Dexamethasone to Treat Acute Chest Syndrome in People With Sickle Cell Disease
Condition: Anemia, Sickle Cell
MeSH terms: Acute Chest Syndrome; Anemia, Sickle Cell
Intervention: Dexamethasone; Placebo
Intervention description: Individuals meeting entry criteria will be randomized to receive dexamethasone 0.3 mg/kg (12 mg maximum single dose). The study drug will be given by mouth every 12 hours until discharge from the hospital or for a maximum of 4 doses (2 days), whichever occurs first. Thereafter, study drug will be tapered over 6 days for a total duration of therapy not to exceed 8 days.; Individuals meeting entry criteria will be randomized to receive 0.3 mg/kg (12 mg maximum single dose) of placebo. The study drug will be given by mouth every 12 hours until discharge from the hospital or for a maximum of 4 doses (2 days), whichever occurs first. Thereafter, study drug will be tapered over 6 days for a total duration of therapy not to exceed 8 days.
Intervention MeSH terms: Dexamethasone
Phase: Phase 3
Study type: Interventional
Primary purpose: Treatment
Allocation: Randomized
Intervention model: Parallel Assignment
Masking: Quadruple (Participant, Care Provider, Investigator, Outcomes Assessor)
Enrollment: 12.0
Sex: All
Minimum age: 5 Years
Healthy volunteers: No
Keywords: Sickle Cell Disease; ACS; Acute Chest Syndrome; Hgb SS; Hgb Sβ0; Dexamethasone

Summary:

      People with sickle cell disease (SCD) may develop acute chest syndrome (ACS), which is a
      common and serious lung condition that usually requires hospitalization. Dexamethasone is a
      medication that may decrease hospitalization time for people with ACS, but it may also bring
      about new sickle cell pain. This study will evaluate the effectiveness of a dexamethasone
      regimen that includes a gradual dose reduction at decreasing hospitalization and recovery
      time in people with SCD and ACS.
    

Eligibility criteria:

        Inclusion Criteria:

          -  Diagnosis of sickle cell anemia (Hgb SS) or sickle-β0-thalassemia (Hgb Sβ0)

          -  Current episode of ACS, defined as a new lobar or segmental pulmonary infiltrate seen
             on a chest radiograph and two or more of the following findings:

               1. Temperature of 38.5°C or higher

               2. Tachypnea (i.e., rapid breathing)

               3. Dyspnea or increased work of breathing

               4. Chest wall pain

               5. Oxygen saturation of less than 90% in room air by pulse oximetry

          -  Current episode of ACS diagnosed in the 24 hours prior to study entry

          -  Ability to take medication in capsule form

        Exclusion Criteria:

          -  Prior participation in this study

          -  Diagnosed with any medical condition that will likely be worsened by corticosteroid
             therapy, including any of the following conditions:

               1. Diabetes mellitus

               2. High blood pressure

               3. Esophageal or gastrointestinal ulceration or bleeding

               4. Known avascular necrosis

          -  Diagnosis of ACS in the 6 months prior to study entry

          -  Treatment with oral or parenteral corticosteroid therapy for any reason in the 14 days
             prior to study entry

          -  Use of inhaled corticosteroids or systemic corticosteroids for respiratory illness in
             the 3 months prior to study entry

          -  Long-term lung condition that requires treatment with corticosteroids

          -  Participation in a program of chronic transfusions that ended fewer than 4 months ago.
             A program of chronic transfusions includes a regimen of serial simple or exchange
             transfusions given at least every 6 weeks for at least three consecutive transfusions
             for the prevention of SCD-related complications.

          -  Pregnant

          -  Treatment with any investigational drug in the 90 days prior to study entry

          -  History of either tuberculosis or a positive skin test for tuberculosis

          -  Known HIV infection or a current systemic fungal infection