8/18/2016 AM Report – Acute Chest Syndrome

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)

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