L-Glutamine Therapy for Sickle Cell Anemia
Condition: Sickle Cell Anemia; Thalassemia
MeSH terms: Anemia; Thalassemia; Anemia, Sickle Cell
Intervention: L-Glutamine; Placebo
Intervention description: L-Glutamine at: 5 g 2X daily (17-33.3 Kg) 10 g 2x daily (33.4-66.6 Kg) 15 g 2x daily (>66.7 kg); Placebo (75% Maltodextrin, 24.5 % Starch, 0.5% Tricalcium Phosphate) - given at the same dosage as L-glutamine
Phase: Phase 2
Study type: Interventional
Primary purpose: Treatment
Allocation: Randomized
Intervention model: Parallel Assignment
Masking: Triple (Participant, Investigator, Outcomes Assessor)
Enrollment: 15.0
Sex: All
Minimum age: 18 Years
Healthy volunteers: No
Keywords: sickle cell disease; sickle cell anemia; L-glutamine; Sickle Cell Anemia (homozygous); Sickle β°-Thalassemia

Summary:

      The primary purpose is to evaluate the effect of L-glutamine therapy on exercise endurance
      and breath by breath exercise response of sickle cell anemia patients

      The secondary purpose is to assess the effect of L-glutamine on incidence of painful crises;
      level of chronic pain, and amount of daily requirement for narcotics.
    

Eligibility criteria:

        Inclusion Criteria:

        To be eligible to participate in the study, a patient must meet all of the following
        inclusion criteria:

          -  Patient is at least 18 years of age.

          -  Patient has been diagnosed with sickle cell anemia or sickle ß0-thalassemia
             (documented by hemoglobin electrophoresis).

          -  Patient has had at least two episodes of painful crises within 12 months of the
             screening visit.

          -  If the patient has been treated with an anti-sickling agent within three months of the
             screening visit, the therapy must have been continuous for at least three months with
             the intent to continue for the next 14 months.

          -  Patient or the patient's legally authorized representative has given written informed
             consent.

          -  If the patient is a female of child-bearing potential, she agrees to practice a
             recognized form of birth control during the course of the study.

          -  Patient is able to perform exercise tolerance test

        Exclusion Criteria:

        If the patient meets any of the following criteria, the patient must not be enrolled:

          -  Patient has a significant medical condition that required hospitalization (other than
             sickle painful crisis) within two months of the screening visit.

          -  Patient has diabetes mellitus with untreated fasting blood sugar >115 mg/dL.

          -  Patient has prothrombin time International Normalized Ratio (INR) > 2.0.

          -  Patient has serum albumin < 3.0 g/dl.

          -  Patient has received any blood products within three weeks of the screening visit.

          -  Patient has a history of uncontrolled liver disease or renal insufficiency.

          -  Patient is pregnant or lactating.

          -  Patient has been treated with an experimental anti-sickling medication/treatment
             (except hydroxyurea) within 30 days of the screening visit.

          -  Patient has been treated with an experimental drug within 30 days of the screening
             visit.

          -  There are factors that would, in the judgment of the investigator, make it difficult
             for the patient to comply with the requirements of the study.