Tapered oral dexamethasone for the acute chest syndrome of sickle cell disease

Abstract

Tapered oral dexamethasone for acute chest syndrome (ACS) in sickle cell anaemia was studied using a novel ACS assessment tool and investigational biomarkers. Twelve participants were randomized (mean age 17·3 years) before early study termination. Dexamethasone decreased duration of hospitalization for ACS by 20·8 h compared to placebo (P = 0·024). Rebound pain occurred in both groups (3 dexamethasone versus 1 placebo). Overall, dexamethasone decreased the leucocyte activation biomarker, sL-selectin; however, participants with rebound pain had higher sL-selectin within 24 h of treatment (dexamethasone or placebo). This ACS assessment tool was feasibly applied, and sL-selectin is a promising biomarker of ACS therapy.

Trial registration: ClinicalTrials.gov NCT00530270.

Figure 1. The Objective Acute Chest Syndrome (ACS) Assessment Tool

This tool was developed for this study and used to define the end of an episode of ACS by serial evaluations of tachypnea, hypoxaemia, work of breathing, thoracic pain, and medical interventions to support respiration. This tool was used and recorded every 4 h until the subject was discharged from the hospital. ACS was defined to have ended when the endpoint criteria for all 5 of the elements of the tool were met. Abbreviations used in the tool: ACS, acute chest syndrome; bpm, breaths per minute; N, No; SpO₂, peripheral oxygen saturation measured by pulse oximetry; VAS, visual analog scale; Y, yes.