Treatment of Vaso-Occlusive Pain Related to Sickle Cell Disease in Pediatric Patients: A Systematic Review

Sickle cell disease is a rare inherited disorder that affects the shape of red blood cells. Symptoms generally begin by three years of age. Complications of sickle cell disease include vaso-occlusive crises, acute chest syndrome, infections, pulmonary hypertension, priapism, stroke, as well as problems with various organ systems. Vaso-occlusive crises are the most common reason for hospitalization in patients with sickle cell disease. A vaso-occlusive crisis is an episode of pain, described as sharp, intense, and throbbing, most commonly occurring in the lower back, leg, hip, abdomen, or chest. It typically begins at night and lasts 3-14 days. No cure for sickle cell disease is widely available; treatment is symptomatic and supportive. No medications have FDA approval for the treatment of vaso-occlusive crises in pediatric patients and no evidence- based guidelines exist to aid in treatment decisions. Trials in pediatric patients are limited because of ethical concerns and pain management is difficult to study due to its subjective nature. This project is a literature review that aims to evaluate the safety and efficacy data of selected trials, focusing on reduction in pain scores in the treatment of pediatric patients with vaso-occlusive crises. Extensive literature searches for clinical trials were performed using four data bases: Pubmed, Embase, Cochrane Controlled Clinical Trials Register, and Clinicaltrials.gov. Trials were included if they enrolled children 18 years old or younger; were randomized, controlled trials; and received a JADAD score of at least 3. Six trials met inclusion criteria for this study. Interventions included in this study were intranasal fentanyl, inhaled nitric oxide, intravenous magnesium sulfate, intravenous ketorolac, and L-arginine. Two studies showed statistical significance in difference in pain scores. A significant difference in pain score at 20 minutes was seen with intranasal fentanyl. Pain scores at discharge were significantly different from placebo with L-arginine. No studies had serious adverse effects. More trials are needed with a larger sample size to detect smaller differences in endpoints