Trial of Oral Glutamine in Patients With Sickle Cell Anemia
Condition: Anemia, Sickle Cell
MeSH terms: Anemia; Anemia, Sickle Cell
Intervention: Glutamine; Placebo
Intervention description: 0.6 gm/kg of oral glutamine per day, in two doses for one year.; Placebo
Phase: Phase 2
Study type: Interventional
Primary purpose: Prevention
Allocation: Randomized
Intervention model: Parallel Assignment
Masking: Triple (Participant, Care Provider, Investigator)
Enrollment: 27.0
Sex: All
Minimum age: 5 Years
Maximum age: 18 Years
Healthy volunteers: No
Keywords: Hemoglobin S Disease; Sickle Cell Anemia

Summary:

      Children with sickle cell anemia (SCA) seem to have higher energy needs than children who do
      not have the disease. This may be the reason why children and teenagers with sickle cell
      anemia tend to be smaller, weigh less, and have less fat and muscle than children and teens
      that do not have the disease.

      This study is being done to find out if giving a supplement called glutamine will help
      children with sickle cell anemia by lowering their energy needs and improving their growth
      and strength. Children will be randomly assigned (like a flip of a coin) to one of two
      groups. One group will take glutamine and one group will take a placebo (a protein mixture
      that looks like glutamine but may not have the same effect in the body). No one will know
      which group is taking which supplement until the study has been completed. Children will be
      in the study for 12 months.
    

Eligibility criteria:

        Inclusion Criteria:

        Active patient (presently receiving medical care for SCA) at the St. Jude Comprehensive
        Sickle Cell Center (SJCSCC) or an affiliate or alliance of St Jude. Patient must not be in
        the high risk category as defined by the SJCSCC. High risk is defined as follows:

          -  3 or more admissions and/or emergency department visits for pain within the past 12
             months, or;

          -  2 or more episodes of acute chest syndrome within the past 24 months, or;

          -  A combination of pain and ACS events > 3 within the past 12 months.

          -  5-18 years of age

          -  Diagnosis of Hb SS or HB0Thal

          -  Weight >15 kg

          -  <50th percentile for height/age or weight/age or weight/ height, or <90% ideal body
             weight, or <90% of BMI for age/ gender.

          -  Is willing to sign informed consent

        Exclusion Criteria

          -  Patients receiving hydroxyurea or any other anti-sickling agent, chronic transfusion,
             or nutrition supplements.A nutrition supplement is any high calorie or high protein
             food additive or oral supplement being used for the purpose of weight gain.

          -  History of poor compliance (Missing two or more clinic appointments in the past year).

          -  Renal or liver dysfunction

          -  Renal dysfunction as defined by serum creatinine >1.5 times normal for age based on
             testing lab.

          -  Hepatic dysfunction as defined by alanine aminotransferase (ALT) >2 times the upper
             limit of normal for age based on testing lab.

          -  Breastfeeding

          -  Pregnancy.Females of childbearing potential must have negative serum or urine
             pregnancy test (record date of test).

          -  Patients enrolled on previous glutamine protocol (SCDGLU).

          -  Patients who are considered high risk