Safety and Efficacy of Sodium Nitrite in Sickle Cell Disease

Inclusion Criteria:

Subjects meeting all of the following criteria will be considered for admission to the study:

1. Male or female subject aged between 8 and 23 years of age; only patients up to the age of 23 will be studied at Childrens Hospital Los Angeles
2. Electrophoretic diagnosis of sickle cell disease;
3. Sudden onset of acute pain involving >=1 sites typical of vaso-occlusive crisis (Vaso-occlusive crisis is defined as acute, severe pain in the extremities, chest, abdomen, or back that can not be explained by other complications of sickle cell disease or by a cause other than sickle cell disease.);
4. Severe pain requiring parenteral analgesics and hospitalization.
5. Informed consent obtained from a legal representative or from subjects 18 years of age or older before enrollment;
6. Being willing and able to be followed for at least 30 days for evaluation.

Exclusion Criteria:

Subjects presenting with any of the following will not be included in the study:

1. Clinically significant bleeding;
2. Current drug abuse or participation in methadone program;
3. Episode of pain requiring hospitalization within 2 weeks prior to current admission;
4. Other complications of sickle cell disease including cerebrovascular accident, pulmonary hypertension, or seizure;
5. Pregnancy (confirmed by a serum human chorionic gonadotropin pregnancy test) or breast feeding;
6. Blood pressure less than 25th percentile for the subject's age and sex on admission or at the time of screening;
7. Methemoglobinemia >3%;
8. Anemia with hemoglobin level less than 6 g/dL;
9. Red blood cell G6PD deficiency, by laboratory testing prior to enrollment;
10. History of allergy to nitrites or allergy to other substances characterized by dyspnea and cyanosis;
11. Treatment with allopurinol, a medication that could interfere with nitrite metabolism, within the past 30 days before screening;
12. Treatment with any investigational drug within the past 30 days;
13. Significant acute or chronic concomitant diseases (including renal, hepatic, cardiovascular, pulmonary, or oncologic disease, sepsis, pulmonary edema, pulmonary embolism) that would be inconsistent with survival for at least 6 months;
14. Any subject judged by the clinical investigator or study manager to be inappropriate for the study.