Trial of Oral Glutamine in Patients With Sickle Cell Anemia

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.

1. The study will compare the effect of glutamine and placebo on resting energy expenditure (REE) in children with sickle cell anemia (SCA) by comparing the change in REE ratio between baseline and 12 months.
2. The study investigates the effect of oral glutamine and placebo on body composition in children with SCA by comparing the difference in body mass indexes (BMIs) and percent of body fat (DEXA Scan) between baseline and 12 months of treatment in the two groups.
3. This Study will investigate the effect of oral glutamine and placebo on growth in children with SCA by comparing the Z scores for one year before baseline to 1 year while on study.
4. This study will investigate the effect of oral glutamine and placebo in children with SCA by comparing the difference in the levels of plasma and red blood cell glutamine between baseline and 12 months of treatment in the two groups.
5. This study will investigate the clinical effects (strength and exercise endurance) of oral glutamine and placebo in children with SCA by comparing the difference between baseline and 12 months of treatment in the two groups.
6. This study will evaluate quality of life in children with SCA who have glutamine or placebo for 12 months.
7. This study will evaluate the changes in REE over time in a small group of patients that will have REE measurement at months 3, 6, and 9. This objective will be offered to all patients, but will be "additional studies" that are not required to participate in the protocol.

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