Intravenous Gammaglobulin for Sickle Cell Pain Crises
Intravenous Gammaglobulin for Sickle Cell Pain Crises
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)
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