Safety and Efficacy of Sodium Nitrite in Sickle Cell Disease

NCT ID: NCT01033227

Last Updated: 2017-10-17

Study Results

Results available

Outcome measurements, participant flow, baseline characteristics, and adverse events have been published for this study.

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Basic Information

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Recruitment Status

TERMINATED

Clinical Phase

PHASE1/PHASE2

Total Enrollment

5 participants

Study Classification

INTERVENTIONAL

Study Start Date

2009-12-31

Study Completion Date

2012-06-30

Brief Summary

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This study will determine if administration of sodium nitrite is safe and can improve small vessel blood flow and tissue oxygenation when given as an additional treatment in patients with acute vaso-occlusive crisis (pain crisis) associated with sickle cell disease.

Detailed Description

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Nitric oxide (NO) is a naturally occuring chemical that relaxes blood vessels and helps improve blood flow.

The pain associated with vaso-occlusive crisis (pain crisis) in sickle cell disease is caused in part by lack of oxygen and increased tissue acid because blood flow is blocked by stiff sickle red cells. Administration of sodium nitrite should generate nitric oxide in this area of hypoxia and acidosis and improve blood flow.

Conditions

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Sickle Cell Disease

Keywords

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sickle cell disease sickle cell anemia pain crisis nitric oxide nitrite vaso occlusive crisis pain medication sickled cells blood flow microcirculation

Study Design

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Allocation Method

RANDOMIZED

Intervention Model

PARALLEL

Primary Study Purpose

TREATMENT

Blinding Strategy

NONE

Study Groups

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No drug

No study drug administered

Group Type NO_INTERVENTION

No interventions assigned to this group

Sodium nitrite injection, USP

Administration if sodium nitrite injection, USP

Group Type EXPERIMENTAL

sodium nitrite injection, usp

Intervention Type DRUG

Sodium nitrite injection, USP will be administered in blocks of six subjects (3 sodium nitrite and 3 no drug). A total of five dose levels are planned, pending safety starting. Drug will be given by continuous infusion infusion for 48 hours starting at 6 nmol/min/kg (10% of the maximal tolerated dose).

Interventions

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sodium nitrite injection, usp

Sodium nitrite injection, USP will be administered in blocks of six subjects (3 sodium nitrite and 3 no drug). A total of five dose levels are planned, pending safety starting. Drug will be given by continuous infusion infusion for 48 hours starting at 6 nmol/min/kg (10% of the maximal tolerated dose).

Intervention Type DRUG

Other Intervention Names

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sodium nitrite

Eligibility Criteria

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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.
Minimum Eligible Age

8 Years

Maximum Eligible Age

23 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Hope Pharmaceuticals

INDUSTRY

Sponsor Role collaborator

Children's Hospital Los Angeles

OTHER

Sponsor Role lead

Responsible Party

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Thomas Coates

Hematology Division Head

Responsibility Role PRINCIPAL_INVESTIGATOR

Principal Investigators

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Thomas Coates, MD

Role: PRINCIPAL_INVESTIGATOR

Children's Hospital Los Angeles

Locations

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Childrens Hospital Los Angeles

Los Angeles, California, United States

Site Status

Countries

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United States

References

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Wood KC, Hsu LL, Gladwin MT. Sickle cell disease vasculopathy: a state of nitric oxide resistance. Free Radic Biol Med. 2008 Apr 15;44(8):1506-28. doi: 10.1016/j.freeradbiomed.2008.01.008. Epub 2008 Jan 26.

Reference Type BACKGROUND
PMID: 18261470 (View on PubMed)

Mack AK, McGowan Ii VR, Tremonti CK, Ackah D, Barnett C, Machado RF, Gladwin MT, Kato GJ. Sodium nitrite promotes regional blood flow in patients with sickle cell disease: a phase I/II study. Br J Haematol. 2008 Sep;142(6):971-8. doi: 10.1111/j.1365-2141.2008.07259.x. Epub 2008 Jul 11.

Reference Type BACKGROUND
PMID: 18671702 (View on PubMed)

Lundberg JO, Weitzberg E. Nitrite reduction to nitric oxide in the vasculature. Am J Physiol Heart Circ Physiol. 2008 Aug;295(2):H477-8. doi: 10.1152/ajpheart.00611.2008. Epub 2008 Jun 27. No abstract available.

Reference Type BACKGROUND
PMID: 18586886 (View on PubMed)

Hachiya T, Blaber AP, Saito M. Near-infrared spectroscopy provides an index of blood flow and vasoconstriction in calf skeletal muscle during lower body negative pressure. Acta Physiol (Oxf). 2008 Jun;193(2):117-27. doi: 10.1111/j.1748-1716.2007.01827.x. Epub 2007 Dec 19.

Reference Type BACKGROUND
PMID: 18162057 (View on PubMed)

Cappellini MD, Fiorelli G. Glucose-6-phosphate dehydrogenase deficiency. Lancet. 2008 Jan 5;371(9606):64-74. doi: 10.1016/S0140-6736(08)60073-2.

Reference Type BACKGROUND
PMID: 18177777 (View on PubMed)

Villagra J, Shiva S, Hunter LA, Machado RF, Gladwin MT, Kato GJ. Platelet activation in patients with sickle disease, hemolysis-associated pulmonary hypertension, and nitric oxide scavenging by cell-free hemoglobin. Blood. 2007 Sep 15;110(6):2166-72. doi: 10.1182/blood-2006-12-061697. Epub 2007 May 29.

Reference Type BACKGROUND
PMID: 17536019 (View on PubMed)

Vanin AF, Bevers LM, Slama-Schwok A, van Faassen EE. Nitric oxide synthase reduces nitrite to NO under anoxia. Cell Mol Life Sci. 2007 Jan;64(1):96-103. doi: 10.1007/s00018-006-6374-2.

Reference Type BACKGROUND
PMID: 17160351 (View on PubMed)

Kato GJ, McGowan V, Machado RF, Little JA, Taylor J 6th, Morris CR, Nichols JS, Wang X, Poljakovic M, Morris SM Jr, Gladwin MT. Lactate dehydrogenase as a biomarker of hemolysis-associated nitric oxide resistance, priapism, leg ulceration, pulmonary hypertension, and death in patients with sickle cell disease. Blood. 2006 Mar 15;107(6):2279-85. doi: 10.1182/blood-2005-06-2373. Epub 2005 Nov 15.

Reference Type BACKGROUND
PMID: 16291595 (View on PubMed)

Ferreira LF, Harper AJ, Townsend DK, Lutjemeier BJ, Barstow TJ. Kinetics of estimated human muscle capillary blood flow during recovery from exercise. Exp Physiol. 2005 Sep;90(5):715-26. doi: 10.1113/expphysiol.2005.030189. Epub 2005 May 20.

Reference Type BACKGROUND
PMID: 15908509 (View on PubMed)

Raj A, Bertolone SJ, Mangold S, Edmonds HL Jr. Assessment of cerebral tissue oxygenation in patients with sickle cell disease: effect of transfusion therapy. J Pediatr Hematol Oncol. 2004 May;26(5):279-83. doi: 10.1097/00043426-200405000-00004.

Reference Type BACKGROUND
PMID: 15111778 (View on PubMed)

Gladwin MT, Crawford JH, Patel RP. The biochemistry of nitric oxide, nitrite, and hemoglobin: role in blood flow regulation. Free Radic Biol Med. 2004 Mar 15;36(6):707-17. doi: 10.1016/j.freeradbiomed.2003.11.032.

Reference Type BACKGROUND
PMID: 14990351 (View on PubMed)

Gladwin MT, Lancaster JR Jr, Freeman BA, Schechter AN. Nitric oxide's reactions with hemoglobin: a view through the SNO-storm. Nat Med. 2003 May;9(5):496-500. doi: 10.1038/nm0503-496. No abstract available.

Reference Type BACKGROUND
PMID: 12724752 (View on PubMed)

Cosby K, Partovi KS, Crawford JH, Patel RP, Reiter CD, Martyr S, Yang BK, Waclawiw MA, Zalos G, Xu X, Huang KT, Shields H, Kim-Shapiro DB, Schechter AN, Cannon RO 3rd, Gladwin MT. Nitrite reduction to nitric oxide by deoxyhemoglobin vasodilates the human circulation. Nat Med. 2003 Dec;9(12):1498-505. doi: 10.1038/nm954. Epub 2003 Nov 2.

Reference Type BACKGROUND
PMID: 14595407 (View on PubMed)

Gladwin MT, Schechter AN, Shelhamer JH, Ognibene FP. The acute chest syndrome in sickle cell disease. Possible role of nitric oxide in its pathophysiology and treatment. Am J Respir Crit Care Med. 1999 May;159(5 Pt 1):1368-76. doi: 10.1164/ajrccm.159.5.9810094. No abstract available.

Reference Type BACKGROUND
PMID: 10228097 (View on PubMed)

Lundberg JO, Gladwin MT, Ahluwalia A, Benjamin N, Bryan NS, Butler A, Cabrales P, Fago A, Feelisch M, Ford PC, Freeman BA, Frenneaux M, Friedman J, Kelm M, Kevil CG, Kim-Shapiro DB, Kozlov AV, Lancaster JR Jr, Lefer DJ, McColl K, McCurry K, Patel RP, Petersson J, Rassaf T, Reutov VP, Richter-Addo GB, Schechter A, Shiva S, Tsuchiya K, van Faassen EE, Webb AJ, Zuckerbraun BS, Zweier JL, Weitzberg E. Nitrate and nitrite in biology, nutrition and therapeutics. Nat Chem Biol. 2009 Dec;5(12):865-9. doi: 10.1038/nchembio.260.

Reference Type BACKGROUND
PMID: 19915529 (View on PubMed)

Other Identifiers

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SN-SC-02-01

Identifier Type: -

Identifier Source: org_study_id