Key Components of Pain Management for Children and Adults with Sickle Cell Disease

Abstract

Sickle cell disease pain manifests as severe acute pain episodes and a debilitating chronic pain syndrome. Acute pain episodes are the most common reason for health care use; however, acute pain episodes are also frequently managed at home. Chronic pain syndrome develops in 30% to 40% of individuals with sickle cell disease, with an increasing incidence and severity with age. We review the critical aspects of pain management that are integral to the comprehensive approach to sickle cell disease pain and are rooted in the biopsychosocial model. The review focuses on opioid pharmacology and psychosocial comorbidities.

Keywords: Acute pain; Anxiety; Chronic pain syndrome; Depression; Opioids; Sickle cell disease; Sleep.

Figure 1.

Trajectory of pain experience from infancy to adulthood for individuals with SCD.

Figure 2.. Biopsychosocial model of pain in individuals with sickle cell disease.

Pain management should be delivered in the context of the biopsychosocial model where interactions between biological, psychological and social influences of pain are addressed.

Figure 3.. Different approaches for opioid infusion for inpatient acute SCD pain management.

(A) Continuous opioid infusion with the goal of keeping the individual in the therapeutic window with the addition of breakthrough opioids administered via patient controlled analgesia (PCA) at one sixth the dose of the continuous infusion given every 20 minutes. Dose adjustment: If the individual self-administers the PCA dose ≥3 times in consecutive 2 hours, then consider increasing the continuous infusion dose. The new continuous dose should be the equivalence of prior hour’s dose (continuous plus cumulative PCA bolus doses). The new PCA dose should be one sixth of the current continuous dose, given every 20 minutes. (B) Low dose continuous infusion without the goal of infusion being in the therapeutic window. PCA dose is given to put the individual in the therapeutic window. This strategy requires the individual to utilize the PCA throughout the day and night and to awaken at night to relieve pain. There is no strategy to distinguish breakthrough pain from a persistent increase in pain intensity.

Figure 3.. Different approaches for opioid infusion for inpatient acute SCD pain management.

(A) Continuous opioid infusion with the goal of keeping the individual in the therapeutic window with the addition of breakthrough opioids administered via patient controlled analgesia (PCA) at one sixth the dose of the continuous infusion given every 20 minutes. Dose adjustment: If the individual self-administers the PCA dose ≥3 times in consecutive 2 hours, then consider increasing the continuous infusion dose. The new continuous dose should be the equivalence of prior hour’s dose (continuous plus cumulative PCA bolus doses). The new PCA dose should be one sixth of the current continuous dose, given every 20 minutes. (B) Low dose continuous infusion without the goal of infusion being in the therapeutic window. PCA dose is given to put the individual in the therapeutic window. This strategy requires the individual to utilize the PCA throughout the day and night and to awaken at night to relieve pain. There is no strategy to distinguish breakthrough pain from a persistent increase in pain intensity.

Figure 4.. Potential timelines of pain treatment for an uncomplicated acute pain event.

Two potential timelines for the receipt of analgesia are depicted: (A) Perceived standard approach to acute pain management and (B) empowered approach to acute pain management.

Figure 4.. Potential timelines of pain treatment for an uncomplicated acute pain event.

Two potential timelines for the receipt of analgesia are depicted: (A) Perceived standard approach to acute pain management and (B) empowered approach to acute pain management.