Intravenous Gammaglobulin for Sickle Cell Pain Crises

Patients will be randomized to a single dose of IVIG versus normal saline placebo during an
uncomplicated pain crisis. Length of VOC and other secondary endpoints will be monitored.

Inclusion Criteria:

- Documented diagnosis of sickle cell disease (SS or S-β thalassemia genotype)

- Age 12-65 years for Phase 1, 8-13.99 years for Phase 2

- Uncomplicated acute pain episode requiring hospital admission and parenteral narcotics

Exclusion Criteria:

- Increased stroke risk as assessed by transcranial Doppler or magnetic resonance
imaging (all subjects undergo testing)

- Concomitant acute process, including fever > 38.5° C with clinical suspicion of
infection

- Increased ALT > 2X ULN

- Serum creatinine ≥1.3 mg/dL, >300 mg/dL protein in spot urinalysis, or known condition
associated with renal dysfunction

- Hb > 10 g/dL and Hct > 30%

- Hb< 5 g/dl

- Known IgA deficiency or known allergy to gamma globulin

- Pregnancy or breastfeeding

- Vaccination with a live attenuated virus in the preceding 6 weeks

- Documented history of illicit (eg. heroin, cocaine) drug abuse or drug-seeking
behavior

- Current participation in another investigational drug study

- Current treatment with chronic transfusion

- Prior thromboses or current estrogen use