Sickle cell pain

A critical reappraisal

Samir K. Ballas, Kalpna Gupta, Patricia Adams-Graves

Research output: Contribution to journalReview article

185 Citations (Scopus)

Abstract

Sickle cell pain includes 3 types: acute recurrent painful crises, chronic pain syndromes, and neuropathic pain. The acute painful crisis is the hallmark of the disease and the most common cause of hospitalization and treatment in the emergency department. It evolves through 4 phases: prodromal, initial, established, and resolving. Each acute painful episode is associated with inflammation that worsens with recurrent episodes, often culminating in serious complications and organ damage, such as acute chest syndrome, multiorgan failure, and sudden death. Three pathophysiologic events operate in unison during the prodromal phase of the crisis: vaso-occlusion, inflammation, and nociception. Aborting the acute painful episode at the prodromal phase could potentially prevent or minimize tissue damage. Our hypothesis is that managing these events with hydration, anti-inflammatory drugs, aggressive analgesia, and possibly vasodilators could abort the crisis and prevent or minimize further damage. Chronic pain syndromes are associated with or accompany avascular necrosis and leg ulcers. Neuropathic pain is not well studied in patients with sickle cell disease but has been modeled in the transgenic sickle mouse. Management of sickle cell pain should be based on its own pathophysiologic mechanisms rather than borrowing guidelines from other nonsickle pain syndromes.

Original languageEnglish (US)
Pages (from-to)3647-3656
Number of pages10
JournalBlood
Volume120
Issue number18
DOIs
StatePublished - Nov 1 2012

Fingerprint

Neuralgia
Pain
Chronic Pain
Acute Chest Syndrome
Vasodilator Agents
Hydration
Inflammation
Leg Ulcer
Anti-Inflammatory Agents
Nociception
Sickle Cell Anemia
Tissue
Sudden Death
Analgesia
Transgenic Mice
Hospital Emergency Service
Hospitalization
Necrosis
Pharmaceutical Preparations
Guidelines

All Science Journal Classification (ASJC) codes

  • Biochemistry
  • Immunology
  • Hematology
  • Cell Biology

Cite this

Sickle cell pain : A critical reappraisal. / Ballas, Samir K.; Gupta, Kalpna; Adams-Graves, Patricia.

In: Blood, Vol. 120, No. 18, 01.11.2012, p. 3647-3656.

Research output: Contribution to journalReview article

Ballas, Samir K. ; Gupta, Kalpna ; Adams-Graves, Patricia. / Sickle cell pain : A critical reappraisal. In: Blood. 2012 ; Vol. 120, No. 18. pp. 3647-3656.
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