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Nitric Oxide Inhalation to Treat Sickle Cell Pain Crises

Phase 2
Completed
Conditions
Anemia, Sickle Cell
Interventions
Drug: Placebo
Registration Number
NCT00094887
Lead Sponsor
Mallinckrodt
Brief Summary

This study will examine whether nitric oxide (NO) gas can reduce the time it takes for pain to go away in patients who are in sickle cell crisis. NO is important in regulating blood vessel dilation, and consequently, blood flow. The gas is continuously produced by cells that line the blood vessels. It is also transported from the lungs by hemoglobin in red blood cells.

Patients 10 years of age or older with sickle cell disease (known SS, S-beta-thalassemia or other blood problems causing sickle cell disease) may be eligible for this study. Patients whose disease is due to hemoglobin (Hgb) SC are excluded. Candidates are screened with blood tests and a chest x-ray to look at the lungs and heart.

Participants are admitted to the hospital in a pain crisis. They are evaluated and then randomly assigned to receive one of two treatments: 1) standard treatment plus NO, or 2) standard treatment plus placebo. The placebo used in this study is nitrogen, a gas that makes up most of the air we breathe and is not known to help in sickle cell disease.

For the first 8 hours of the study, patients receive placebo or NO through a facemask. The mask may be taken off for 5 minutes every hour and for not more than 20 minutes to eat a meal. After the first 8 hours, the gas is delivered through a nasal cannula (small plastic tubing that rests under the nose) that may be taken off only while showering or using the restroom. Patients are questioned about the severity of their pain when they start the study and then every few hours while they are in the hospital. Their vital signs (temperature, breathing rate, and blood pressure) and medicines are checked. Patients will breathe the gas for a maximum of 3 days, but will stay hospitalized until the patient feels well enough to go home. Patients are followed up about 1 month after starting the study by a return visit to the hospital or by a phone call.

Detailed Description

The object of this study is to determine the safety and efficacy of nitric oxide for inhalation in the treatment of vaso-occlusive pain crisis (VOC) in patients with sickle cell disease. The study population will include patients with sickle cell disease (SS, S-beta-Thalassemia) presenting with vaso-occlusive pain crisis. Patients will be administered either placebo or inhaled nitric oxide to see if the experimental agent, inhaled nitric oxide, can reduce the time it takes for resolution of the vaso-occlusive crisis.

Recruitment & Eligibility

Status
COMPLETED
Sex
All
Target Recruitment
150
Inclusion Criteria

Each subject must meet all of the following inclusion criteria during the screening process in order to participate in the study:

  • Patient must have a diagnosis of SCD (known SS, S-Beta-thalassemia or other hemoglobinopathies causing sickle cell disease). Patients with disease due to Hgb SC are not permitted.
  • Must present to the ED/EC or other appropriate unit in VOC.
  • Greater than or equal to 10 years old.
  • Written informed consent/assent has been obtained.
Exclusion Criteria

Subjects meeting any of the following criteria during baseline evaluation will be excluded from entry into the study:

  • Exposure to therapeutic nitric oxide within the past 12 hours.
  • Patient has received sildenafil or other phosphodiesterase 5 inhibitors, therapeutic L-arginine, nitroprusside or nitroglycerine within the past 12 hours.
  • Patient has received previous ED/EC or other appropriate unit treatment for a vaso-occlusive crisis less than 48 hours or hospitalization less than 14 days ago (patients transferred directly from another ED or clinic may be enrolled).
  • Patient has visited the ED/EC or other appropriate unit greater than 10 times in the past year having a vaso-occlusive crisis.
  • Patients presenting with clinically diagnosed bacterial infection (e.g., osteomyelitis, pneumonia, sepsis or meningitis).
  • Patients who are currently enrolled in any other investigational drug study except for hydroxyurea studies.
  • Pregnant women (urine HCG + )/ nursing mothers.
  • Patients who have received an exchange transfusion (not simple transfusion) in the last 30 days or are on a chronic simple or exchange transfusion program.
  • Suspected splenic sequestration.
  • Acute chest syndrome or pneumonia: Abnormal new pulmonary infiltrate (alveolar infiltration and not atelectasis) and one or more pulmonary signs and/or symptoms (fever, rales, wheezing, cough, shortness of breath, retractions).
  • Previous participation in this study.

Study & Design

Study Type
INTERVENTIONAL
Study Design
PARALLEL
Arm && Interventions
GroupInterventionDescription
PlaceboPlaceboParticipants receive Nitrogen gas
Inhaled Nitric OxideNitric OxideParticipants receive Inhaled nitric oxide (INO)
Primary Outcome Measures
NameTimeMethod
Time to Vaso-occlusive Pain Crisis (VOC) Resolutionwithin 30 days

VOC resolution was defined by all of the following conditions:

* Pain relief - Visual Analog Scale (VAS) pain scores of 6 or less, (6 as worst and 0 as best)

* Freedom from parenteral narcotic use,

* Ability to walk unless the subject was not able to walk for any reason other than acute VOC prior to the onset of crisis,

* Subject and/or family's belief that the painful crisis could be managed at home with or without oral analgesic use, and the physician concurred with that assessment.

Secondary Outcome Measures
NameTimeMethod
Length of Hospitalizationwithin 40 days

Length of hospitalization is defined as the length of time from admission to discharge order

Total Dose of Opioids Receivedwithin 8 hours and within 40 days

The total dose (mg) of opioid medications received during the trial

Number of Participants With Acute Chest Syndrome/Pneumonia Requiring Blood Transfusionwithin 40 days

Number of participants who required a blood transfusion before discharge because of acute chest syndrome/pneumonia

Number of Participants Discharged to Home Within the First 24 Hourswithin 24 hours
Number of Participants Readmitted to Hospital Within 30 Days After Dischargeduring first 24 hours and during 30 day follow-up

The number of participants readmitted to the hospital for any reason within 30 days after discharge

Trial Locations

Locations (11)

National Institutes of Health Clinical Center, 9000 Rockville Pike

🇺🇸

Bethesda, Maryland, United States

Johns Hopkins University

🇺🇸

Baltimore, Maryland, United States

Colorado Sickle Cell Treatement and Research Center

🇺🇸

Aurora, Colorado, United States

Brigham and Women's Hospital

🇺🇸

Boston, Massachusetts, United States

Childrens Hospital, Boston

🇺🇸

Boston, Massachusetts, United States

Case Western Reserve University Hospital

🇺🇸

Cleveland, Ohio, United States

Childrens Hospital, Pittsburgh

🇺🇸

Pittsburgh, Pennsylvania, United States

University of Alabama

🇺🇸

Birmingham, Alabama, United States

Howard University Hospital

🇺🇸

Washington, District of Columbia, United States

Children's Hospital Oakland

🇺🇸

Oakland, California, United States

St. Christopher's Hospital for Children

🇺🇸

Philadelphia, Pennsylvania, United States

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