Arginine Therapy for Sickle Cell Disease Pain

The purpose of this study is to determine the effects of IV L-arginine hydrochloride therapy
in children with sickle cell disease (SCD) and vaso-occlusive pain events (VOE).
Specifically, the impact on total opioid use (mg/kg) over the duration of their emergency
department (ED) visit and hospital stay will be evaluated.

Inclusion Criteria:

- Established diagnosis of sickle cell disease (SCD); all genotypes

- Pain requiring medical care in an acute care setting (such as the emergency department
or ED, hospital ward, day hospital, clinic) not attributable to non-sickle cell
causes, that is moderate-to-severe requiring parenteral opioids

Exclusion Criteria:

- Decision to discharge home from the acute care setting

- Hemoglobin less than 5 gm/dL or immediate need for red cell transfusion anticipated
within next 12 hours

- Hepatic dysfunction of SGPT greater than 3 times the upper value

- Renal dysfunction of creatinine greater than 1.0

- Mental status or neurological changes

- Acute stroke or clinical concern for stroke

- Pregnancy

- Allergy to arginine

- Two (2) or more ED visits for VOE within the last 7 days prior to CURRENT ED visit

- Hospitalization within 14 days

- Previous randomization in this arginine RCT (patient consented and screen failed
before receiving study drug or placebo remains eligible for future participation).

- Use of inhaled nitric oxide, sildenafil or arginine within the last month

- PICU admission from the emergency department

- Hypotension requiring treatment with clinical intervention

- Acidosis with Co2≤ 16

- Newly started on HU for <3 months

- Not an appropriate candidate in the investigator's judgment

- Patient refusal