Overview
The purpose of this study is to determine whether intravenous immune globulin is safe and effective in the acute treatment of pain crises in sickle cell disease.
Funding Source: Food and Drug Administration (FDA), Office of Orphan Products Development (OOPD)
Description
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.
Eligibility
Inclusion Criteria:
- Documented diagnosis of sickle cell disease (SS or S-β thalassemia genotype)
- Age 12-65 years for Phase 1 (Completed), 6-13.99 years for Phase 2 (Ongoing)
- 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