Defibrotide in Sickle Cell Disease-Related Acute Chest Syndrome

Inclusion Criteria: * SCD-associated ACS with the presence of any two or more of the following signs not explained by other etiologies: Fever, Chest pain, Cough, Dyspnea, Tachypnea for age, Pulmonary infiltrate on CXR and/or Chest CT scan, Decreased O2 saturation with or without oxygen supplement; * Age 2 to 40 years of age; * Homozygous Hemoglobin S Disease, Hemoglobin SC Disease or Hemoglobin S 0/+ thalassemia; * Informed consent/assent; * Consent of patient/parent within ≤72 hours after inpatient admission for SCD-associated ACS. * Females of childbearing age will have a negative pregnancy test. Exclusion Criteria: * Current Grade III or IV hemorrhage; * Previous hypersensitivity reaction to defibrotide; * Current systemic anti-coagulant therapy and/or fibrinolytic therapy; * Consent of patient/parent greater than 72 hours of inpatient admission for SCD-associated ACS; * No signed informed consent