Adenosine 2A Agonist Lexiscan in Children and Adults With Sickle Cell Disease
Condition: Sickle Cell Disease
MeSH terms: Anemia, Sickle Cell
Intervention: Lexiscan
Intervention description: Given as an infusion
Intervention MeSH terms: Regadenoson
Phase: Phase 1
Study type: Interventional
Primary purpose: Treatment
Allocation: Non-Randomized
Intervention model: Parallel Assignment
Masking: None (Open Label)
Enrollment: 39.0
Sex: All
Minimum age: 10 Years
Maximum age: 70 Years
Healthy volunteers: No
Keywords: lexiscan

Summary:

      Sickle cell disease (SCD) is an inherited blood disorder that causes the red blood cells to
      change their shape from a round shape to a half-moon/crescent or sickled shape. People who
      have SCD have a different type of protein that carries oxygen in their blood (hemoglobin)
      then people without SCD. This different type of hemoglobin makes the red blood cells change
      into a crescent shape under certain conditions. Sickle-shaped cells are a problem because
      they often get stuck in blood vessels blocking the flow of blood, and cause inflammation and
      injury to the important areas in the body. Lexiscan is drug that may prevent this
      inflammation and injury caused by the sickle shaped cells. This drug is approved by the FDA
      to be used as a fast infusion during a heart stress test in people who are unable to exercise
      enough to put stress on their heart by making it beat faster. Lexiscan has never been studied
      in patients with SCD and has never been given as a long infusion.
    

Eligibility criteria:

        Inclusion Criteria Stage I/II/IIb: (COMPLETE AND CLOSED TO ACCRUAL)

          -  Participants must have sickle cell anemia confirmed by hemoglobin analysis

          -  Participants must report that their pain is at baseline. Additionally, they cannot
             report an increase in dose or frequency of opioid use in the last 2 weeks prior to
             drug administration

          -  Age 21-70 years

          -  Participants must have the laboratory indices as outlined in the protocol

          -  Participants must have reliable IV access as determined by the investigator

          -  Women of child-bearing potential and men must agree to use adequate contraception
             prior to study entry and for the duration of the study.

        Inclusion Criteria Stage III: (COMPLETE AND CLOSED TO ACCRUAL)

          -  Participants must have sickle cell anemia confirmed by hemoglobin analysis

          -  Participant is admitted to the hospital for a pain episode

          -  Age 21-70 years

          -  Participants must have the laboratory indices as outlined in the protocol

          -  Participants must have reliable IV access as determined by the investigator

          -  Women of child-bearing potential and men must agree to use adequate contraception
             prior to study entry and for the duration of study participation

        Inclusion Criteria Stage IV: (open, still accruing volunteers)

          -  Participants must have sickle cell disease confirmed by hemoglobin analysis

          -  Participant is admitted to the hospital for a pain episode

          -  Ages of assent (10 to 17 years at DFCI, but different depending on institution)

          -  Participants must have the laboratory indices as outlined in the protocol

          -  Participants must have reliable IV access as determined by the investigator

          -  Participants and parents must have the ability to understand and the willingness to
             sign a written informed consent and assent document

          -  Women of child-bearing potential and men must agree to use adequate contraception
             prior to study entry and for the duration of study participation

        Exclusion Criteria Stage I/II/IIb: (COMPLETE AND CLOSED TO ACCRUAL)

          -  Participants with a current physician diagnosis of asthma (within last 12 months),
             require continuous supplemental oxygen, or predicted or current use of some asthma
             medications.

          -  Participants with second- or third-degree AV block or sinus node dysfunction

          -  Have a history of bleeding diathesis

          -  Have a history of clinically overt stroke

          -  Have a history of severe hypertension not adequately controlled with anti-hypertensive
             medications

          -  Participants who are receiving chronic anti-coagulation or anti-platelet therapy

          -  Participants with a history of metastatic cancer

          -  Participants who have had a hospitalization or emergency room visit for any reason in
             the past 2 weeks

          -  Participants may not be receiving any other study agents or have received a study
             agent in the past 30 days

          -  Uncontrolled intercurrent illness

          -  Pregnant or breastfeeding women

          -  Participants with HIV

          -  Participants who have previously enrolled and received the investigational agent as
             part of this study

          -  Participants who are taking medications that may interact with the investigational
             agent

        Exclusion Criteria Stage III: (COMPLETE AND CLOSED TO ACCRUAL)

          -  Participants with a current physician diagnosis of asthma (within last 12 months),
             require continuous supplemental oxygen, or predicted or current use of some asthma
             medications.

          -  Participants with second- or third-degree AV block or sinus node dysfunction

          -  Have a history of bleeding diathesis

          -  Have a history of clinically overt stroke

          -  Have a history of severe hypertension not adequately controlled with anti-hypertensive
             medications

          -  Participants who are receiving chronic anti-coagulation or anti-platelet therapy

          -  Participants with a history of metastatic cancer

          -  Participants may not be receiving any other study agents or have received a study
             agent in the past 30 days

        Exclusion Criteria Stage IV: (open, still accruing volunteers)

          -  Participants with a current physician diagnosis of asthma (within last 12 months),
             require continuous supplemental oxygen, or predicted or current use of some asthma
             medications.

          -  Participants with second- or third-degree AV block or sinus node dysfunction

          -  Have a history of bleeding diathesis

          -  Have a history of clinically overt stroke

          -  Have a history of hypertension not adequately controlled with anti-hypertensive
             medications

          -  Participants who are receiving chronic anti-coagulation or anti-platelet therapy

          -  Participants with a history of metastatic cancer

          -  Participants may not be receiving any other study agents or have received a study
             agent in the past 30 days

          -  Participants with HIV

          -  Participants who have previously enrolled and received the investigational agent as
             part of this study

          -  Participants who are taking medications that may interact with the investigational
             agent