Leading cause of death for patients with sickle cell disease!

Etiology

●Vaso-occlusion within the pulmonary microvasculature and can be from triggers for vaso-occlusion (eg: infection, asthma, hypoventilation) or consequence of vaso-occlusion (from bone marrow and fat embolism)

Diagnosis
New radiographic consolidation AND at least one of the following
Temperature ≥38.5°C
●>2 percent decrease in SpO₂ (O₂ saturation) from steady state at RA
●PaO2 <60 mmHg
●Tachypnea (per age-adjusted normal)
●Intercostal retractions, nasal flaring, or use of accessory muscles of respiration
●Chest pain
●Cough
●Wheezing
●Râles

DDX

●Acute Coronary Syndrome vs. Pneumonia vs. PE most common differential diagnoses.

*Note that PE can be in-situ thrombosis OR thrombo-embolic from lower extremities.

Treatment

●Fluid management (avoid over-resuscitation, use hypotonic fluids)
●Pain control
●Antibiotics to cover atypical and CAP (usually cefotaxime/azithromycin or CTX/azithromycin)
●VTE prophylaxis (high risk for VTE!)
●Supplemental O2 and incentive spirometry (to prevent atelectasis)
●If refractory, simple transfusion to increase hemoglobin to 10 or exchange transfusion for severe cases to achieve Hemoglobin S <30 % and goal hemoglobin of 10

Prevention of ACS
●Hydroxyurea (not for acute episodes)
●Chronic transfusion therapy (only if refractory to above)