Pain Management in Sickle Cell Disease: Palliative Care Begins at Birth?

Abstract

People with sickle cell disease (SCD) are living longer, but their lives are impacted even more by the unpredictable intermittent or constant pain that is often poorly managed over a lifetime. To address this problem, an interdisciplinary team approach is needed that brings the medical professionals together for optimal compassionate care that is coordinated from the beginning of life and throughout the patient's lifespan.The hematologist, whenever possible, should take the lead. Effective models that have been developed to address SCD pain, such as the Day Hospital, The Recidivism Project and various Home Care models, need to be integrated into an overarching plan on a continuum with an underpinning philosophy that will sustain such a program. While the focus of palliative care has been end of life, its philosophies fit the chronic disease model and support an integrated team approach to the SCD pain management dilemma. The patient-focused and family-oriented interventions can be modeled to fit "any place, any time," outpatient, inpatient or the home, with homecare and other support systems. Such are the needs in SCD: appropriate, timely and compassionate care to prevent or relieve suffering and to preserve dignity, meaning, value and quality of life with improvement that is translational from birth until the end of life.

Mast cell activation contributes to sickle cell pathobiology and pain in mice

“…It has been hypothesized that the neural response to unrelieved pain may in turn contribute to sickle pathophysiology and further amplify pain. 5 Unmyelinated C-fiber nociceptors serve a sensory afferent function to transmit information about noxious (painful) stimulation to the central nervous system (CNS), but also serve an efferent function that causes vasodilatation and plasma extravasation, a phenomenon termed neurogenic inflammation. 6 Upon activation of these fibers, action potentials travel orthodromically along the axon to the CNS as well as antidromically to invade endings of their arborizations, leading to the release of the neuropeptides substance P (SP) and calcitonin gene-related peptide (CGRP) in the periphery that produce neurogenic inflammation.…”

The management of sickle cell pain

“…Until that time, health care practitioners that care for people with SCD will need to partner with pain specialists to help develop treatment approaches that are individualized for each patient. This is a lifelong endeavor and has been compared to the palliative care approach used in cancer care [25]. Ultimately, cell therapybased approaches, such as cord blood transplant and induced pluripotent stem cells, will be necessary to affect a permanent cure.…”

Palliative Care Teams as Advocates for Adults with Sickle Cell Disease

“…4,8,9 Despite this variability, all forms of SCD have the potential to develop a pain crisis, even those with traditionally milder clinic presentations. 8,10 In a meta-analysis, the majority of patients reported having one vasocclusive crisis per year, while others had two crises per month. 2 Adults with SCD often die in the hospital with causes of death related to pulmonary hypertension, renal or multiorgan failure, acute coronary syndrome, stroke, iron overload, or thromboembolism.…”