Vaso-occlusive crisis in sickle cell disease: current paradigm on pain management

Abstract

This narrative review aims to highlight the current paradigm on pain management in sickle cell vaso-occlusive crisis. It specifically examines the pathophysiologic mechanisms of sickle cell pain as well as the pharmacologic and nonpharmacologic methods of pain management. Recurrent painful episodes constitute the major morbidity in sickle cell disease (SCD). While adolescents and young adults experience mostly acute episodic nociceptive pain, it is now recognized that a significant number of adult patients develop chronic neuropathic and centralized pain. In fact, current evidence points to an age-dependent increase in the frequency of SCD patients with chronic pain. Management of disease-related pain should be based on its pathophysiologic mechanisms instead of using recommendations from other non-SCD pain syndromes. Pain management in vaso-occlusive crisis is complex and requires multiple interventions such as pharmacologic, nonpharmacologic, and preventive therapeutic interventions. Pharmacologic treatment involves the use of non-opioid and opioid analgesics, and adjuvants – either singly or in combination – depending on the severity of pain. The basic approach is to treat SCD pain symptomatically with escalating doses of non-opioid and opioid analgesics. Given the moderate-to-severe nature of the pain usually experienced in this form of SCD crisis, opioids form the bedrock of pharmacologic treatment. Multimodal analgesia and structured, individualized analgesic regimen appear more effective in achieving better treatment outcomes. Although the current evidence is still limited on the supportive role of cognitive behavioral therapy in pain management, this nonpharmacologic approach is reportedly effective, but needs further exploration as a possible adjunct in analgesia.

Early Detection of Acute Chest Syndrome Through Electronic Recording and Analysis of Auscultatory Percussion

“…However, those that do not have a pulmonary infiltrate are considered to have uncomplicated VOC. Although labeled uncomplicated VOC, the treatment and pain management is quite complex and requires multiple pharmacologic, nonpharmacologic, and preventative therapeutic interventions [13]. In the simplest cases, the patient is treated, recovers, and is discharged from the hospital.…”

Targeting Neutrophil Adhesive Events to Address Vaso-Occlusive Crisis in Sickle Cell Patients

“…Contributing to microvascular occlusion is a greater capacity for neutrophil extracellular trap (NET) formation characterized by decondensed chromatin enriched in citrullinated histones and bound to granular enzymes ( 9 ). The pathophysiology and requirement for pain management directly associated with VOC in patients has been extensively reviewed ( 2 , 4 , 6 , 10 , 11 ). As such, the focus of this review is to highlight the role of neutrophils in the initiation and propagation of VOC, as well as targeted pharmacological interventions against adhesion molecules critical for neutrophil recruitment and heterotypic adhesion with other blood cells during flow within vessels.…”

Efficacy of Sucrose in Reducing Pain during Immunization among 10- to 18-Month-Old Infants and Young Children: A Randomized Controlled Trial

“…immunization injections) cause the release of mediators from the injured tissue which, in turn, activate and/ or sensitize the nociceptors to the noxious stimuli (Omoigui, 2007). Activation of nociceptors generates an action potential (de-polarisation and re-polarisation) at the cell membranes of injured tissue which, in turn, stimulates the nociceptive nerve terminals (Orhurhu et al, 2019;Uwaezuoke et al, 2018). Activation of the nerve terminals promotes the release of several neurotransmitters (exhibitory chemicals) that permit transduction and transmission of the pain stimuli through the spinal cord to the brain pathway where pain is perceived and responses are elicited (Caterina & Julius, 2001;Orhurhu et al, 2019;Szallasi & Blumberg, 1999;Uwaezuoke et al, 2018).…”