3. Semantic Qualifiers Symptoms Acute /subacuteChronic LocalizedDiffuse SingleMultiple StaticProgressive ConstantIntermittent Single EpisodeRecurrent AbruptGradual SevereMild PainfulNonpainful BiliousNonbilious Sharp/StabbingDull/Vague Problem Characteristics Ill-appearing/ Toxic Well-appearing/ Non-toxic Localized problemSystemic problem AcquiredCongenital New problem Recurrence of old problem

4. CXR 1: LUL consolidation

5. CXR 2: Worsening of the LUL consolidation with development of a small pleural effusion

6. 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

7. 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

8. 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

9. 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

10. 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

11. Acute Chest Syndrome  2 nd leading cause of admissions after VOC**  More common in children but more severe in adults

12. 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)

13. 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

14. Acute Chest Syndrome Treatment  Simple transfusion  Goal Hgb close to 10g/dL  EARLY!!  Exchange transfusion  Progressive illness despite treatment  Significant hypoxia  Multi-lobe infiltrates

15. 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

16. NOON CONFERENCE: HEME/ONC EMERGENCIES DR. VELEZ Thanks for your attention